Regragui Meriem, Karkouri Mehdi, Ibahioin Khadija, El Azhari Abdessamad
Laboratoire d'anatomie et de cytologie pathologique, centre hospitalier universitaire lbn Rochd, 1, rue des hôpitaux, Casablanca, Maroc.
Laboratoire d'anatomie et de cytologie pathologique, centre hospitalier universitaire lbn Rochd, 1, rue des hôpitaux, Casablanca, Maroc.
Ann Pathol. 2018 Dec;38(6):381-385. doi: 10.1016/j.annpat.2018.06.003. Epub 2018 Jul 4.
Astroblastoma is a rare neuroepithelial tumor most commonly seen in children and young adults. Due to its rarity, this tumor can be easily misdiagnosed as its classification, histogenesis and therapeutic management are still being discussed. We report the case of a 21 year old man, who presented at the Emergency Room for loss of consciousness. He reported a history of headaches, vomiting and decreased visual acuity. The CT and MRI showed a left temporoparietal solid-cystic mass with heterogeneous enhancement and perilesional edema. The patient underwent a total mass resection. On histopathological examination, tumor cells were organized in perivascular pseudorosettes which are typically encountered in astroblastoma, without neither necrosis nor endothelial hyperplasia. They had broad processes and rounded nuclei without any mitotic activity. Immunochemistry stains confirmed the diagnosis by showing a positive reactivity for GFAP, EMA, vimentin and S100. Astroblastoma is a rare glial tumor of uncertain origin. Clinical presentation and imaging are nonspecific. Therefore, its diagnosis is based on histopathologic findings: typical perivascular pseudorosettes. However, similar histological pattern may be seen in other glial neoplasms. In the 2016 WHO Classification, astroblastoma is among the "other glial neoplasms" without a grading system. So far, there are no reliable prognosis factors for this tumor; however, two entities have been described: well differenciated astroblastoma (considered as low grade) and anaplastic/malignant astroblastomas (considered as high grade). Gross total resection is the treatment of choice for astroblastomas. Adjuvant therapy is still controversial. This case illustrates a cerebral tumor which is rarely encountered in practice and that can cause diagnostic problems and subsequently, inadequate treatment.
成星形细胞瘤是一种罕见的神经上皮肿瘤，最常见于儿童和青年。由于其罕见性，该肿瘤容易被误诊，因为其分类、组织发生和治疗管理仍在讨论中。我们报告一例21岁男性病例，该患者因意识丧失就诊于急诊室。他有头痛、呕吐和视力下降的病史。CT和MRI显示左颞顶叶实性囊性肿块，有不均匀强化和瘤周水肿。患者接受了肿瘤全切术。组织病理学检查显示，肿瘤细胞呈血管周围假菊形团排列，这是成星形细胞瘤的典型表现，无坏死和内皮细胞增生。它们有宽阔的突起和圆形核，无任何有丝分裂活性。免疫组化染色显示GFAP、EMA、波形蛋白和S100呈阳性反应，从而确诊。成星形细胞瘤是一种起源不明的罕见胶质肿瘤。临床表现和影像学表现不具有特异性。因此，其诊断基于组织病理学发现：典型的血管周围假菊形团。然而，其他胶质肿瘤也可能出现类似的组织学模式。在2016年世界卫生组织分类中，成星形细胞瘤属于“其他胶质肿瘤”，没有分级系统。到目前为止，该肿瘤尚无可靠的预后因素；然而，已描述了两种类型：高分化成星形细胞瘤（被认为是低级别）和间变性/恶性成星形细胞瘤（被认为是高级别）。肿瘤全切术是成星形细胞瘤的首选治疗方法。辅助治疗仍存在争议。该病例说明了一种在实践中很少遇到的脑肿瘤，它可能导致诊断问题，进而导致治疗不足。
