Case report: Human chorionic gonadotropin-producing germinal sella tumor

Reporte de caso: Tumor germinal selar productor de gonadotropina coriónica humana

Abstract

cells. Germinomas represent 65-75 % of GCTs in the central nervous system, they are classified according to specific tumor markers: alpha-fetoprotein (AFP) and fraction b of human chorionic gonadotropin (b-hCG). In males, the pathophysiological mechanism of hCG producing tumors, the homology between luteinizing hormone (LH) and hCG activates the LH receptor in testicular Leydig cells, increasing testosterone production causing precocious puberty and virilization.

The case of a 6-year-old male patient is reported, who started with polydipsia, weight loss, nocturia and an increased urination frequency, later adding headaches, for which the screening test for polyuric polydipsic syndrome was performed. Physical examination revealed male genitalia with macrogenitalism and testicles with Tanner III. Extension studies were performed, finding elevation of hCG (10.3 IU/ml) and AFP (1.4 IU/ml), and the cranial magnetic resonance imaging (MRI) reports thickening of the pituitary stalk without the presence of neurohypophysis.

Diagnosis included sellar germ cell tumor, central diabetes insipidus and peripheral precocious puberty are included. Treatment started with desmopressin, spironolactone as antiandrogen and anastrozole. The presentation of diabetes insipidus and precocious puberty in males should make suspect tumoral causes. Therefore, it is always pertinent to carry out imaging studies and laboratories to evaluate the function of the hypothalamus-pituitary axis.