Ovarian steroid cell tumour (NOS) causing Cushing's syndrome in an extremely young girl: A case report

Steroid cell tumours are rare type of sex cord-stromal tumour of the ovary, with the average age at diagnosis during the fourth decade of life. A 2-year-old girl presented with virilisation, precocious pseudopuberty and Cushing syndrome. Her morning cortisol level was elevated and there was left lower abdominal mass found in radiological examination. An explorative laparotomy followed by a left salpingo-oophorectomy was performed. Histopathological examination revealed an encapsulated solid yellow-tan tumour composed of polygonal cells arranged in nests/lobes, containing numerous vascular channels, with abundant, clear-to-granular eosinophilic cytoplasm, severe nuclear pleomorphism and no Reinke crystals present consistent with an ovarian steroid cell tumour (NOS). The tumours cells were diffusely positive with calretinin, weak focal positive for inhibin, melan-A, pankeratin, chromogranin and negative for AFP, HMB-45 and androgen receptors. Following the tumour removal, blood pressure regressed to normal. The patient was discharged and advised for outpatient care.