Clinicopathological characteristics and prognosis of pheochromocytomas and paragangliomas (PPGLs) in 235 patients.

Abstract

e15175 Background: Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors. Clinical and pathological features are closely interrelated with prognosis. To study the Clinicopathological characteristics and prognosis of pheochromocytomas and paragangliomas (PPGLs) in 235 patients. Methods: We conducted a retrospective analysis of 235 patients with PPGLs who were treated and followed up for 66 months in a single Oncology center. Analyze the relationship between clinicopathological features and survival. Results: Patients presented with various symptoms: hypertension (57%), headache, palpitations and sweating (20%), Stomachache/abdominal distension (15.3%), Neck mass (5.1%), backache 10 (4.3), Asymptomatic 88/235 (37.4%). Among 235 patients, 110 (47%) were male and 125 (53%) were female, with mean (SD) age 50±12 years. There were 135 cases of adrenal pheochromocytoma and 100 cases of extra-adrenal paraganglioma (68 retroperitoneal, 16 carotid body, and 16 at other sites). Recurrence occurred in 21 patients (14 retroperitoneal, 6 adrenal, 1 carotid body; tumor size (6.33 ± 3.99) cm; 18 cases with SDHB negative, Ki67 proliferation index (8.39 ± 10.69)%; hypertension 11/21). 5 patients died (4 retroperitoneal, 1 adrenal; mean tumor size 9.46 cm; 2 with SDHB negative, 3 with weak diffuse expression; mean Ki67 19%; 3 with hypertension, 1 had hypertension complicated by cerebral hemorrhage). SDHB immunohistochemistry was performed on 217 of the 235 cases. Negative/weak diffuse expression was observed in 37 cases (17%). The recurrence rate was 43% in the SDHB negative/weak diffuse expression group and 2% in the positive group. 5-year DFS 93%, 5-year OS 98%; 10-year DFS 83%, 10-year OS 94%. Younger age (48 years) and extra-adrenal location were predictors of poor prognosis. Large tumor size (6cm), high Ki67 index (5%), and SDHB negative/weak diffuse expression were independent risk factors for postoperative recurrence in PPGLs. Conclusions: Large tumor size, high Ki67 index, and SDHB negative/weak diffuse expression are independent risk factors for postoperative recurrence in PPGLs.