Fukita Yosho, Yasuda Ikuma, Ishibashi Hiroyuki, Asaki Tsutoshi, Adachi Seitaro, Toyomizu Michifumi, Takeda Takefumi, Suematsu Naomi
Department of Gastroenterology, Seirei Yokohama Hospital.
Department of Pathology, Seirei Yokohama Hospital.
Nihon Shokakibyo Gakkai Zasshi. 2017;114(9):1665-1674. doi: 10.11405/nisshoshi.114.1665.
An 80-year-old man presented to our hospital with complaints of tarry stool and shortness of breath. A blood test confirmed marked anemia. On abdominal contrast-enhanced computed tomography, neither hemorrhagic lesions nor tumorous lesions could be pointed out. Upper gastrointestinal endoscopy revealed multiple erythematous flat elevated lesions, which were about 10mm in diameter, located between the stomach and the horizontal part of the duodenum. Colonoscopy revealed similar lesions throughout the entire colon. Pathological examination of biopsy specimens demonstrated the proliferation of neoplastic cells positive for immunostaining of factor VIII-related antigen, CD31, and CD34. Accordingly, the patient was diagnosed with angiosarcoma of the gastrointestinal tract. Although the patient was transferred to another hospital for chemotherapy, he died shortly after the transfer because of deterioration of his bleeding symptoms. Angiosarcoma is a soft-tissue neoplasm of vascular endothelium origin, accounting for less than 2% of all sarcomas. It usually occurs in the skin of the head and neck and in soft tissues. Angiosarcoma of the gastrointestinal tract is rare and is described only in case reports and small series. These tumors are characterized by an extremely aggressive course, with a high tendency to metastasize, leaving patients with a poor prognosis. When angiosarcoma is found in multiple sites of the gastrointestinal tract, it is sometimes difficult to differentiate between primary and metastatic occurrences. We analyzed reported cases of multifocal angiosarcoma of the digestive tract, of which there are 43 so far. In 24 cases, the angiosarcoma was thought to originate from the gastrointestinal tract (primary angiosarcoma). In 13 cases, angiosarcoma of other organs metastasized to the digestive tract (metastatic angiosarcoma). In the remaining 6 cases, whether the multifocal angiosarcoma of the digestive tract was primary or metastatic was unclear. In the current case, no primary lesion was found outside the gastrointestinal tract. Therefore, he was diagnosed with primary multifocal angiosarcoma.
一名80岁男性因黑便和呼吸急促前来我院就诊。血液检查证实有明显贫血。腹部增强CT未发现出血性病变或肿瘤性病变。上消化道内镜检查发现胃和十二指肠水平部之间有多个直径约10mm的红斑性扁平隆起病变。结肠镜检查发现整个结肠均有类似病变。活检标本的病理检查显示肿瘤细胞增殖，对VIII因子相关抗原、CD31和CD34免疫染色呈阳性。因此，该患者被诊断为胃肠道血管肉瘤。尽管患者被转至另一所医院进行化疗，但转院后不久因出血症状恶化死亡。血管肉瘤是一种起源于血管内皮的软组织肿瘤，占所有肉瘤的比例不到2%。它通常发生在头颈部皮肤和软组织。胃肠道血管肉瘤罕见，仅在病例报告和小系列研究中有描述。这些肿瘤的特点是病程极具侵袭性，转移倾向高，患者预后差。当胃肠道多个部位发现血管肉瘤时，有时难以区分原发性和转移性病变。我们分析了已报道的消化道多灶性血管肉瘤病例，目前共有43例。其中24例血管肉瘤被认为起源于胃肠道（原发性血管肉瘤）。13例为其他器官的血管肉瘤转移至消化道（转移性血管肉瘤）。其余6例消化道多灶性血管肉瘤是原发性还是转移性尚不清楚。在本例中，胃肠道外未发现原发性病变。因此，他被诊断为原发性多灶性血管肉瘤。
