Benyakhlef Salma, Tahri Abir, Khlifi Asmaa, Abdelouahab Hajar, Imane Kamaoui, Moufid Fayçal, Rouf Siham, Latrech Hanane
Department of Endocrinology, Mohammed VI Hospital, Medical School, Mohamed First University, Oujda, Morocco.
Department of Radiology, Mohammed VI Hospital, Medical School, Mohamed First University, Oujda, Morocco.
Case Rep Pediatr. 2021 Sep 27;2021:6670585. doi: 10.1155/2021/6670585. eCollection 2021.
Pilomyxoid astrocytoma (PMA) is a freshly described figure of low-grade neoplasms encountered in early childhood. Nevertheless, its precise classification by the World Health Organization (WHO) is still debatable. Making an exact diagnosis relies on histological and immunohistochemical pathognomonic features with specific radiological findings. PMA behaves aggressively with a shorter progression-free survival, and its management is unfortunately still arguable. We describe a rare case of PMA involving the suprasellar region who displays symptoms consistent with diencephalic syndrome. The diagnosis was made by magnetic resonance imaging (MRI) focused on the hypothalamic-pituitary axis, and the patient underwent a subtotal tumor resection combined with chemotherapy. Diagnosis of brain tumors should be kept in mind in young children with generalized and severe unexplained loss of subcutaneous fat with failure to thrive after ruling out classical causes.
毛黏液样星形细胞瘤（PMA）是一种新近描述的在幼儿期出现的低级别肿瘤类型。然而，世界卫生组织（WHO）对其精确分类仍存在争议。准确诊断依赖于具有特定影像学表现的组织学和免疫组化特征。PMA具有侵袭性，无进展生存期较短，遗憾的是其治疗方法仍存在争议。我们描述了一例罕见的累及鞍上区域的PMA病例，该病例表现出与间脑综合征一致的症状。通过聚焦下丘脑 - 垂体轴的磁共振成像（MRI）做出诊断，患者接受了肿瘤次全切除联合化疗。对于排除经典病因后出现全身严重不明原因皮下脂肪减少且生长发育迟缓的幼儿，应考虑脑肿瘤的诊断。
