Introduction:

Adrenal tumors in children are rare, and adrenal carcinoma accounts for less than 10% of cases. The typical manifestation is precocious puberty.

Case description:

An older female infant presented with acute gastroenteritis; she had a remote history of increased abdominal diameter and pubic, facial, and abdominal hair growth. Physical examination revealed a distended abdomen and a palpable mass in the left lower quadrant, approximately 15 cm in diameter, with a firm, elastic consistency, regular borders, non-mobile, and tender to palpation. Bowel sounds were present. Abdominal and pelvic CT scan: The left adrenal gland showed an expansive process with soft tissue density measuring 103 x 95 x 111 mm. Laboratory results: Cortisol 43.53 µg/dL, DHEA >3000 µg/dL, Testosterone 2191 µg/dL. Surgical treatment: Excision of the tumor mass. Pathology: High-grade adrenal cortex carcinoma, 14 cm, 616 grams. TNM pT2 pN0.

Discussion:

Pediatric adrenocortical carcinoma is a rare and mostly functional neoplasm. It often presents with signs of virilization and, to a lesser extent, hypercortisolism; both conditions can coexist, as in our patient. Computed tomography is key for diagnosis and staging, revealing tumors generally larger than 6 cm and with heterogeneous morphology. The treatment of choice is complete surgical resection, the main determinant of prognosis. We present the case of a girl with an adrenal tumor larger than 10 cm, managed with complete excision and histologically confirmed as high-grade adrenocortical carcinoma.

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