Birkhaeuser Frederic, Ackermann Christoph, Flueckiger Tobias, Guenin Marc-Olivier, Kern Beatrice, Tondelli Peter, Peterli Ralph
Department of Surgery, St. Claraspital, Basel, Switzerland.
Dis Colon Rectum. 2004 Oct;47(10):1734-7. doi: 10.1007/s10350-004-0637-5.
We describe a young female patient suffering from a PEComa (perivascular epithelioid cell tumor) of the cecum, incidentally found at an examination made by her family physician. The perivascular epithelioid cell tumor is a very rare tumor, until today reported in a few cases in falciform ligament, uterus, jejunum, terminal ileum, rectum, liver, kidney, lung, pancreas, prostate, and soft tissue of the thigh. This tumor is part of a new group of tumors, comprised of angiomyolipoma, lymphangiomyolipoma, and clear-cell myomelanocytic "sugar" tumor. Defined by coexpression of melanocytic (HMB-45) and muscle markers (smooth muscle actin and desmin) the perivascular epithelioid cell tumor does not have predictable histopathologic behavior. Some cases of metastasis are described, comorbidities such as tuberous sclerosis of the brain "Bourneville" and lymphangioleiomyomatosis have to be excluded. The therapy consists of the radical resection. An adjuvant therapy is not known. Recommended is a close and long-term follow-up clinically and by CT scan.
我们描述了一名年轻女性患者，她患有盲肠的PEComa（血管周上皮样细胞瘤），这是在家庭医生进行的一次检查中偶然发现的。血管周上皮样细胞瘤是一种非常罕见的肿瘤，迄今为止仅在镰状韧带、子宫、空肠、回肠末端、直肠、肝脏、肾脏、肺、胰腺、前列腺以及大腿软组织中的少数病例中有报道。该肿瘤是一组新的肿瘤的一部分，包括血管平滑肌脂肪瘤、淋巴管平滑肌瘤和透明细胞肌黑素细胞性“糖”瘤。血管周上皮样细胞瘤由黑素细胞标记物（HMB-45）和肌肉标记物（平滑肌肌动蛋白和结蛋白）的共表达所定义，其组织病理学行为不可预测。有一些转移病例的描述，必须排除诸如脑部结节性硬化症“博恩维尔病”和淋巴管平滑肌瘤病等合并症。治疗方法为根治性切除。目前尚无辅助治疗方法。建议进行密切的长期临床随访并通过CT扫描进行监测。
