肝门周围丛状神经纤维瘤病；NF1 的一种罕见腹腔内表现。
Periportal Plexiform Neurofibromatosis; a rare intra-abdominal manifestation of NF1.
机构信息
Shaukat Khanam Memorial Cancer Hospital, Lahore, Pakistan.
出版信息
J Pak Med Assoc. 2020 Mar;70(3):557-558.
PMID:32207449
Abstract
Periportal Plexiform Neurofibromatosis (PPNF) is a rare visceral manifestation of Neurofibromatosis Type 1 (NF1) or Von Recklinghausen disease. Neurofibromas are the most common lesions in NF1. We present a case of a young female diagnosed with NF1 who initially presented with hard abdominal mass. Contrast enhanced CT revealed the unusual lintrahepatic periportal plexiform neurofibromatosis in addition to a typical large retroperitoneal lumbar neurofibroma.
摘要
肝门周围丛状神经纤维瘤病（PPNF）是神经纤维瘤病 1 型（NF1）或冯·雷克林豪森病的一种罕见内脏表现。神经纤维瘤是 NF1 中最常见的病变。我们报告了一例年轻女性 NF1 的病例，该患者最初表现为腹部硬块。增强 CT 显示除了典型的大型腹膜后腰椎神经纤维瘤外，还存在不常见的肝内肝门周围丛状神经纤维瘤病。
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