8410/3 Sebaceous adenocarcinoma


Synonyms

Sebaceous carcinoma

Definitions

Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Sebaceous carcinoma (SC) is a malignant adenocarcinoma with sebaceous differentiation. The lesion typically arises in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. SC presents as a painless, typically slow-growing, nodular mass and may be multifocal. Histologically, the tumour is characterized by dermal lobules of malignant sebocytic cells with multivesicular and vacuolated clear cytoplasm, surrounded by a fibrovascular stroma. The centre of the tumour cell nests may be necrotic. SCs may be associated with overlying carcinoma in-situ or with extramammary Paget disease, or both, in the surface epithelium and in other epidermal appendages.
Sebaceous carcinoma usually arises in middle-aged to elderly patients (average age 62 years). Women are twice as frequently affected. The risk for local recurrence is 30-40%, the risk for distant metastases 20-25%. Tumour-related mortality is 10-20% 1
Click to access Pubmed
Moreno C, Jacyk WK, Judd MJ, Requena L (2001)
Highly aggressive extraocular sebaceous carcinoma.
Am J Dermatopathol 23: 450-5



2
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.

Despite numerous sebaceous glands present on the vulva, vulvar sebaceous carcinoma is rare. The tumour resembles its cutaneous counterpart. It is composed of cords and nests of basaloid appearing neoplastic glandular elements with cellular features of sebaceous epithelium. It may be associated with vulvar intraepithelial neoplasia(VIN)
3
Click to access Pubmed
Escalonilla P, Grilli R, Cañamero M, Soriano ML, Fariña MC, Manzarbeitia F, Sáinz R, Matsukura T, Requena L (1999)
Sebaceous carcinoma of the vulva.
Am J Dermatopathol 21: 468-72



4
Click to access Pubmed
Carlson JW, McGlennen RC, Gomez R, Longbella C, Carter J, Carson LF (1996)
Sebaceous carcinoma of the vulva: a case report and review of the literature.
Gynecol Oncol 60: 489-91



5
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



.



Breast
ICD-O-3 topography code: C50
ICD10: C50
           

A breast carcinoma with prominent sebaceous differentiation in no less than 50% of cells. There should be no evidence of origin from cutaneous adnexal sebaceous glands.





Penis
ICD-O-3 topography code: C60
ICD10: C60
           

An adenocarcinoma with sebaceous differentiation. It presents as a painless mass and it may be multifocal. It grows in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. It is characterized by the presence of malignant cells with multivesicular and clear cytoplasm. It may recur and metastasize.



Skin
ICD-O-3 topography code: C44
ICD10: C44
           

Sebaceous carcinoma (SC) is a malignant adenocarcinoma with sebaceous differentiation. The lesion typically arises in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. SC presents as a painless, typically slow-growing, nodular mass and may be multifocal. Histologically, the tumour is characterized by dermal lobules of malignant sebocytic cells with multivesicular and vacuolated clear cytoplasm, surrounded by a fibrovascular stroma. The centre of the tumour cell nests may be necrotic. SCs may be associated with overlying carcinoma in-situ or with extramammary Paget disease, or both, in the surface epithelium and in other epidermal appendages.
Sebaceous carcinoma usually arises in middle-aged to elderly patients (average age 62 years). Women are twice as frequently affected. The risk for local recurrence is 30-40%, the risk for distant metastases 20-25%. Tumour-related mortality is 10-20%
9
Click to access Pubmed
Moreno C, Jacyk WK, Judd MJ, Requena L (2001)
Highly aggressive extraocular sebaceous carcinoma.
Am J Dermatopathol 23: 450-5



10
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.



Ovary
ICD-O-3 topography code: C56
ICD10: C56
           

An adenocarcinoma with sebaceous differentiation. It presents as a painless mass and it may be multifocal. It grows in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. It is characterized by the presence of malignant cells with multivesicular and clear cytoplasm. It may recur and metastasize.



Salivary glands
ICD-O-3 topography code: C07-C08
ICD10: C07-C08
           

An adenocarcinoma with sebaceous differentiation. It presents as a painless mass and it may be multifocal. It grows in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. It is characterized by the presence of malignant cells with multivesicular and clear cytoplasm. It may recur and metastasize.