Germinoma

Jai Grewal MD (Dr. Grewal of the Long Island Brain Tumor Center at Neurological Surgery, PC, received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Harpreet K Grewal MD (Dr. Grewal of JGMDPC in Roslyn, New York, has no relevant financial relationships to disclose; Dr. Grewal's spouse received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Edward J Dropcho MD, editor. (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released August 29, 1995; last updated February 2, 2015; expires February 2, 2018

This article includes discussion of germinoma, dysgerminoma, and seminoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Intracranial germinomas are relatively rare tumors that most often occur in childhood and adolescence, where they typically arise in the pineal and suprasellar regions and present with hydrocephalus and diabetes insipidus. In this article, the authors expand our view of the molecular pathogenesis of this neoplasm, highlight novel markers used in the diagnosis of this tumor, and review recent modifications in treatment strategies.

Historical note and terminology

Tumors arising in the posterior third ventricular and suprasellar regions represent a variety of cell types, including those derived from ectopic primordial germ cells, and those derived from cellular elements intrinsic to the CNS (Kleihues and Cavenee 2000) (see Table 1). In addition, pineal and suprasellar metastases from systemic malignancies can occur. Tumors derived from primordial germ cells are designated primary CNS germ cell tumors. This group encompasses a number of histologic tumor types, including the germinoma and the “nongerminomatous” germ cell tumors: embryonal carcinoma, yolk sac (endodermal sinus) tumor, choriocarcinoma, teratoma, and mixed germ cell tumors, in which components of 2 or more cell types are present. Histologic, electron microscopic, immunohistochemical, and molecular biological studies have confirmed the similarity of intracranial germinoma and nongerminomatous germ cell tumors to their extraneural analogues (Bentley et al 1990).

Table 1. Nongerm-Cell Tumors Occurring in the Suprasellar and Pineal Regions

Suprasellar region

 

Astrocytic tumors

   

- Astrocytoma
- Anaplastic astrocytoma
- Glioblastoma multiforme

• Oligodendrogliomas
• Optic nerve gliomas
Meningiomas
• Craniopharyngiomas
• Chordoid glioma
• Lymphoma
• Epidermoid cysts
• Pituitary tumors
• Rathke cleft cyst
• Esthesioneuroblastomas
• Metastases (breast, lung, kidney, and melanoma are most common)

Pineal region

 

Astrocytic tumors

   

- Astrocytoma
- Anaplastic astrocytoma
- Glioblastoma multiforme

• Oligodendrogliomas
• Ependymomas
Choroid plexus papillomas
• Pineal cysts
• Dermoid cysts
• Primitive neuroectodermal tumors
• Pineocytomas and pineoblastomas
• Hamartomas
• Primary malignant melanoma
• Metastases (breast, lung, kidney and melanoma are most common)

Germinomas are the most common of the intracranial germ cell tumors, accounting for 54% (range: 29% to 65%) of germ cell tumors in several large series (Jennings et al 1985; Russell and Rubinstein 1989; Hoffman et al 1991; Balmaceda et al 1996; Schild et al 1996; Matsutani et al 1997). Germ cell tumors, both neural and extraneural have a predilection for midline structures, occurring primarily in the pineal and suprasellar regions in the CNS and peripherally in the sacrococcygeal region, retroperitoneum, and mediastinum. Uncommonly, germinomas may occur in the basal ganglia and thalamus (3% of cases), fourth and lateral ventricles, cerebellum, cerebellopontine angle, and spinal canal (Jennings et al 1985; Russell and Rubinstein 1989; Hoffman et al 1991). Although extremely rare, germinoma in the brainstem has also been described (Neelima et al 2010). Multifocal tumors involving both the pineal gland and neurohypophysis, and sometimes diffusely involving the third ventricle and adjacent structures, have been reported in 2% to 8% of cases. Series over the last 5 years, using current neuroimaging techniques, have recorded bifocal (pineal and suprasellar) or multifocal involvement in about 18% of cases (Allen et al 1994; Balmaceda et al 1996; Matsutani et al 1997; Sawamura et al 1998a; Bamberg et al 1999; Bouffet et al 1999). The incidence of CSF dissemination at presentation is difficult to determine because of various methods of assessment, but in series since 1989, about 14% ) of patients were diagnosed with disseminated disease by MRI scanning or CSF cytology (Legido et al 1989; Dattoli and Newall 1990; Shibamoto et al 1994a; Balmaceda et al 1996; Huh et al 1996; Schild et al 1996; Haddock et al 1997; Aoyama et al 1998; Sawamura et al 1998a; Bamberg et al 1999; Bouffet et al 1999; Buckner et al 1999); the true incidence may be somewhat higher.

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