Paragangliomas (PGLs) and pheochromocytomas (PCCs) are rare neuroendocrine tumors that derived from pluripotent neural crest stem cells. PCCs are derived from the adrenal medulla, while PGLs are histologically identical tumors derived from ganglia outside the adrenal gland. Even though the majority of tumors are benign, they are associated with high morbidity rates caused by excessive catecholamine secretion. We retrospectively analyzed 71 patients (36 female and 35 males) with PGLs between January 2002 and December 2016. Twenty three patients were lost to follow up. Kaplan-Meier curve was used as the univariate version of survival analysis. The median age at diagnosis was 47.0 years (1175) and the mean tumor size was 53 mm (26170 mm). PGLs in our group were mostly distributed in abdomen (60%), head and neck (26.2%), rarely in spine (9.2%) and chest (4.6%). Hereditary form of disease was present in 22.5% of patients. The most common mutation was in SDHB gene (10/16), hereafter in VHL gene (3/16); two patients had mutation in SDHD gene and one in SDHC. Multiple PGLs were identified in six patients. Hormonally secreting tumors were present in 28%; 16/30 patients had positive finding on I-123 MIBG scintigraphy. Surgical resection was performed in 79% of patients, tumor embolization in 11%. At 22 patients the median proliferation index Ki67 was 1.8% (0.118%) and median PASS score 3(16). Metastatic disease was present in 18.8% of patient at the time of diagnosis and five patients had acquired metastasis. These patients were treated with PRRT (6/14), chemotherapy (3/14) and sutent (3/14). Median overall survival (OS) was 284 months (95%CI 251318) with 10-year OS 88%. Mean disease free survival was estimated 20 months (95%CI 831). We presented clinical characteristics, genetic profile, treatment options and survival of our patients with PGLs, our experience as single tertiary center.
20 - 23 May 2017
European Society of Endocrinology