Radić Sonja, Zovak Mario, Galović Marić Anita, Baturina Stjepan, Kirigin Monica Stephany, Krušlin Božo
Department of Pathology and Cytology, General Hospital Karlovac, Karlovac, Croatia.
Department of Surgery, Sestre Milosrdnice University Hospital Center, Zagreb, Croatia.
Case Rep Pathol. 2021 Sep 9;2021:7237379. doi: 10.1155/2021/7237379. eCollection 2021.
Gastrointestinal angiosarcomas are rare and represent less than 1% of all gastrointestinal tract malignancies, with most occurring in the stomach and small intestine. Occurrence in the colorectal segments is considered extremely rare. . We describe the case of a 61-year-old male with multiple primary angiosarcomas of the colon who presented with fever and abdominal pain. The patient was initially hospitalized and treated as having an infectious disease. A multislice computed tomography (MSCT) scan revealed multiple soft tissue tumors in the region of the left iliopsoas and gluteus medius muscles. After developing hematochezia, a colonoscopy was performed which found an ulcerated tumor in the sigmoid colon. The small tissue biopsy taken during the procedure presented diagnostic difficulties and was given a preliminary diagnosis of gastrointestinal stromal tumor (GIST). Examination of the resected colon segment and surrounding fat tissue revealed four separate tumors. Microscopically, the tumors were composed of solid sheets of spindle and epithelioid neoplastic cells with prominent nucleoli and numerous mitotic figures and immunohistochemically positive for ERG, CD31, CD34, vimentin, and CD117, while negative for CK7, CK20, CD20, CD3, CD45, TTF-1, PAN-CK, ALK, Mpox, S-100, and DOG1, leading to the final diagnosis of multiple colonic angiosarcomas. The patient's condition declined rapidly and he passed away from multiple organ failures 60 days after initial hospitalization.
Both clinical and pathological diagnoses of colorectal angiosarcoma are challenging. Patients are present with nonspecific symptoms leading to mismanagement and late diagnosis. A definitive pathological diagnosis relies on immunohistochemical staining for endothelial markers. Misdiagnosis as poorly differentiated adenocarcinoma or GIST is possible in limited tissue biopsies.
胃肠道血管肉瘤较为罕见,占所有胃肠道恶性肿瘤的比例不到1%,大多数发生在胃和小肠。发生在结直肠段的情况极为罕见。我们描述了一例61岁男性患有多发原发性结肠血管肉瘤,表现为发热和腹痛。患者最初住院并按传染病进行治疗。多层螺旋计算机断层扫描(MSCT)显示左髂腰肌和臀中肌区域有多个软组织肿瘤。出现便血后,进行了结肠镜检查,发现乙状结肠有一个溃疡性肿瘤。手术过程中获取的小组织活检诊断困难,初步诊断为胃肠道间质瘤(GIST)。对切除的结肠段和周围脂肪组织检查发现有四个独立的肿瘤。显微镜下,肿瘤由实性片状的梭形和上皮样肿瘤细胞组成,核仁突出,有许多有丝分裂象,免疫组化ERG、CD31、CD34、波形蛋白和CD117呈阳性,而CK7、CK20、CD20、CD3、CD45、TTF-1、泛细胞角蛋白、ALK、猴痘、S-100和DOG1呈阴性,最终诊断为多发结肠血管肉瘤。患者病情迅速恶化,初次住院60天后因多器官功能衰竭去世。
结直肠血管肉瘤的临床和病理诊断都具有挑战性。患者表现为非特异性症状,导致管理不当和诊断延迟。明确的病理诊断依赖于内皮标志物的免疫组化染色。在有限的组织活检中可能误诊为低分化腺癌或GIST。