Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery

Abstract

Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla. Malignant pheochromocytoma is defined as the presence of metastatic spread in tissues where chromaffin cells are not usually present. This case report describes the case of a relapsed malignant pheochromocytoma, spread to the right liver lobe, superior pole of the right kidney, posterior right hemidiaphragm, right hemidiaphragmatic pillar, inferior vena cava, and regional lymph nodes. After evaluation, an extensive surgery was performed, with resection of all the affected tissues and regional lymphadenectomy. No adjuvant treatment (radiotherapy or chemotherapy) was given. Complete clinical, biochemical, and radiological remission was achieved, with normalisation of metanephrine and normetanephrine. To date, six years after surgery, the patient remains asymptomatic and normotensive without taking any antihypertensive medication. We conclude that the therapeutic approach should be individualized in the case of metastatic pheochromocytoma. Extensive surgery can be considered as a treatment option, even in the case of multiple metastases, as it may be able to achieve complete remission of the disease, avoiding costly and potentially dangerous adjuvant therapies.

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

“…Moreover, several studies have shown that surgical resection of the primary tumor is associated with improved survival even with metastatic disease [104,106,107]. In addition, complete metastatic surgery may be considered in oligo-metastatic PCC/PGL on a case-by-case decision, although there is only little evidence for such an approach from single case reports [108,109].…”

Epic battles in endocrinology — malignant pheochromocytoma: a case report

“…Although surgical resection is the only potentially curative approach, it is rarely feasible due to delayed diagnosis of malignancy. In some cases metastasectomy proved to be a sufficient treatment, leading to complete remission and patients remaining asymptomatic for years [18,19]. Our patient underwent multiple debulking surgeries which, together with long-term lanreotide treatment, enabled the almost six-year follow-up that she achieved.…”

Systematic treatment based on evidence for malignant pheochromocytoma: one case report and Literature review

“…It's estimated annual incidence is about 8 per 100,000 people a year [5]. The highest incidence is in the age of 40-50 with no significant different between males and females [6].…”