Steroid Cell NOS in a Child: A Rare Case Report

Introduction: Steroid cell tumors are rare sex chord tumours of the ovary with malignant potential. Here we report a case of steroid cell NOS in a 12-year-old girl & similarity of the clinical presentation with non-classic congenital adrenal hyperplasia. Clinical Case: 12-yr girl came with complaints of hirsutism since many years and a recent change in voice. There was no past history suggestive of adrenal crisis. On examination she had muscular body habitus, moderate hirsutism (FG score 19), acne. SMR B1, P4, A3. Karyotype 46 XX. BA 13yrs. DHEAS- 144, serum cortisol 8am-14ug/dl. Testosterone-8.35ng/ml (normal 0.1-0.9ng/ml). serum 17 (OH) Progesterone basal-16.37 ng/ml(N-0.2-1.3ng/ml). Post synacthen 17OHP >35ng/ml, LH- <0.1, FSH- 0.2(prepubertal range). U/S-adrenals normal, right ovary was enlarged in size, 4x3cm cystic lesion. A CECT ABDOMEN showed Rt ovary -homogenously enhancing fairly well-defined solid lesion. Differential diagnosis was: NC/SVCAH with ovarian rest tumours vs ovarian virilising tumour. Since there was no non-invasive way of confirming between both the diagnosis, we gave a trial with hydrocortisone to see if her 17OHP reduces. After 1 month of therapy with hydrocortisone,17 OHP was still elevated -17.08ng/ml. Since there was no response, she underwent ovarian biopsy/excision. Right ovarian cystectomy was done. HPE showed -consider1. Steroid cell tumour NOS 2. Adrenal rest tumour. Note that these 2 tumours have identical morphology & IHC features. Followup:2 mon post-surgery: B5 P4 A3, mild facial hair,17 OHP - 1.53 ng/ml, Testosterone 2.5 ng/dl.12 months post-surgery child attained menarche had regular menstrual cycles, Serum 17OHP- 0.41 ng/ml, testosterone-11ng/dl. Conclusion: The usual differential diagnosis in 46 XX female children presenting with signs of androgen excess is CAH, it is not uncommon to find SVCAH presenting in childhood with symptoms & signs of virilisation. Clinically both SV CAH and Steroid cell tumours can present with hyperandrogenic features in the childhood. Amongst steroid cell tumours, 3 subtypes are seen, they include 1. stromal luteoma 2. Leydig cell tumour 3. steroid cell tumour NOS. The steroid cell tumours of the ovary secrete androstenedione, alpha hydroxy progesterone & testosterone .17OHP is raised in both the conditions. The HPE of both adrenal rest tumours & steroid cell NOS is similar & a clear-cut differentiation cannot be made. They are similar in clinical presentation, biochemistry & HPE. It is important to recognise that steroid cell tumour NOS is a differential to SVCAH in childhood. Since these tumours can occur at any age, clues to differentiate both conditions are-raised 17OHP but normal DHEAS & on imaging- normal adrenals with enlarged ovaries with or without solid cystic lesions in steroid cell NOS of the ovary.