Niamathullah Sadiya, Sivaselvam S, Ghosh Mitra, Ghosh Siddhartha
Department of Histopathology, SRM Institute of Medical Sciences, Vadapalani, Chennai, India.
Indian J Pathol Microbiol. 2014 Jan-Mar;57(1):101-4. doi: 10.4103/0377-4929.130913.
Pleomorphic xanthoastrocytoma has been considered as an astrocytic tumor with relatively favorable prognosis. It corresponds to WHO Grade-II neoplasm. Recently, several patterns with relatively poor prognosis have been recorded and a new concept of "PXA with anaplastic features" has been proposed. The present case is about a 9-year-old girl who presented with symptoms of recurrent headache, seizures and poor academic performance. MRI revealed left fronto-parietal irregular enhancing mass lesion with callosal involvement and right mid-brain arteriovenous malformation. Clinical and radiological examination was suggestive of a high grade glial neoplasm/PNET. A diagnosis of high grade glial neoplasm was rendered on the squash smears submitted for frozen sections based on the presence of spindle cells, admixed with pleomorphic bizarre, giant cells with multilobated nuclei showing few atypical mitosis and abundant eosinophilic cytoplasm. Frontal craniotomy with debulking of the tumor was performed and permanent sections revealed a biphasic glial neoplasm with spindle cells arranged in fascicles admixed with bizarre multinucleated giant cells showing abundant vacuolated and lipidized cytoplasm, nuclear hyperchromasia with intranuclear inclusions. Eosinophilic granular bodies, mitosis of 7/10 HPF, micro vascular proliferation, necrosis and invasion into the underlying brain parenchyma were noted. With these histomorphological findings a diagnosis of pleomorphic xanthoastrocytoma with anaplastic features was rendered.
多形性黄色星形细胞瘤一直被认为是一种预后相对良好的星形细胞肿瘤。它相当于世界卫生组织二级肿瘤。最近，已记录到几种预后相对较差的模式，并提出了“具有间变性特征的多形性黄色星形细胞瘤”这一新概念。本病例是一名9岁女孩，出现反复头痛、癫痫发作和学业成绩不佳的症状。磁共振成像显示左额顶叶不规则强化肿块病变，累及胼胝体，右侧中脑动静脉畸形。临床和影像学检查提示为高级别胶质瘤/原始神经外胚层肿瘤。根据送检冰冻切片的压片涂片上存在梭形细胞，混有形态多样的奇异巨细胞，核呈多叶状，显示少量非典型有丝分裂且胞质嗜酸性丰富，做出了高级别胶质瘤的诊断。进行了额部开颅肿瘤部分切除术，永久切片显示为双相性胶质瘤，梭形细胞成束排列，混有奇异的多核巨细胞，显示丰富的空泡化和脂质化胞质，核深染并伴有核内包涵体。可见嗜酸性颗粒小体，每10个高倍视野有7个有丝分裂象，微血管增生、坏死以及侵犯至下方脑实质。根据这些组织形态学表现，诊断为具有间变性特征的多形性黄色星形细胞瘤。
