Endocrine Abstracts

Introduction: Pheochromocytoma (PHEO) is an adrenal medulla tumor secreting catecholamines. Malignancy is defined by the presence of metastases in non-chromaffin tissue. Its optimal management requires experienced multidisciplinary teams.We report the case of a patient followed for malignant pheochromocytoma

Comment: 58-year-old patient operated for a pheochromocytoma in 2016, then lost sight of. The evolution was marked 6 years later by the installation of right lumbar pain associated with a Menard triad evolving in a context of weight loss not figure. Clinical examination found right lumbar contact with tenderness on palpation, associated with swelling next to the left scapula. The dosage of urinary methoxylated derivatives shows a normetanephrine level twice the normal. The adrenal CT shows a right interhepato-renal lesional process invading the hepatic parenchyma measuring: 8.4 × 10.5 cm. As part of the extension assessment, a CTAP CT scan was performed showing an inter hepatorenal tissue mass invading the liver and the thoracic wall in favor of tumor recurrence with secondary pancreatic and bone localizations at the level of the femoral head, and lysis scapular bone. MIBG scintigraphy and PET FDG are not available The file was staffed in mulidisciplinary meeting, the therapeutic decision was to complete with palliative chemotherapy with VP 16 cisplatine A genetic study is requested in search of a mutation of the SDHB gene

Discussion: The prevalence of metastatic pheochromocytomas varies between 5 and 26%. Only the presence of lymph node or distant metastases affirms malignancy; histological examination does not provide certain information Secondary localizations are most often pulmonary, hepatic and bone.The diagnosis of recurrence can be made through MIBG scintigraphy despite a normal hormonal balance. Surgery is rarely curative, but tumor reduction or resection of metastases particularly reduces cardiovascular risk. Treatment with 131I-MIBG is an interesting alternative in the management of malignant pheochromocytomas when chemotherapy is indicated as first-line treatment in the event of an inoperable tumor or failure of metabolic radiotherapy.

Conclusion: Our case illustrates the importance of clinical, biological and even morphological monitoring of all patients operated for pheochromocytoma, which must be continued for a long time (15 years) given the possibility of late revelation of metastases. In spontaneous evolution, the 5-year survival is generally less than 50%. Due to the current absence of an effective treatment for malignant tumors, several palliative therapies allow tumor reduction and improved survival.