Primary cutaneous adenoid cystic carcinoma in the United States: incidence, survival, and associated cancers, 1976 to 2005.
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.


CONCLUSIONS
A pathologic review of reported cases was not undertaken.


CONCLUSIONS
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.
Dores
Graça M
GM
Medical Service, Department of Veterans Affairs Medical Center, Oklahoma City, Oklahoma 73104, USA. doresg@mail.nih.gov
Huycke
Mark M
MM
Devesa
Susan S
SS
Garcia
Carlos A
CA
D003528:Carcinoma, Adenoid Cystic / Q000453:epidemiology* / Q000401:mortality; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000453:epidemiology; D012878:Skin Neoplasms / Q000453:epidemiology* / Q000401:mortality; D015996:Survival Rate; D014481:United States / Q000453:epidemiology
J Am Acad Dermatol. 2003 Nov;49(5):832-41
14576661
Int J Dermatol. 2001 Nov;40(11):724-5
11737443
Proc R Soc Med. 1975 Oct;68(10):649-50
1208516
Lancet. 2002 Feb 9;359(9305):497-8
11853800
Plast Reconstr Surg. 1991 Jul;88(1):140-4
1647038
Am J Dermatopathol. 1990 Apr;12(2):169-74
2158756
Arch Dermatol. 1975 Jun;111(6):793-4
1137427
Am J Dermatopathol. 1998 Dec;20(6):571-7
9855352
Clin Exp Dermatol. 1987 Sep;12(5):378-80
2833370
Int J Hematol. 1993 Dec;59(1):67-71
8161737
Am J Dermatopathol. 1986 Feb;8(1):2-13
3010759
AIDS. 2009 Jan 28;23(3):385-93
19114864
Am J Clin Pathol. 1967 Jun;47(6):767-80
4164856
Am J Epidemiol. 1995 Feb 15;141(4):300-4
7840107
Arch Dermatol. 1971 Jan;103(1):68-78
4321803
J Invest Dermatol. 2008 May;128(5):1340-2
18408748
Cancer Epidemiol Biomarkers Prev. 2006 Aug;15(8):1545-9
16896047
Am J Ophthalmol. 2001 Feb;131(2):281-3
11228319
J Am Acad Dermatol. 1999 Apr;40(4):640-2
10188691
Radiat Res. 1995 Mar;141(3):259-77
7871153
J Am Acad Dermatol. 2008 Apr;58(4):636-41
18342709
Cancer. 2008 Dec 15;113(12):3372-81
18932259
Clin Exp Dermatol. 1996 May;21(3):249-50
8914382
J Dermatol Surg Oncol. 1992 Mar;18(3):175-6
1311729
Arch Pathol Lab Med. 1992 Jan;116(1):76-9
1310381
Jpn J Ophthalmol. 2006 Jul-Aug;50(4):374-376
16897224
J Laryngol Otol. 2001 Aug;115(8):673-5
11535157
Arch Dermatol. 1969 Aug;100(2):234-42
5797968
Acta Derm Venereol. 2004;84(3):243-4
15202851
Cutis. 2008 Mar;81(3):243-6
18441847
Arch Dermatol. 1984 Jun;120(6):774-7
6326693
Arch Dermatol. 1978 Mar;114(3):421-4
204257
Br J Dermatol. 1988 Apr;118(4):567-77
2837268
Dermatol Surg. 2003 Jun;29(6):647-9
12786711
J Am Acad Dermatol. 2008 Dec;59(6):1081-5
19022107
Acta Derm Venereol. 2008;88(3):316-8
18480953
Br J Neurosurg. 1999 Dec;13(6):601-3
10715732
J Dermatol. 1993 Mar;20(3):164-70
7683029
J Eur Acad Dermatol Venereol. 2004 May;18(3):383-5
15096169
Cancer. 1951 May;4(3):521-36
14839609
J Dermatol. 1989 Jun;16(3):227-30
2551943
Cutis. 2006 Mar;77(3):157-60
16610734
Tumori. 1991 Jun 30;77(3):264-7
1650509
Head Neck. 2000 Jan;22(1):84-6
10585609
Br J Ophthalmol. 2003 Jan;87(1):118-9
12488275