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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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Shi B, Gaebelein G, Hildebrandt B, Weichert W, Glanemann M. Adult jejunojejunal intussusception caused by metastasized pleomorphic carcinoma of the lung: report of a case. Surg Today 2009; 39:984-989. [PMID: 19882322 DOI: 10.1007/s00595-008-3954-9] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2008] [Accepted: 07/06/2008] [Indexed: 12/21/2022]
Abstract
Adult small-intestinal intussusception is rare and very different from childhood intussusception. Both benign and malignant pathologies can underlie small intestinal intussusception in adults, but malignancy is much less frequent. We report a case of jejunojejunal intussusception caused by an intestinal metastasis of the sarcomatoid component of pleomorphic carcinoma of the right lung. The patient, a 61-year-old man, underwent successful segmental jejunal resection. Adult small bowel intussusception, though an unusual cause of acute abdomen, requires early diagnosis and timely management. To our knowledge, this is the first report of adult jejunojejunal double intussusception caused by metastatic sarcomatoid carcinoma of the lung.
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Affiliation(s)
- Baomin Shi
- Department of General, Visceral, and Transplantation Surgery, Universitätsmedizin Berlin, 13353 Berlin, Germany
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Avramut M, Parwani AV. Metastatic angiosarcoma in an ileal conduit: an unusual presentation. Int J Surg Pathol 2008; 17:60-4. [PMID: 18499689 DOI: 10.1177/1066896908316382] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
The authors in this study describe the case of a patient with a history of multiple malignancies who underwent total cystectomy with ileal loop urinary diversion and presented with a lower extremity angiosarcoma on the background of lymphedema a decade later. Shortly thereafter, she was diagnosed with metastatic ileal conduit angiosarcoma. The authors state that to their knowledge, this is the first case of ileal conduit angiosarcoma reported in the English literature.
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Affiliation(s)
- Mihaela Avramut
- Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
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