Tumores suprarrenales feminizantes en España: aportación de un caso y revisión de los cinco pacientes descritos / Feminizing adrenal tumors in Spain: report of a case and review of the five previously published patients

Los tumores adrenales feminizantes son muy raros. Se presenta el estudio clínico y hormonal de un varón de 49 años hasta su fallecimiento, 6 años después del diagnóstico, junto con una revisión de los otros 5 casos descritos en España. El paciente consultó por ginecomastia y disminución de la libido; se halló gran elevación de estrógenos, testosterona en el límite bajo de la normalidad y un tumor suprarrenal derecho que, al extirparlo, se observó que era de histología benigna, informado como adenoma. Tres años después, reapareció la clínica junto con aumento de estrógenos, glucocorticoides y andrógenos; se objetivó recidiva tumoral en el polo superior del riñón derecho y metástasis peritoneales, hepáticas y pulmonares. Tratado con mitotano y aminoglutetimida, las concentraciones hormonales descendieron transitoriamente, pero volvieron a aumentar hasta su fallecimiento a los 6 años del diagnóstico inicial. Resalta en este caso el carácter aparentemente benigno y unisecretor del tumor para recidivar a los 3 años con carácter maligno y plurisecretor y metástasis generalizadas sin respuesta al tratamiento médico (AU)

Feminizing adrenal tumours are very rare. We report the clinical and hormonal study of a case, a 49 years old male, since his first consultation until his death 6 years after the initial diagnosis, and a review of the other 5 Spanish patients previously published. His initial symptoms were gynecomastia and libido decrease, with increase of plasmatic and urinary oestrogen levels, plasma testosterone near low normal level and a right adrenal gland tumour that, after its removal, showed a benign histology and was classified as an adrenocortical adenoma. Three years after, initial symptoms returned, with oestrogen, glucocorticoid and androgen hypersecretion, tumour local relapse and peritoneal, liver and lung metastasis. After mitotane and aminoglutethimide therapy, hormonal concentrations fell temporary and then raised again until his death 3 years later. The main special feature of this case is the apparently benign initial adrenal tumour with only oestrogen hypersecretion, and its relapse 3 years later with secretion of several steroid hormones, generalized metastasis and no response to medical therapy (AU)

[Feminizing adrenal tumours in Spain: report of a case and review of the five previously published patients]. / Tumores suprarrenales feminizantes en España: aportación de un caso y revisión de los cinco pacientes descritos.

Feminizing adrenal tumours are very rare. We report the clinical and hormonal study of a case, a 49 years old male, since his first consultation until his death 6 years after the initial diagnosis, and a review of the other 5 Spanish patients previously published. His initial symptoms were gynecomastia and libido decrease, with increase of plasmatic and urinary oestrogen levels, plasma testosterone near low normal level and a right adrenal gland tumour that, after its removal, showed a benign histology and was classified as an adrenocortical adenoma. Three years after, initial symptoms returned, with oestrogen, glucocorticoid and androgen hypersecretion, tumour local relapse and peritoneal, liver and lung metastasis. After mitotane and aminoglutethimide therapy, hormonal concentrations fell temporary and then raised again until his death 3 years later. The main special feature of this case is the apparently benign initial adrenal tumour with only oestrogen hypersecretion, and its relapse 3 years later with secretion of several steroid hormones, generalized metastasis and no response to medical therapy.