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[A case of infantile virilizing adrenocortical tumor]
Y Nakagawa1, Y Hirao, S Tsujimoto
1Department of Urology, Nara Medical University.
Insights
This case report details an infantile virilizing adrenocortical tumor in a 16-month-old girl. Surgical removal of the left adrenal tumor was successful, confirming adrenocortical adenoma.
Area of Science:
- Pediatric Endocrinology
- Oncology
- Surgical Pathology
Background:
- Infantile virilizing adrenocortical tumors are rare neoplasms causing precocious pseudopuberty in children.
- Early diagnosis and surgical intervention are crucial for favorable outcomes.
Observation:
- A 16-month-old female presented with hirsutism, obesity, acne, and virilization.
- Elevated androstenedione, dehydroepiandrosterone-sulfate, cortisol, urinary 17-KS, and 17-OHCS were noted.
- Abdominal CT and adrenal scintigraphy identified a left adrenal tumor.
Findings:
- The patient underwent left adrenalectomy for a 5.0 x 4.5 x 3.7 cm adrenocortical adenoma.
- Histopathological examination confirmed the diagnosis of adrenocortical adenoma.
- The tumor was non-responsive to dexamethasone suppression and ACTH stimulation tests.
Implications:
- This case highlights the importance of considering adrenal tumors in the differential diagnosis of pediatric virilization.
- Successful surgical management of adrenocortical adenoma can reverse hormonal abnormalities and virilization symptoms.
- Further research into the genetic and molecular basis of these tumors may improve diagnostic and therapeutic strategies.
Abstract:
The patient was a 16-month-old girl, born by mature natural delivery and weighing 3,320 g. Hirsutism was noted on birth. Development of pubic hair and hypertrophy of the labia minora were noted after 8 months. At the time of admission, the height was 80 cm and body weight 14.5 kg. Systemic obesity, facial acne, systemic hirsutism, low pitched voice and hypertrophied clitoris were observed. Androstenedione, dehydroepiandrosterone-sulfate and cortisol showed high levels in the blood, and the urinary 17-KS was obviously high, along with an increase in urinary 17-OHCS. The subject did not respond to either the dexamethasone inhibition test or ACTH load test. The abdominal CT revealed a tumor in the front upper position of the left kidney, and adrenal scintigraphy disclosed an obvious accumulation image in the adrenal gland on the left side. Based on the diagnosis of a left adrenal tumor, left adrenalectomy was performed. The tumor measured 5.0 x 4.5 x 3.7 cm, and weighed 57 g. Histopathologically it was diagnosed as adrenocortical adenoma. The infantile virilizing adrenocortical tumor is reported together with some discussion of the literature.