Skovrlj Branko, Pain Margaret, Bederson Joshua B, Fowkes Mary
Department of Neurosurgery, Mount Sinai School of Medicine, New York, NY, USA.
Department of Pathology, Mount Sinai School of Medicine, New York, NY, USA.
Surg Neurol Int. 2014 Feb 27;5:29. doi: 10.4103/2152-7806.127968. eCollection 2014.
Pilomyxoid astrocytoma (PMA) has recently been accepted as an aggressive variant of pilocytic astrocytoma with distinct histopathological features. PMAs have been frequently described in the pediatric population with a predilection for the hypothalamic/chiasmatic region.
A 72-year-old African American male presented with 6 months of memory loss, difficulty expressing himself, and a progressively worsening gait. Magnetic resonance imaging of the brain demonstrated a heterogeneously enhancing cystic mass centered within the cerebellar vermis with mass effect on the fourth ventricle and ventriculomegaly. The patient underwent placement of a ventriculoperitoneal shunt followed by a surgical resection of the lesion, which after immunohistopathologic evaluation, was diagnosed as a World Health Organization grade II PMA. The patient refused further treatment of the lesion and expired 11 months after initial symptom presentation and 4 months after surgery.
To our knowledge, this is the first report of PMA of the cerebellar vermis in a previously unreported age group. This case report describes the natural history of this type of tumor in a patient who refused adjuvant therapy following surgical resection.
黏液乳头样星形细胞瘤（PMA）最近被认为是具有独特组织病理学特征的毛细胞型星形细胞瘤的侵袭性变体。PMA在儿童人群中经常被描述，好发于下丘脑/视交叉区域。
一名72岁非裔美国男性，出现6个月的记忆力减退、表达困难和逐渐加重的步态。脑部磁共振成像显示，一个不均匀强化的囊性肿块位于小脑蚓部中央，对第四脑室有占位效应并导致脑室扩大。患者接受了脑室腹腔分流术，随后对病变进行了手术切除，经免疫组织病理学评估，诊断为世界卫生组织II级PMA。患者拒绝进一步治疗该病变，在出现初始症状11个月后及手术后4个月死亡。
据我们所知，这是首次报道在一个此前未报道的年龄组中发生的小脑蚓部PMA。本病例报告描述了该类型肿瘤在手术切除后拒绝辅助治疗的患者中的自然病程。
