Baisakh Manas R, Mohapatra Nachiketa, Adhikary Samiran D, Routray Debasis
Department of Pathology, Apollo Hospital, Bhubaneswar, Odisha, India.
Indian J Pathol Microbiol. 2014 Jan-Mar;57(1):130-2. doi: 10.4103/0377-4929.130924.
Malignant peripheral nerve sheath tumor (MPNST) of the adrenal gland is extremely rare. Most of them occur in association with neurofibromatosis, ganglioneuroma or as part of a composite tumor such as pheochromocytoma. Only seven cases of MPNST of the adrenal gland have been reported in the literature till date. Discriminating this entity from other soft tissue sarcomas and gastrointestinal stromal tumor of the adrenal gland has important diagnostic and therapeutic implications. Moreover, the tumor size and pattern of expression for certain immunohistochemical markers may serve as independent predictors of aggressiveness. Herein we present a 24-years-old male with features of Von Recklinghausen's disease who presented with large left adrenal gland malignant peripheral nerve sheath tumor.
肾上腺恶性外周神经鞘瘤（MPNST）极为罕见。大多数病例与神经纤维瘤病、神经节神经瘤相关，或作为诸如嗜铬细胞瘤等复合肿瘤的一部分出现。迄今为止，文献中仅报道了7例肾上腺MPNST。将该实体与其他软组织肉瘤及肾上腺胃肠道间质瘤进行鉴别具有重要的诊断和治疗意义。此外，肿瘤大小及某些免疫组化标志物的表达模式可能是侵袭性的独立预测指标。在此，我们报告1例患有冯·雷克林豪森病的24岁男性，其表现为左侧肾上腺巨大恶性外周神经鞘瘤。
