Retrospective Evaluation of The Cases with Malignant Pheochromocytoma: A Single Center Experience

Abstract

IIntroduction The aim of this study was to evaluate the clinicodemographic factors and features of tumors in patients with malignant pheochromocytoma. Methods: We retrospectively evaluated the data of 5 cases admitted to our department between 2013 and 2020 and diagnosed with malignant pheochromocytoma. Results: The patients included three females with mean age of 45 years at presentation. Three cases were diagnosed with pheochromocytoma after hypertensive attack and two cases were diagnosed after detection of adrenal mass. The average diameter of the adrenal mass of the cases was 58 mm. Extesion to periadrenal adipose tissue was detected in two patients, vascular invasion in one patient, and capsule invasion in 1 patient in the surgery-resected specimen. Intraabdominal lymph node metastasis was found in 1 patient and metastatic focus was seen in the liver in 1 patient at the time of the diagnosis. During follow-up, perirenal region metastasis was detected in 1 patient. Bone metastasis was seen in 3 patients, lymph node metastasis in 2 patients and lung metastasis in 1 patient. Transperitoneal surrenalectomy was performed in all cases. Lutetium-177, radiotherapy and surgery were the preferred adjuvant therapies. Three patients died because of progression of the disease or acute complications and the other 2 patients are still under follow-up in our clinic. Conclusion: Malignant pheochromocytoma is rare and definitive criteria for malignancy is not established. Prognosis is poor and there is no curative treatment. Resection of malignant pheochromocytoma with intent to cure, which may improve symptoms and possibly survival is important.