Endocrine Abstracts

Introduction: The composite pheochromocytoma is a rare adrenal medullatumour that combines a pheochromocytome and a neuroblastic tumour. We present the case of a composite phenochromocytoma discovered in a patient with neurofibromatosis type 1.

Case report: Patient aged 64 years referred to our department by his dermatologist for the exploration of a left adrenal mass discovered during the practice of a CT scan and then an 18F-FDG MRImade as part of an exploration of his neurofibromatosis. The patient hada high blood pressure and neurofibromatosis type 1 for which he is followed since 1995 The radiological characteristics at the 18F-FDG MRIwas in favor of a moderately hyper-metabolic formation fixing to SUV max 3.1 of the left adrenal of 18 × 12 mm and then the Adrenal-centered scanner was in favor of a left adrenal nodule that has a spontaneous density, an absolute and relative wash against the diagnosis of adenoma. In biology we noticed a slight rise in urinary and plasma metadrenaline on two successive samples. The patient was operated (left adrenalectomy) because we consideredthe mass as a pheochromocytoma. He had simple surgical suites with the need to stop antihypertensive treatment. Anatomopathological examination was in favour of a composite phenochromocytoma with a ganglioneuroma with no sign of malignancy

Discussion: The largest series of composite pheochromocytoma studied was published by Gupta et al., it included a single case of neurofibromatosis, the mean age atdiagnosis was 67years,and the average size was 5.2 cm. The majority were functionnaly activewith a moderate elevation of catecholamines. This published case is similar to our patient in clinical and biological characteristics.

Conclusion: Pheochromocytoma, as part of a neurofibromatosis, would have more specific characteristics that need to be better studied in order to refine the management of this chronic disease.