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累及中线及中线外颅内结构的生殖细胞瘤。

Germinoma with involvement of midline and off-midline intracranial structures.

作者信息

Loto Monica Graciela, Danilowicz Karina, González Abbati Santiago, Torino Rafael, Misiunas Alejandro

机构信息

Division of Endocrinology, Metabolism, Nutrition and Diabetes, 1828 Caseros Avenue, 1152 Buenos Aires, Argentina.

Department of Neurosurgery, British Hospital, 1828 Caseros Avenue, 1152 Buenos Aires, Argentina.

出版信息

Case Rep Endocrinol. 2014;2014:936937. doi: 10.1155/2014/936937. Epub 2014 Feb 9.

Abstract

Germinomas are malignant intracranial germ tumors, usually found in suprasellar regions. Less than 10% are localized in off-middle structures, and synchronous involvement of both structures has only exceptionally been published. A case of an 18-year-old male patient with progressive right-sided hemiparesis and panhypopituitarism was reviewed. Brain MRI showed a solid mass involving pituitary and hypothalamus with thickening of pituitary stalk, high intensity lesions on T2-weighted imaging in left internal capsule, caudate nucleus, globus pallidus, and mild atrophy of the left internal capsule and cerebral peduncle. Nonadenomatous lesions were considered in the differential diagnosis. Alfa-fetoprotein (AFP) levels were negative in both serum and cerebrospinal fluid (CSF), while β -human chorionic gonadotrophin ( β -HCG) levels were slightly increased in CSF. A transsphenoidal biopsy identified a germinoma. Four cycles of chemotherapy with bleomicine, etoposide, and cysplatin were given, followed by radiotherapy, but patients died due to a recidiva. Conclusion. Germinoma must be considered in patients with insipidus diabetes with a sellar mass with thickening of pituitary stalk; and ectopic germinoma must be suspected in patients with slowly progressive hemiparesis with cerebral hemiatrophy. Even with a rare condition, colocalization of midline and off-midline germinoma must be suspected in the presence of these typical signs of both localizations.

摘要

生殖细胞瘤是恶性颅内生殖细胞肿瘤,通常位于鞍上区域。不到10%位于非中线结构,且两个结构同时受累的情况仅有极少的报道。回顾了一例18岁男性患者,其有进行性右侧偏瘫和全垂体功能减退。脑部磁共振成像(MRI)显示一个实性肿块累及垂体和下丘脑,垂体柄增粗,左侧内囊、尾状核、苍白球在T2加权成像上有高信号病变,左侧内囊和大脑脚轻度萎缩。鉴别诊断时考虑了非腺瘤性病变。血清和脑脊液(CSF)中的甲胎蛋白(AFP)水平均为阴性,而脑脊液中的β-人绒毛膜促性腺激素(β-HCG)水平略有升高。经蝶窦活检确诊为生殖细胞瘤。给予了四个周期的博来霉素、依托泊苷和顺铂化疗,随后进行放疗,但患者因复发死亡。结论。对于患有尿崩症且鞍区有肿块、垂体柄增粗的患者,必须考虑生殖细胞瘤;对于有缓慢进展性偏瘫伴脑半球萎缩的患者,必须怀疑异位生殖细胞瘤。即使是罕见情况,在出现这两种定位的典型体征时,也必须怀疑中线和非中线生殖细胞瘤的共定位。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c581/3934446/216c58562ff9/CRIM.ENDOCRINOLOGY2014-936937.001.jpg

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