Endocrine Abstracts

Introduction: Adrenocortical carcioma, is an aggressive cancer originating in the cortex of the adrenal gland. Adrenocortical carcinoma is a rare tumor, in United State <25 new cases of adrenocortical tumours (benign and malignant) are diagnosed annually (0.1–0.4 cases/million per year) and malignant adrenal tumours comprise ~1% of all carcinomas diagnosed prior to 20 years of age. Adrenocortical carcinoma has a bimodal distribution by age, with cases clustering in children under five, and in adults 30–40 years old. Adrenocortical carcinoma is remarkable for the many hormonal syndromes which can occur in patients with steroid hormone-producing (‘functional’) tumors, including Cushing’s syndrome, Conn syndrome, virilization, and feminization. Because of the relative rarity of these tumors, little is known about their cause and the influence of genetic factors, adrenocortical carcinomas are associated with numerous constitutional syndromes, including Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Carney complex, multiple endocrine neoplasia 1, and hemihypertrophy syndrome. The incidence is associated with a mutation in the P53 gene.

Case report: A 8.5-year-old boy evaluated in our clinic for excessive pubic hair (pubarche), his condition dated back to 1 year ago. On examination: he looks well, normal blood pressure, the height 121 cm (above 50th percentile for the family target), weight 30 kg (75th percentile). Tanner stages for pubic hair, penile size and testes were Iv, Iv, II respectively (Fig. 1A and B). No palpable abdominal mass, the rest of his physical examination unremarkable. Laboratory findings: FSH 0.10 IU/l normal for stage Iv (1.4–15.4), LH 0.15 IU/l normal (1.2–7.8), testosterone 8 nmol/l normal (0.10–1.04), 17 hydroxyprogesterone 2.1 ng/ml normal (0.2–3.5), cortisol level 346 mmol/l normal (168–728). Thyroid function tests, renal function tests and serum electrolytes tests were all normal. Medical imaging data: bone age 11 years, abdominal ultrasonography revealed well defined mass at right suprarenal region heterogeneous texture with some calcification measures (49×48) mm, abdominal magnetic resonance revealed well defined mass at right suprarenal region measures about (5×5) cm (Fig. 2A and B) Interpretation was adrenal carcinoma or adenoma. Dynamic computed tomography of adrenal glands revealed: right adrenal mass (5×4.4) cm well defined, heterogeneous, associated with perilesional vessels, no invasion to surrounding structures or lymph nodes without distant metastasis in the visualized area. Interpretation: most probably adrenocortical carcinoma. Brain magnetic resonance imaging was normal. Scrotal ultrasonography revealed both testes slightly enlarged in size with normal echogenesity. Chest X-ray normal. Based on these clinical, imaging data and laboratory tests, our diagnosis after consulting the radiologist, paediatric surgeon and paediatric oncologist was adrenal androgen-secreting tumour either adenoma or carcinoma, surgical management was decided and the patient underwent right open adrenaloectomy at 14 April 2012. Pathology report revealed grossly single piece measuring 5×5×4 cm, soft yellow cut section with hemorrhagic foci. Microscopically section shows malignant tumour compose of atypical cells with dense compact eosinophilic cytoplasm, some cells have intranuclear inclusion, capsular invasion, sinusoidal invasion, focal clear cell component and fibrous brands (Fig. 3A and B). The histological picture is consistent with adrenocortical carcinoma, confirmed by immunohistochemistry (epithelial membrane antigen and cytokeratin both positive). Tumour staging: stage I; can removed completely. Post-operatively: serum cortisol 0.500 nmol/l for morning and evening (N: 171–536), so he put on hydrocortisone replacement therapy, and he was scheduled for follow-up examination monthly for the first 2 years, scanning every 3 months for the first 2 years, 4 months for the next 2 years and every 6 months during the 5th year.

Conclusion: To our knowledge, this is the first case report of virilizing adrenocortical carcinoma with unique pathologic findings presented with peripheral precocious puberty in our country in this age group. Three years ago, we submitted a project to establish a center of excellence for the specialty of the paediatric endocrinology and diabetes in Iraq to Minister of Health. as a project for change management after our clinical attachment course in UK for two months, and we gained the agreement for that. Now it is a center for teaching, training of postgraduate medical students, referral center for management and consultation of paediatric endocrine diseases, diabetes and sex disorders. Our case is one example for that, this considered a success especially in a developing country suffered and still suffers from wars, terrorism and violence.