8407/0 Syringoma, NOS


Definitions

Skin
ICD-O-3 topography code: C44

Syringomas are common benign sweat gland neoplasms. The lesions are small (1-3 mm in diameter) and almost always multiple. They present as numerous firm, smooth, dome-shaped papules in the upper reticular dermis. The tumour papules are composed of numerous solid nests, cords, and tubules of epithelial cells surrounded by a densely sclerotic stroma. The epithelial aggregates branch and anastomose. The tumour papules may become confluent.
Syringomas arise most frequently on the lower eyelids, followed by the upper cheeks. Less commonly affected sites are neck, chest, axillae, pubic area, periumbilical region, penis, vulva, hands and forehead.
Syringomas affect women more frequently than men and have a predilection for Asians. They usually develop in adolescence and early adulthood. The majority of cases are sporadic. Syringomas are benign tumours; progression to carcinoma has not been reported1
 
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



.



Vulva
ICD-O-3 topography code: C51

Syringomas are common benign sweat gland neoplasms. The lesions are small (1-3 mm in diameter) and almost always multiple. They present as numerous firm, smooth, dome-shaped papules in the upper reticular dermis. The tumour papules are composed of numerous solid nests, cords, and tubules of epithelial cells surrounded by a densely sclerotic stroma. The epithelial aggregates branch and anastomose. The tumour papules may become confluent.
Syringomas arise most frequently on the lower eyelids, followed by the upper cheeks. Less commonly affected sites are neck, chest, axillae, pubic area, periumbilical region, penis, vulva, hands and forehead.
Syringomas affect women more frequently than men and have a predilection for Asians. They usually develop in adolescence and early adulthood. The majority of cases are sporadic. Syringomas are benign tumours; progression to carcinoma has not been reported
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



.

In the vulva, syringomas typically present as asymptomatic or pruritic small, clustered, nog-pigmented papules in the deeper skin layers of the labia majora.
The nodules are frequently bilateral
3
 
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

Syringomatous tumour (SyT) is a non-metastasizing, locally invasive tumour of the nipple/areolar region that shows sweat-duct differentiation. Although originally named ?syringomatous adenoma?
4
Click to access Pubmed
Rosen PP (1983)
Syringomatous adenoma of the nipple.
Am J Surg Pathol 7: 739-45



, ?syringomatous tumour? is favoured owing to the invasion into stroma and occasional local recurrence exhibited by these lesions 5
 
Tavassoli FA, Eusebi V, eds
Tumors of the mammary gland. AFIP atlas of tumor pathology. Fourth series ; fasc. 10
American Registry of Pathology in collaboration with the Armed Forces Institute of Pathology: Washington, D.C. 2009



.