Ahmad Gaity F, Athavale Ram, Hamid Bushra N A, Davies-Humphreys John
Department of Obstetrics and Gynaecology, Countess of Chester Hospital, Liverpool Road, Chester, UK.
J Reprod Med. 2004 May;49(5):404-7.
Malignant hemangiopericytoma (MHPC) is a rare vascular tumor and has been reported to occur in the musculature of the extremities, retroperitoneum and pelvis. Omental hemangiopericytomas (HPCs) are extremely rare. Synovial sarcomas and solitary fibrous tumors share histologic features with HPCs, causing diagnostic difficulties. Immunohistochemistry is essential for the diagnosis.
A 74-year-old woman presented with an abdominopelvic mass. A malignant ovarian tumor was suspected on clinical features, ultrasound and computed tomography. Staging laparotomy revealed a large, vascular tumor adherent to loops of small bowel, colon, cecum and appendix, but the ovaries and uterus were normal. The tumor was completely removed after extensive dissection. Histopathology and detailed immunohistochemistry established the diagnosis of a malignant hemangiopericytoma arising from the omentum. The patient developed recurrent subacute bowel obstruction and died 4 months after the initial diagnosis.
MHPCs are rare tumors and not likely to be diagnosed preoperatively. Treatment is therefore individualized and based on the findings at laparotomy. Some tumors, such as the one described here, exhibit very aggressive behavior.
恶性血管外皮细胞瘤(MHPC)是一种罕见的血管肿瘤,据报道可发生于四肢肌肉组织、腹膜后和骨盆。大网膜血管外皮细胞瘤(HPC)极为罕见。滑膜肉瘤和孤立性纤维瘤与HPC具有共同的组织学特征,导致诊断困难。免疫组织化学对诊断至关重要。
一名74岁女性因腹盆腔肿块就诊。根据临床特征、超声和计算机断层扫描怀疑为恶性卵巢肿瘤。分期剖腹探查发现一个大的血管性肿瘤,附着于小肠、结肠、盲肠和阑尾肠袢,但卵巢和子宫正常。经过广泛解剖后肿瘤被完全切除。组织病理学和详细的免疫组织化学检查确诊为起源于大网膜的恶性血管外皮细胞瘤。患者出现复发性亚急性肠梗阻,并在初次诊断后4个月死亡。
MHPC是罕见肿瘤,术前不太可能被诊断。因此,治疗是个体化的,基于剖腹探查的结果。一些肿瘤,如本文所述的肿瘤,表现出非常侵袭性的行为。