Malignant pheochromocytoma: Hepatectomy for liver metastases

Abstract

Malignant pheochromocytoma accounts for approximately 10% of pheochromocytoma cases. The main site of distant metastasis is the liver. Hypertensive crisis due to catecholamine oversecretion is potentially fatal. We present a case of malignant pheochromocytoma with multiple liver metastases. A 60-year-old female with repeated hypertensive episodes was diagnosed with malignant pheochromocytoma. She underwent a left adrenalectomy and partial hepatectomy with resection of segment 6. Catecholamine levels remained high after surgery and she received repeated cycles of chemotherapy. Four months after surgery, multiple liver metastases were detected. In spite of ongoing chemotherapy, catecholamine levels eventually became uncontrollable. Serum and urine noradrenaline and vanillylmandelic acid levels increased, but adrenaline and dopamine levels stayed within the normal range. Preoperative liver imaging revealed multiple metastases in all segments except segment 4. Percutaneous transhepatic portal vein embolization (PTPE) of the right and lateral branches of the portal vein was performed. The functional liver volume of segment 4 increased after PTPE. Right hepatectomy, lateral segmentectomy and partial resection of segment 1 were performed 10 mo after the initial surgery. Intraoperative ultrasonography detected two small tumors in segment 4, which were treated with intraoperative microwave coagulation therapy. Noradrenaline levels normalized immediately after the second hepatectomy. As there was increased telomerase activity in the resected specimen, she received adjuvant chemotherapy. She remained in good health for 2 years. However, further metastases eventually occurred and she subsequently died due to a brain hemorrhage. Hepatectomy may be a therapeutic option for reduction of tumor mass in pheochromocytoma with liver metastases.

Malignant Pheochromocytoma With Intravascular Extension to the Heart

“…Treatment involves surgical removal of the primary tumor. In malignant cases, different therapeutic techniques are employed to reduce tumor mass in metastatic locations, including hepatectomy for hepatic metastasis, 20 tumor embolization, and use of radionuclide agents. For cases that involve the IVC, a thoracolaparotomy has been performed for partial resection of the IVC with successful results.…”

Synchronous liver metastasis at initial diagnosis of adrenal pheochromocytoma by CT: A case report

“…Long-term hypertension can cause significant damage to vital organs such as the heart, brain and kidneys, and sudden severe hypertension may also lead to pheochromocytoma crisis, which is life threatening. Malignant pheochromocytoma accounts for ~10-15% of all pheochromocytoma cases (5). Distinguishing between benign and malignant tumors using histopathological methods is often challenging.…”

Incidentally Detected Inoperable Malignant Pheochromocytoma with Hepatic Metastasis Treated by Transcatheter Arterial Chemoembolization

“…Even if a PCC is initially diagnosed as benign on the basis of histopathological findings, it is possible for metastasis to occur in other organs after resection [ 2 ]. Malignant PCC accounts for approximately 10% to 15% of all cases of PCC [ 3 ]. The standard treatments for malignant PCC include surgical resection, radiotherapy, local ablation, chemotherapy, radionuclide therapy (e.g., metaiodobenzylguanidine [MIBG]) and molecular targeted therapy.…”

“…Accordingly, some patients are overtreated, while others are undertreated. Despite the liver being the second most frequent site of distant metastasis [ 3 ], reported cases of liver metastasis in PCC are relatively rare [ 2 ]. Additional case reviews and long-term studies are necessary to reach a consensus on the most appropriate treatment strategy for these lesions.…”