具有横纹肌母细胞分化的恶性周围神经鞘瘤的罕见组织学变异型。
Unusual histological variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation.
作者信息
Shete Smita, Bolde Saroj, Pandit Gopal, Matkari Pushkar, Ingle Sachin B
机构信息
Smita Shete, Saroj Bolde, Gopal Pandit, Pushkar Matkari, Department of Pathology, Dr. Vaishampayan Memorial Government Medical College, Solapur, Maharashtra 4132512, India.
出版信息
World J Clin Cases. 2015 Apr 16;3(4):389-92. doi: 10.12998/wjcc.v3.i4.389.
Malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation is called as malignant triton tumor (MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with neurofibromatosis (NF-1). We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an enlarged mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.
具有横纹肌母细胞分化的恶性周围神经鞘膜瘤（MPNST）被称为恶性蝾螈瘤（MTT）。它是一种侵袭性很强的软组织肿瘤，局部复发率较高。MTT的预后比MPNST差。MTT在神经纤维瘤病（NF-1）患者中似乎更具侵袭性。我们在此报告一例有趣的病例，一名55岁男性，自30岁起全身患有多发性神经纤维瘤和多个咖啡牛奶斑，被诊断为NF-1。6年来，他左大腿有一个肿块增大。对肿块进行了广泛切除。组织病理学检查显示诊断为MTT，免疫组织化学证实了这一诊断。
Zotero 插件