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Ma XM, Yang BS, Yang Y, Wu GZ, Li YW, Yu X, Ma XL, Wang YP, Hou XD, Guo QH. Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature. World J Gastroenterol 2023; 29:561-578. [PMID: 36688020 PMCID: PMC9850938 DOI: 10.3748/wjg.v29.i3.561] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/11/2022] [Revised: 12/03/2022] [Accepted: 12/23/2022] [Indexed: 01/12/2023] Open
Abstract
BACKGROUND Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues. Small-intestinal angiosarcomas are rare, and the prognosis is poor.
CASE SUMMARY We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma. Small intestinal angiosarcoma is more common in elderly and male patients. Gastrointestinal bleeding, anemia, abdominal pain, weakness, and weight loss were the common symptoms. CD31, CD34, factor VIII-related antigen, ETS-related gene, friend leukemia integration 1, and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma. Small-intestinal angiosarcoma most commonly occurs in the jejunum, followed by the ileum and duodenum. Radiation and toxicant exposure are risk factors for angiosarcoma. After a definite diagnosis, the mean and median survival time was 8 mo and 3 mo, respectively. Kaplan-Meier survival analysis showed that age, infiltration depth, chemotherapy, and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma. Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.
CONCLUSION Angiosarcoma should be considered for unexplained melena and abdominal pain, especially in older men and patients with a history of radiation exposure. Prompt treatment, including surgery and adjuvant chemotherapy, is essential to prolonging patient survival.
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Affiliation(s)
- Xiao-Mei Ma
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
- The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Bao-Shun Yang
- General Surgery Ward 5, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Yuan Yang
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
- The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Guo-Zhi Wu
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
- The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Ying-Wen Li
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
- The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Xiao Yu
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
- The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Xiao-Li Ma
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
- The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Yu-Ping Wang
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Xu-Dong Hou
- General Surgery Ward 5, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Qing-Hong Guo
- Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
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Squillaci S, Marasco A, Pizzi G, Chiarello M, Brisinda G, Tallarigo F. Primary post-radiation angiosarcoma of the small bowel. Report of a case and review of the literature. Pathologica 2020; 112:93-101. [PMID: 32760052 PMCID: PMC7931569 DOI: 10.32074/1591-951x-3-20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2020] [Accepted: 02/19/2020] [Indexed: 01/12/2023] Open
Abstract
Angiosarcomas developing in unusual sites such as the small bowel are rare, and fewer than 65 cases have been reported in the literature. They are not uncommonly associated with a known eliciting factor. Thus, among hitherto described cases of angiosarcoma of the small bowel, 16 were radiation-induced. One additional example of ileal post-irradiation angiosarcoma (PRA) in a 72-year-old female patient with a past history of uterine leiomyosarcoma is herein reported as a reminder of this causal association. The morphologic and immunohistochemical clues leading to the correct diagnosis of PRA of the small bowel and the differential diagnostic problems are discussed; a comprehensive review of the literature has also been performed with a focus on survival.
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Affiliation(s)
- Salvatore Squillaci
- Division of Anatomic Pathology, Hospital of Vallecamonica, Esine (Bs), Italy
| | - Angela Marasco
- Division of Anatomic Pathology, Hospital “San Giovanni di Dio”, Crotone, Italy
| | - Giandomenico Pizzi
- Division of Anatomic Pathology, Hospital “San Giovanni di Dio”, Crotone, Italy
| | | | | | - Federico Tallarigo
- Division of Anatomic Pathology, Hospital “San Giovanni di Dio”, Crotone, Italy
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Liu Z, Yu J, Xu Z, Dong Z, Suo J. Primary angiosarcoma of the small intestine metastatic to peritoneum with intestinal perforation: a case report and review of the literature. Transl Cancer Res 2019; 8:1635-1640. [PMID: 35116908 PMCID: PMC8799189 DOI: 10.21037/tcr.2019.06.40] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2019] [Accepted: 05/14/2019] [Indexed: 01/12/2023]
Abstract
The study reports on a case of primary angiosarcoma of the small intestine in a 43-year-old woman presenting with intestinal perforation and metastasis to the peritoneum, along with a pertinent literature review. After hospitalization, an exploratory laparotomy was undertaken to aid in her diagnosis, followed by palliative intestinal resection with enteroenterostomy. The pathological examination and immunohistochemistry of resected tumor tissues confirmed the diagnosis. Moreover, analysis of seven reported cases of primary intestinal angiosarcoma in the literature revealed that patients often present with abdominal pain or recurrent gastrointestinal (GI) bleeding, dying within six months of diagnosis or resection surgery. Altogether, these findings illustrate that, despite volume-reducing palliative surgery, the prognosis of primary angiosarcoma of the small intestine remains poor for the majority of patients.
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Affiliation(s)
- Zhicheng Liu
- Department of Gastrointestinal Surgery, The First Hospital of Jilin University, Changchun 130021, China
| | - Jinhai Yu
- Department of Gastrointestinal Surgery, The First Hospital of Jilin University, Changchun 130021, China
| | - Zhonghang Xu
- Department of Gastrointestinal Surgery, The First Hospital of Jilin University, Changchun 130021, China
| | - Zhiwei Dong
- Department of Gastrointestinal Surgery, The First Hospital of Jilin University, Changchun 130021, China
| | - Jian Suo
- Department of Gastrointestinal Surgery, The First Hospital of Jilin University, Changchun 130021, China
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Nai Q, Ansari M, Liu J, Razjouyan H, Pak S, Tian Y, Khan R, Broder A, Bagchi A, Iyer V, Hamouda D, Islam M, Sen S, Yousif A, Hu M, Lou Y, Duhl J. Primary Small Intestinal Angiosarcoma: Epidemiology, Diagnosis and Treatment. J Clin Med Res 2018; 10:294-301. [PMID: 29511417 PMCID: PMC5827913 DOI: 10.14740/jocmr3153w] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2017] [Accepted: 10/09/2017] [Indexed: 02/06/2023] Open
Abstract
Angiosarcoma is an aggressive mesenchymal sarcoma of endothelial cell origin with high mortality. Its occurrence in the small intestine is exceedingly low. In addition to the rarity of small intestine angiosarcoma, the nonspecific early clinical symptoms obscure the suspicion of such tumors and thereby delay the diagnosis. In a hope to improve the knowledge of this rare but fatal neoplasm, we report one case of angiosarcoma of duodenum and jejunum in a 73-year-old man. Furthermore, we summarize and analyze the common clinical features, tumor markers, treatment, and survival of previous reported cases of this malignancy. Small bowel angiosarcoma occurs more often in men than women (1.6:1). The median age at diagnosis is 68.5 years. The overall median survival time is 150 days; the median survival time in female (300 days) is longer than that of male patients (120 days). Von Willebrand factor (vWF), CD31, CD34, vimentin, and Ulex europaeus agglutinin 1 appear to be the most useful markers for the diagnosis. The majority of the patients underwent surgical resection alone or surgery with subsequent chemotherapy. The patients treated with surgery plus chemotherapy survive longer than those underwent surgical resection only (median 420 days, n = 7 vs. 96.5 days, n = 26, respectively; P = 0.0275). Further studies of more cases are needed for a better understanding of this rare entity, as well as the development of effective strategies for prevention, early diagnosis, and treatment.
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Affiliation(s)
- Qiang Nai
- University of Toledo Medical Center, Toledo, OH 43614, USA
- These authors contributed equally
| | - Mohammad Ansari
- Raritan Bay Medical Center, Perth Amboy, NJ 08861, USA
- These authors contributed equally
| | - Jing Liu
- School of Public Health, Shandong University, Jinan 250012, China
| | - Hadi Razjouyan
- Saint Peter's University Hospital, New Brunswick, NJ 08901, USA
| | - Stella Pak
- University of Toledo Medical Center, Toledo, OH 43614, USA
| | - Yufei Tian
- University of Toledo Medical Center, Toledo, OH 43614, USA
| | - Rafay Khan
- Raritan Bay Medical Center, Perth Amboy, NJ 08861, USA
| | - Arkady Broder
- Saint Peter's University Hospital, New Brunswick, NJ 08901, USA
| | - Arindam Bagchi
- University of Toledo Medical Center, Toledo, OH 43614, USA
| | - Veena Iyer
- University of Toledo Medical Center, Toledo, OH 43614, USA
| | - Danae Hamouda
- University of Toledo Medical Center, Toledo, OH 43614, USA
| | | | - Shuvendu Sen
- Raritan Bay Medical Center, Perth Amboy, NJ 08861, USA
| | | | - Man Hu
- Department of Radiation Oncology and Shandong Province Key Laboratory of Radiation Oncology, Shandong Cancer Hospital Affiliated to Shandong University, Jinan, China
- Shandong Academy of Medical Sciences, Jinan 250117, China
| | - Yali Lou
- Department of Neurology, Brigham and Women's Hospital, 60 Fenwood Road, Boston, MA 02115, USA
| | - Jozsef Duhl
- Raritan Bay Medical Center, Perth Amboy, NJ 08861, USA
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Wang Q, Zhao T, Mi BT, Zhang YL, Wei R, Tong HL, Wu M. Primary Colonic Angiosarcoma Seen in a Patient on Calcium Channel Blocker: A Case Report with Summary Analysis of 32 Other Cases from the Literature. AMERICAN JOURNAL OF CASE REPORTS 2018; 19:254-261. [PMID: 29511155 PMCID: PMC5850845 DOI: 10.12659/ajcr.907287] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023]
Abstract
Patient: Female, 54 Final Diagnosis: Primary colonic angiosarcoma Symptoms: Rectal bleeding Medication: Levamlodipine Besylate Clinical Procedure: Hemicolectomy Specialty: Gastroenterology and Hepatology
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Affiliation(s)
- Qi Wang
- Division of Gastroenterology, Department of Internal Medicine, Second Hospital of Shanxii Medical University, Taiyuan, Shanxi, China (mainland)
| | - Ting Zhao
- Division of Gastroenterology, Department of Internal Medicine, Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China (mainland)
| | - Bian-Tao Mi
- Division of Gastroenterology, Department of Internal Medicine, Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China (mainland)
| | - Yong-Lin Zhang
- Department of Radiology, Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China (mainland)
| | - Rong Wei
- Department of Pathology, Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China (mainland)
| | - Hong-Lin Tong
- Department of Surgery, Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China (mainland)
| | - Maoxin Wu
- Department of Pathology, Stony Brook University Hospital, Stony Brook, NY, USA
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Zacarias Föhrding L, Macher A, Braunstein S, Knoefel WT, Topp SA. Small intestine bleeding due to multifocal angiosarcoma. World J Gastroenterol 2012; 18:6494-6500. [PMID: 23197897 PMCID: PMC3508646 DOI: 10.3748/wjg.v18.i44.6494] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/23/2012] [Accepted: 09/28/2012] [Indexed: 02/06/2023] Open
Abstract
We report a case of an 84-year-old male patient with primary small intestinal angiosarcoma. The patient initially presented with anemia and melena. Consecutive endoscopy revealed no signs of upper or lower active gastrointestinal bleeding. The patient had been diagnosed 3 years previously with an aortic dilation, which was treated with a stent. Computed tomography suggested an aorto-intestinal fistula as the cause of the intestinal bleeding, leading to operative stent explantation and aortic replacement. However, an aorto-intestinal fistula was not found, and the intestinal bleeding did not arrest postoperatively. The constant need for blood transfusions made an exploratory laparotomy imperative, which showed multiple bleeding sites, predominately in the jejunal wall. A distal loop jejunostomy was conducted to contain the small intestinal bleeding and a segmental resection for histological evaluation was performed. The histological analysis revealed a less-differentiated tumor with characteristic CD31, cytokeratin, and vimentin expression, which led to the diagnosis of small intestinal angiosarcoma. Consequently, the infiltrated part of the jejunum was successfully resected in a subsequent operation, and adjuvant chemotherapy with paclitaxel was planned. Angiosarcoma of the small intestine is an extremely rare malignant neoplasm that presents with bleeding and high mortality. Early diagnosis and treatment are essential to improve outcome. A small intestinal angiosarcoma is a challenging diagnosis to make because of its rarity, nonspecific symptoms of altered intestinal function, nonspecific abdominal pain, severe melena, and acute abdominal signs. Therefore, a quick clinical and histological diagnosis and decisive measures including surgery and adjuvant chemotherapy should be the aim.
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Lewis C, Gerrand C, Barnes D, Murray S, Milner R, Ragbir M. Experience of angiosarcoma in the North of England Bone and Soft Tissue Tumour Service. J Plast Reconstr Aesthet Surg 2011; 64:884-91. [DOI: 10.1016/j.bjps.2010.11.016] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2010] [Revised: 11/08/2010] [Accepted: 11/16/2010] [Indexed: 12/22/2022]
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Grewal JS, Daniel ARM, Carson EJ, Catanzaro AT, Shehab TM, Tworek JA. Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literature. Int J Colorectal Dis 2008; 23:745-56. [PMID: 18080128 DOI: 10.1007/s00384-007-0420-x] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/21/2007] [Indexed: 02/06/2023]
Abstract
BACKGROUND Angiosarcoma is a rare high-grade neoplasm that frequently involves the skin and subcutaneous tissue. Rarely, angiosarcoma can occur in the gastrointestinal tract where it frequently exhibits multicentric epithelioid morphology. DESIGN We report a case of multicentric epithelioid angiosarcoma (EAS) of the small intestine in a 73-year-old male patient who presented with weakness and melena, and was found to have bleeding lesions in the small intestine on upper gastrointestinal endoscopy. In addition to this case, we extensively reviewed the clinical and pathological features of previously reported cases of angiosarcoma of the small intestine in the English literature since 1970. RESULTS Our patient presented with rare and aggressive EAS of the small intestine. Despite surgical resection of the lesions, the patient continued to worsen and developed rapidly progressive metastatic disease. He died within 4 months of the diagnosis. CONCLUSIONS Angiosarcoma, especially of the deep tissues and the gastrointestinal tract, is very aggressive and rapidly metastatic. The survival rate in these patients is extremely poor, and most patients die within 6 months to 1 year of the diagnosis. Treatment usually involves surgical resection of the bleeding lesions and frequent blood transfusions for symptom alleviation.
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Affiliation(s)
- Jaspreet S Grewal
- Department of Internal Medicine, St. Joseph Mercy Hospital, Reichert Health Center, 5333 McAuley Drive, Suite 3009, Ann Arbor, MI 48106-0995, USA.
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Abstract
Availability of KIT tyrosine kinase inhibitors for specific treatment of GISTs has magnified the importance of accurate differential diagnosis of GIST from other tumors occurring in the GI tract and abdomen. The general problems in this distinction include histological mimicry of other mesenchymal tumors with GIST, occasional KIT-negativity of GIST, and KIT-positivity of non-GISTs. Up to 5% to 10% gastric GISTs and <2% of intestinal GISTs can be KIT-negative. The identification of these tumors as GISTs is based on knowledge of the spectrum of GIST morphology, and can be supported by molecular diagnosis of KIT and PDGFRA mutations (the latter pertain to gastric tumors). True smooth muscle tumors (rare in GI tract except in esophagus and colon) can be separated from GISTs by the eosinophilic tinctorial quality of tumor cells, positivity for smooth muscle markers, and negativity for KIT. Desmoids can form large GIST-like masses, but are composed of spindled or stellate-shaped cells in a densely collagenous stroma. Negativity for KIT and nuclear positivity for beta-catenin are differentiating features. GI schwannomas, melanoma, and rare primary clear cell sarcoma are S100-positive, usually with characteristic histology. The latter two can be KIT-positive. KIT-positive non-GISTs include some sarcomas, especially angiosarcoma and Ewing sarcoma, extramedullary myeloid tumor, seminoma, and a few carcinomas, notably small cell carcinoma of lung. Spurious KIT-positivity, seen with some polyclonal KIT antibodies, has been a source of confusion leading to probable false-positive results in fibroblastic tumors and occasional other sarcomas, such as leiomyosarcomas. Integration of histological features with carefully standardized immunohistochemistry, supported by KIT and PDGFRA mutation analysis, is the cornerstone of state-of-the art differential diagnosis of GIST. To comprehensively capture all GISTs, KIT immunostains should be performed on all unclassified epithelioid and mesenchymal tumors of the abdomen. This is a US government work. There are no restrictions on its use.
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Affiliation(s)
- Nancy Dow
- Division of Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
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Hinterseher I, Pistorius S, Zietz C, Bergert H. Epithelioid hemangiosarcoma of the rectum. Int J Colorectal Dis 2005; 20:385-7. [PMID: 15959792 DOI: 10.1007/s00384-004-0673-6] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/28/2004] [Indexed: 02/04/2023]
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Al-Nafussi A, Wong NA. Intra-abdominal spindle cell lesions: a review and practical aids to diagnosis. Histopathology 2001; 38:387-402. [PMID: 11422475 DOI: 10.1046/j.1365-2559.2001.01119.x] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Intra-abdominal spindle cell lesions are uncommon and often present a diagnostic challenge. An important group of such lesions are the gastrointestinal stromal tumours. Other intra-abdominal spindle cell lesions include fibromatosis, various sarcomas-in particular, leiomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumour-and, in women, endometrial stromal sarcoma. Less common lesions are inflammatory myofibroblastic tumours, the mesenteric spindle cell reactive lesions, retroperitoneal fibrosis, and solitary fibrous tumour. A variety of intra-abdominal tumours of nonmesenchymal origin may have a spindle cell/sarcomatoid morphology; these include sarcomatoid carcinoma, malignant melanoma and, in women, sarcomatoid granulosa cell tumour. Finally, metastatic sarcomas from pelvic or extra-abdominal organs need also be considered. A set of practical aids to the diagnosis of intra-abdominal spindle cell lesions is presented to assist pathologists dealing with such lesions, particularly with regards to the consideration of differential diagnoses.
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Affiliation(s)
- A Al-Nafussi
- Department of Pathology, University of Edinburgh Medical School, Teviot Place, Edinburgh EH8 9AG, UK.
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