Endocrine Abstracts

Paragangliomas (PGLs) and pheochromocytomas (PCCs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. We retrospectively analyzed 82 patients (43 female and 38 males) predominantly with PGLs who were hospitalized between January 2002 and December 2022. Twenty one patients were lost to follow up. The median age at diagnosis was 47 years (11–75) and the mean tumor size was 52.5 mm (26–170 mm). PGLs in our group were mostly distributed in abdomen (57%), head and neck (24.5%), rarely in spine (8.2%) and chest (3.2%). Four of our patients had coexistence of PCC. Hereditary form of disease was present in 19.5% of patients. The most common mutation was in SDHB gene (10/16), hereafter in VHL gene (3/16); 2 patients had mutation in SDHD gene and one in SDHC. Multiple PGLs at diagnosis were identified in 8 patients. Hormonally secreting tumors were present in 32% and 51% of patients had hypertension. Patients were treated mostly with surgery (86%) and tumor embolization (11.6%) in case of head and neck PGLs. At 30 patients, the median proliferation index Ki67 was 2.25% (0.1–25%). Median PASS score was 5 (1–14). Relapse of disease was present in 17% of patients with mean time to relapse 26 month (0–168). Metastatic disease was present in 18.8% of patients at the time of diagnosis and 6 patients had acquired metastasis. Mean time between initial and malignancy diagnosis was 28 months (range 0–300). These patients were treated mostly with PRRT (6/17), chemotherapy (4/17) and sutent (3/17). Median overall survival (OS) was 339 months (95%CI 305–373) with 10-year OS 93%.

We presented clinical characteristics, genetic profile, treatment options and survival of patients with PGLs, our experience as single tertiary center.