Original article
Epidemiology of malignant cutaneous granular cell tumors: A US population-based cohort analysis using the Surveillance, Epidemiology, and End Results (SEER) database

https://doi.org/10.1016/j.jaad.2017.09.062Get rights and content

Background

Malignant cutaneous granular cell tumors (mcGCTs) are rare and associated with substantial morbidity and mortality. The literature includes single-institution studies.

Objective

To examine the incidence, secondary malignancies, treatment, overall survival, and disease-specific survival (DSS) of patients with mcGCT.

Methods

A population-based cohort analysis was conducted in the Surveillance, Epidemiology, and End Results database from 1973 to 2013 for patients with a diagnosis of mcGCT. Risk-adjusted associations between overall survival/DSS and patient characteristics and treatment modalities were assessed by Cox proportional hazard regression. Quantile regression was used to determine median survival times.

Results

The 5-year DSS rate was 62.8%. Patients demonstrated an increased risk for renal and pancreatic cancers. In risk-adjusted models, male sex (hazard ratio [HR], 0.21; 95% confidence interval [CI], 0.06-0.82; P = .02), advanced cancer stage (HR, 2.29; 95% CI, 1.40-3.72; P < .01), and surgical resection (HR, 0.06; 95% CI, 0.01-0.59; P = .02) predicted DSS. Median survival time in years increased for males (1.39), earlier stage (0.60), and surgical intervention (5.34).

Limitations

Absent or incorrect reporting in retrospective Surveillance, Epidemiology, and End Results data is possible. The database is more likely to include academic centers. Some subanalyses may be underpowered because of the limited sample size for a rare cancer.

Conclusions

Our study presents an in-depth assessment of factors that identify high-risk patients. Residency in a nonmetro area, black race, female sex, and no surgical resection were each associated with poorer DSS.

Section snippets

Methods

Data on patients with a diagnosis of mcGTC were obtained from the SEER program for the years 1973 to 2013. The database is a widely used cancer registry that covers an estimated 27.8% of the US population. Geographic regions covered include San Francisco-Oakland, metropolitan Detroit, Seattle (Puget Sound), metropolitan Atlanta, San Jose–Monterey, Los Angeles, greater California, Connecticut, Hawaii, Iowa, New Mexico, Utah, Kentucky, New Jersey, rural and greater Georgia, Kentucky, and Alaska.

Results

The SEER database search yielded 113 patients with granular cell tumors from 1973 to 2013, of which 82 patients (72.6%) presented with tumors on the cutis or subcutis. Of the selected cohort, 65.0% were female and 70.8% were white. The mean age at diagnosis was 49.2 years. At presentation, 15.9% of tumors were metastatic (stage IV) and 17.1% demonstrated regional involvement (stage II/III). The most frequent body site of the lesion was the trunk (in 32.5% of the cohort). Surgery was performed

Discussion

Our findings support the previously reported high mortality associated with mcGTCs,11, 18 with a 5-year DSS of 62.8%. This study also reveals a number of prognostic factors, of which the most important is thought to be cancer staging. Although there is no clear staging scheme for these tumors, the stratification used here may be clinically useful for physicians in relaying a prognosis to patients with mcGTCs. The divisions introduced (ie, local, regional, and distant) are consistent with SEER

Conclusions

Herein, we have reported on the characteristic epidemiology of mcGTCs by using available population-level data. Determinants of survival include age at diagnosis, sex, stage, and surgical resection, and there are apparent demographic, race, and sex-based disparities in survival. Patients in this population also are at an increased risk for pancreatic or renal cancers. These data underlie the importance of early diagnosis and surgical treatment of mcGTCs for optimal outcomes and outline

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    Funding sources: None.

    Conflicts of interest: None disclosed.

    Ms Mirza and Dr Tuggle had full access to all of the data in the study and take responsibility for integrity of the data and accuracy of the data analysis. Ms Mirza, Dr Tuggle, and Dr Narayan take responsibility for the study concept and design. Ms Mirza, Dr Tuggle, and Ms Zogg take responsibility for data acquisition, analysis, and interpretation, as well as for statistical analysis. Ms Mirza, Dr Tuggle, Ms Zogg, and Mr Mirza take responsibility for drafting of the manuscript. Dr Narayan takes responsibility for critical revision of manuscript for important intellectual content and for study supervision. Ms Mirza and Drs Tuggle and Narayan take responsibility for administrative, technical, or material support.

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