Delayed Diagnosis and False Relapse Due to Paternal Testosterone Use in Adrenocortical Carcinoma

Abstract

The prognosis of pediatric adrenocortical carcinoma often depends on prompt diagnosis to begin treatment before metastatic progression. We discuss a girl who presented at 8 months of age with virilization, which was thought to be due to exposure to a topical testosterone preparation being used by her father. Her testosterone level did not decrease promptly after her father discontinued the medication, however, and when she followed up with signs of Cushing syndrome 5 months later, metastatic adrenocortical carcinoma was diagnosed. The patient was successfully treated with surgery and multiagent chemotherapy. Nine months after the end of treatment, her testosterone level was again found to be elevated. Testosterone precursors were now absent, however, and there were no imaging signs of recurrence. Further history showed that her father had restarted topical testosterone, and this time, exogenous exposure was correctly diagnosed. As use of topical testosterone becomes more prevalent, exogenous exposure must be considered in the differential diagnosis of childhood virilization. Any persistent testosterone elevation after exposure ceases or signs of hypercortisolism, however, are inconsistent with this diagnosis. We believe that the risk-benefit ratio favors abdominal ultrasound to rule out malignancy in all children presenting with virilization.

Pubertal outcome in a female with virilizing adrenocortical carcinoma

“…This included assessment for defects in adrenal steroidogenesis causing adrenal hyperplasia or for adrenal tumors. Exogenous administration of androgen compounds was ruled out by history and by elevation of testosterone precursors (11). …”

Pubertad precoz periférica por transferencia interpersonal de testosterona

“…Lo anterior sugiere la necesidad de realizar más estudios, cuando los niveles de testosterona no disminuyen después de suspender la administración exógena. 10 La Administración de Alimentos y Medicamentos (FDA, por sus siglas en inglés) de Estados Unidos de América en el año 2009 sugirió colocar una etiqueta de advertencia en los productos que contienen testosterona; sin embargo, la mayoría de los casos reportados de transferencia no intencional de testosterona a niños, ocurrieron porque los adultos no revisaron los etiquetados y olvidan lavarse las manos o cubrir el área de la piel donde se aplicó el producto. 5…”

A review of adverse events in animals and children after secondary exposure to transdermal hormone‐containing medicinal products

“…The clinical signs persisted despite discontinuation of the TTCP and an adrenocortical carcinoma was subsequently diagnosed. 40 …”

“…The clinical signs persisted despite discontinuation of the TTCP and an adrenocortical carcinoma was subsequently diagnosed. 40 It is important to recognise that secondary exposure to THCPs can have wider impacts within households. In one report, which was retrieved from EVDAS, two young boys were virilised following exposure to the father's TTCP.…”

“…Initially, the clinical signs in the child had been associated with secondary exposure to a TTCP being used by her father. The clinical signs persisted despite discontinuation of the TTCP and an adrenocortical carcinoma was subsequently diagnosed 40 …”