What determines mortality in malignant pheochromocytoma? – Report of a case with eighteen-year survival and review of the literature

Abstract

Pheochromocytoma (PCC) is a tumor derived from adrenomedullary chromaffin cells. Prognosis of malignant PCC is generally poor due to local recurrence or metastasis. We aim to report a case of malignant PCC with 18-year survival and discuss which factors may be related to mortality and long-term survival in malignant pheochromocytoma. The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors. Diagnosis of PCC was established biochemically and a tumor with infiltration of renal parenchyma was resected. No genetic mutation or copy number variations were identified in SDHB, SDHD, SDHC, MAX and VHL. Over 18 years, tumor progression was managed with 131I-MIBG (iodine-metaiodobenzylguanidine) and 177Lutetium-octreotate therapy. Currently, the patient is asymptomatic and presents sustained stable disease, despite the presence of lung, para-aortic lymph nodes and femoral metastases. Adequate response to treatment with control of tumor progression, absence of significant cardiovascular events and other neoplasms, and lack of mutations in the main predisposing genes reported so far may be factors possibly associated with the prolonged survival in this case. Early diagnosis and life-long follow-up in patients with malignant pheochromocytoma are known to be crucial in improving survival.

Pheochromocytoma

“…Pheos are rare tumors, with an annual incidence of 2 to 9.1 per 1 million adults and may correspond up to 60% of all adrenal incidentalomas (epinephromas) (Farrugia et al 2016) according to various studies (Kudva et al 1999;Mantero et al 2000;Harari and Inabnet 2011;Ramachandran and Rewari 2017;Andrade et al 2018). The majority are benign but up to 25% may be malignant (Dahia 2017).…”

Metastatic pheochromocytoma to the pancreas diagnosed by endoscopic ultrasound‐guided fine needle aspiration: A case report and review of literature

“…9 Malignant pheochromocytoma carries a poor prognosis due to local recurrence or metastasis, with 5-year survival rates ranging from 40% to 70% and a median survival of 7 years, though there are a few reports of survival to more than 15 years after diagnosis. 3 Pheochromocytomas are typically diagnosed by laboratory and imaging modalities. Increased levels of plasma or urinary catecholamines and their metabolites are essential to making the diagnosis.…”

“…3 The most common sites of metastases are lymph nodes, bone, liver, lung, and kidney. 3 The most common sites of metastases are lymph nodes, bone, liver, lung, and kidney.…”

“…1,2 Malignancy is defined by the presence of metastases, evidence of local invasion, or recurrence. 3 The most common sites of metastases are lymph nodes, bone, liver, lung, and kidney. 3,4 The use of fine needle aspiration (FNA) in diagnosing pheochromocytoma is controversial due to the potential risk of hemorrhage within the lesion, precipitating a hypertensive crisis; however, this complication occurs rarely.…”

“…3 The most common sites of metastases are lymph nodes, bone, liver, lung, and kidney. 3,4 The use of fine needle aspiration (FNA) in diagnosing pheochromocytoma is controversial due to the potential risk of hemorrhage within the lesion, precipitating a hypertensive crisis; however, this complication occurs rarely. 2 We report the first case in the literature of metastatic pheochromocytoma to the pancreas initially suspected after endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) and cytology.…”