Endocrine Abstracts

Aim

Pheochromocytoma and paraganglioma are rare neuroendocrine tumors, that can be diagnosed incidentally or with symptoms that may be confused with other diseases. In our study, we aimed to demonstrate our clinical biochemical and pathological experiences with pheochromocytoma and paraganglioma cases.

Method

The clinical, biochemical, radiological, and pathological data of a total of 79 patients diagnosed with pheochromocytoma between 2006 and 2021 at Marmara University School of Medicine Endocrinology and Metabolic Diseases Polyclinic were evaluated retrospectively.

Results

The mean age of the 79 patients was 51.7 ± 15.0 years, and 46.8 ± 15.6 years at the time of diagnosis. The majority of patients were female (Female/Male:48/31). The percentage of patients diagnosed incidentally was 53.2%, while 32.9% of patients were diagnosed due to hyperadrenargic symptoms, and 14.0% of them were diagnosed during screening for hereditary conditions. The most frequent symptoms were hypertension (49.4%), palpitation (39.2%) and perspriation (30.4%). Median tumor size in MR imaging was 45.5 mm (range: 7–170 mm) and the lesion size correlated significantly (P < 0.0001, r = 0.5390) with the urinary normetanephrine levels. Tumor locatization was left adrenal in 30.4%, right adrenal in 27.8%, bilateral adrenal in 17.7%, and paraganglioma in 21.5% of patients. Eight patients (10.1%) were Von Hippel-Lindau, three patients (3.8%) were multiple endocrine neoplasia (MEN) type 2A, two patients (2.5%) were MEN type 2B, one patient (%1.3) each was neurofibromatosis type 1 and familial pheochromocytoma. Hereditary pheochromocytomas were diagnosed at younger ages, and bilateral lesions were more prevalent in them (both P < 0.0001). 64 patients were operated, others refused the operation or left the follow-up. The mean of tumor size in pathology report was 57.2 ± 27.5 mm. Malign pheochromocytoma was detected in 12 patients (15.2%), half of them was hereditary. Postoperative in the first year, urinary metanephrine, normetanephrine and vanilmandelik acid values of all patients decreased compared to preoperative values significantly (all of them P < 0.0001). A total of 5 patients died during the follow-up period, one of them was malignant. Three of the malignant ones received chemoradiotherapy. The clinical and biochemical characteristics of malignant and benign ones were similar. Pathological findings will be re-evaluated.

Conclusion

Hereditary syndromes should be kept in mind in young and bilateral cases. Since there is no clinical or biochemical test to distinguish malignant and bening, malign pheochromocytoma should also be considered, especially in hereditary cases.