胃肠道血管周上皮样细胞肿瘤的研究进展

Progress of perivascular epithelioid cell tumor occurring in gastrointestinal tract

摘要:

血管周上皮样细胞瘤是一种罕见的间叶组织肿瘤，由组织学和免疫组织化学有独特表现的血管周 上皮样细胞组成，良性多见，好发于成年女性。血管周上皮样细胞瘤主要起源于女性的子宫，可发生于肺、肝、 肾脏、膀胱及胃肠道等部位。血管周上皮样细胞瘤肿瘤家族包括血管平滑肌脂肪瘤、肺及肺外组织的透明细 胞糖瘤、淋巴管平滑肌瘤病及镰状韧带/ 韧带的透明细胞肌黑质细胞瘤。血管周上皮样细胞瘤的临床表现缺 乏特异性，发病机制不明，病理学上具有独特的形态，对肌细胞和黑素细胞标记具有免疫反应性。发生于胃肠 道的血管周上皮样细胞瘤少见，该文就发生于胃肠道的血管周上皮样细胞瘤的临床表现、临床病理特征、诊断、 治疗及预后做一综述。

Abstract:

Perivascular epithelioid cell tumor is a rare mesenchymal tumor composed of unique histological and immunohistochemical perivascular epithelioid cells, which is benign more than malignant and frequently occurs in adult women. Perivascular epithelioid cell tumor mainly originates from the female uterus and can occur in other parts such as lung, liver, kidney, bladder and gastrointestinal tract. The family of perivascular epithelioid cell tumor tumors include angiomyolipoma (AML), clear cell "sugar" tumors (CCST) of lung and extrapulmonary tissues, lymphangioleiomyomatosis (LAM) and clear cell myomelanocytic tumor (CCMMT) in falciform ligament/ ligaments. However, the clinical manifestations of perivascular epithelioid cell tumor are lack of specificity, and the pathogenesis is unknown. It has unique morphological features and immunoreactivity for myocytes markers and melanocyte markers. Perivascular epithelioid cell tumor, which occurs in the gastrointestinal tract, were rare. In this review, we carry out a comprehensive survey based on published data and discuss our current understanding of the clinicopathologic features, diagnosis and differential diagnosis, treatment and prognosis of perivascular epithelioid cell tumor occurring in the gastrointestinal tract.