ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI An Unusual Cause of Upper Gastrointestinal Bleeding Jiong Lu, Xian-Ze Xiong, and Nan-Sheng Cheng Department of Bile Duct Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
Question: A 79-yearold man presented with a 4-month history of intermittent melena, weakness, and abdominal pain was admitted to our hospital. His past medical history was significant for hypertension and cholecystectomy. He denied previous radiotherapy or industrial chemical exposure. Physical examination showed an ill-appearing man with pale conjunctivae. His abdomen was soft, not tender or distended, and had no organomegaly or palpable masses. Laboratory examinations revealed hemoglobin of 56 g/L and a hematocrit of 18%. Serum levels of tumor markers were within normal range. Contrast-enhanced CT of the abdomen revealed no abnormality. Upper gastrointestinal endoscopy combined with endoscopic ultrasonography was performed. In the second part of the duodenum proximal to the papilla, a 6-mm mucous prominence lesion with friable mucosa and easy-contact bleeding was identified and biopsied (Figure A). Endoscopic ultrasonography further confirmed it was derived from the submucosa (Figure B). Because biopsy was highly suggestive of malignancy, the patient underwent an exploratory laparotomy. Three separate lesions in the duodenum, ileum, and transverse mesocolon were found during the surgery. He eventually underwent the pancreaticoduodenectomy and partial enterectomy. What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.11.049
Gastroenterology 2015;149:e5–e6
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to: Image 3: Multifocal Angiosarcoma of the Gastrointestinal Tract
Microscopically, the tumors were characterized by sheets of plump polygonal cells with abundant eosinophilic cytoplasm and hyperchromatic nuclei (Figure C). Mitoses were frequent, and primitive anastomosing vascular channels were identified. On immunohistochemical staining, the tumor cells were strongly positive for CD31 (Figure D) and Fli-1 (Figure E). These findings were consistent with the diagnosis of angiosarcoma. This patient’s postoperative recovery was uneventful, but he died of multiple metastases 6 months postoperatively. Angiosarcoma is a rare, malignant neoplasm of vascular endothelium, accounting for 1%-2% of all sarcomas.1 It most frequently occurs in the skin and the subcutaneous tissues, but extremely rarely in the gastrointestinal tract. Although the etiology of angiosarcoma is unclear, several factors such as vinyl chloride, Thoratrast, prior chemotherapy, radiation, and lymphedema have been implicated in its pathogenesis.2 The clinical diagnosis of gastrointestinal angiosarcoma is difficult, mainly because of the nonspecific symptoms at presentation. Operative resection remains the most effective treatment for this malignancy, but complete surgical excision is virtually impossible because of its infiltrative and multifocal nature.2 Palliative resection of the bleeding lesions can also improve functional status with aggressive blood transfusions. The neoadjuvant chemotherapy or radiotherapy may be beneficial for improving survival, but it has yet to be determined. Despite all efforts, the overall prognosis of gastrointestinal angiosarcoma is extremely poor, and few cases can survive >1 year.3
References 1. 2. 3.
e6
Chen JL, Mok KT, Tseng HH, et al. Duodenal angiosarcoma: an unusual cause of severe gastrointestinal bleeding. J Chin Med Assoc 2007;70:352–355. Allison KH, Yoder BJ, Bronner MP, et al. Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. Am J Surg Pathol 2004;28:298–307. Zacarias Föhrding L, Macher A, Braunstein S, et al. Small intestine bleeding due to multifocal angiosarcoma. World J Gastroenterol 2012;18:6494–6500.
Question: A 79-yearold man presented with a 4-month history of intermittent melena, weakness, and abdominal pain was admitted to our hospital. His past medical history was significant for hypertension and cholecystectomy. He denied previous radiotherapy or industrial chemical exposure. Physical examination showed an ill-appearing man with pale conjunctivae. His abdomen was soft, not tender or distended, and had no organomegaly or palpable masses. Laboratory examinations revealed hemoglobin of 56 g/L and a hematocrit of 18%. Serum levels of tumor markers were within normal range. Contrast-enhanced CT of the abdomen revealed no abnormality. Upper gastrointestinal endoscopy combined with endoscopic ultrasonography was performed. In the second part of the duodenum proximal to the papilla, a 6-mm mucous prominence lesion with friable mucosa and easy-contact bleeding was identified and biopsied (Figure A). Endoscopic ultrasonography further confirmed it was derived from the submucosa (Figure B). Because biopsy was highly suggestive of malignancy, the patient underwent an exploratory laparotomy. Three separate lesions in the duodenum, ileum, and transverse mesocolon were found during the surgery. He eventually underwent the pancreaticoduodenectomy and partial enterectomy. What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.11.049
Gastroenterology 2015;149:e5–e6
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to: Image 3: Multifocal Angiosarcoma of the Gastrointestinal Tract
Microscopically, the tumors were characterized by sheets of plump polygonal cells with abundant eosinophilic cytoplasm and hyperchromatic nuclei (Figure C). Mitoses were frequent, and primitive anastomosing vascular channels were identified. On immunohistochemical staining, the tumor cells were strongly positive for CD31 (Figure D) and Fli-1 (Figure E). These findings were consistent with the diagnosis of angiosarcoma. This patient’s postoperative recovery was uneventful, but he died of multiple metastases 6 months postoperatively. Angiosarcoma is a rare, malignant neoplasm of vascular endothelium, accounting for 1%-2% of all sarcomas.1 It most frequently occurs in the skin and the subcutaneous tissues, but extremely rarely in the gastrointestinal tract. Although the etiology of angiosarcoma is unclear, several factors such as vinyl chloride, Thoratrast, prior chemotherapy, radiation, and lymphedema have been implicated in its pathogenesis.2 The clinical diagnosis of gastrointestinal angiosarcoma is difficult, mainly because of the nonspecific symptoms at presentation. Operative resection remains the most effective treatment for this malignancy, but complete surgical excision is virtually impossible because of its infiltrative and multifocal nature.2 Palliative resection of the bleeding lesions can also improve functional status with aggressive blood transfusions. The neoadjuvant chemotherapy or radiotherapy may be beneficial for improving survival, but it has yet to be determined. Despite all efforts, the overall prognosis of gastrointestinal angiosarcoma is extremely poor, and few cases can survive >1 year.3
References 1. 2. 3.
e6
Chen JL, Mok KT, Tseng HH, et al. Duodenal angiosarcoma: an unusual cause of severe gastrointestinal bleeding. J Chin Med Assoc 2007;70:352–355. Allison KH, Yoder BJ, Bronner MP, et al. Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. Am J Surg Pathol 2004;28:298–307. Zacarias Föhrding L, Macher A, Braunstein S, et al. Small intestine bleeding due to multifocal angiosarcoma. World J Gastroenterol 2012;18:6494–6500.
