Unusual Evolution of Bilateral Neuroblastoma

Introduction: Bilateral neuroblastoma is extremely rare. To date, only 60 cases are reported in the literature. The association of neuroblastoma with paraneoplastic Cushing's syndrome is exceptional. The average age at diagnosis is 3 months. The majority of patients are classified according to INSS stage IVs. The prognosis is generally favorable with 90% survival at 5 years. The treatment is similar to unilateral neuroblastoma. Case Report: 3 months girl, admitted for excessive weight gain with bluish skin nodules. These symptoms started on the 40th day of life. There were no gastrointestinal symptoms, nor exophthalmia, obesity with a weight of 7.7 kg, the face puffy with telangiectasias, arterial hypertension (15/08cmHg) and hepatomegaly at 6 cm below the costal margin. Abdominal ultrasound showed a right adrenal mass of 7.7 / 9 cm heterogeneous structure, second adrenal mass was found on the left side with the same characteristics. The liver is nodular and increased size. The rest of the radiological assessment was normal (thorax and skeleton). The bone marrow smear was normal. The level of cortisol was high at 8pm, urinary catecholamine were normals, and biopsy of subcutaous nodules showed a malignant tumor proliferation suggesting at first around cell neuroblastoma or PNET. Research NMYC amplification was not done. The diagnosis of bilateral neuroblastoma with liver metastasis associated with Cushing's syndroma is retained. We had proceeded with resection at the left side and partial at the right one. The child is placed on antihypertensive therapy and ultrasound monitoring. The short-term trend showed a decline in blood pressure with decreased volume of subcutaneous nodules and weight. After 8 months, the patient has presented a cerebral metastasis and died. Conclusion: The bilateral nature and association with Cushing's syndrome does not seem to have a negative impact on prognosis neuroblastoma. Frequently reported spontaneous regression in neuroblastoma stage IVs is observed in the case reported here; but the cerebral complication wasn't expected in such cases.