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Primary cutaneous adenoid cystic carcinoma in the United States: Incidence, survival, and associated cancers, 1976 to 2005 - 24/04/13

Doi : 10.1016/j.jaad.2009.07.027 
Graça M. Dores, MD, MPH a, b, , Mark M. Huycke, MD a, c, Susan S. Devesa, PhD b, Carlos A. Garcia, MD d
a Medical Service, Department of Veterans Affairs Medical Center, Oklahoma City, Oklahoma 
b Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland 
c Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 
d Department of Dermatology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 

Correspondence to: Graça M. Dores, MD, MPH, Department of Veterans Affairs Medical Center, Medical Service (111), 921 NE 13th St, Oklahoma City, OK 73104.

Abstract

Background

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare appendageal tumor of uncertain origin. Details on epidemiologic features of PCACC are sparse and largely based on clinical reports.

Objective

We sought to develop an understanding of PCACC incidence, survival, and associated cancers using population-based data.

Methods

We used the Surveillance, Epidemiology, and End Results program to calculate age-adjusted incidence rates (IRs), IR ratios, 95% confidence intervals, standardized incidence ratios (SIRs), and 5-year relative survival of PCACC diagnosed during 1976 to 2005.

Results

In a population of 723,174,580 person-years, the overall PCACC IR was 0.23 per 1 million person-years (n = 152), with similar IRs among male and female patients (IR = 0.24). Most cases of PCACC presented at a localized stage and arose on the face/head/neck. Among 122 of the 2-month survivors of PCACC and more than 2.4 million 2-month cancer survivors, risk of associated cancers overall was not significantly increased (SIR = 1.17 [n = 24] and SIR = 1.43 [n = 16], respectively). However, PCACC was associated with significantly increased risks of subsequent lymphohematopoietic (n = 6; SIR = 3.70) and thyroid (n = 2; SIR = 15.25) cancers, whereas the converse associations were not observed. Five-year relative survival was excellent (96.1%; n = 122) with more favorable survival noted for PCACC involving the face/head/neck than the trunk.

Limitations

A pathologic review of reported cases was not undertaken.

Conclusion

PCACC is a rare appendageal tumor that affects male and female individuals equally, primarily presents at localized stage, predominates in the face/head/neck, and is associated with favorable survival. Immunosuppression does not appear to contribute to the development of PCACC, and the observed associated cancer patterns will need to be confirmed in larger studies.

Le texte complet de cet article est disponible en PDF.

Key words : adenoid cystic carcinoma, epidemiology, incidence, second primary cancers, skin cancer, survival

Abbreviations used : API, CI, IR, PCACC, PY, SEER, SEER-9, SEER-11, SIR


Plan


 Supported by the Department of Veterans Affairs Medical Center in Oklahoma City, and the Intramural Research Program of the National Cancer Institute, National Institutes of Health.
 Conflicts of interest: None declared.
 Reprints not available from the authors.


© 2009  American Academy of Dermatology, Inc.. Publié par Elsevier Masson SAS. Tous droits réservés.
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Vol 63 - N° 1

P. 71-78 - juillet 2010 Retour au numéro
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