Endocrine Abstracts

Introduction: SACT are rare in childhood and present with variable signs depending on the type of hormone excess. We describe the unusual presentation of a teenager with SAT presenting with slow progressing puberty.

Methods: A pre-menarchal 13.5 years old girl with high BMI (28 kg/m²) presented with slow progressing puberty. She started her puberty at least 3 years previously with breast changes, then progressed to develop pubic and axillary hair over 2 years before presentation. Her Tanner staging for puberty was B4,P4 without virilisation, acne and cushingoid features. There was a strong family history of PCOS and Type2 Diabetes. Investigations were done to rule out PCOS.

Results: Blood investigations showed raised levels of testosterone (3.6 nmol/l) and DHEAS (27 μmol/l). Abdominal US and MRI scan showed the presence of a left sided adrenal mass. 24 h urinary profile was suggestive of a SACT. The adrenal mass was removed surgically with intact capsule. Tumour size 125×95×75 mm, weight 585 g. Histology showed uncertain malignant potential with the presence of three potentially malignant absolute histological criteria (size/weight/presence of necrosis). She had a mild menstrual bleeding 1 month after surgery without further menses. PCOS was confirmed biochemically and by ovarian US morphology at the age of 15.5. At 2 year follow-up, there were no biochemical/radiological evidence of tumour relapse. She has recently developed glucose intolerance and started on Metformin. Current BMI is 27.4 kg/m².

Discussion: SACT is one of the most aggressive endocrine tumour with a poor prognosis. Distinction between adenoma and carcinoma is difficult and relies on tumour size, imaging and histopathological criteria. An early diagnosis of SACT is crucial but often delayed because of atypical presentation. Alertness should be maintained in using virilisation as a clinical criteria to exclude SACT when suspecting PCOS.