Introduction: Germ cell tumors (GCTs) are classified as extragonadal if there is no evidence of a primary tumor in the gonads. They typically occur in the midline structures. Newly diagnosed adult cases of pituitary or pineal glands germinomas are very rare since most of the cases are diagnosed in the mid teens, presenting 14:1, on behalf of the male gender. The estimated incidence of this tumor in western countries is between 0.43.4%, being more frequent in the Asia.
Case report: A 30-year-old man, was referred to Endocrinology in August 2007, complaining of lack of energy, asthenia, decreased body hair, decreased libido and erectile dysfunction, with no ejaculate, since December 2005. He was previously observed by Urology, carried out thyroid function and was medicated with levothyroxine 100 μg/day since March 2007. Decreased hairiness (axillary, trunk and beard) and decreased testicular volume, without other major changes on physical exam. In October 2007, starts complaints of headaches and blurred left vision. The patient was observed by Ophthalmology, which diagnosed bitemporal hemianopsia, and performed pituitary MRI which revealed a bulky suprasellar lesion with 16×23×20 mm. The endocrinological evaluation revealed hypopituitarism. He underwent pituitary surgery at May 2008, with near total resection, because of proximity to the optic chiasm. The histological exam revealed germ cell tumor. According to the radiosensitive of these tumors treatment was completed with external radiotherapy. After surgery, the hormonal evaluation showed panhypopituitarism and diabetes insipidus. The patient remains clinically stable, on hormonal replacement therapy and without evidence of recurrence.
Discussion: Owing to its rarity in adulthood, not only by location but also to its multifaceted clinical presentation, requires multidisciplinary assessment. The cranial location requires surgical treatment which is often near total resection, because of risk of surgical complications. Therefore, radiation therapy is usually necessary. Intracranial germinomas have a reported 90% five years survival, so follow-up should be extensive.
27 Apr - 01 May 2013
European Society of Endocrinology