Phi Ji Hoon, Koh Eun Jung, Kim Seung-Ki, Park Sung-Hye, Cho Byung-Kyu, Wang Kyu-Chang
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 101 Daehakro, Jongno-gu, Seoul 110-744, Republic of Korea.
Childs Nerv Syst. 2011 Dec;27(12):2177-81. doi: 10.1007/s00381-011-1587-6. Epub 2011 Sep 25.
Desmoplastic infantile astrocytoma (DIA) is an uncommon brain tumor of early infancy. The tumor is characterized by a lobar location, glial histology, and excellent prognosis after surgical removal. DIA and a similar tumor, desmoplastic infantile ganglioglioma (DIG) have been considered to be benign neoplasms, but the prognosis of DIA and DIG is currently under question as atypical and aggressive clinical features of the tumors have been reported. We encountered a patient who was diagnosed with DIA at the age of 22 months and exhibited tumor recurrence 8 years later. Surgical removal of the recurred tumor revealed that the tumor had transformed to overt glioblastoma. This case demonstrates that DIA is not an absolutely benign tumor and that careful clinical surveillance is needed during the follow-up period.
促纤维增生性婴儿星形细胞瘤(DIA)是一种婴幼儿期少见的脑肿瘤。该肿瘤的特点是位于脑叶、具有神经胶质组织学特征,手术切除后预后良好。DIA和一种类似的肿瘤,促纤维增生性婴儿节细胞胶质瘤(DIG)曾被认为是良性肿瘤,但由于已报道肿瘤具有非典型和侵袭性临床特征,目前DIA和DIG的预后受到质疑。我们遇到一名患者,其在22个月大时被诊断为DIA,8年后出现肿瘤复发。手术切除复发肿瘤显示肿瘤已转变为明显的胶质母细胞瘤。该病例表明DIA并非绝对良性肿瘤,随访期间需要仔细的临床监测。