Faculty Collaboration Database

Evolution of a pheochromocytoma. Endocr Pract 2006;12(5):545-51

Date

09/28/2006

Pubmed ID

17002930

DOI

10.4158/EP.12.5.545

Scopus ID

2-s2.0-33947384600 (requires institutional sign-in at Scopus site)   2 Citations

Abstract

OBJECTIVE: To present a case that demonstrates the evolution of a pheochromocytoma over a several-year period and to emphasize the importance of a thorough work-up for pheochromocytoma in patients with neurofibromatosis type 1 (NF1) and hypertension.

METHODS: We review the long-term clinical, biochemical, and imaging findings in a man with a complex medical history of hypertension, NF1, and cardiomyopathy.

RESULTS: A 44-year-old man, with a well-documented history of headaches, hypertension, and NF1, was referred for evaluation of a right adrenal enlargement. He had developed cardiomyopathy and undergone an evaluation for cardiac transplantation. Initial computed tomography revealed subtle asymmetry in the upper right adrenal gland. Biochemical studies for pheochromocytoma yielded equivocal findings, with a 1.5-fold elevation in the urinary norepinephrine and near-normal urinary metanephrine level. Because 131I-metaiodobenzylguanidine imaging showed no tracer uptake in the area of the right adrenal gland, the patient was thought not to have a pheochromocytoma. The patient eventually underwent cardiac transplantation and did well. On reassessment 3 1/2 years later, he was found to have a larger right adrenal mass. The second endocrine evaluation demonstrated substantial elevation in the urinary metanephrine level, and the patient underwent laparoscopic right adrenalectomy to remove the tumor (3.5 by 3.0 by 2.5 cm), which proved to be a pheochromocytoma.

CONCLUSION: This case shows that a pheochromocytoma can be difficult to diagnose and can evolve to become a large, biochemically active tumor. It is imperative that patients with an adrenal tumor undergo periodic reevaluation to ensure that the tumor remains stable in size. If the tumor enlarges, further biochemical testing is warranted.

Author List

Magill SB, Fritsche CM, Klinger DE, Tector AJ, Shaker JL

Authors

Dean Edward Klinger MD Professor in the Surgery department at Medical College of Wisconsin
Steven B. Magill MD, PhD Staff Physician in the Medicine department at Medical College of Wisconsin
Joseph L. Shaker MD Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

3-Iodobenzylguanidine
Abdomen
Adrenal Gland Neoplasms
Adult
Creatinine
Disease Progression
Dopamine
Epinephrine
Humans
Hypertension
Male
Metanephrine
Neurofibromatosis 1
Norepinephrine
Normetanephrine
Pheochromocytoma
Tomography, Emission-Computed
Tomography, X-Ray Computed