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儿童促结缔组织增生性婴儿星形细胞瘤:两例报告

A Rare Tumor in Childhood Desmoplastic Infantile Astrocytoma: Two Case Reports.

作者信息

Tanık Canan, Devecioğlu Elif Gökçe, Hasçiçek Seyhan, Meltem Can Songül, Halefoğlu Ahmet Mesrur, Kabukçuoğlu Fevziye

机构信息

Department of Pathology, Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.

Department of Neurology, Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.

出版信息

Sisli Etfal Hastan Tip Bul. 2018 Sep 28;52(3):224-228. doi: 10.14744/SEMB.2018.29292. eCollection 2018.

Abstract

Desmoplastic infantile astrocytomas (DIAs), are rare supratentorial tumors, usually observed in the first 24 months of life. Despite their aggressive appearance, they tend to follow a favorable clinical course. Total or near total resection of tumor is usually the treatment option. Desmoplastic Infantile Ganglioglioma (DIG) and DIA are WHO grade I tumors that have similar clinical and morphological findings. The only criterion in differential diagnosis is the neural component of DIG. These tumors both have dense fibroblastic stroma and positive staining with glial fibrillar acidic protein (GFAP) and CD34. A rare case of desmoplastic infantile astrocytoma presenting with right side partial seizures presented in a 1-year-old child. A rare case of desmoplastic infantile astrocytoma presenting with focal onset generalized seizures presented in a 1-year-old child. Despite their radiological and histological properties, these tumors have a benign course. After 3-year follow-up for the first case and 1-year follow-up for the second case, there was no recurrence.

摘要

促纤维增生性婴儿星形细胞瘤(DIAs)是罕见的幕上肿瘤,通常在生命的前24个月出现。尽管其外观具有侵袭性,但它们往往具有良好的临床病程。肿瘤的全切除或近全切除通常是治疗选择。促纤维增生性婴儿节细胞胶质瘤(DIG)和DIA是世界卫生组织I级肿瘤,具有相似的临床和形态学表现。鉴别诊断的唯一标准是DIG的神经成分。这些肿瘤都有致密的成纤维细胞基质,胶质纤维酸性蛋白(GFAP)和CD34染色呈阳性。一名1岁儿童出现右侧部分性癫痫发作,诊断为促纤维增生性婴儿星形细胞瘤,此为罕见病例。一名1岁儿童出现局灶性发作全身性癫痫发作,诊断为促纤维增生性婴儿星形细胞瘤,此为罕见病例。尽管具有放射学和组织学特征,但这些肿瘤病程呈良性。第一例患者随访3年,第二例患者随访1年,均无复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a0a5/7315091/ac8e4a2d3ba5/MBSEH-52-224-g001.jpg

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