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(3): 561-578 [PMID: 36688020 DOI: 10.3748/wjg.v29.i3.561]
Corresponding Author of This Article
Qing-Hong Guo, PhD, Chief Physician, Department of Gastroenterology, The First Hospital of Lanzhou University, No. 1 Donggang West Road, Chengguan District, Lanzhou 730000, Gansu Province, China. gqh@lzu.edu.cn
Research Domain of This Article
Oncology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Xiao-Mei Ma, Yuan Yang, Guo-Zhi Wu, Ying-Wen Li, Xiao Yu, Xiao-Li Ma, Yu-Ping Wang, Qing-Hong Guo, Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
Xiao-Mei Ma, Yuan Yang, Guo-Zhi Wu, Ying-Wen Li, Xiao Yu, Xiao-Li Ma, The First Clinical Medical College, Lanzhou University, Lanzhou 730000, Gansu Province, China
Bao-Shun Yang, Xu-Dong Hou, General Surgery Ward 5, The First Hospital of Lanzhou University, Lanzhou 730000, Gansu Province, China
Author contributions: Ma XM contributed to data analysis and manuscript writing; Yang BS contributed to conceptualization and methodology; Yang Y contributed to data curation and visualization; Wu GZ contributed to data curation, writing-original draft preparation; Li YW, Yu X, and Ma XL contributed to literature search and screening; Wang YP and Hou XD contributed to revised the manuscript and approved the final version; Guo QH contributed to supervision and final confirmation.
Informed consent statement: Informed written consent was obtained from the patients for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Qing-Hong Guo, PhD, Chief Physician, Department of Gastroenterology, The First Hospital of Lanzhou University, No. 1 Donggang West Road, Chengguan District, Lanzhou 730000, Gansu Province, China. gqh@lzu.edu.cn
Received: November 11, 2022 Peer-review started: November 11, 2022 First decision: November 24, 2022 Revised: December 3, 2022 Accepted: December 23, 2022 Article in press: December 23, 2022 Published online: January 21, 2023 Processing time: 61 Days and 20.5 Hours
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.
Core Tip: Small intestinal angiosarcoma is a rare malignant soft tissue tumor. We report a primary multifocal ileal angiosarcoma with metastases to the adrenal gland and lumbar spine. The patient died 4 mo after surgical resection. Further, we collected relevant case reports and analyzed statistically. We concluded that small intestinal angiosarcoma tend to occur in elderly men. Melena and anemia were the most common symptoms. The diagnosis depended on microscopic morphology and immunohistochemistry. CD31, CD34, factor VIII-related antigen, ETS-related gene, friend leukemia integration 1, and von Willebrand factor were valuable diagnostic markers. Surgery and chemotherapy could improve the prognosis of patients.
