Mortality in patients with Pheochromocytoma: a population-based study 1977-2016

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Background: Pheochromocytomas and catecholamine-secreting paragangliomas (PPGL) are rare catecholamine-producing tumors. Due to the rarity, limited data on prognosis exists and data are mainly from tertiary centers with potential referral bias. Here, we present population-based mortality data over an observation period of 40 years.

Materials and methods: We identified a cohort of 198 PPGL patients diagnosed 1 January 1977 to 31 December 2016 in North and Central Denmark (population 1.75 million). Data on tumor size, tumor location, surgery and recurrence were obtained from health records. Date of death or emigration was obtained from the Civil Registration System. Hazard rate-ratios (HRR) for death or tumor recurrence were calculated using Cox regression and adjusted for sex and age at diagnosis.

Results: Radically operated (n=162, 81.8%) patients (median age 52.0 years (Q1–Q3: 39.3–63.0)) had an overall 5- and 10-year survival after surgery of 90.8% (CI95%: 84.5–94.6) and 78.7% (CI95%: 69.8–85.2) and a recurrence-free 5- and 10-year survival of 87.0% (CI95%: 80.1–91.7) and 75.7% (CI95%: 66.7–82.7), respectively. HRR for death or recurrence was not associated with tumor size or with extra-adrenal location of tumor. Twelve (6.1%) PPGL patients did not undergo radical surgery as they either died before surgery (n=2), had disseminated disease (n=4), abstained from surgery (n=4) or since radical surgery was not technically possible (n=2). For these patients, median time from diagnosis to death was 5.3 years (Q1–Q3: 0.3–16.4). Twenty-four (12.1%) PPGL patients were diagnosed at autopsy. Based on autopsy reports, PPGL was regarded the underlying cause of death in 5 (20.8%) patients, a contributing cause in 14 (58.3%) patients and an incidental finding in 5 (20.8%) patients.

Conclusion: PPGL patients who undergo radical surgery have a good long-term prognosis with three-fourths of patients living more than ten years after surgery. Those who are not radically operated are a heterogeneous patient group with a relatively short life expectancy. This, combined with the fact that PPGL was considered a direct or contributing cause of death in many patients diagnosed at autopsy, underlines the importance of early diagnosis and treatment of PPGL.
Original languageEnglish
JournalEndocrine Abstracts
Volume56
ISSN1470-3947
DOIs
StatePublished - 22 May 2018

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