Endocrine Abstracts

Introduction: Pheochromocytoma represents only 5% of adrenal incidentalomas. It is malignant in only 10% of cases. We report a case of pheochrompcytoma with suspucion of double cortico-meullary component or an ectopic secretion of CRH in the context of neurofibromatosis type1

Observation: A26year old woman with a history of unilateral renal lithiasis, colic and breast neoplasia, with no notable personal history, consulted for right lumbar pain. An abdominal CT scan showed a solidocystic right adrenal incidentaloma with a spontaneous density of 40 HU measuring 79×75 mm and a slightly irregular contour. The fleshy portion had an absolute washout of 33%. It displaces the liver above and the upper pole of the kidney below. It displaces the inferior vena cava and the right renal vein medially. The left adrenal gland is unremarkable. Pheochromocytoma was suspected in the presence of paroxysmal malaise with headaches, palpitations, and weight loss. Her BMI was of 25 kg/m². ambulatory blood pressure measurement had shown blood pressure paroxysms with concomitant tachycardia In addition, she had café au lait spots, axillary lentigines and cutaneous and subcutaneous neurofibromas. However, she had no signs of hypercorticism or recent virilisation. Hotmonal exploration concluded to a mixed secretion of catecholamin and cortisol. ACTH was 34.8 pg/ml. 17 OH progesterone and SDHEA levels were normal fter confirming the diagnosis of pheochromocytoma, a thoracic-abdominal-pelvic CT scan did not reveal any metastases or secondary locations. Indeed, our patient neurofibromatosis type 1 and Slit lamp examination revealed lish nodules. she had no flush syndrome and Calcitonin was normal. She underwent a right adrenalectomy by right laparotomy The anapatomopathological examination showed a pheochromocytoma of 10 cm with a PASS score of 5 with cystic and haemorrhagic changes without necrosis nor capsule infiltrationThe immunohistochemical study was strongly positive for chromogranin, and negative for ACTH, but not unavailable for CRH. At 2 months postoperatively, normalization of Metanephrine and Normetanephrine levels as well as a positive minute braking were noted. ACTH was normal and The CT scan performed showed postoperative remodelling of the right adrenal gland without signs of recurrence.

Conclusion: Mixed corticomedullary tumour (MCMT) of the adrenal gland is an extremely rare tumour characterised by a mixture of steroidogenic and chromaffin cells in a single tumour mass. It is associated with ectopic adrenocorticotropic hormone in some cases. Current theories regarding the pathogenesis of MCMT should be further investigated by genetic testing