Pheochromocytoma: 16 Years of Experience in a Single Center

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Ozgen Saydam B., ADIYAMAN S. C., BOZKURT O., Demir O., KOÇDOR M. A., YÖRÜKOĞLU K., ...More

TURKISH JOURNAL OF ENDOCRINOLOGY AND METABOLISM, vol.25, no.1, pp.54-67, 2021 (ESCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 25 Issue: 1
  • Publication Date: 2021
  • Doi Number: 10.25179/tjem.2020-79054
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, Academic Search Premier, CINAHL, EMBASE, Directory of Open Access Journals, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.54-67
  • Keywords: Hereditary pheochromocytoma, sporadic pheochromocytoma, paroxysmal hypertension, multiple endocrine neoplasia type 2, von Hippel-Lindau disease, MULTIPLE ENDOCRINE NEOPLASIA, CUSHINGS-SYNDROME, LAPAROSCOPIC ADRENALECTOMY, SECONDARY HYPERTENSION, CASE SERIES, PARAGANGLIOMA, PREVALENCE, PRECURSOR, SPECTRUM, TYPE-2
  • Dokuz Eylül University Affiliated: Yes


Objective: Reviewing the 16-year experience of pheochromocytoma in a tertiary referral center. Material and Methods: The demographics data and the results of clinical, biochemical, and radiological evaluations of 67 patients who received a diagnosis of pheochromocytoma between the years 2004 and 2020 were obtained retrospectively. Results: The mean age (+/- SD) of the patients at the time of diagnosis was 46 years (+/- 16.1) with a slight female predominance. The percentage of patients diagnosed due to complaints was 50.8%, while 31.2% were diagnosed during the adrenal incidentaloma screening, and 18% were diagnosed during screening for hereditary conditions. Pre-existing hypertension was detected in 56.7% of the patients, while 11.9% of the patients were diagnosed to have hypertension at the time of diagnosis. Paroxysmal pattern was observed in 53.7% of the patients and was accompanied by the classical triad of palpitation (32.8%), headache (20.9%), and sweating (14.9%) as the leading symptoms. Median tumor size was 40 mm (range: 9-90 mm) and the lesion size correlated significantly (p<0.001) with the urinary catecholamine metabolite levels. The overall rate of hemodynamic instability in both perioperative and postoperative periods was 6%. Hereditary syndromes, including multiple endocrine neoplasia type 2A (MEN 2A), MEN 2B, von Hippel-Lindau (VHL), and neurofibromatosis type 1 (NF1), were diagnosed in 24% of these patients. Hereditary pheochromocytomas were diagnosed at younger ages, and bilateral lesions were more prevalent in hereditary pheochromocytomas (p=0.003 and p<0.001, respectively). In addition, patients with hereditary pheochromocytomas were more asymptomatic rather than sporadic (p=0.016). Metastasis was detected in 3% of these patients. Conclusion: Pheochromocytoma is a rare, life-threatening condition, and therefore, it is important to suspect and test for pheochromocytoma in patients with clinical suspicion. In addition, hereditary syndromes associated with pheochromocytomas should be considered while evaluating patients with pheochromocytoma. A life-long annual follow-up is recommended for the detection of recurrent or metastatic disease, and its evaluation, treatment, and follow-up should involve a multidisciplinary approach in experienced centers.