PMON319 A Pure Androgen Secreting Adrenal - Cortical Tumor in a 13 Year Old Female

Abstract

Abstract Background Adrenal cortical adenomas are exceedingly rare representing 0.2% of pediatric malignancies and often present with signs of Cushing syndrome, virilization and mixed elevated hormones.[1] We present a rare case of a virilizing androgen only secreting adrenal cortical adenoma with resolution after surgical removal. Clinical Case A 13-year-old female was referred to endocrine clinic for 1 year history of deep masculine voice, hirsutism and elevated DHEA-S levels. She also reported facial hair requiring removal every 3 to 4 days, back hair, acne, and dull aching back pain. Pubertal changes began at age 9 with breast development and menarche at age 11 followed by amenorrhea. On physical exam, she had a significantly deep masculine voice, axillary hair, female genitalia with prominent clitoris, Tanner Stage IV pubic hair and Stage III breast development. Blood pressure was within normal limits and weight was historically consistent in the 90th percentile. Initial labs were significant for elevated 17-Hydroxypregnenolone 353 ng/dL (ref: 44-235), Androstenedione LCMS 903 ng/dL (ref: 50-170), Testosterone 100 ng/dL (ref: 15-35), DHEA 5460 ng/dL (ref: <202) and DHEA-S >1000 ug/dL (ref: 67.8–328.6). LH, FSH, Estradiol, Cortisol LCMS, and Chromogranin A were within normal limits for her sex and age. Further work up ruled out pheochromocytoma, paraganglioma, Cushing's syndrome and congenital adrenal hyperplasia. Magnetic resonance imaging revealed a well-circumscribed 5.5×5.8×6.4 cm cystic and solid left adrenal mass and displacement of the left kidney. She was referred to endocrine oncology and surgery specialists. Genetic testing showed absence of TP53 involvement. Patient underwent open left adrenalectomy, and remained hospitalized for 3 days in recovery. The surgical pathology identified an adrenal cortical adenoma Stage II (pT2, pN0, cM0) measuring 8.7×6.0×3.3cm with slight increase of ki67 index at 3.7% (average 8044 nuclei counted) and hot spot with 4.6% (1266 nuclei counted). Follow up revealed resumption of regular periods, improvement in acne and male-pattern hair growth, and voice becoming less deep beginning 3 months post-operatively. DHEA-S returned to normal ranges at 220 ug/dL (ref: 67.8–328.6). Patient did well post-operatively except for occasional sharp back pain. Discussion Rare amongst adrenal tumors are isolated androgen-secreting sub types. To our knowledge, this is one of the first documented cases of a pure androgen secreting adrenal cortical tumor without symptoms of Cushing's syndrome in the pediatric population.