A 63-Year-Old Man with Neurofibromatosis Type 1 and Intracerebral Astrocytoma: A Case Report

Iman Hakim Wicaksana, Geizar Arsika Ramadhana
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Abstract

Background: Neurofibromatosis type 1 (NF-1) is one of the hereditary neurocutaneous disorders. Neurofibromatosis type 1 has broad clinical manifestations that can occur in the skin, bones, eyes, and nervous system. In progressive cases, NF-1 can malign and cause intracerebral tumor lesions. Astrocytoma, especially pilocytic astrocytoma, is one of the tumor classes that patients with NF-1 can develop with far-reaching disease progression. This case report presents a case of NF-1 with intracerebral astrocytoma. Case presentation: A 63-year-old man with whole body lumps since childhood presented with weakness in the right limbs 1 month ago. 3 months earlier, the patient also had a full-body seizure for 1 minute twice. The patient then underwent a magnetic resonance imaging (MRI) examination of the brain and found a mass in the left centrum semiovale, left corona radiata, left insular, left external capsule, and left frontotemporoparietal lobe with a size of 7.2 x 5.3 x 6.2 cm. The patient then underwent a VP shunt followed by tumor resection. The tumor was successfully evacuated, and histopathological examination with hematoxylin & eosin (HE) staining revealed glioblastoma multiformis. The patient also underwent histopathologic examination with Ki-67 immunohistochemistry (IHC) staining and showed pilocytic astrocytoma. Conclusion: Craniotomy of tumor excision is the definitive therapy in neurofibromatosis patients accompanied by intracerebral astrocytoma.
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一名 63 岁男性神经纤维瘤病 1 型和脑内星形细胞瘤患者的病例报告
背景:神经纤维瘤病 1 型(NF-1)是遗传性神经皮肤疾病之一。神经纤维瘤病 1 型具有广泛的临床表现,可发生在皮肤、骨骼、眼睛和神经系统。在进展期病例中,NF-1 可恶变并引起脑内肿瘤病变。星形细胞瘤,尤其是柔毛细胞性星形细胞瘤,是NF-1患者可发展为影响深远的肿瘤类型之一。本病例报告介绍了一例NF-1合并脑内星形细胞瘤的病例。 病例介绍:一名自幼全身肿块的 63 岁男性患者 1 个月前出现右侧肢体无力。3 个月前,患者还曾两次全身抽搐 1 分钟。患者随后接受了脑部磁共振成像(MRI)检查,发现左半叶中心、左放射冠、左岛叶、左外囊和左额颞顶叶有肿块,大小为 7.2 x 5.3 x 6.2 厘米。患者随后接受了 VP 分流术,然后进行了肿瘤切除术。肿瘤被成功切除,经苏木精和伊红(HE)染色的组织病理学检查显示为多形性胶质母细胞瘤。患者还接受了 Ki-67 免疫组织化学(IHC)染色组织病理学检查,结果显示为柔毛细胞性星形细胞瘤。 结论对于伴有脑内星形细胞瘤的神经纤维瘤病患者,肿瘤切除的开颅手术是最终的治疗方法。
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