Capsuloganglionic germinoma: A rare site for uncommon childhood tumor

Abstract

Germ cell tumors (GCTs) are rare intracranial tumors with a strong predilection for children. Commonly, these tumors arise either in the suprasellar or the pineal region. The basal ganglia-thalamus complex represents a rare site of nonmidline intracranial GCTs. Such basal ganglionic GCTs have been reported to produce certain interesting clinico-radiological features, the knowledge of which may provide important diagnostic clues preoperatively. We present the case of a 9.5-year-old boy who presented with right hemiparesis and precocious puberty. Imaging revealed a heterogeneously enhancing mass involving the left capsuloganglionic region, sparing the thalamus. There was little perilesional edema and midline shift. Because of the deep location of the mass and a lack of mass effect, a neuronavigation-guided tumor biopsy was performed which unraveled a pure germinoma. The child was referred for adjuvant radiotherapy following an uneventful postoperative course. At the time of writing the report, the child was on radiotherapy and doing well.

Molecular Pathology and Targeted Therapies for Personalized Management of Central Nervous System Germinoma

“…GH (growth hormone) deficiency and hypogonadism are the most frequent endocrine insufficiencies (89–95% of cases), followed by hypothyroidism and hypocortisolaemia, in approximately 50% of patients [ 40 ]. Boys with intracerebral germ tumours can develop precocious puberty (isosexual pseudoprecocity) because of beta-human chorionic gonadotropin (β-hCG) secretion of the syncytiotrophoblasts component of the tumour, which stimulates Leydig cells, with subsequent production of testosterone [ 41 , 42 ].…”