弥漫性肺脑膜上皮瘤病临床病理学观察

Clinicopathologic observation of diffuse pulmonary meningotheliomatosis

收集2例弥漫性肺脑膜上皮瘤病(diffuse pulmonary meningotheliomatosis,DPM)的临床与病理资料,并复习相关文献。此类疾病老年女性好发,胸部CT表现为两肺弥漫性结节,组织学上表现为多灶弥漫小结节,由脑膜上皮样细胞组成,与周围肺实质境界清楚、呈结节状生长。细胞形态温和,肺泡上皮细胞无异形。免疫组织化学显示上皮样细胞PR(+),EMA(+),vimentin(+)和CD56(+)。DPM是一种罕见的、以肺内微型脑膜上皮样结节为主要特征的良性病变。病理组织学结合免疫组织化学可有助于该病的正确诊断。

The clinicopathologic features of 2 cases of diffuse pulmonary meningotheliomatosis(DPM)were retrospectively analysed,and revelant literatures were reviewed.DPM was seen more common in older women.CT scanning of chest showed diffuse pulmonary nodules within both lungs.Histopathologically,DPM showed diffuse small multiple nodules composed of meningothelial-like cells,well-circumscribed margin,and nodular pattern.The neoplastic cells were bland,alveolar cells had no atypia.Immunohistochemically,epithelioid cells were all positive for PR,EMA,vimentin,CD56.DPM is a rare benign lesion characterized by pulmonary micromeningoepithelioid nodules.The combination of histopathology and immunohistochemistry may helpful for the correct diagnosis of DPM.