ESPE Abstracts (2016) 86 P-P1-734

ESPE2016 Poster Presentations Pituitary and Neuroendocrinology P1 (36 abstracts)

Pseudopuberty in a Young Girl with Adrenocortical Carcinoma During Mitotane Therapy

Romana Marini a , Debora De Pasquale b , Carla Bizzarri a , Elisa Santoro a , Raffaele Cozza b & Marco Cappa a


aDepartment of Endocrinology and Diabetes, Bambino Gesù Children’s Hospital, Rome, Italy; bDepartment of Oncology and Hematology, Bambino Gesù Children’s Hospital, Rome, Italy

Background: The Adrenocortical Neoplasm (ACN) is a rare condition in childhood (0,3 cases/1000000). In paediatric age, ACN at stage 1 is treated by complete adrenalectomy, while at stages 2 and 3 surgery is followed by adjuvant treatment with Mitotane (M). Chemotherapy is required in metastatic cases (stage 4).

Objective and hypotheses: M is an adrenal cytotoxic agent which has both adrenolytic action on ACN cells and inhibition on steroid hormone synthesis apparently without cellular destruction. Furthermore M it seems to modify the peripheral metabolism of steroid. It was been hypothesized that the M may also have a partial suppressive effect on pituitary ACTH-secreting cells. Gynecomastia (G) was described as side effect of M in men and in one little boy. It was associated with increased binding capacity of SHBG in the plasma compartment modulating hormonal disposal for target cells.

Method: In the 2008–2016 period we observed 8 patients affected by ACN (3 males and 5 females, aged 0–32 months at diagnosis). Histologic results: malignant lesion in 5 cases, neoplasm with uncertain behaviour in 2 cases, and adenoma in 1 patient. All children affected by malignant or uncertain lesions underwent surgery, followed by M adjuvant treatment. One child was also treated with chemotherapy due to advanced disease (stage 4).

Results: We report a young girl, affected by ACN with uncertain behaviour (stage 2), who, at the age of 2.5 years, underwent M therapy developing adrenal insufficiency, treated with hydrocortisone, and progressive telarche. The full hormonal testing (LH-RH Test, basal 17-Beta-estradiol, progesterone, adrenocortical hormones and their precursors) showed a normal pre-pubertal panel. The telarche completely reverted after the stop of M therapy (2 gr/m2 per daily for 1 year).

Conclusion: To our knowledge this is the first reported case of abnormal breast development in a young female child treated with Mitotane for adrenocortical neoplasm.

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