Komotar Ricardo J, Mocco J, Carson Benjamin S, Sughrue Michael E, Zacharia Brad E, Sisti Alexander C, Canoll Peter D, Khandji Alexander G, Tihan Tarik, Burger Peter C, Bruce Jeffery N
Department of Neurosurgery, Columbia University, New York, USA.
MedGenMed. 2004 Dec 9;6(4):42.
Pilomyxoid astrocytoma (PMA) is a recently described type of brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous system (CNS) tumor in the pediatric population, yet displays subtle histologic differences. Previous studies have shown PMA to behave more aggressively than PA, with shorter progression-free and overall survival as well as a higher rate of recurrence and CNS dissemination. These findings suggest that PMA may be a unique and distinct neoplasm. This review summarizes the histologic, clinical, and radiographic characteristics of PMA. In addition, the current treatment options and research endeavors involving this disease are described. Increased recognition of PMA within the medical community has the potential to affect the treatment and prognosis of pediatric low-grade astrocytomas.
毛黏液样星形细胞瘤(PMA)是一种最近才被描述的脑肿瘤类型。PMA与毛细胞型星形细胞瘤(PA)具有相似特征,PA是儿科人群中最常见的中枢神经系统(CNS)肿瘤,但二者在组织学上存在细微差异。先前的研究表明,PMA的行为比PA更具侵袭性,无进展生存期和总生存期更短,复发率和CNS播散率更高。这些发现表明PMA可能是一种独特且不同的肿瘤。本综述总结了PMA的组织学、临床和影像学特征。此外,还描述了目前针对该疾病的治疗选择和研究进展。医学界对PMA认识的提高有可能影响小儿低级别星形细胞瘤的治疗和预后。