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Germinoma with Involvement of Midline and Off-Midline Intracranial Structures

DOI: 10.1155/2014/936937

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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. 1. Introduction Germ cell tumors (GCT) represent approximately 3% of neoplasms in children’s cancer registries [1]. They constitute 0.1 to 2.4% of all childhood intracranial tumors in North America and Europe, while they account for almost 2.1 to 9.5% in Japan and the Far East [2, 3]. Central nervous system germ cell tumors (CNSGCT) are rare and most of them occur in patients under 20 years of age [1, 4]. CNSGCTs have been classified in “secreting” and “nonsecreting” tumors. Secreting tumors are defined as those presenting with an elevated CSF AFP ≥ 10?ng/mL or above the local laboratory’s normal range and/or a CSF -HCG level ≥ 50?IU/l or greater than the accepted laboratory normal range. This has been shown to be related to prognosis and treatment response [1]. Brain germinomas are usually serologically negative for these markers [5]. The most common sites of involvement of intracranial germinomas are the pineal or suprasellar regions, while some patients have both localizations at the time of diagnosis [1, 4]. Off-midline germinomas arising in the basal ganglia, thalami, and internal capsule, also called ectopic

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