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Thaveenthiran P, Bae L, Wewelwala C, Zhou K. Primary small intestine angiosarcoma mimicking Crohn's disease. BMJ Case Rep 2024; 17:e255424. [PMID: 38290985 PMCID: PMC10828878 DOI: 10.1136/bcr-2023-255424] [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: 12/27/2023] [Indexed: 02/01/2024] Open
Abstract
A man in his 40s presented to an emergency department after experiencing worsening abdominal pain for 2 days. Contrast-enhanced CT of the abdomen and pelvis revealed circumferential mural thickening and luminal narrowing of the distal ileum and upstream dilatation of the small intestine, indicating small intestine obstruction. This prompted emergency laparotomy, where two lesions in the distal ileum were identified as the source of his bowel obstruction and resected. Immunohistochemistry of the resected segment revealed a primary small intestine angiosarcoma acting positively for vascular markers ERG and CD31. A subsequent positron emission tomography (PET) scan revealed positive mediastinal metastatic lymphadenopathy without organ metastases.Following his surgery, the patient recovered well and was promptly referred to an oncology unit at a specialised health centre for further treatment. Primary small intestine angiosarcoma is a rare entity in which patients present with non-specific symptoms requiring prompt tissue diagnosis to facilitate multidisciplinary management.
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Affiliation(s)
- Prasanthan Thaveenthiran
- General Surgery, Monash Health, Clayton, Victoria, Australia
- Trauma, National Trauma Research Institute, The Alfred Hospital, Melbourne, Victoria, Australia
| | - Lily Bae
- General Surgery, Monash Health, Clayton, Victoria, Australia
| | | | - Kevin Zhou
- Radiology, Monash Health, Clayton, Victoria, Australia
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2
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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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Sharko A, Samuel S, Ying GW, Prasad S, Baig S. Gastric Epithelioid Angiosarcoma: An Unexpected Tumor in an Unexpected Location. Cureus 2021; 13:e15049. [PMID: 34150400 PMCID: PMC8208171 DOI: 10.7759/cureus.15049] [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] [Indexed: 11/28/2022] Open
Abstract
Angiosarcomas are aggressive neoplasms that arise from endothelial cells and can develop in any part of the body. Gastrointestinal angiosarcomas are very uncommon and can have a variable clinical presentation. We report a case of an 84-year-old female who presented with acute blood loss anemia. She underwent a gastrointestinal workup with esophagogastroduodenoscopy (EGD), which revealed two polyps in the stomach. Histologic evaluation of the polyps was indicative of angiosarcoma, and the diagnosis was eventually confirmed by immunohistochemical analysis with positive CD31, ERG, and FLI1 stains. This case is reported to demonstrate the importance of considering angiosarcoma in the differential for patients presenting with gastrointestinal bleeding.
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Affiliation(s)
- Artem Sharko
- Internal Medicine, Northwestern Medicine McHenry Hospital, Rosalind Franklin University of Medicine and Science, McHenry, USA
| | - Shirly Samuel
- Internal Medicine, Northwestern Medicine McHenry Hospital, Rosalind Franklin University of Medicine and Science, McHenry, USA
| | - Grace W Ying
- Internal Medicine, Chicago Medical School Internal Medicine Residency Program at Northwestern Medicine McHenry Hospital, McHenry, USA
| | - Sonika Prasad
- Internal Medicine, Northwestern Medicine McHenry Hospital, Rosalind Franklin University of Medicine and Science, McHenry, USA
| | - Shaji Baig
- Internal Medicine, Northwestern Medicine McHenry Hospital, Rosalind Franklin University of Medicine and Science, McHenry, USA
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Schizas D, Mastoraki A, Giannakodimos I, Giannakodimos A, Ziogou A, Katsaros I, Frountzas M, Koutelidakis I, Vassiliu P, Pikoulis E. Primary Angiosarcoma of the Gastrointestinal Tract: A Systematic Review of the Literature. J INVEST SURG 2020; 35:400-408. [PMID: 33342318 DOI: 10.1080/08941939.2020.1853283] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/20/2023]
Abstract
Primary Gastrointestinal Angiosarcoma (PGAS) constitutes a rare malignant tumor arising from vascular or lymphatic endothelial cells. The aim of this study was to review the available literature on PGAS and to elucidate its biological behavior and optimal therapeutic approach. PubMed and Embase bibliographical databases were systematically searched (last search April 8th, 2020) for studies concerning PGAS. Ninety-eight studies met our inclusion criteria, involving 110 patients (male/female = 1.5) with an age of 62.40 ± 17.84 (mean, SD) years. They were most frequently located at small (44.5%) and large intestine (35.5%), while 12.7% were multifocal. Surgical resection of the tumor was conducted at 84.0% of the cases combined with adjuvant therapy at 12.3%. One-year cumulative survival was 55.18% (95% CI: 34.33%-71.84%) for large intestine, 30.2% (95% CI: 17.1%-44.5%) for small intestine, whereas multifocal PGAS had a 6-months cumulative survival of 23.08% (95% CI: 5.58%-47.46%). Therefore, PGAS is an extremely rare entity with atypical clinical presentation, challenging diagnosis and aggressive behavior. High clinical suspicion is crucial for its prompt management. Further studies and the development of novel therapeutic agents are required in order to improve survival.
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Affiliation(s)
- Dimitrios Schizas
- First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
| | - Aikaterini Mastoraki
- First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
| | - Ilias Giannakodimos
- First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
| | - Alexios Giannakodimos
- First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
| | - Afroditi Ziogou
- Third Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Greece
| | - Ioannis Katsaros
- First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
| | - Maximos Frountzas
- Laboratory of Experimental Surgery and Surgical Research N.S. Christeas, Athens Medical School, Athens, Greece
| | - Ioannis Koutelidakis
- Second Department of Surgery, Aristotle University of Thessaloniki, G.Gennimatas General Hospital, Thessaloniki, Greece
| | - Pantelis Vassiliu
- Fourth Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Greece
| | - Emmanouil Pikoulis
- Third Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Greece
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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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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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Huntington JT, Jones C, Liebner DA, Chen JL, Pollock RE. Angiosarcoma: A rare malignancy with protean clinical presentations. J Surg Oncol 2015; 111:941-50. [PMID: 26031299 DOI: 10.1002/jso.23918] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2015] [Accepted: 03/19/2015] [Indexed: 02/06/2023]
Abstract
Angiosarcoma is a rare form of soft tissue sarcoma. Primary small intestinal angiosarcomas are especially uncommon. The clinical presentations of small intestinal angiosarcomas vary but gastrointestinal (GI) bleeding is a frequent finding. We present a case of persistent GI bleeding of unknown etiology culminating in operative exploration demonstrating a primary small intestinal angiosarcoma. A discussion of the diagnosis, pathology, and management of angiosarcoma with a review of the current literature is provided including molecular genetics, difficult cases, and current treatment options.
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Affiliation(s)
- Justin T Huntington
- Department of General Surgery, The Ohio State University Wexner Medical Center, Columbus, Ohio
| | - Christian Jones
- Department of Surgery, Division of Trauma, Critical Care and Burn, The Ohio State University Wexner Medical Center, Columbus, Ohio
| | - David A Liebner
- Division of Medical Oncology, The Ohio State University Wexner Medical Center, Columbus, Ohio.,Department of Biomedical Informatics, The Ohio State University, Columbus, Ohio
| | - James L Chen
- Division of Medical Oncology, The Ohio State University Wexner Medical Center, Columbus, Ohio.,Department of Biomedical Informatics, The Ohio State University, Columbus, Ohio
| | - Raphael E Pollock
- Department of Surgery, Division of Surgical Oncology, The Ohio State University Wexner Medical Center - James Cancer Hospital and Solove Research Institute, Columbus, Ohio
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8
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Navarro-Chagoya D, Figueroa-Ruiz M, López-Gómez J, Nava-Leyva H, Álvarez-Ponce CE, Guzmán-Sombrero G, Velazquez-Garcia J. Obscure gastrointestinal bleeding due to multifocal intestinal angiosarcoma. Int J Surg Case Rep 2015; 10:169-72. [PMID: 25853844 PMCID: PMC4430205 DOI: 10.1016/j.ijscr.2015.03.049] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2015] [Revised: 03/21/2015] [Accepted: 03/21/2015] [Indexed: 02/07/2023] Open
Abstract
INTRODUCTION Intestinal angiosarcomas are an extremely rare and aggressive vascular tumors, with a few cases reported in the literature. PRESENTATION OF CASE A 45 years-old male arrived to our hospital with intermittent gastrointestinal bleeding presenting melena and weight loss, he has antecedent of pelvic radiotherapy ten years before admission for an unknown pelvic tumor. Emergency surgery was required because of uncontrolled bleeding and hemodynamic instability. Histopathological findings revealed a multifocal high-grade epithelioid angiosarcoma, with cells reactive for CD31, keratins CKAE 1/AE3 and factor VIII. DISCUSSION Angiosarcomas are aggressive tumors with a high rate of lymph node metastasis and peripheral organs. The diagnosis is difficult because it present nonspecific clinical presentation, radiological and histopathological findings. There are few reports of angiosarcoma involving the small intestine and the most common presentation are abdominal pain and gastrointestinal bleeding. There is not enough information for intestinal angiosarcoma secondary to radiation therapy, but there have been proposed criteria for diagnosis: no microscopic or clinical evidence of antecedent malignant lesion, angiosarcoma presented in the field of irradiation, long latency period between radiation and angiosarcoma and histological confirmation. We suspect our patient course with a secondary form of angiosarcoma. Therapy for bleeding angiosarcoma consists in control of bleeding and medical management to stabilize the patient. Once accomplished surgical resection is required. CONCLUSION We should keep in mind this tumors as a cause of obscure intestinal bleeding in patients with medical history of radiation therapy.
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Affiliation(s)
- Dolores Navarro-Chagoya
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico.
| | - Marco Figueroa-Ruiz
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico
| | - Javier López-Gómez
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico
| | - Héctor Nava-Leyva
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico
| | - Carlos Eduardo Álvarez-Ponce
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico
| | - Gustavo Guzmán-Sombrero
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico
| | - José Velazquez-Garcia
- Instituto Mexicano Del Seguro Social, Department of General Surgery, Hospital de Especialidades Centro Médico Nacional La Raza, Seris y Zaachila SN, Col. La Raza, México D.F. CP 2990, Mexico
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Takahashi M, Ohara M, Kimura N, Domen H, Yamabuki T, Komuro K, Tsuchikawa T, Hirano S, Iwashiro N. Giant primary angiosarcoma of the small intestine showing severe sepsis. World J Gastroenterol 2014; 20:16359-16363. [PMID: 25473196 PMCID: PMC4239530 DOI: 10.3748/wjg.v20.i43.16359] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2014] [Revised: 05/14/2014] [Accepted: 07/16/2014] [Indexed: 02/06/2023] Open
Abstract
Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40 °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.
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Ni Q, Shang D, Peng H, Roy M, Liang G, Bi W, Gao X. Primary angiosarcoma of the small intestine with metastasis to the liver: a case report and review of the literature. World J Surg Oncol 2013; 11:242. [PMID: 24067058 PMCID: PMC3851255 DOI: 10.1186/1477-7819-11-242] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/24/2013] [Accepted: 09/03/2013] [Indexed: 02/08/2023] Open
Abstract
Angiosarcoma is a rare disease with a poor prognosis; significantly, patients with intestinal angiosarcomas who survive over 1 year after diagnosis are extraordinarily rare. This article describes the case of a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which had increased in severity 2 weeks prior to presentation. After a complicated diagnostic and therapeutic process, the diagnosis of primary angiosarcoma of the small intestine with metastasis to the liver was made by pathological and immunohistochemical examinations. We reviewed previous cases of angiosarcoma described in the English literature to determine their risk factors, diagnosis and treatment, and we found that angiosarcoma is extremely rare, especially in the small intestine. To the best of our knowledge, this may be the youngest case of primary angiosarcoma of the small intestine with metastasis to the liver reported in the English literature.
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Affiliation(s)
- Qingqiang Ni
- Department of General Surgery, First Affiliated Hospital, Dalian Medical University, Dalian, Liaoning 116011, P,R, China.
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