Pheochromocytoma diagnosed pathologically with previous negative serum markers

J Surg Oncol. 2015 Oct;112(5):492-5. doi: 10.1002/jso.24031. Epub 2015 Sep 18.

Abstract

Background and objectives: Patients presenting with adrenal masses require workup with catecholamine or metabolite measurements to rule out pheochromocytoma. There is a select portion of patients with marker negative pheochromocytoma. The aim of this study is to compare patient characteristics and presentations between marker positive and marker negative tumors.

Methods: We performed an IRB-approved retrospective chart review of 88 cases of pheochromocytoma excised at our institution from 1995 to 2013. We considered any abnormal elevation in diagnostic test to be marker-positive.

Results: Seventy-eight cases had laboratory results available. Among these, seven had no elevations in laboratory testing. There was no difference in age or tumor size, but marker-negative patients had higher BMI than marker-positive patients. Marker negative patients were more likely to present with vertigo/dizziness (P = 0.003). Neither was more likely to have a genetic syndrome associated with risk of pheochromocytoma.

Conclusions: Marker-negative pheochromocytoma is uncommon, representing 9% of cases in our series. Of patients with adrenal masses or presentation suggesting catecholamine excess with normal labs, those with vertigo/dizziness may warrant a metaiodobenzylguanidine scan or repeat testing to avoid missing pheochromocytoma. Clinicians may need a high degree of suspicion for pheochromocytoma in patients with negative testing and elevated BMI.

Keywords: adrenal; metanephrines; pheochromocytoma.

Publication types

  • Comparative Study

MeSH terms

  • 3-Iodobenzylguanidine
  • Adrenal Gland Neoplasms / blood
  • Adrenal Gland Neoplasms / diagnosis*
  • Adult
  • Aged
  • Biomarkers, Tumor / blood*
  • Body Mass Index
  • Catecholamines / blood*
  • Dizziness
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Pheochromocytoma / blood
  • Pheochromocytoma / diagnosis*
  • Prognosis
  • Retrospective Studies
  • Vertigo
  • Young Adult

Substances

  • Biomarkers, Tumor
  • Catecholamines
  • 3-Iodobenzylguanidine