Milken Institute School of Public Health Poster Presentations (Marvin Center & Video)

Title

Genitourinary Paraganglioma: Demographic, Pathologic, and Clinical Characteristics in the Surveillance, Epidemiology and End Results (SEER) Database (2000-2012)

Poster Number

64

Document Type

Poster

Status

Graduate Student - Masters

Abstract Category

Epidemiology and Biostatistics

Keywords

oncology; paraganglioma; genitourinary pathology; urology

Publication Date

4-2017

Abstract

Background: Extra-adrenal paragangliomas (PGL) are infrequent, benign, 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. While most PGL develop above the umbilicus, they have been reported in the genitourinary (GU) tract. Due 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.

Methods: The SEER 18 database was utilized to identify all cases of PGL from 2000-2012. 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 between GU and non-GU PGL. Statistical significance was defined as p

Results: 299 cases of PGL were retrieved from SEER. 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). 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 women, and it was more common in whites than all other races. While 50% of GU PGL was organ confined, only 5.7% of non-GU PGL was localized at diagnosis. All cases of PGL were treated with surgery. There were 2 (10%) cause-specific deaths in the GU PGL groups from 2000-2012. 5-year overall survival was 93.3% for GU PGL versus 65.5% in non-GU PGL (p=0.062).

Conclusions: Genitourinary PGL remains rare, with low incidence (6.7% of all PGL cases) in the US population between 2000 and 2012. Also, it had better 5-year overall survival compared to PGL developing outside of the GU tract. The bladder represents the most common site of involvement and surgery is the mainstay of treatment for GU PGL. Clearer prognostic factors 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.

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Poster to be presented at GW Annual Research Days 2017.

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Genitourinary Paraganglioma: Demographic, Pathologic, and Clinical Characteristics in the Surveillance, Epidemiology and End Results (SEER) Database (2000-2012)

Background: Extra-adrenal paragangliomas (PGL) are infrequent, benign, 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. While most PGL develop above the umbilicus, they have been reported in the genitourinary (GU) tract. Due 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.

Methods: The SEER 18 database was utilized to identify all cases of PGL from 2000-2012. 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 between GU and non-GU PGL. Statistical significance was defined as p

Results: 299 cases of PGL were retrieved from SEER. 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). 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 women, and it was more common in whites than all other races. While 50% of GU PGL was organ confined, only 5.7% of non-GU PGL was localized at diagnosis. All cases of PGL were treated with surgery. There were 2 (10%) cause-specific deaths in the GU PGL groups from 2000-2012. 5-year overall survival was 93.3% for GU PGL versus 65.5% in non-GU PGL (p=0.062).

Conclusions: Genitourinary PGL remains rare, with low incidence (6.7% of all PGL cases) in the US population between 2000 and 2012. Also, it had better 5-year overall survival compared to PGL developing outside of the GU tract. The bladder represents the most common site of involvement and surgery is the mainstay of treatment for GU PGL. Clearer prognostic factors 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.