Pubertal outcome in a female with virilizing adrenocortical carcinoma

Abstract

Adrenocortical tumors are neoplasms that rarely occur in pediatric patients. Adrenocortical carcinoma (ACC) is even more uncommon, and is an aggressive malignancy with 5-year survival of 55% in a registry series. There is a lack of information on long-term endocrine outcome in survivors. We describe a 10-year follow-up in a patient who presented at 3 years 5 months with a 1-year history of axillary odor and 6 months’ history of pubic hair development with an increased clitoral size. Androgen levels were increased and a pelvic sonogram revealed a suprarenal mass of the left kidney. The tumor was successfully removed. At 6 years 11 months, androgen levels increased again. Workup for tumor recurrence was negative and the findings likely represented early adrenarche. The patient had menarche at an appropriate time and attained a height appropriate for her family.

Gonadotropin-dependent pubertal disorders are common in patients with virilizing adrenocortical tumors in childhood

“…This predominance may explain why the majority of patients in this cohort had normal (79.3%) or tall stature (17.5%) and advanced BA (63.5%) at diagnosis of ACT, although most had M-ACT. This finding is in accordance with previous reports (18, 19, 24, 30, 31, 32, 34, 42, 43, 47). In addition, the combination of androgen and glucocorticoid effects may have accounted for the lack of difference in initial stature SDS, BMI SDS and Δ BA between V-ACT and M-ACT.…”

“…Despite the acceleration of linear growth and skeletal maturation at diagnosis of ACT, there was a reduction of stature SDS from initial to final height, and the majority of patients (17 out of 19) reached normal FH. These results reinforce that growth tends to return to the patient’s predetermined channel after successful removal of an androgen-secreting ACT (47), with good prognosis on FH (18, 19, 30, 32).…”

“…Although morbidity (metastasis, recurrence and complications of ACT treatment) and mortality in patients with ACTs are well documented (17, 19, 23, 24), the late effects of early exposure to androgen excess on gonadotropin-dependent puberty and on growth have been scarcely presented in case reports (30, 31, 32, 33), in small series of patients (18, 34) or in studies with other purposes (19, 24).…”

Erythrocytosis as the first manifestation of adrenal carcinoma

“…In females, severe hyperandrogenism causes virilization and hirsutism. In both sexes, it induces pseudopuberty and growth accelerations followed by growth arrest, muscle hypertrophy, deepening of the voice, and polycythemia . Data in the literature about the association between severe hyperandrogenism and erythrocytosis are scanty overall, and completely lacking in children .…”

“…In both sexes, it induces pseudopuberty and growth accelerations followed by growth arrest, muscle hypertrophy, deepening of the voice, and polycythemia. [10][11][12] Data in the literature about the association between severe hyperandrogenism and erythrocytosis are scanty overall, and completely lacking in children. 12 Previous studies suggested that testosterone stimulates hematopoiesis in bone marrow stem cells and increases iron absorption at the intestinal level and its incorporation into erythrocytes.…”