|
1
|
Dincer A, Snyder MH, Burke SM, Arkun K, Heilman CB, Wu JK, Malek AM. Stent Retriever and Aspiration Catheter Biopsy of an Intraluminal Transverse Sinus Meningioma: Case Report. Oper Neurosurg (Hagerstown) 2025:01787389-990000000-01539. [PMID: 40257248 DOI: 10.1227/ons.0000000000001580] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2024] [Accepted: 12/26/2024] [Indexed: 04/22/2025] Open
Abstract
BACKGROUND AND IMPORTANCE Intracranial intraluminal mass lesions are rare and challenging to biopsy using noninvasive methods. Obtaining tissue for diagnosis is critical for guiding treatment, yet traditional approaches such as craniotomy pose significant risks. In this case report, we describe the first use of an aspiration catheter and stent retrieval system to safely and successfully perform an endovascular biopsy in a patient with an exclusively intraluminal meningioma. CLINICAL PRESENTATION The patient had a history of medulloblastoma, which was resected and treated with craniospinal radiation. During routine surveillance MRI, an incidental filling defect was identified in the superior sagittal sinus, suspected to be either thrombus or intraluminal meningioma. The lesion demonstrated rapid growth despite anticoagulation therapy, prompting the decision to pursue tissue sampling to guide further treatment. A combined approach using a large-bore suction thrombectomy catheter and a 6-mm Solitaire X stentriever was used through a transvenous femoral approach. Although no tissue was obtained from the suction effluent, sufficient tissue fragments were captured on the stentriever to diagnose meningioma. The patient tolerated the procedure well, with no periprocedural complications. CONCLUSION Obtaining intravascular tissue with a stent retrieval system, particularly for firmer sinus wall lesions such as a meningioma may offer a safe alternative technique to craniotomy for intraluminal sinus tissue diagnosis.
Collapse
Affiliation(s)
- Alper Dincer
- Department of Neurosurgery, Tufts Medical Center, Boston, Massachusetts, USA
| | - M Harrison Snyder
- Department of Neurosurgery, Tufts Medical Center, Boston, Massachusetts, USA
| | - Shane M Burke
- Department of Neurosurgery, Tufts Medical Center, Boston, Massachusetts, USA
| | - Knarik Arkun
- Department of Pathology, Tufts Medical Center, Boston, Massachusetts, USA
| | - Carl B Heilman
- Department of Neurosurgery, Tufts Medical Center, Boston, Massachusetts, USA
| | - Julian K Wu
- Department of Neurosurgery, Tufts Medical Center, Boston, Massachusetts, USA
| | - Adel M Malek
- Department of Neurosurgery, Tufts Medical Center, Boston, Massachusetts, USA
| |
Collapse
|
|
2
|
Huang Y, Wang Y, Kong B, Zheng F, Ding Y, Hayford B, Guo J, Qin R. Pulmonary artery intimal sarcoma mimicking pulmonary thromboembolism: A case report and literature review. Oncol Lett 2025; 29:169. [PMID: 39963321 PMCID: PMC11831714 DOI: 10.3892/ol.2025.14915] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2024] [Accepted: 01/09/2025] [Indexed: 02/20/2025] Open
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare and highly aggressive form of hemangiosarcoma. The non-specific clinical manifestations, signs and routine imaging findings of this sarcoma often mimic those of pulmonary thromboembolism (PTE), resulting in frequent misdiagnosis as PTE prior to pathological confirmation in most patients. This delay in achieving an accurate diagnosis poses challenges for promptly initiating optimal treatment and further contributes to the unfavorable prognosis. The current study presents the case of a 68-year-old female who presented with acute chest tightness and dyspnea. Transthoracic echocardiography revealed the presence of atypical echogenic structures within the primary pulmonary artery, while computed tomography pulmonary angiography showed marked filling defects in the main trunk and branches of the pulmonary artery. The patient was initially misdiagnosed with PTE but did not respond well to anticoagulant therapy. Subsequent surgical resection confirmed the diagnosis of PAIS through pathological examination. Despite postoperative treatment with molecular-targeted antitumor drugs, the patient experienced tumor recurrence and intrapulmonary metastasis, ultimately succumbing to disease progression. This exceptional case is being presented to enhance the clinical understanding of PAIS, to encourage further extensive research for reliable diagnostic approaches and to provide further data that will help form efficacious therapeutic strategies to ameliorate the unfavorable prognosis of affected patients.
Collapse
Affiliation(s)
- Yan Huang
- Department of Cardiology, Jiangsu University Affiliated People's Hospital, Zhenjiang Clinical Medical College of Nanjing Medical University, Zhenjiang, Jiangsu 212002, P.R. China
| | - Yiming Wang
- Department of Cardiology, Jiangsu University Affiliated People's Hospital, Zhenjiang Clinical Medical College of Nanjing Medical University, Zhenjiang, Jiangsu 212002, P.R. China
| | - Biko Kong
- Department of Cardiology, Jiangsu University Affiliated People's Hospital, Zhenjiang Clinical Medical College of Nanjing Medical University, Zhenjiang, Jiangsu 212002, P.R. China
| | - Feng Zheng
- Department of Ultrasound, Jiangsu University Affiliated People's Hospital, Zhenjiang, Jiangsu 212002, P.R. China
| | - Yi Ding
- Department of Medical Imaging, Jiangsu University Affiliated People's Hospital, Zhenjiang, Jiangsu 212002, P.R. China
| | - Boamah Hayford
- Research Center of Fluid Machinery Engineering and Technology, Jiangsu University, Zhenjiang, Jiangsu 212013, P.R. China
| | - Junfang Guo
- Department of Cardiology, Jiangsu University Affiliated People's Hospital, Zhenjiang Clinical Medical College of Nanjing Medical University, Zhenjiang, Jiangsu 212002, P.R. China
| | - Rong Qin
- Department of Medical Oncology, Jiangsu University Affiliated People's Hospital, Zhenjiang, Jiangsu 212002, P.R. China
| |
Collapse
|
|
3
|
Nagai T, Misumi Y, Yoshioka D, Kawamura M, Kawamura T, Matsuura R, Kawamura A, Taira M, Shimamura K, Sakai D, Sato T, Miyagawa S. Salvage Right Ventricular Outflow Tract Reconstruction for Pulmonary Embolization with Pulmonary Artery Sarcoma. Surg Case Rep 2025; 11:24-0068. [PMID: 40160478 PMCID: PMC11950829 DOI: 10.70352/scrj.cr.24-0068] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2024] [Accepted: 03/04/2025] [Indexed: 04/02/2025] Open
Abstract
INTRODUCTION Pulmonary artery intimal sarcoma (PAIS) is extremely rare and highly malignant. Although improved outcomes have been reported after complete surgical resection and chemotherapy, limited information is available regarding the indications, procedures, and prognosis of palliative surgery for PAIS. This report describes a successful salvage surgical case for rapid hemodynamic deterioration due to PAIS obstructing the pulmonary artery trunk. CASE PRESENTATION A 64-year-old woman, complaining of dyspnea for a month, was referred for a pulmonary artery tumor. Imaging studies confirmed an intraluminal tumor that obstructs the pulmonary artery trunk and extends to the right ventricular wall and interventricular septum, suspecting a malignancy. During preoperative workups, she developed acute hemodynamic and respiratory deterioration due to pulmonary embolization, so emergency surgery was planned on a salvage basis. The tumor originated from the pulmonary artery intima just distal to the pulmonary valve, obstructed the pulmonary artery trunk, and extensively involved the left main coronary artery and the interventricular septum, where complete resection of the tumor was not achieved. Reconstruction of the pulmonary valve, the right ventricular outflow tract (RVOT), and bilateral pulmonary arteries were performed using a composite of a prosthetic valve and vascular grafts. The patient's postoperative course was uneventful, and she was discharged home asymptomatic. Pathological diagnosis of the operative specimen confirmed pulmonary intimal sarcoma. After 4 months of postoperative chemotherapy, tumor progression was confirmed. The patient passed away at home 8 months after surgery. CONCLUSION We reported a case of PAIS presenting with RVOT obstruction and rapid hemodynamic and respiratory deterioration, who underwent succeeding emergent surgery and was discharged home asymptomatic. Palliative RVOT reconstruction can be a useful surgical option for PAIS accompanying pulmonary embolization on a salvage basis.
Collapse
Affiliation(s)
- Tsubasa Nagai
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Yusuke Misumi
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Daisuke Yoshioka
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Masashi Kawamura
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Takuji Kawamura
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Ryohei Matsuura
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Ai Kawamura
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Masaki Taira
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Kazuo Shimamura
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Daisuke Sakai
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Taro Sato
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| | - Shigeru Miyagawa
- Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
| |
Collapse
|
|
4
|
Liang G, Zhang Q, Luo Y, Zhao Y, Luo B. Efficacy of anlotinib and chemotherapy combination as neoadjuvant therapy in the treatment of pulmonary artery intimal sarcoma: a case report. Front Oncol 2025; 15:1507281. [PMID: 40078190 PMCID: PMC11897571 DOI: 10.3389/fonc.2025.1507281] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2024] [Accepted: 02/07/2025] [Indexed: 03/14/2025] Open
Abstract
Pulmonary arterial intimal sarcoma (PAIS) is a rare malignant mesenchymal tumor often associated with an unfavorable prognosis and lacks a standardized treatment approach to date. This report presents a notable case of PAIS treated with neoadjuvant therapy involving anlotinib concomitantly administered with chemotherapy of ifosfamide and pirarubicin, which resulted in a favorable outcome. A 38-year-old man was admitted to our hospital with chest tightness, cough, and dyspnea, all of which had persisted for more than a week. Initial evaluation via chest computed tomography (CT) revealed a sizable posterior mediastinal tumor measuring 11.9 × 7.6 cm. A CT-guided biopsy was performed, and pathological findings confirmed the diagnosis of PAIS. Efficacy evaluation showed slow progress after one cycle of chemotherapy with ifosfamide and pirarubicin. To enhance treatment outcomes, we incorporated anlotinib as a neoadjuvant therapy alongside ifosfamide and pirarubicin. Subsequent CT imaging demonstrated a significant reduction in tumor size, and the patient experienced notable alleviation of symptoms. The patient then underwent surgery, radiation, and subsequently, maintenance treatment with anlotinib for one year. No severe drug-related side effects were observed. The patient achieved progression-free survival of 25 months following administration of anlotinib. Thus, the combination of anlotinib with ifosfamide and pirarubicin demonstrated significant efficacy and safety. This approach holds promise as an effective therapeutic strategy for managing unresectable, locally advanced, or advanced PAIS. However, further clinical studies are necessary to validate these findings.
Collapse
Affiliation(s)
| | | | | | - Yuanhua Zhao
- Department of Radiation Oncology, Hubei Cancer Hospital, TongJi Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Bo Luo
- Department of Radiation Oncology, Hubei Cancer Hospital, TongJi Medical College, Huazhong University of Science and Technology, Wuhan, China
| |
Collapse
|
|
5
|
Lee P, Ling N, Lamb CR. Endovascular Forceps Biopsy of Pulmonary Artery Intimal Sarcoma: A Safer Approach. Am J Respir Crit Care Med 2025; 211:287-288. [PMID: 39586105 PMCID: PMC11812534 DOI: 10.1164/rccm.202407-1460le] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2024] [Accepted: 11/20/2024] [Indexed: 11/27/2024] Open
Affiliation(s)
- Pyng Lee
- Respiratory and Critical Care Medicine, National University Hospital, Singapore; and
| | - Norris Ling
- Respiratory and Critical Care Medicine, National University Hospital, Singapore; and
| | - Carla R. Lamb
- Pulmonary and Critical Care Medicine, Lahey Clinic, Burlington, Massachusetts
| |
Collapse
|
|
6
|
Luan T, Hao J, Gu Y, He P, Li Y, Wang L, Deng H, Guan W, Lin X, Xie X, Deng Y, Wang S, Wang C, Li J, Li R, Luan Y, Yang G, Zhang Y, Zhong N, Zhou C. A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma. BMC Cancer 2024; 24:1597. [PMID: 39741266 DOI: 10.1186/s12885-024-13277-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2024] [Accepted: 12/02/2024] [Indexed: 01/02/2025] Open
Abstract
OBJECTIVE The aim of this study is to explore the clinicopathological features, radiographic manifestations, treatment options, and prognosis of primary pulmonary angiosarcoma (PPAS). METHOD We summarized and analyzed the clinical data of 11 patients with primary pulmonary angiosarcoma treated at the First Affiliated Hospital of Guangzhou Medical University between January 2018 and January 2024. A retrospective analysis was conducted in conjunction with a review of the relevant literature. RESULTS Among the 11 cases, the primary symptoms were nonspecific respiratory issues, with chest pain, cough, and shortness of breath being the most common. Initial diagnosis was correct in only 2 cases, while the remaining 9 cases were misdiagnosed as other conditions, such as lung nodules, infections, or pulmonary embolism. All patients were ultimately diagnosed via pathology, with CD31 and CD34 showing a high positivity rate of 60%. Of the 11 patients, 9 have died, and 2 remain alive. The average survival period was 13.2 months.Based on the number of lesions, PPAS was categorized into three types: solitary, multiple, and invasive pulmonary artery. The average survival period for the solitary type (2 cases) was 15.5 months, for the multiple type (5 cases) was 6.6 months, and for the pulmonary artery type (4 cases) was 20.5 months. Among the 3 patients who underwent surgery, the median overall survival (OS) was 23.0 months, whereas for the 8 non-surgical patients, the median OS was 9.7 months. CONCLUSION Primary pulmonary angiosarcoma is a rare, highly aggressive vascular malignancy characterized by nonspecific clinical symptoms and a tendency for metastasis and pulmonary artery embolism. Currently, no effective treatment exists, and the overall prognosis is poor.
Collapse
Affiliation(s)
- Tao Luan
- Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650500, China
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Jianqing Hao
- Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650500, China
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Yingying Gu
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Ping He
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Yijia Li
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Liqiang Wang
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Haiyi Deng
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Wenhui Guan
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Xinqing Lin
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Xiaohong Xie
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Yu Deng
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Shuaiying Wang
- Anesthesia Surgery Center, Zhejiang Cancer Hospital, Hangzhou, 310022, China
| | - Changchun Wang
- Anesthesia Surgery Center, Zhejiang Cancer Hospital, Hangzhou, 310022, China
| | - Jingxu Li
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Ru Li
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China
| | - Yang Luan
- Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650500, China
| | - Gang Yang
- Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650500, China
| | - Yunhui Zhang
- Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650500, China.
| | - Nanshan Zhong
- Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650500, China.
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China.
| | - Chengzhi Zhou
- National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, 510000, China.
| |
Collapse
|
|
7
|
Kashizaki F, Chen H, Tsuchiya N, Matsumoto S, Osada R, Miyasaka A, Kaneko M, Kikuchi A, Kojima Y, Yumoto K, Hashiyama N, Osawa H, Koizumi H, Mo M, Takahashi K, Kaneko T. Clinical Features of Primary Pulmonary Artery Sarcoma: A Systematic Review and Pooled Analysis. Arch Bronconeumol 2024:S0300-2896(24)00484-8. [PMID: 39827013 DOI: 10.1016/j.arbres.2024.12.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2024] [Revised: 12/07/2024] [Accepted: 12/13/2024] [Indexed: 01/22/2025]
Abstract
OBJECTIVES Primary pulmonary artery sarcoma (PPAS) is a rare disease with unclear clinical manifestations. Advances in imaging devices have improved diagnostic capabilities, potentially affecting clinical characteristics and overall survival (OS); however, details remain unclear. This study conducted a pooled analysis of case reports and series to analyse the clinical characteristics and OS of PPAS in the era of advanced medical devices. METHODS Data were sourced from PubMed and CINAHL, focusing on studies published between 1 January 2014 and 31 December 2023. The study included patients diagnosed with PPAS, with extracted data covering demographics, diagnosis, treatments, and survival. RESULTS Overall, 643 patients were included (mean age: 52.6±13.1 years; 50.4% were female). Initially, 70.6% were diagnosed with pulmonary thromboembolism (PTE), and 15.4% were suspected of having PPAS. Among these, 93.9% and 55.2% showed suggestive findings on computed tomography (CT)-integrated positron emission tomography with 2-deoxy-2-18F-fluoro-d-glucose (18F-FDG PET/CT) and CT, respectively, with 98.2% confirmed before death. The right main pulmonary artery was the most affected site on CT (72.3%). Surgery and chemotherapy were performed in 81.4% and 66.4% of patients, respectively. The median OS was 31 months, with surgery extending OS across all stages and chemotherapy benefiting stages III-IV. Longer OS was achieved in patients who underwent complete surgical resection. CONCLUSIONS 18F-FDG PET/CT and multi-detector-row CT can differentiate PTE from PPAS. These medical devices may contribute to improved OS.
Collapse
Affiliation(s)
- Fumihiro Kashizaki
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan.
| | - Hao Chen
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Nanami Tsuchiya
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Sachiko Matsumoto
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Reeko Osada
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Atsushi Miyasaka
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Mai Kaneko
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Akitomo Kikuchi
- Department of General Thoracic Surgery, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Yui Kojima
- Department of Pathology, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Kentaro Yumoto
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Naoki Hashiyama
- Department of Cardiovascular Surgery, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Hiroyuki Osawa
- Department of General Thoracic Surgery, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Harumi Koizumi
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Makoto Mo
- Department of Cardiovascular Surgery, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Kenichi Takahashi
- Department of Respiratory Medicine, Yokohama Minami Kyosai Hospital, Yokohama, Japan
| | - Takeshi Kaneko
- Department of Respiratory Medicine, Yokohama City University Hospital, Yokohama, Japan
| |
Collapse
|
|
8
|
Miyazaki K, Matsumoto I, Nishikawa S, Wada T, Takayama T, Saito D, Yoshida S, Iino K, Takemura H. Reoperation of Pulmonary Artery Intimal Sarcoma for Recurrence After Pulmonary Artery Replacement. ANNALS OF THORACIC SURGERY SHORT REPORTS 2024; 2:484-487. [PMID: 39790400 PMCID: PMC11708652 DOI: 10.1016/j.atssr.2024.02.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Accepted: 02/26/2024] [Indexed: 01/12/2025]
Abstract
Pulmonary artery intimal sarcoma has very poor prognosis, for which radical resection may be difficult. A 68-year-old man with pulmonary artery intimal sarcoma underwent bilateral pulmonary artery resection and replacement. At 31 months postoperatively, recurrence occurred at the anastomotic site of the right pulmonary artery. Therefore, he underwent right upper lobectomy and combined resection and reconstruction of the right pulmonary artery. The tumor was removed, while preserving the lungs as much as possible. He is still alive 42 months after the initial surgery.
Collapse
Affiliation(s)
- Kentaro Miyazaki
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Isao Matsumoto
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Satoshi Nishikawa
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Takashi Wada
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Tetsuya Takayama
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Daisuke Saito
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Shuhei Yoshida
- Department of Thoracic Surgery, Kanazawa University, Kanazawa, Japan
| | - Kenji Iino
- Department of Cardiovascular Surgery, Kanazawa University, Kanazawa, Japan
| | - Hirofumi Takemura
- Department of Cardiovascular Surgery, Kanazawa University, Kanazawa, Japan
| |
Collapse
|
|
9
|
Gao X, Xie A, Xiao W, Wei Z, Yu S. Pulmonary Artery Sarcoma Misdiagnosed as Pulmonary Embolism. J Cardiothorac Vasc Anesth 2024; 38:2041-2046. [PMID: 38937171 DOI: 10.1053/j.jvca.2024.06.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/17/2024] [Revised: 05/23/2024] [Accepted: 06/03/2024] [Indexed: 06/29/2024]
Affiliation(s)
- Xue Gao
- Guizhou Medical University, Guiyang, China; Department of Ultrasound Medicine, the First People's Hospital of Guiyang City, Guiyang, China
| | - Aihua Xie
- Department of Ultrasound Medicine, Affiliated Hospital of Guizhou Medical University, Guiyang, China
| | - Weiwei Xiao
- Guizhou Medical University, Guiyang, China; Department of Ultrasound Medicine, Affiliated Hospital of Guizhou Medical University, Guiyang, China
| | - Zhihong Wei
- Department of Ultrasound Medicine, Affiliated Hospital of Guizhou Medical University, Guiyang, China
| | - Shaomei Yu
- Department of Ultrasound Medicine, Affiliated Hospital of Guizhou Medical University, Guiyang, China.
| |
Collapse
|
|
10
|
Atahan C, Güral Z, Yücel S, Ağaoğlu F. Pulmonary artery intimal sarcoma: Case report of a patient managed with multimodality treatment and a comprehensive literature review. Strahlenther Onkol 2024; 200:725-729. [PMID: 38866999 PMCID: PMC11272804 DOI: 10.1007/s00066-024-02250-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2024] [Accepted: 05/20/2024] [Indexed: 06/14/2024]
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare and aggressive malignancy originating from the intimal layer of the pulmonary artery with poor prognosis due to its aggressive nature. The management of PAIS poses both diagnostic and therapeutic challenges. It presents with nonspecific symptoms and is often misdiagnosed as pulmonary embolism. While surgical resection is the primary treatment modality, the role of adjuvant chemotherapy and radiotherapy remains uncertain. However, given the high recurrence rate, adjuvant chemotherapy and/or radiotherapy have been utilized in a limited number of case reports. We present the case of a 46-year-old woman who was diagnosed with PAIS and underwent surgical resection followed by adjuvant chemotherapy (ChT) and radiotherapy (RT), demonstrating good tolerance to this multimodal treatment approach.
Collapse
Affiliation(s)
- C Atahan
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
| | - Z Güral
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
| | - S Yücel
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
| | - F Ağaoğlu
- Department of Radiation Oncology, Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey.
| |
Collapse
|
|
11
|
Zhang Z, Zhen Y, Liu J, Liu X, Yang L, Xu M, Wen J, Liu P. Outcomes of pulmonary endarterectomy for patients with pulmonary artery sarcoma. Front Cardiovasc Med 2024; 11:1302372. [PMID: 39015681 PMCID: PMC11250646 DOI: 10.3389/fcvm.2024.1302372] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2024] [Accepted: 05/22/2024] [Indexed: 07/18/2024] Open
Abstract
Objective Pulmonary artery sarcoma (PAS) is an exceedingly rare and insufficiently investigated disease, leading to uncertain in its optimal management. This study aims to present our institutional experience and the outcomes of pulmonary endarterectomy for PAS. Methods We gathered clinical characteristics, intraoperative data, postoperative outcomes, and prognosis information from PAS patients who underwent surgical treatment at our institution between December 2016 and September 2023. Results A total of 20 patients with PAS underwent pulmonary endarterectomy. The median age of the patients was 52 (IQR 45, 57) years, with 12 patients (60%) being female. Intimal sarcoma was confirmed in 19 patients, while the remaining one was diagnosed with large cell neuroendocrine carcinoma. The perioperative mortality rate was three cases (15%). Follow-up was conducted for a median duration of 14 months (range: 1-61). During the follow-up period, 11 patients experienced recurrence or metastasis, and 5 patients succumbed to the disease. The estimated cumulative survival rates at 1 and 2 years for all 20 patients were 66.4% and 55.3%, respectively. Conclusion Pulmonary endarterectomy emerges as a palliative but effective approach for managing PAS, particularly when complemented with postoperative therapies such as chemotherapy and targeted therapy, which collectively contribute to achieving favorable long-term survival outcomes.
Collapse
Affiliation(s)
- Zhaohua Zhang
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Yanan Zhen
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Jingwen Liu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Xiaopeng Liu
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Liang Yang
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Mingyuan Xu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Jianyan Wen
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| | - Peng Liu
- Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China
- Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Beijing, China
| |
Collapse
|
|
12
|
Bourlond B, Maurizi N, Antiochos P, Skalidis I, Auf Der Springe K, Royer C, Monney P, Muller O, Kirsch M. Recurrence of an undifferentiated pleomorphic pulmonary artery sarcoma 8 years after initial presentation: a case report. Front Cardiovasc Med 2024; 11:1378333. [PMID: 38984354 PMCID: PMC11232353 DOI: 10.3389/fcvm.2024.1378333] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2024] [Accepted: 05/20/2024] [Indexed: 07/11/2024] Open
Abstract
Background Primary cardiac tumors remain exceptionally rare, characterized by a poor prognosis. Among them, sarcomas originating in the pulmonary arteries constitute the most infrequent subgroup within primary cardiac sarcomas. Case summary This report presents the case of a 76-year-old female experiencing a recurrence of an undifferentiated pleomorphic intracardiac pulmonary artery sarcoma located in the right ventricular outflow tract, manifesting 8 years after initial remission. Successful outcomes were attained through a combination of surgical resection, state-of-the-art radiotherapy, and chemotherapy. This comprehensive approach proved essential for optimizing both survival and quality of life. Discussion The unexpectedly prolonged recurrence-free survival observed in this case underscores the effectiveness of the comprehensive multimodal treatment approach outlined in the existing literature. This highlights the pivotal role of a multidisciplinary strategy in addressing primary cardiac sarcomas, particularly those arising in the pulmonary arteries.
Collapse
Affiliation(s)
- Baudouin Bourlond
- Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Niccolo Maurizi
- Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Panagiotis Antiochos
- Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Ioannis Skalidis
- Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Katarina Auf Der Springe
- Department of Nuclear Medicine and Molecular Imaging, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
| | - Claire Royer
- Department of Anatomopathology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Pierre Monney
- Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Olivier Muller
- Department of Cardiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| | - Mathias Kirsch
- Department of Anatomopathology, Lausanne University Hospital (CHUV), Lausanne, Switzerland
| |
Collapse
|
|
13
|
Din Abdul Jabbar MA, Bishop GJ, Raja S, Tong MZ, Cameron SJ. Pulmonary Sarcoma: A Wolf in Sheep's Clothing. Am J Med 2024; 137:500-502. [PMID: 38307151 DOI: 10.1016/j.amjmed.2023.12.029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2023] [Revised: 12/29/2023] [Accepted: 12/29/2023] [Indexed: 02/04/2024]
Affiliation(s)
- Muzammil Arif Din Abdul Jabbar
- Heart Vascular and Thoracic Institute, Department of Cardiovascular Medicine, Section of Vascular Medicine, Cleveland Clinic Foundation, Ohio; School of Clinical Medicine, University of Cambridge, United Kingdom
| | - G Jay Bishop
- Heart Vascular and Thoracic Institute, Department of Cardiovascular Medicine, Section of Vascular Medicine, Cleveland Clinic Foundation, Ohio
| | - Siva Raja
- Heart, Vascular and Thoracic Institute, Department of Thoracic and Cardiovascular surgery, Cleveland Clinic Foundation, Ohio
| | - Michael Z Tong
- Heart, Vascular and Thoracic Institute, Department of Thoracic and Cardiovascular surgery, Cleveland Clinic Foundation, Ohio
| | - Scott J Cameron
- Heart Vascular and Thoracic Institute, Department of Cardiovascular Medicine, Section of Vascular Medicine, Cleveland Clinic Foundation, Ohio; Taussig Institute, Department of Hematology, Cleveland Clinic Foundation, Ohio; Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Ohio.
| |
Collapse
|
|
14
|
Rashidi F, Bilehjani E, Mousavi-Aghdas SA, Parvizi R. Massive primary pulmonary artery rhabomyosarcoma: A case report. ROMANIAN JOURNAL OF INTERNAL MEDICINE = REVUE ROUMAINE DE MEDECINE INTERNE 2024; 62:67-74. [PMID: 38044271 DOI: 10.2478/rjim-2023-0032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 05/31/2023] [Indexed: 12/05/2023]
Abstract
BACKGROUND Pulmonary artery sarcomas (PAS) are rare tumours causing an insidiously progressive obstruction of the pulmonary circulation. The clinical presentation is often indistinguishable from chronic thromboembolic pulmonary hypertension (CTEPH). However, the atypical appearance of a heterogeneous filling defect in CT pulmonary angiography (CTPA) should prompt further investigation. CASE PRESENTATION A previously healthy young man presented with massive haemoptysis, acute respiratory distress, and progressive exertional dyspnea since the year before. Echocardiography demonstrated severe right ventricular dysfunction and highly probable pulmonary hypertension. CTPA revealed an extensive filling defect with an appearance concerning PAS. Due to syncopal episodes at rest, the patient underwent urgent pulmonary artery endarterectomy (PEA). A massive tree-like tumour was excised as a result. Post-operatively, reperfusion injury and refractory pulmonary oedema mandated extracorporeal membrane oxygenation (ECMO). Unfortunately, ECMO was complicated with massive haemolysis and acute kidney injury. The patient succumbed to multi-organ failure. Through tissue analysis established a diagnosis of embryonal rhabdomyosarcoma. DISCUSSION Unfortunately, the patient had not reached out for his worsening dyspnea. PASs should not be mistaken for a thrombus and anticoagulation should be avoided. The urgent condition precluded biopsy and tissue diagnosis. Similarly, neoadjuvant chemotherapy was not feasible. Post-operatively, reperfusion injury and pulmonary oedema ensued, which mandated ECMO. This complication should be anticipated preoperatively. There is a need for more data on PASs to establish a consensus for management.
Collapse
Affiliation(s)
- Farid Rashidi
- Tuberculosis and Lung Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
| | - Eissa Bilehjani
- Cardiovascular Research Center, Madani Heart Center, Tabriz University of Medical Sciences, Tabriz, Iran
| | - Seyed Ali Mousavi-Aghdas
- Tuberculosis and Lung Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
| | - Rezayat Parvizi
- Department of Anesthesiology, Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
| |
Collapse
|
|
15
|
Sato S, Ichimura H, Kobayashi K, Kawabata S, Kawamura T, Suzuki H, Imai A, Matsuzaki K, Sakata A, Matsubara D, Sato Y. Pulmonary artery sarcoma and severe valvular diseases in late-septuagenarian women: was 2-stage surgery an appropriate strategy? A case report. Surg Case Rep 2024; 10:10. [PMID: 38190036 PMCID: PMC10774505 DOI: 10.1186/s40792-023-01805-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2023] [Accepted: 12/26/2023] [Indexed: 01/09/2024] Open
Abstract
BACKGROUND Pulmonary artery sarcomas (PASs) are rare, and complete tumor resection is often difficult at the time of detection. We encountered a case of PAS that was thought to be resectable; however, the patient had severe symptomatic valvular disease. We faced a difficult decision regarding the surgical strategy. CASE PRESENTATION A 76-year-old female presented with a history of polysurgery for multiple primary cancers. She was referred to our department with a calcified mass in the right pulmonary artery (PA) and severe symptomatic valvular disease. After a discussion with the cardiovascular surgeon, we decided to perform a two-stage surgery. She underwent valvuloplasty through a median sternotomy, resulting in an improvement in her exertional dyspnea. The tumor was removed three months later with a right upper lobectomy and PA patch reconstruction through a posterolateral thoracotomy. When the PA was opened, the edge of the tumor was entrapped by vascular clamp forceps because of insufficient dissection of the adhesions between the superior vena cava and the right main PA resulting from the first operation. The patient underwent proton therapy twice for chest wall metastases which recurred three months after surgery, and local recurrence in the PA was diagnosed five months after surgery. The patient was alive with stable disease 25 months after surgery. CONCLUSION Two-stage surgery for PAS and valvular disease resulted in incomplete resection of the PAS in the right PA. It is important not to underestimate surgical adhesions due to the initial surgery and to consider and implement measures to prevent adhesions of critical vessels during the second operation.
Collapse
Affiliation(s)
- Sakiko Sato
- Department of Thoracic Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Hideo Ichimura
- Department of Thoracic Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan.
- Department of Thoracic Surgery, Institute of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.
| | - Keisuke Kobayashi
- Department of Thoracic Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Shuntaro Kawabata
- Department of Thoracic Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Tomoyuki Kawamura
- Department of Thoracic Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Hisashi Suzuki
- Department of Thoracic Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Akito Imai
- Department of Cardiovascular Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Kanji Matsuzaki
- Department of Cardiovascular Surgery, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Akiko Sakata
- Department of Pathology, Hitachi General Hospital, Hitachi, Ibaraki, 317-0077, Japan
| | - Daisuke Matsubara
- Department of Diagnostic Pathology, University of Tsukuba, Tsukuba, Ibaraki, 305-8575, Japan
| | - Yukio Sato
- Department of Thoracic Surgery, Institute of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| |
Collapse
|
|
16
|
Goldberg A, Chen JN, Breaux J, Pope H. Spindly reason for a pulmonary embolism. BMJ Case Rep 2024; 17:e258084. [PMID: 38182162 PMCID: PMC10773297 DOI: 10.1136/bcr-2023-258084] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/15/2023] [Indexed: 01/07/2024] Open
Abstract
Pulmonary artery intimal sarcomas (PAISs) are rare, malignant tumours that arise from the intimal or intramural wall of the pulmonary artery and are often mistaken for pulmonary emboli. Diagnosis and treatment of this condition are difficult due to the lack of formal guidelines. Initiating treatment as soon as possible after diagnosis is essential for maximising survival. Here, we present the case of a patient with a PAIS that initially presented similar to pulmonary thromboembolic disease and was treated with a multimodal approach.
Collapse
Affiliation(s)
- Alec Goldberg
- Internal Medicine, Tulane University School of Medicine, New Orleans, Louisiana, USA
| | - Jason N Chen
- Internal Medicine, NYU Grossman School of Medicine, New York, New York, USA
| | - John Breaux
- Cardiothoracic Surgery, Lakeview Regional Medical Center, Covington, Louisiana, USA
| | - Helen Pope
- Internal Medicine, Tulane University School of Medicine, New Orleans, Louisiana, USA
| |
Collapse
|
|
17
|
Gumus A, De Caevel A, Trifan BF. Pulmonary Artery Sarcoma with Extensive Invasion of the Right Ventricle: A Case Report and Review of Therapeutic Options. Ann Thorac Cardiovasc Surg 2024; 30:24-00106. [PMID: 39505543 PMCID: PMC11550908 DOI: 10.5761/atcs.cr.24-00106] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2024] [Accepted: 09/16/2024] [Indexed: 11/08/2024] Open
Abstract
Pulmonary artery sarcoma (PAS) is a rare, aggressive cancer originating from the intimal layer of the pulmonary artery (PA), often mistaken for pulmonary thromboembolism. This case report underscores the complex management of PAS and the necessity of a multidisciplinary approach for accurate diagnosis and treatment. A 52-year-old woman with PAS was diagnosed using imaging and therapeutic tests to distinguish it from pulmonary embolism. Primary treatment included surgical resection of the pulmonary trunk, valve, and tumor, followed by reconstruction. Complete resection was impossible due to extensive endocardial infiltration in the right ventricle, precluding cardiac transplant. The patient underwent adjuvant radiotherapy; however, the disease recurred, and she died 3 years post-diagnosis. This case highlights the rarity of an extensive right ventricle invasion, the absence of clear PAS management guidelines, and the limited evidence on the effectiveness of adjuvant therapies. It concludes that multidisciplinary teams are vital for decision-making and stresses the need for further research to establish effective treatment protocols.
Collapse
Affiliation(s)
- Alev Gumus
- Department of Cardiovascular Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Alain De Caevel
- Department of Cardiology, Clinique Notre Dame de Grâce, Gosselies, Charleroi, Belgium
| | - Bogdan F Trifan
- Department of Cardiovascular Surgery, Grand Hôpital de Charleroi, Charleroi, Belgium
| |
Collapse
|
|
18
|
Chan EY, Ali A, Zubair MM, Nguyen DT, Ibarra-Cortez SH, Graviss EA, Shapira OM, Ravi V, MacGillivray TE, Reardon MJ. Primary cardiac sarcomas: Treatment strategies. J Thorac Cardiovasc Surg 2023; 166:828-838.e2. [PMID: 35219517 DOI: 10.1016/j.jtcvs.2021.10.070] [Citation(s) in RCA: 14] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/14/2021] [Revised: 09/28/2021] [Accepted: 10/13/2021] [Indexed: 10/19/2022]
Abstract
OBJECTIVE Our multidisciplinary cardiac tumor team now has an experience of operating on 122 cases of primary cardiac sarcoma over a 23-year period. The purpose of this study is to present our short- and long-term outcomes for cardiac sarcoma. METHODS We performed a retrospective review of a prospectively collected Institutional Review Board-approved cardiac tumor database for cardiac sarcoma. Patient characteristics, surgical factors, and patient outcomes were analyzed. Perioperative data were collected from direct patient communication and all available medical records. The primary end point was all-cause mortality at 1, 3, and 5 years from the time of our surgery and 1, 3, and 5 years from the initial diagnosis. The secondary end point was all-cause mortality between the first and second halves of the study. RESULTS From October 1998 to April 2021, we operated on 122 patients with a primary cardiac sarcoma. The mean age was 45.3 years old, and 52.5% were male. Tumors were most frequently found in the left atrium (40.2%) and right atrium (32.0%). The most common type of tumor histologically was an angiosarcoma (38.5%), followed by high-grade sarcoma (14.8%). Survival from initial diagnosis at 1, 3, and 5 years was 88.4%, 43.15%, and 27.8%, respectively. Survival from surgery at our institution at 1 and 3 years was 57.1% and 24.5%, respectively. When comparing outcomes from different time periods, we found no significant difference in survival between the previous era (1998-2011) and the current era (2011-2021). CONCLUSIONS Management of these complex patients can show reasonable outcomes in centers with a multidisciplinary cardiac tumor team. Mortality has not improved with time and is likely related to the systemic nature of this disease.
Collapse
Affiliation(s)
- Edward Y Chan
- Division of Thoracic Surgery, Department of Surgery, Houston Methodist Hospital, Houston, Tex
| | - Areeba Ali
- Department of Structural Heart and Interventional Cardiology, Houston Methodist Research Institute, Houston Methodist Hospital, Houston, Tex
| | - M Mujeeb Zubair
- Department of Cardiovascular Surgery, Houston Methodist Hospital, Houston, Tex
| | - Duc T Nguyen
- Department of Pathology and Genomic Medicine, Houston Methodist Research Institute, Houston Methodist Hospital, Houston, Tex
| | - Sergio H Ibarra-Cortez
- Department of Structural Heart and Interventional Cardiology, Houston Methodist Research Institute, Houston Methodist Hospital, Houston, Tex
| | - Edward A Graviss
- Department of Pathology and Genomic Medicine, Houston Methodist Research Institute, Houston Methodist Hospital, Houston, Tex
| | - Oz M Shapira
- Department of Cardiothoracic Surgery, Hebrew University, Hadassa Medical Center, Jerusalem, Israel
| | - Vinod Ravi
- Department of Sarcoma Medical Oncology, MD Anderson Cancer Center, Houston, Tex
| | | | - Michael J Reardon
- Department of Cardiovascular Surgery, Houston Methodist Hospital, Houston, Tex.
| |
Collapse
|
|
19
|
Başar V, Ermerak NO, Olgun Yıldızeli Ş, Bozkurtlar E, Ercelep Ö, Mutlu B, Kocakaya D, Bekiroğlu GN, Taş S, Yanartaş M, Sunar H, Ak K, Küçükoğlu S, Yıldızeli B. Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival? TURK GOGUS KALP DAMAR CERRAHISI DERGISI 2023; 31:388-397. [PMID: 37664762 PMCID: PMC10472466 DOI: 10.5606/tgkdc.dergisi.2023.23906] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 08/02/2022] [Accepted: 12/16/2022] [Indexed: 09/05/2023]
Abstract
Background In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease. Methods Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery. Results Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median followup was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas. Conclusion Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.
Collapse
Affiliation(s)
- Veysel Başar
- Department of Cardiovascular Surgery, University of Health Sciences, Koşuyolu High Specialization Education and Research Hospital, Istanbul, Türkiye
| | - N. Onur Ermerak
- Department of Thoracic Surgery, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - Şehnaz Olgun Yıldızeli
- Department of Pulmonology and Intensive Care, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - Emine Bozkurtlar
- Department of Pathology, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - Özlem Ercelep
- Department of Medical Oncology, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - Bülent Mutlu
- Department of Cardiology, Istanbul University Institute of Cardiology, Istanbul, Türkiye
| | - Derya Kocakaya
- Department of Pulmonology and Intensive Care, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - G. Nural Bekiroğlu
- Department of Biostatistics, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - Serpil Taş
- Department of Cardiovascular Surgery, University of Health Sciences, Koşuyolu High Specialization Education and Research Hospital, Istanbul, Türkiye
| | - Mehmed Yanartaş
- Department of Cardiovascular Surgery, University of Health Sciences, Koşuyolu High Specialization Education and Research Hospital, Istanbul, Türkiye
| | - Hasan Sunar
- Department of Cardiovascular Surgery, University of Health Sciences, Koşuyolu High Specialization Education and Research Hospital, Istanbul, Türkiye
| | - Koray Ak
- Department of Cardiovascular Surgery, Marmara University Faculty of Medicine, Istanbul, Türkiye
| | - Serdar Küçükoğlu
- Department of Cardiology, Istanbul University Institute of Cardiology, Istanbul, Türkiye
| | - Bedrettin Yıldızeli
- Department of Thoracic Surgery, Marmara University Faculty of Medicine, Istanbul, Türkiye
| |
Collapse
|
|
20
|
Giner F, Machado I, Rubio-Martínez LA, López-Guerrero JA, Claramunt-Alonso R, Navarro S, Ferrández A, Mayordomo-Aranda E, Llombart-Bosch A. Intimal Sarcoma with MDM2/CDK4 Amplification and p16 Overexpression: A Review of Histological Features in Primary Tumor and Xenograft, with Immunophenotype and Molecular Profiling. Int J Mol Sci 2023; 24:ijms24087535. [PMID: 37108696 PMCID: PMC10141691 DOI: 10.3390/ijms24087535] [Citation(s) in RCA: 11] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2023] [Revised: 04/12/2023] [Accepted: 04/18/2023] [Indexed: 04/29/2023] Open
Abstract
Intimal sarcomas (IS) are rare malignant mesenchymal tumors arising in large blood vessels of the systemic and pulmonary circulation and also in the heart. They are morphologically similar to other spindle cell, poorly differentiated sarcomas. The prognosis is poor and depends mainly on surgical options. Three cases of IS were collected from two institutions. Clinical data were retrieved and histological study was performed. A wide immunohistochemical panel was analyzed. FISH of MDM2 gene was performed, and a molecular study with NGS was implemented in all cases. The mean age of our cases was 54 years. Histologically, the tumors presented a diffuse growth pattern with heterogeneous atypical epithelioid or spindle cells and extensive thrombosed areas. All cases presented intense immunoexpression for MDM2, CDK4, CD117, c-myc, PDGFRA, and p16. PDGFRA, HTERT, and pan-TRK gained expression, while p16 lost intensity, being weaker in both the local recurrences and xenografts. The three cases showed amplification of MDM2 by FISH. NGS analysis revealed amplifications in the CDK4, PDGFRA, and KIT genes, together with BRAF mutation and KRAS amplification. P16 was expressed in all cases, losing intensity in local recurrence and xenografts. Two new alterations, a BRAF mutation and a KRAS amplification, were detected by NGS in different tumors, opening up new therapeutic options for these patients.
Collapse
Affiliation(s)
- Francisco Giner
- Pathology Department, University of Valencia, 46010 Valencia, Spain
- Pathology Department, Hospital Universitari i Politècnic La Fe of Valencia, 46026 Valencia, Spain
| | - Isidro Machado
- Pathology Department, Instituto Valenciano de Oncología, 46009 Valencia, Spain
| | | | | | | | - Samuel Navarro
- Pathology Department, Hospital Clínic Universitari, 46010 Valencia, Spain
| | - Antonio Ferrández
- Pathology Department, Hospital Clínic Universitari, 46010 Valencia, Spain
| | - Empar Mayordomo-Aranda
- Pathology Department, Hospital Universitari i Politècnic La Fe of Valencia, 46026 Valencia, Spain
| | | |
Collapse
|
|
21
|
Koike T, Iwata H, Hirose K, Minamino T. A case report of pulmonary artery intimal sarcoma negative for 18F-FDG mimicking pulmonary thromboembolism. Eur Heart J Case Rep 2023; 7:ytad140. [PMID: 37123654 PMCID: PMC10141459 DOI: 10.1093/ehjcr/ytad140] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2022] [Revised: 09/22/2022] [Accepted: 03/24/2023] [Indexed: 05/02/2023]
Abstract
Background Pulmonary artery sarcoma is a rare malignant neoplasm arising from intimal mesenchymal cells in the pulmonary artery wall and is often difficult to differentiate from pulmonary embolism, however, 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can be useful for a differential diagnosis. Here, we present a rare case of pulmonary sarcoma undetectable by PET. Case summary A 77-year-old woman who had worsening dyspnoea on effort for a month and progressive chest discomfort with nocturnal cough for a week presented to our hospital. Contrast-enhanced computed tomography (CT) demonstrated a massive filling defect in the left pulmonary artery (PA). Two major differential diagnoses were considered; pulmonary thromboembolism and tumour-like lesions. Positron emission tomography-computed tomography (PET-CT) revealed that there was no abnormal accumulation of 18F-FDG in the mass. However, even after effective anti-thrombotic treatment for 3 weeks, a follow-up CT showed no reduction at all in the size of the lesion in the pulmonary artery. Therefore, surgery for diagnostic therapeutic purposes was performed. Discussion The present case is informative because it supports the idea that being aware of PA angiosarcoma as a potential differential diagnosis of pulmonary thromboembolism is essential, particularly in cases of no evident peripheral venous thrombosis and a negative D-dimer test, even if neither heterogenous contrast enhancement in CT and magnetic resonance imaging nor accumulation of 18-FDG in PET-CT is evident.
Collapse
Affiliation(s)
- Takuma Koike
- Department of Cardiovascular Biology and Medicine, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan
| | - Hiroshi Iwata
- Corresponding author. Tel: +81-3-3813-3111, Fax: +81-3-5689-0627,
| | - Kuniaki Hirose
- Department of Cardiovascular Biology and Medicine, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan
- The Department of Cardiology, Koshigaya Municipal Hospital, 10-32, Higashikoshigaya, Koshigaya, Saitama 343-8577, Japan
| | | |
Collapse
|
|
22
|
Catalano M, Crimi L, Galioto F, Coronella M, Foti PV, Palmucci S, Basile A. Not always embolism: A case of pulmonary artery intimal sarcoma - The role of the radiologist in early diagnosis. Respir Med Case Rep 2023; 42:101822. [PMID: 36874264 PMCID: PMC9975687 DOI: 10.1016/j.rmcr.2023.101822] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2023] [Revised: 02/14/2023] [Accepted: 02/16/2023] [Indexed: 02/22/2023] Open
Abstract
Introduction Pulmonary artery intimal sarcoma (PAIS) is a rare malignant neoplasm with imaging features that can mimic pulmonary embolism (PE). It must be recognized early because a radical resection may be useful to prolong survival. Case description A clinical case of a 57-year-old Caucasian male affected by PAIS is presented, which describes the computed tomography (CT) findings found in PAIS and the elements of overlap and differentiation with PE. The main common element is represented by the endoluminal filling defect of the pulmonary arterial vessels in contrast-enhanced CT examinations; a characteristic polypoid morphology or polylobulated contours are typical findings of PAIS. Other specific elements of the neoplasm such as wall eclipse sign, extension beyond the arterial wall, and metastasis are also explained. Conclusions The overlap of the clinical-radiological findings and the epidemiological difference between PAIS and PE cause a diagnostic delay. By knowing the differential elements, the radiologist can detect the neoplasm early to accelerate diagnosis and suggest optimal management.
Collapse
Affiliation(s)
- Marco Catalano
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| | - Luca Crimi
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| | - Federica Galioto
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| | - Maria Coronella
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| | - Pietro Valerio Foti
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| | - Stefano Palmucci
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| | - Antonio Basile
- Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, Radiology Unit 1, University Hospital Policlinico “G. Rodolico-San Marco”, Catania, 95123, Italy
| |
Collapse
|
|
23
|
Chan EY, Ravi V, Ali A, Nguyen DT, Graviss EA, MacGillivray TE, Reardon MJ. Surgical Management of Primary Pulmonary Artery Sarcoma. Semin Thorac Cardiovasc Surg 2023; 35:53-64. [PMID: 34743005 DOI: 10.1053/j.semtcvs.2021.10.013] [Citation(s) in RCA: 9] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2021] [Accepted: 10/22/2021] [Indexed: 11/11/2022]
Abstract
Primary pulmonary artery sarcoma is a rare cardiac tumor with a dismal prognosis without surgical therapy. It is often confused with the more common chronic pulmonary emboli which may delay the appropriate diagnosis or lead to suboptimal surgery. The objective of this study was to evaluate the short and long-term survival and local recurrence rate of pulmonary artery sarcoma cases operated on at our institution using an anatomic resection approach for the pulmonary trunk and main pulmonary arteries rather than endarterectomy. We searched our prospectively collected cardiac tumor database for cases of primary pulmonary sarcoma operated at our institution between June 2000 and September 2018 and followed until January 3, 2021. We used an anatomic resection and replacement technique for involved pulmonary root and main pulmonary arteries with endarterectomy used only for disease distal to the first arterial branch when lung preservation was possible. The primary endpoints for our study were survival from the time of initial diagnosis and survival from the time of our surgery. Secondary endpoints were operative 30-day mortality and incidence of local recurrence or metastatic disease. We identified 20 consecutive cases of surgical resection of primary pulmonary sarcoma. The median age at surgery was 52.5 years (IQR 43.5-60.5). Complete pulmonary root resection and reconstruction using a pulmonary homograft were needed in 16/20 (80%) of cases. All resections employed cardiopulmonary bypass with cardioplegic arrest. A pneumonectomy was needed in 7/20 (35%) of patients. A negative margin (R0) resection was achieved in 9 patients (45%) and margins were microscopically positive (R1) on final pathology in 9 patients (45%). Two patients (10%) had gross tumor (R2) at the resection margin. Operative mortality was 2/20 (10%). Median survival was 2.8 years from diagnosis (95% CI 1.3-8.8) and 2.7 years from surgery by our team (95% CI 0.8-5.9). Survival from first initial diagnosis at 1, 3, 5, and 10 years was 85.0%, 49.1%, 49.1%, and 16.4%. Survival from our surgery by our team at 1, 3, 5, and 10 years was 70%, 48.8%, 41.8%, and 8.4%. Surgical resection of primary pulmonary artery sarcoma with an approach utilizing an anatomic resection of the pulmonary root and main pulmonary arteries when involved and pneumonectomy or endarterectomy when there is disease distal to the first branch artery can be done with a reasonable operative risk and long-term survival when compared to the natural history of the disease.
Collapse
Affiliation(s)
- Edward Y Chan
- Department of Surgery, Houston Methodist Hospital, Houston, Texas
| | - Vinod Ravi
- Department of Sarcoma Oncology, MD Anderson Cancer Center, Houston, Texas
| | - Areeba Ali
- Houston Methodist Research Institute, Houston, Texas
| | - Duc T Nguyen
- Department of Pathology and Genomic Medicine, Houston Methodist Research Institute
| | - Edward A Graviss
- Department of Pathology and Genomic Medicine, Houston Methodist Research Institute, Houston Methodist Hospital, Houston, Texas
| | | | - Michael J Reardon
- Department of Cardiovascular Surgery, Houston Methodist Hospital, Houston, Texas.
| |
Collapse
|
|
24
|
Arslanhan G, Ak K, Öztürk F, Türker A, Bozkurtlar E, Arsan S, Yıldızeli B. Pulmonary artery sarcoma treated with pulmonary endarterectomy and leaflet reconstruction using Ozaki technique. J Card Surg 2022; 37:5630-5633. [PMID: 36378914 DOI: 10.1111/jocs.17172] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2022] [Revised: 10/31/2022] [Accepted: 10/31/2022] [Indexed: 11/16/2022]
Abstract
Pulmonary arterial intimal sarcomas (PAIS) are rare malignancies with a poor prognosis. Sarcomas present with signs and symptoms mimicking pulmonary thromboembolic disease, delaying the diagnosis. We present a 29-year-old male patient diagnosed with PAIS in the right and main pulmonary arteries extending to the left pulmonary leaflet. The patient was treated with pulmonary endarterectomy and pulmonary leaflet reconstruction using the Ozaki technique.
Collapse
Affiliation(s)
- Gokhan Arslanhan
- Department of Cardiovascular Surgery, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| | - Koray Ak
- Department of Cardiovascular Surgery, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| | - Fatih Öztürk
- Department of Cardiovascular Surgery, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| | - Alperen Türker
- Department of Cardiovascular Surgery, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| | - Emine Bozkurtlar
- Department of Pathology, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| | - Sinan Arsan
- Department of Cardiovascular Surgery, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| | - Bedrettin Yıldızeli
- Department of Thoracic Surgery, Marmara University School of Medicine Pendik Research and Training Hospital, Istanbul, Turkey
| |
Collapse
|
|
25
|
Zhao M, Nie P, Guo Y, Chen H. Pulmonary artery intimal sarcoma: A rare cause of filling defects in pulmonary arteries. Am J Med Sci 2022; 364:655-660. [PMID: 35588894 DOI: 10.1016/j.amjms.2022.05.009] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2021] [Revised: 10/07/2021] [Accepted: 05/11/2022] [Indexed: 01/25/2023]
Abstract
Pulmonary artery intimal sarcomas are very rare and arise from primitive pluripotent mesenchymal cells. They are often misdiagnosed as pulmonary thromboembolism, leading to futile anticoagulation treatment and delayed diagnosis. We present a case of a patient who showed nonspecific pulmonary symptoms and characteristic imaging manifestation. Progressive symptoms and additional imaging led to the suspicion of a pulmonary artery intimal sarcoma, which was finally confirmed by pathological biopsy. This case serves as a reminder to consider pulmonary artery intimal sarcomas in the differential diagnosis of patients with dyspnea and filling defects on computed tomography pulmonary angiography or contrast-enhanced computed tomography.
Collapse
Affiliation(s)
- Mimi Zhao
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003
| | - Pei Nie
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003
| | - Yonghua Guo
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003
| | - Haisong Chen
- Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao, China, 266003.
| |
Collapse
|
|
26
|
Oliveira MF, Sá I, Gonçalves F, Santos M, Reis A. A Not So Innocent Murmur. Heart Lung Circ 2022; 31:e147-e148. [PMID: 35927192 DOI: 10.1016/j.hlc.2022.04.055] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2021] [Revised: 06/11/2021] [Accepted: 04/04/2022] [Indexed: 01/27/2023]
Affiliation(s)
- Marta Fontes Oliveira
- Cardiology Department, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal.
| | - Isabel Sá
- Cardiology Department, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| | - Fabienne Gonçalves
- Pulmonary Vascular Disease Unit, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| | - Mário Santos
- Cardiology Department, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal; Pulmonary Vascular Disease Unit, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| | - Abílio Reis
- Pulmonary Vascular Disease Unit, Centro Hospitalar Universitário do Porto, Largo do Prof. Abel Salazar, Porto, Portugal
| |
Collapse
|
|
27
|
Murphy CG, Goldstein JM, Besharati S, Kobsa S, Salvatore MM, Rosenzweig EB, Ingham M, Del Portillo A, Takeda K, Chandra S, Furfaro D. A 52-Year-Old Man With Chest Pain and Dyspnea. Chest 2022; 162:e259-e264. [PMID: 36344135 PMCID: PMC9808716 DOI: 10.1016/j.chest.2022.05.020] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2022] [Accepted: 05/14/2022] [Indexed: 11/06/2022] Open
Abstract
CASE PRESENTATION A 52-year-old man came to the cardiac surgery clinic for pulmonary thromboendarterectomy (PTE) evaluation. He had initially appeared at an outside hospital 1 year earlier, with chest pain and shortness of breath. He had no known chronic conditions. A CT pulmonary angiogram (CTPA) at that time showed a filling defect at the bifurcation of the main pulmonary artery. A transthoracic echocardiogram revealed mild mitral valve regurgitation, but otherwise the results were normal. As he was hemodynamically stable and not hypoxemic, he was treated solely by anticoagulation. Despite adhering to prescribed apixaban, he developed progressive dyspnea and reduced exercise tolerance over the subsequent year. A repeat CTPA performed 12 months after the initial presentation showed a persistent filling defect at the level of the pulmonary artery bifurcation, with a new extension now completely occluding the right main pulmonary artery. A pulmonary angiogram confirmed this complete occlusion, and right heart catheterization revealed precapillary pulmonary hypertension, with a mean pulmonary artery pressure of 50 mm Hg. His anticoagulation was transitioned to enoxaparin for presumed apixaban treatment failure, and an investigation for hypercoagulable conditions was initiated. His lupus anticoagulant test result was positive, but he did not meet the criteria for antiphospholipid syndrome because he was negative for anticardiolipin and β2-glycoprotein antibodies. Assays for antithrombin III, protein C, prothrombin gene, and factor V Leiden mutations produced normal results.
Collapse
Affiliation(s)
- Charles G Murphy
- Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, Columbia University Irving Medical Center, New York, NY.
| | - Jonathan M Goldstein
- Department of Radiology, Columbia University Irving Medical Center, New York, NY
| | - Sepideh Besharati
- Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, NY
| | - Serge Kobsa
- Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Columbia University Irving Medical Center, New York, NY
| | - Mary M Salvatore
- Department of Radiology, Columbia University Irving Medical Center, New York, NY
| | - Erika B Rosenzweig
- Division of Pediatric Cardiology, Department of Pediatrics, Columbia University Irving Medical Center, New York, NY
| | - Matthew Ingham
- Division of Hematology and Oncology, Department of Medicine, Columbia University Irving Medical Center, New York, NY
| | - Armando Del Portillo
- Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, NY
| | - Koji Takeda
- Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Columbia University Irving Medical Center, New York, NY
| | - Subani Chandra
- Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, Columbia University Irving Medical Center, New York, NY
| | - David Furfaro
- Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
| |
Collapse
|
|
28
|
Shao N, Deng C. Pulmonary thromboembolic disease or pulmonary artery intimal sarcoma: Case report and literature review. Oncol Lett 2022; 24:350. [PMID: 36168308 PMCID: PMC9478618 DOI: 10.3892/ol.2022.13470] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2022] [Accepted: 07/11/2022] [Indexed: 11/11/2022] Open
Abstract
The present case study reported on a patient initially diagnosed with pulmonary embolism at the First Affiliated Hospital of Fujian Medical University (Fuzhou, China) in May 2021. Furthermore, a relevant literature review was performed. The patient was a 57-year-old Chinese male who presented with dyspnea and wheezing following exercise. Physical examination revealed pulmonary valve second heart sound > aortic valve second heart sound but lack of swelling on both lower limbs, while the imaging results suggested pulmonary artery filling defects. Initially, the patient was diagnosed with pulmonary embolism and was administered anticoagulation treatment, which lasted for 3 months but proved to be ineffective. Subsequent re-examination via chest computed tomography further indicated multiple nodules in the left hilum and lung. Therefore, the patient was hospitalized for lung aspiration biopsy, which led to the final diagnosis of pulmonary artery intimal sarcoma based on the pathological review.
Collapse
Affiliation(s)
- Nan Shao
- Division of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Fujian Medical University, Fuzhou, Fujian 350005, P.R. China
| | - Chaosheng Deng
- Division of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Fujian Medical University, Fuzhou, Fujian 350005, P.R. China
| |
Collapse
|
|
29
|
Improving the imaging diagnostic strategy for pulmonary artery masses based on 18F-FDG PET/CT integrated with CTPA. Eur J Nucl Med Mol Imaging 2022; 49:4109-4121. [PMID: 35732973 DOI: 10.1007/s00259-022-05851-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2022] [Accepted: 05/24/2022] [Indexed: 11/04/2022]
Abstract
OBJECTIVE To evaluate the diagnostic accuracy of computed tomography pulmonary angiography (CTPA) and 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) for pulmonary artery (PA) masses. METHODS Of 2889 patients with PA filling defects of PA on CTPA, 79 consecutive patients suspicious for PA malignancy who subsequently underwent 18F-FDG PET/CT were enrolled. All masses were diagnosed on the basis of pathological findings or clinical imaging follow-up. For each mass, morphological CT signs, standardized uptake value (SUVmax and SUVmean), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) on 18F-FDG PET/CT were used as diagnostic markers. RESULTS Expansive growth, irregular margin, invasion, CT contrast uptake, and wall eclipse sign were strongly associated with the malignant nature of masses. The coexistence of at least 5 CT signs perfectly identified malignant masses, whereas the detection of no more than 4 CT signs did not accurately discriminate between the natures of masses. Mean SUVmax, SUVmean, MTV, and TLG values were significantly higher in malignant masses compared to those in benign masses. The diagnostic accuracy of 18F-FDG PET/CT parameters (SUV, MTV, and TLG) was excellent in detecting malignant masses. Among patients with 3 or 4 pathological CT signs, SUVmax > 3.4 significantly increased the identification of malignancies. CONCLUSIONS CTPA is a useful imaging modality for diagnosing PA masses, especially when at least 5 abnormal CT signs are identified. Similarly, 18F-FDG PET/CT accurately identified malignant masses and provided additional valuable information on diagnostic uncertainties after CTPA.
Collapse
|
|
30
|
Lashari BH, Kumaran M, Aneja A, Bull T, Rali P. Beyond Clots in the Pulmonary Circulation: Pulmonary Artery Tumors Mimicking Pulmonary Embolism. Chest 2022; 161:1642-1650. [PMID: 35041833 DOI: 10.1016/j.chest.2022.01.013] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/30/2021] [Revised: 01/02/2022] [Accepted: 01/10/2022] [Indexed: 10/19/2022] Open
Abstract
Pulmonary embolism (PE) is the most common filling defect seen on CT scan pulmonary angiography. Pulmonary artery (PA) tumors can mimic PE on imaging and clinical presentation. One classic feature of tumors is failure to improve on anticoagulation. PA tumors, particularly malignant ones, have radically different treatments and usually have a grim prognosis. Thus, it is essential that PA tumors, when suspected, receive an expedited confirmatory diagnosis followed by multidisciplinary treatment at an expert center. In this review, we present clinical, imaging, and histopathologic features of benign and malignant PA tumors, emphasizing differentiating features from PE. We also describe available diagnostic and treatment methods for PA tumors.
Collapse
Affiliation(s)
- Bilal Haider Lashari
- Department of Thoracic Medicine and Surgery, Temple University Hospital, Philadelphia, PA.
| | - Maruti Kumaran
- Department of Radiology, Lewis Katz School of Medicine at Temple University, Philadelphia, PA
| | - Amandeep Aneja
- Department of Pathology, Lewis Katz School of Medicine at Temple University, Philadelphia, PA
| | - Todd Bull
- Department of Medicine, Pulmonary Sciences and Critical Care, University of Colorado, Aurora, CO
| | - Parth Rali
- Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, PA
| |
Collapse
|
|
31
|
Monteagudo-Vela M, Alayza AR, Santín EM, Cecconi A, Copa GR. PULMONARY ARTERY SARCOMA WITH EXTENSIVE LEIOMYOSARCOMA DIFFERENTIATION AND HETEROLOGOUS OSTEOSARCOMATOUS ELEMENTS. Cardiovasc Pathol 2022; 60:107436. [PMID: 35597407 DOI: 10.1016/j.carpath.2022.107436] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2022] [Revised: 05/13/2022] [Accepted: 05/15/2022] [Indexed: 11/29/2022] Open
Abstract
Pulmonary artery sarcomas are exceptionally unusual. Their clinic, diagnosis and treatment play a very important role in the ultimate outcome and long-term survival. We present the case of a 70-year-old gentleman diagnosed with a leiomyosarcoma of the pulmonary artery with osteosarcoma differentiation that underwent surgical resection and subsequent chemotherapy, with good recovery at 9 months follow-up. Late diagnosis and incomplete surgical resection will worsen the short- and long-term prognosis of these patients.
Collapse
Affiliation(s)
- María Monteagudo-Vela
- Cardiothoracic Surgery Department, Hospital Universitario de la Princesa, Madrid, Spain.
| | | | - Emilio Monguió Santín
- Cardiothoracic Surgery Department, Hospital Universitario de la Princesa, Madrid, Spain
| | - Alberto Cecconi
- Cardiology Department, Hospital Universitario de la Princesa, Madrid, Spain
| | - Guillermo Reyes Copa
- Cardiothoracic Surgery Department, Hospital Universitario de la Princesa, Madrid, Spain
| |
Collapse
|
|
32
|
Funauchi Y, Takase T, Miyoshi T, Miyashita N, Kimura M, Nakazawa G. The Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma: Two Case Reports. Intern Med 2022; 61:667-671. [PMID: 34471021 PMCID: PMC8943374 DOI: 10.2169/internalmedicine.7666-21] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
Pulmonary artery sarcoma (PAS) is considered a very rare tumor with a poor prognosis. We herein report two cases of PAS that were diagnosed by positron emission tomography (PET)/computed tomography (CT). In both cases, PET was an effective option for diagnosing tumors, and surgical resection was a valid treatment for these diseases. If a pulmonary artery tumor is suspected, PET/CT is useful for diagnosing PAS and very helpful for choosing the surgical treatment strategy.
Collapse
Affiliation(s)
- Yohei Funauchi
- Division of Cardiology, Kindai University Faculty of Medicine, Japan
| | - Toru Takase
- Division of Cardiology, Kindai University Faculty of Medicine, Japan
| | - Tatsuya Miyoshi
- Division of Cardiology, Kindai University Faculty of Medicine, Japan
| | - Naoya Miyashita
- Division of Cardiovascular Surgery, Kindai University Faculty of Medicine, Japan
| | - Masatomo Kimura
- Division of Pathology, Kindai University Faculty of Medicine, Japan
| | - Gaku Nakazawa
- Division of Cardiology, Kindai University Faculty of Medicine, Japan
| |
Collapse
|
|
33
|
Saxena A, Laycock A, Leong J, Merry CJ. Rare case of pulmonary artery intimal sarcoma managed by pneumonectomy. ANZ J Surg 2022; 92:3075-3077. [PMID: 35188318 DOI: 10.1111/ans.17542] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2020] [Revised: 01/02/2022] [Accepted: 02/05/2022] [Indexed: 12/01/2022]
Affiliation(s)
- Akshat Saxena
- Department of Cardiothoracic Surgery, Fiona Stanley Hospital, Perth, Australia.,Department of Respiratory Medicine, Fiona Stanley Hospital, Perth, Australia
| | - Andrew Laycock
- Department of Pathology, Fiona Stanley Hospital, Perth, Australia
| | - Jeanie Leong
- Department of Respiratory Medicine, Fiona Stanley Hospital, Perth, Australia
| | - Christopher J Merry
- Department of Cardiothoracic Surgery, Fiona Stanley Hospital, Perth, Australia
| |
Collapse
|
|
34
|
Alozie A, Werner D, Schafmayer C, Yilmaz K, Skusa K, Meinel FG, Zimpfer A, Öner A, Dohmen P. Management of Pulmonary Artery Synovial Sarcoma: The 3-Step Surgical Approach. Ann Thorac Surg 2022; 114:e443-e445. [DOI: 10.1016/j.athoracsur.2022.02.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/05/2021] [Revised: 02/05/2022] [Accepted: 02/13/2022] [Indexed: 11/01/2022]
|
|
35
|
Tuft C, Maheepala K, Raguparan A, Naeem A, Lodh S, Lindstrom S. Pulmonary artery sarcoma: An important mimic of pulmonary embolism-Case reports and literature review. Respirol Case Rep 2022; 10:e0897. [PMID: 35028154 PMCID: PMC8743422 DOI: 10.1002/rcr2.897] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2021] [Revised: 11/27/2021] [Accepted: 12/06/2021] [Indexed: 11/10/2022] Open
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d-dimer, troponin T or NT-proBNP, as well as characteristic findings on CT pulmonary angiography such as the 'wall eclipsing sign' and an non-dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2-year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.
Collapse
Affiliation(s)
- Colin Tuft
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Krishan Maheepala
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Ajantha Raguparan
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Anas Naeem
- Intensive Care UnitSt George HospitalSydneyNew South WalesAustralia
| | - Suhrid Lodh
- Radiology DepartmentSt George HospitalSydneyNew South WalesAustralia
| | - Steven Lindstrom
- Respiratory and Sleep Medicine DepartmentSt George HospitalSydneyNew South WalesAustralia
| |
Collapse
|
|
36
|
Hassler KR, Puig CA, Cangut B, Pochettino A, Robinson SI, Morris JM, Blackmon SH. Pulmonary Artery Sarcoma Complete Resection Facilitated by Three-Dimensional Printed Model. Ann Thorac Surg 2022; 114:e375-e378. [PMID: 35051392 DOI: 10.1016/j.athoracsur.2021.11.072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2021] [Revised: 11/18/2021] [Accepted: 11/20/2021] [Indexed: 11/01/2022]
Abstract
Primary pulmonary (PA) artery sarcomas are rare tumors and are commonly misdiagnosed as pulmonary embolism. PA sarcomas demonstrate intraluminal growth into the vessel rather than through the wall, require complete resection to enhance survival, and require complex surgical planning. The purpose of this case report is to describe an optimal team approach with multidisciplinary planning facilitated by a customized three-dimensional (3D) model to guide intervention and enhance communication.
Collapse
Affiliation(s)
| | - Carlos A Puig
- Department of Thoracic Surgery, Mayo Clinic, Rochester, MN
| | - Busra Cangut
- Department of Cardiac Surgery, Mayo Clinic, Rochester, MN
| | | | | | - Jonathan M Morris
- Department of Radiology and the Anatomic Modeling Lab, Mayo Clinic, Rochester, MN
| | | |
Collapse
|
|
37
|
Chuang TM, Hsiao HH, Tsai KB. Primary intimal sarcoma with chondrosarcoma differentiation of the pulmonary artery. JOURNAL OF CANCER RESEARCH AND PRACTICE 2022. [DOI: 10.4103/jcrp.jcrp_37_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
|
|
38
|
Darawsha F, Kramer R, Raanani E, Saute M. OUP accepted manuscript. Interact Cardiovasc Thorac Surg 2022; 35:6570591. [PMID: 35438178 PMCID: PMC9419682 DOI: 10.1093/icvts/ivac096] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2021] [Revised: 03/14/2022] [Accepted: 04/14/2022] [Indexed: 11/13/2022] Open
Abstract
Pulmonary artery angiosarcoma is a rare malignant vascular tumour with a poor prognosis and a grim clinical course. Common clinical presentations include shortness of breath, coughing and haemoptysis. Differential diagnosis includes thromboembolism and lung carcinoma. Rarity of the tumour and the consequent lack of treatment guidelines further worsen the prognosis. We report a case of pulmonary artery angiosarcoma involving the main pulmonary artery and its bifurcation with emphasis on the surgical treatment.
Collapse
Affiliation(s)
- Firas Darawsha
- Division of Cardio-Thoracic and Vascular Surgery, Sheba Medical Center, Tel Hashomer, Israel
| | - Ran Kramer
- Division of Cardio-Thoracic and Vascular Surgery, Sheba Medical Center, Tel Hashomer, Israel
| | - Ehud Raanani
- Division of Cardio-Thoracic and Vascular Surgery, Sheba Medical Center, Tel Hashomer, Israel
| | - Milton Saute
- Division of Cardio-Thoracic and Vascular Surgery, Sheba Medical Center, Tel Hashomer, Israel
- Corresponding author. Department of Thoracic Surgery, Sheba Medical Center, Tel Hashomer 52621, Israel. Tel: +972 522697767; e-mail: (M. Saute)
| |
Collapse
|
|
39
|
Sharma MS. Commentary: Pulmonary artery sarcoma-an invitation to the masquerade ball. JTCVS Tech 2021; 10:315-316. [PMID: 34984393 PMCID: PMC8691909 DOI: 10.1016/j.xjtc.2021.11.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2021] [Revised: 10/29/2021] [Accepted: 11/01/2021] [Indexed: 11/22/2022] Open
Affiliation(s)
- Mahesh S. Sharma
- Department of Surgery, The University of North Carolina at Chapel Hill, Chapel Hill, NC
| |
Collapse
|
|
40
|
Rackauskas M, Beaver T. Commentary: Are we always ready for a challenging surprise? JTCVS Tech 2021; 10:320-321. [PMID: 34977747 PMCID: PMC8691815 DOI: 10.1016/j.xjtc.2021.09.046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2021] [Revised: 09/19/2021] [Accepted: 09/24/2021] [Indexed: 11/22/2022] Open
Affiliation(s)
- Mindaugas Rackauskas
- Division of Thoracic Surgery, Department of Surgery, University of Florida College of Medicine, Gainesville, Fla
| | - Thomas Beaver
- Division of Cardiac Surgery, Department of Surgery, University of Florida College of Medicine, Gainesville, Fla
| |
Collapse
|
|
41
|
Pupovac SS, Hemli JM, Sarmiento IC, Lazzaro RS, Scheinerman SJ, Brinster DR. The imitation game: Pulmonary artery sarcoma masquerading as an acute pulmonary embolism. JTCVS Tech 2021; 10:317-319. [PMID: 34977746 PMCID: PMC8691795 DOI: 10.1016/j.xjtc.2021.08.039] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2021] [Accepted: 08/27/2021] [Indexed: 11/18/2022] Open
Affiliation(s)
- Stevan S. Pupovac
- Department of Cardiothoracic Surgery, North Shore University Hospital/Northwell Health, Manhasset, NY
- Address for reprints: Stevan S. Pupovac, MD, Department of Cardiothoracic Surgery, North Shore University Hospital, 300 Community Dr, Manhasset, NY 11030.
| | - Jonathan M. Hemli
- Department of Cardiothoracic Surgery, North Shore University Hospital/Northwell Health, Manhasset, NY
| | - I. Claire Sarmiento
- Department of Cardiovascular & Thoracic Surgery, Lenox Hill Hospital/Northwell Health, New York, NY
| | - Richard S. Lazzaro
- Department of Cardiovascular & Thoracic Surgery, Lenox Hill Hospital/Northwell Health, New York, NY
| | - S. Jacob Scheinerman
- Department of Cardiovascular & Thoracic Surgery, Lenox Hill Hospital/Northwell Health, New York, NY
| | - Derek R. Brinster
- Department of Cardiovascular & Thoracic Surgery, Lenox Hill Hospital/Northwell Health, New York, NY
| |
Collapse
|
|
42
|
Malik MI, Rabbani M, Hage F, Inculet R, Chu MW. Missed pulmonary artery sarcoma requiring radical excision and pneumonectomy. JTCVS Tech 2021; 10:309-312. [PMID: 34984391 PMCID: PMC8691900 DOI: 10.1016/j.xjtc.2021.09.055] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2021] [Accepted: 09/27/2021] [Indexed: 11/28/2022] Open
Affiliation(s)
- Mohsyn Imran Malik
- Division of Cardiac Surgery, Western University, London, Ontario, Canada
| | - Mohamad Rabbani
- Division of Cardiac Surgery, Western University, London, Ontario, Canada
| | - Fadi Hage
- Division of Cardiac Surgery, Western University, London, Ontario, Canada
| | - Richard Inculet
- Division of Thoracic Surgery, Western University, London, Ontario, Canada
- Division of Oncology, Western University, London, Ontario, Canada
| | - Michael W.A. Chu
- Division of Cardiac Surgery, Western University, London, Ontario, Canada
- Address for reprints: Michael W. A. Chu, MD, MEd, London Health Sciences Centre, B6-106 University Hospital, 339 Windermere Rd, London, Ontario, Canada N6A 5A5.
| |
Collapse
|
|
43
|
Takauchi T, Murai R, Musiake K, Akaike Y, Hirayama M, Ueda A, Komiya T, Kadota K. Pedunculated pulmonary artery intimal sarcoma with poor uptake in 18F-FDG PET/CT: A case report. J Cardiol Cases 2021; 24:110-113. [PMID: 34466172 DOI: 10.1016/j.jccase.2021.02.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2020] [Revised: 02/07/2021] [Accepted: 02/13/2021] [Indexed: 11/17/2022] Open
Abstract
Pulmonary artery intimal sarcoma (PAIS) is a rare tumor with an incidence of 0.001%-0.03% that usually grows along artery walls and absorbs fluorodeoxyglucose. It is difficult to distinguish PAIS from pulmonary thromboembolism due to the similarities of their symptoms. Therefore, contrast-enhanced computed tomography and positron emission tomography-computed tomography (PET-CT) should be used to establish a correct diagnosis. Here we report a case of an extremely rare type of PAIS, pedunculated PAIS, which could not be visualized on PET-CT. Histological features of a tumor with a low accumulation of fluorodeoxyglucose revealed low-cellularity and necrotizing background. Multimodal imaging was useful to diagnose PET-CT negative PAIS accurately. <Learning objective: Pedunculated pulmonary artery intimal sarcoma (PAIS) is a rare form of neoplasm. You need to know that PAIS which has low cellularity with marked interstitial myxoid tissue cannot be detected on positron emission tomography-computed tomography.>.
Collapse
Affiliation(s)
- Takumi Takauchi
- Department of Cardiology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki 710-8602, Japan
| | - Ryosuke Murai
- Department of Cardiology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki 710-8602, Japan
| | - Kazunori Musiake
- Department of Cardiology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki 710-8602, Japan
| | - Yoko Akaike
- Department of Pathology, Kurashiki Central Hospital, Kurashiki, Japan
| | - Masaya Hirayama
- Department of Cardiovascular Surgery, Kurashiki Central Hospital, Kurashiki, Japan
| | - Atsushi Ueda
- Department of Hematology, Kurashiki Central Hospital, Kurashiki, Japan
| | - Tatsuhiko Komiya
- Department of Cardiovascular Surgery, Kurashiki Central Hospital, Kurashiki, Japan
| | - Kazushige Kadota
- Department of Cardiology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki 710-8602, Japan
| |
Collapse
|
|
44
|
Kourouni I, Aesif SW, Tamarkin SW, Bolen M, Sivak E, Shaman Z, Tamaskar I, Florou V. A 51-year-old man with chronic cough and left hilar prominence. Breathe (Sheff) 2021; 17:210018. [PMID: 34295422 PMCID: PMC8291951 DOI: 10.1183/20734735.0018-2021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2021] [Accepted: 03/31/2021] [Indexed: 11/16/2022] Open
Abstract
A 51-year-old male in good overall health presented with a dry cough of 5 months' duration. He was working as a long-distance truck driver and was a life-long nonsmoker. He had no associated dyspnoea, wheezing, rhinosinusitis, haemoptysis or syncope, nor constitutional symptoms such as weight loss or fevers. Physical examination and vital signs were normal on presentation. Chest radiographs with posterior–anterior and lateral views are shown in figure 1. What is the diagnosis of this man with a chronic dry cough and left hilar prominence on chest radiography?https://bit.ly/3fL7QMx
Collapse
Affiliation(s)
- Ismini Kourouni
- Division of Pulmonary, Critical Care and Sleep Medicine, Metro Health Medical Center, Case Western Reserve University, Cleveland, OH, USA
| | - Scott W Aesif
- Division of Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Stephen W Tamarkin
- Dept of Radiology, Metro Health Medical Center, Case Western Reserve University, Cleveland, OH, USA
| | - Michael Bolen
- Division of Imaging Institute, Cleveland Clinic, Cleveland, OH, USA
| | - Edward Sivak
- Division of Pulmonary, Critical Care and Sleep Medicine, Metro Health Medical Center, Case Western Reserve University, Cleveland, OH, USA
| | - Ziad Shaman
- Division of Pulmonary, Critical Care and Sleep Medicine, Metro Health Medical Center, Case Western Reserve University, Cleveland, OH, USA
| | - Ila Tamaskar
- Division of Oncology, Metro Health Medical Center, Case Western Reserve University, Cleveland, OH, USA
| | - Vaia Florou
- Dept of Medicine, Division of Oncology, Huntsman Cancer Institute, University of Utah School of Medicine, Salt Lake City, UT, USA
| |
Collapse
|
|
45
|
Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J. Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I. Cancer Treat Rev 2021; 99:102259. [PMID: 34311246 DOI: 10.1016/j.ctrv.2021.102259] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2021] [Revised: 07/03/2021] [Accepted: 07/06/2021] [Indexed: 12/22/2022]
Affiliation(s)
| | - Josefina Cruz Jurado
- Hospital Universitario Canarias, Medical Oncology Department, Santa Cruz de Tenerife, Spain
| | | | - Daniel Bernabeu
- Hospital Universitario La Paz, Radiology Department, Madrid, Spain
| | - Luis Javier Floría
- Hospital Universitario Miguel Servet, Orthopedic and Traumatology Department, Zaragoza, Spain
| | - Javier Lavernia
- Instituto Valenciano de Oncología, Medical Oncology Department, Valencia, Spain
| | - Ana Sebio
- Hospital Universitario Santa Creu i Sant Pau, Medical Oncology Department, Barcelona, Spain
| | | | - Rosa Álvarez
- Hospital Universitario Gregorio Marañón, Medical Oncology Department, Madrid, Spain
| | - Raquel Correa
- Hospital Virgen de la Victoria, Radiation Oncology Department, Malaga, Spain
| | | | - Gloria Marquina
- Hospital Universitario Clínico San Carlos, Medical Oncology Department, Madrid, Spain
| | - Nadia Hindi
- University Hospital "Fundacion Jimenez Diaz" Madrid, Medical Oncology Department, Madrid, Research Institute FJD-UAM, Madrid (Spain), TBsarc, CITIUS III, Seville, Spain
| | - Andrés Redondo
- Hospital Universitario La Paz, Medical Oncology Department, Madrid, Spain
| | - Virginia Martínez
- Hospital Universitario La Paz, Medical Oncology Department, Madrid, Spain
| | | | - Cristina Mata
- Hospital Universitario Gregorio Marañón, Pediatric and Adolescent Hemato-oncology Department, Madrid, Spain
| | | | - Javier Martin-Broto
- University Hospital "Fundacion Jimenez Diaz" Madrid, Medical Oncology Department, Madrid, Research Institute FJD-UAM, Madrid (Spain), TBsarc, CITIUS III, Seville, Spain
| |
Collapse
|
|
46
|
Qin J, Ng CS, He P, Lin X, Lin X, Hou P. Pulmonary artery intimal sarcoma - A primeval or rediscovered tumor? A report of 14 new cases with literature review. Pathol Res Pract 2021; 224:153548. [PMID: 34280751 DOI: 10.1016/j.prp.2021.153548] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/26/2021] [Revised: 07/06/2021] [Accepted: 07/08/2021] [Indexed: 11/28/2022]
Abstract
Pulmonary artery sarcomas (PAS) are rare with many being undifferentiated pleomorphic or spindle cell (UPSC) sarcomas with variable atypia. The term pulmonary artery intimal sarcoma (PAIS) was rarely coined in the early literature and sometimes used for luminal sarcomas. With the advent of immunohistochemistry and molecular genetics, many of these UPSC sarcomas were found to frequently overpress MDM2 and/or CDK4 and PDGFRA with genetic alterations in 12q12-15 and 4q12, where the MDM2, CDK4 and PDGFRA genes are located. These recent developments enabled refinement in diagnosis of PAIS. We diagnosed 14 cases of PAIS (6 males and 8 females, mean age 44 years) in 2015 - 2020 in our institution. Six were initially misdiagnosed as thromboembolism and the remaining pulmonary artery tumors. The tumors were pulmonary artery intraluminal polypoid masses with histology of spindle cell sarcomas exhibiting immunohistochemical positivity for MDM2 (100%) and CDK4 (79%) with MDM2 gene amplification (100%). Ten surgically treated patients fared better than four other biopsy only and not surgically treated patients, who died of disease within 5-11 months. PAIS needs to be differentiated from other spindle cell tumors and those exhibiting MDM2 gene amplification, especially dedifferentiated liposarcoma. The use of biopsy to provide diagnostic material poses a sampling error problem and correlation with clinical, radiologic, histologic, immunophenotypic and genotypic features are essential for accurate diagnosis and early surgical intervention of PAIS.
Collapse
Affiliation(s)
- Jilong Qin
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Chi Sing Ng
- Department of Pathology, St. Teresa's Hospital, 327 Prince Edward Road, Kowloon, Hong Kong, China.
| | - Ping He
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Xiaodong Lin
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Xina Lin
- Department of Pathology, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| | - Peng Hou
- PET-CT Center, First Affiliated Hospital of Guangzhou Medical University, 151 Yanjiang West Road, Guangzhou 510120, China
| |
Collapse
|
|
47
|
Altshuler E, Lowther G, Jantz M. Primary Pulmonary Artery Sarcoma Confined to the Left Pulmonary Artery. J Investig Med High Impact Case Rep 2021; 9:23247096211014687. [PMID: 33969719 PMCID: PMC8113362 DOI: 10.1177/23247096211014687] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Abstract
Primary pulmonary artery sarcomas (PPAS) are extremely rare tumors that are often mislabeled as pulmonary emboli (PE). PPAS usually involve the pulmonary trunk and are histologically classified as leiomyosarcoma, spindle cells sarcoma, fibrous histiocytoma, or undifferentiated sarcoma. Our case involved a 78-year-old man with an undifferentiated PPAS confined to the left pulmonary artery that was initially misdiagnosed as a PE. After a month-long delay in treatment in which the patient was prescribed warfarin, the correct diagnosis was made. Pulmonary artery endarterectomy and left lung pneumonectomy were performed, and he survived for 18 months before disease recurrence and death. Our case helps illustrate some of the clinical and radiographic findings that help distinguish PPAS from PE.
Collapse
|
|
48
|
Vanni F, Scheggi V, Marchionni N, Stefàno PL. A new-onset pulmonary artery stenosis in a young man: case report. EUROPEAN HEART JOURNAL-CASE REPORTS 2021; 5:ytab118. [PMID: 33937621 PMCID: PMC8068665 DOI: 10.1093/ehjcr/ytab118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/03/2020] [Revised: 11/30/2020] [Accepted: 03/04/2021] [Indexed: 11/14/2022]
Abstract
Background Poorly differentiated and undifferentiated sarcomas are the most common primary tumours of the pulmonary arteries. They usually affect large-calibre vessels and present with predominantly intraluminal growth. Dyspnoea, cough, chest pain, and haemoptysis are the most common presenting symptoms. Clinical and imaging manifestations can mimic pulmonary embolisms and correct diagnosis may require multimodal imaging. The overall prognosis is poor; however, early diagnosis and complete surgical resection seem to improve the prognosis. Case summary A 31-year-old male was admitted to our department after a pre-syncopal episode associated with dyspnoea of recent onset. Echocardiography showed a mass with irregular borders attached to the pulmonary artery trunk, almost obliterating its lumen and determining a flow acceleration with a peak velocity and gradient, respectively, of 3.8 m/s and 60 mmHg. At cardiac magnetic resonance imaging and positron emission tomography-computed tomography scan, the mass had inhomogeneous contrast impregnation and an intense 18-fluorodeoxyglucose uptake, both findings are highly suggestive of an angiosarcoma of the pulmonary artery. Biopsy specimens were taken through bronchoscopy but the material was insufficient for diagnosis. The patient decided to continue treatment in another hospital, where he died a few months later. Discussion The presence of a unique mass involving the main trunk of the pulmonary artery or proximal branches associated with rapidly progressive dyspnoea in a patient at low risk for pulmonary embolism should raise the suspicion of primary sarcoma of the pulmonary artery. There are no guidelines for the treatment. Surgery and neo/adjuvant chemotherapy are reported in literature but burdened by bias and concerning a small number of cases.
Collapse
Affiliation(s)
- Francesco Vanni
- Division of Cardiovascular and Perioperative Medicine, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| | - Valentina Scheggi
- Division of Cardiovascular and Perioperative Medicine, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| | - Niccolò Marchionni
- Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Division of General Cardiology, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| | - Pier Luigi Stefàno
- Cardiothoracovascular Department, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy.,Division of Cardiac Surgery, Azienda Ospedaliero-Universitaria Careggi and University of Florence, Largo Brambilla 3, 50134 Florence, Italy
| |
Collapse
|
|
49
|
Ropp AM, Burke AP, Kligerman SJ, Leb JS, Frazier AA. Intimal Sarcoma of the Great Vessels. Radiographics 2021; 41:361-379. [PMID: 33646906 DOI: 10.1148/rg.2021200184] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Intimal sarcomas of the pulmonary artery and aorta are rare entities with a poor prognosis. In many instances, pulmonary artery sarcomas are misinterpreted as acute or chronic pulmonary thromboembolism, whereas aortic intimal sarcomas are often misdiagnosed as protuberant atherosclerotic disease or intimal thrombus. Discernment of intimal sarcomas from these and other common benign entities is essential for the timely initiation of aggressive therapy. The most useful imaging modalities for assessment of a suspected intimal sarcoma include CT angiography, fluorine 18-fluorodeoxyglucose PET, and MRI. The authors discuss the clinical features, current treatment options, characteristic imaging findings, and underlying pathologic features of intimal sarcomas. The authors emphasize imaging discernment of intimal sarcomas and how their differential diagnosis is informed by knowledge of radiologic-pathologic correlation. The most reliable distinguishing imaging features are also emphasized to improve accurate and timely diagnosis. Online supplemental material is available for this article. ©RSNA, 2021.
Collapse
Affiliation(s)
- Alan M Ropp
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Allen P Burke
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Seth J Kligerman
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Jay S Leb
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| | - Aletta A Frazier
- Department of Radiology and Medical Imaging, University of Virginia School of Medicine, 1215 Lee St, Charlottesville, VA 22903 (A.M.R.); Departments of Pathology (A.P.B.) and Diagnostic Radiology and Nuclear Medicine (A.A.F.), University of Maryland School of Medicine, Baltimore, Md; Department of Diagnostic Radiology, University of California San Diego School of Medicine, San Diego, Calif (S.J.K.); Department of Diagnostic Radiology, Columbia University Medical Center, New York, NY (J.S.L.); and American Institute for Radiologic Pathology Program (AIRP), American College of Radiology, Silver Spring, Md, and Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences (USUHS), Bethesda, Md (A.A.F.)
| |
Collapse
|
|
50
|
Gnanalingam C, Wright M, Ahmed Y. Pulmonary artery sarcoma masquerading as a pulmonary embolism. BMJ Case Rep 2021; 14:e232676. [PMID: 33637489 PMCID: PMC7919568 DOI: 10.1136/bcr-2019-232676] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/10/2020] [Indexed: 11/04/2022] Open
Abstract
A 52-year-old woman was referred to the respiratory team with worsening shortness of breath for the last 3 months, which had deteriorated significantly over the last 2-3 weeks. She underwent a CT pulmonary angiogram, which was reported locally as showing a large pulmonary embolism. Given the chronic history and appearance of the thrombus, the patient was referred to a specialist pulmonary vascular disease multidisciplinary team and underwent a PET-CT scan with the diagnosis being felt to be more consistent with a pulmonary artery sarcoma. Within 4 weeks, she underwent a pulmonary endarterectomy which confirmed the presence of an extensive mass. She underwent bilateral endarterectomy and pulmonary artery valve replacement, with subsequent improvement of her breathlessness back to premorbid baseline. Postoperative histology has confirmed a pulmonary artery angiosarcoma. Alternate imaging modalities and early referral to a specialist unit allowed as early a diagnosis as possible with good symptomatic benefit.
Collapse
Affiliation(s)
| | - Matthew Wright
- Respiratory Medicine, Southend Hospital, Westcliff-on-Sea, Essex, UK
| | - Yasser Ahmed
- Respiratory Medicine, Southend Hospital, Westcliff-on-Sea, Essex, UK
| |
Collapse
|