Pathology of Syringoma - Dr Sampurna Roy MD

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Pathology of Syringoma

Dr Sampurna Roy MD

Dermatopathology Quiz Case: 182

Answer - Syringoma

Syringoma is a benign adnexal tumour originating from the intraepidermal portion of the eccrine duct.

The name syringoma is derived from the Greek word syrinx, which means pipe or tube.

These lesions are common in patients with Down syndrome.

Clear cell syringoma are commonly associated with diabetes mellitus.

Clinical presentation:

i) Multiple papules on the lower eyelids and cheeks of adolescent females

ii) Solitary and giant lesions, plaque form or milia-like lesions limited to the vulva, penis, buttock, neck, scalp, axilla, abdomen or extremities.

Eruptive syringoma is a rare, clinically distinct variant of syringoma.

Microscopic features:

Dermal tumours are composed of small ducts lined by two layers of cuboidal epithelium ;

Ducts have a comma-like tail (tadpole appearance);

Solid nests and strands of basaloid cells may be present in the dermis ;

Some ducts contain eosinophilic material ;

Backround stroma is usually fibrotic ;

The lesion is well demarcated from the surrounding dermis ;

Numerous mast cells are present in the stroma in some cases;

Clear cell variant- ducts are lined by epithelial cells with pale or clear cytoplasm.

Immunohistochemistry:

Tumour cells express cytokeratin10 in the intermediate cells, cytokeratin 6 and 19 and Carcinoembryonic antigen (CEA) in the luminal cells and epithelial membrane antigen (EMA) in the peripheral cells of the duct.

(Differential diagnosis - Carcinoembryonic antigen (CEA) is negative in desmoplastic trichoepithelioma).

Progesterone receptors are expressed in most syringomas.

Differential diagnosis:

Microcystic adnexal carcinoma (no perineural invasion or deep extension in syringoma). It is impossible to make the distinction in a shave biopsy.

Chondroid syringoma.

Tadpole shaped ducts in Syringoma -Pathology Infographic

Further reading:
An unusual presentation and distribution of generalized eruptive syringomas.

Multiple giant vulvar syringoma: an extraordinary report.

Vulvar nonclear cell syringoma associated with pruritus and diabetes mellitus.

Cutaneous syringoma: a clinicopathologic study of 34 new cases and review of the literature.

Penile syringoma: reports and review of patients with syringoma located on the penis.

Linearly distributed syringoma: a case report.

"Eruptive" facial syringomas: an inflammatory skin reaction?