Extra-adrenal paragangliomas (PGLs) are infrequent, benign, and neuroendocrine tumors arising from chromaffin cells of the autonomic nervous system. Most PGLs are sporadic, but up to 32% are associated with inherited syndromes such as neurofibromatosis type 1, von Hippel-Lindau disease, and familial PGL. Although most PGLs develop above the umbilicus, they have been reported in the genitourinary (GU) tract. Owing to the paucity of literature on the rates of GU PGL, the objective of our study is to describe the demographic, pathologic, and clinical characteristics of GU PGL, and compare them to non-GU sites of PGL using the surveillance, epidemiology, and end results (SEER) database.
The SEER 18 database was used to identify all cases of PGL from 2000 to 2012. Demographic, pathologic, and clinical characteristics were described using chi-square and t-test for categorical and continuous variables, respectively. The Kaplan-Meier method was used to compare overall survival (OS) between GU and non-GU PGL. Statistical significance was defined as P<0.05. All analyses were performed using excel and SAS/Stat version 9.4.
A total of 299 cases of PGL were retrieved from SEER, and 20 (6.7%) of the total PGL arose from the GU tract. The mean age at diagnosis was higher in non-GU than GU PGL (50.4±17.2 vs. 40.8±15.6, P = 0.026). Furthermore, 83.3% of GU PGLs developed in the bladder, followed by the kidneys/renal pelvis (16.7%), and spermatic cord (2%). Non-GU PGL developed most frequently within the endocrine system (43%). PGL, overall, was more common in men than in women, and it was more common in whites than all other races. Although 55.5% of GU PGLs were organ confined, only 22.2% of non-GU PGLs were localized at diagnosis. All cases of PGL were treated with surgery. There were 2 cause-specific deaths in the GU PGL groups between 2000 and 2012. The 5-year OS was 93.3% for GU PGL vs. 65.5% in non-GU PGL (P = 0.062).
GU PGL remains rare with low incidence (6.7% of all PGL cases) in the US population between 2000 and 2012. Bladder PGL represents just 5% of all PGL. Moreover, GU PGL had better OS compared to PGL developing outside of the GU tract although the P-value only approached statistical significance. The bladder represents the most common site of involvement, and surgery is the mainstay of treatment for GU PGL. Clearer prognostic factors, including tumor grade and stage, are needed to better elucidate PGL management in the future; thus, pooled studies from various institutions with detailed clinical information are needed to delineate these prognostic factors.
Urologic oncology. 2017 Mar 18 [Epub ahead of print]
Stephanie Purnell, Abhinav Sidana, Mahir Maruf, Campbell Grant, Piyush K Agarwal
Urologic Oncology Branch, National Cancer Institute, Bethesda, MD., Department of Urology, George Washington University Hospital, Washington, DC., Urologic Oncology Branch, National Cancer Institute, Bethesda, MD. Electronic address: .