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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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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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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: 21] [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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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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