PJEOCHROMOCYTOME MALIN : A PROPOS DE 3 OBSERVATIONS CLINIQUES ET REVUE DE LA LITTERATURE

Abstract

Malignant pheochromocytoma is a rare and aggressive tumor of chromaffin cells, often revealed by metastases. The objective of this work is to better understand this pathology through the analysis of clinical cases, describing its diagnostic, evolutionary, and therapeutic aspects, and confronting them with data from the literature to improve its management and prognosis. Patients and Methods: Three clinical cases of malignant pheochromocytoma managed in endocrinology department in University hospital of Rabat were studied. The analysis was based on clinical, biological, morphological, histopathological, and genetic data. Results and Discussion: The reported cases illustrate the clinical variability of malignant pheochromocytomas, ranging from forms discovered due to paroxysmal hypertension to slowly progressing metastases. The results confirm: • The diagnostic difficulty of malignancy as no histological criterion is definitive. • The major role of genetics, particularly SDHB mutations, which are associated with a high risk of malignancy. • Functional imaging (MIBG, 18F-FDOPA PET, Ga-68 DOTATATE) as tools for localization and follow-up. • Therapeutic options are multimodal: surgery when possible, radiometabolic therapy, chemotherapy (CVD, temozolomide) and targeted therapies (. • Prognosis is heterogeneous: 50% of metastatic patients live beyond 5 years -as was the case in our 3rd patient-, some progress rapidly –as observed in two of our patients- (death within 4-5 years), while others survive over 20 years without cytotoxic treatment. Conclusion: Malignant pheochromocytoma is a rare and complex entity posing diagnostic and therapeutic challenges. Genetics plays a central role in screening, prognosis, and follow-up. Future perspectives rely on biomarkers, targeted therapies, and nuclear medicine to improve survival and quality of life for patient