Epinephrine-secreting cystic pheochromocytoma presenting with an incidental adrenal mass - A case report and a review of the literature


Erem C., Kocak M., Ersoz H., Ersoz S., Yucel Y.

ENDOCRINE, vol.28, no.2, pp.225-230, 2005 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 28 Issue: 2
  • Publication Date: 2005
  • Doi Number: 10.1385/endo:28:2:225
  • Journal Name: ENDOCRINE
  • Journal Indexes: Science Citation Index Expanded, Scopus
  • Page Numbers: pp.225-230
  • Keywords: cystic pheochromocytoma, epinephrine, incidental adrenal mass, MANAGEMENT, DIAGNOSIS

Abstract

Cystic adrenal masses are a relatively rare condition, and are usually nonfunctioning and asymptomatic. Differential diagnosis includes pheochromocytorna (PHEO) and adrenal carcinoma; 8-10% of patients with PHEO may be completely asymptomatic. Moreover, fewer than 10% of PHEOs secrete pure epinephrine. We report a case of a E-secreting pure cystic PHEO presenting with an incidental adrenal mass. A 49-year-old Turkish woman was hospitalized at Farabi Hospital for further examinations of a right adrenal cystic mass with a thick wall that was incidentally discovered by abdominal ultrasonography during examination for nausea, vomiting, headache, and angina-like chest pain in another hospital. On admission, her blood pressure was 100/60 mmHg. Tension Holter monitoring revealed paroximal hypertension (178/136 mmHg) and hypotension (78/54 mmHg) attacks. Of urinary catecholamines and its metabolites, only urine metanephrine was markedly increased, despite a urine epinephrine level near the upper limit of normal ranges. Abdominal computed tomography and magnetic resonance imaging studies revealed a cystic round tumor approx 5 cm in diameter, located in the right adrenal gland. Right adrenalectomy was performed; the surgical specimen revealed pure cystic PHEO. Postoperatively, the urine metanephrine level returned to normal range and urine epineprine level was decreased approx 60%. In conclusion, a diagnosis of E-secreting PHEO should be considered in patients with nonspecific symptoms, presenting with an incidental cystic adrenal mass, even in the absence of hypertension.