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Dermpath-India

Pathology of Primary Mucinous Carcinoma of the Skin

Dr Sampurna Roy MD        

 

Path Quiz Case 72 - History and images:

Diagnosis: Primary Mucinous Carcinoma of the Skin

 

                                                                                                                      

 

 

Primary mucinous carcinoma of the skin is a rare low grade variant of sweat gland tumour. 

Site:  Commonly arises in the head or neck, with the eyelid being the most common site. It is also found on the scalp, axilla and trunk.

Clinical presentation : Painless, gray or reddish nodules, measuring 0.5 to 7cm in diameter. Cut surface has a gelatinous appearance.

Microscopic features:  

Dermal tumour demonstrates large pools of basophilic mucin separated by thin fibrovascular septa ;

Small islands of epithelial cells are present in the mucinous pool ;

Cells are dense at the periphery; 

Tumour cells are small, cuboidal and bland ;

May have vacuolated cytoplasm;

Arranged in cribriform, papillary, tubular or glandular patterns ;

Almost no mitotic figures are present.

Mucinous carcinomas originate from in situ lesions that may represent, ductal hyperplasia, atypical ductal hyperplasia, or ductal carcinoma in situ  or a combination of the three.

The presence of an in situ component defines the neoplasm as primary cutaneous, but its absence does not exclude the diagnosis.

Cytologic diagnosis of primary mucinous carcinoma of the skin is possible; however, correlation of clinical, radiologic and pathologic features is necessary to arrive at an accurate diagnosis.

Special stain: The mucin is PAS-positive and diastase resistant (consistent with sialomucin). Mucicarmine is also positive.

Immunohistochemistry: 

Tumour is positive for low- molecular weight cytokeratin, CEA, EMA and sometimes S100. 

Some cases demonstrated estrogen and progesterone receptors.

CK20 is negative which might significantly contribute to the differentiation of this tumour from cutaneous metastases from gastrointestinal carcinomas

In all cases of mucinous carcinoma of skin a full clinical assessment is essential.

This tumour must be distinguished from metastatic visceral mucinous carcinoma (breast, colon, stomach, lung, ovary or pancreas).

There should be no evidence of primary tumour at these sites.

 

Differential diagnosis:  Metastatic mucinous carcinoma ;

Mucinous basal cell carcinoma - PAS negative

Colonic carcinoma metastatic to skin usually involves the anterior abdominal unlike primary cutaneous mucinous carcinoma which usually involves the head and neck area.

Mucinous carcinomas of gastrointestinal origin produce nonsulfated, neutral and sulfated mucins.

Primary mucinous carcinomas of the skin produce a nonsulfated mucin.

Dirty necrosis is a constant histologic finding in most cases of intestinal mucinous carcinomas involving the skin.

Mammary mucinous carcinoma involving the skin usually present with lesions on chest wall, breast, axilla and these locations can serve as clue to the breast origin.

Endocrine mucin-producing sweat gland tumour is an extremely rare tumour usually located on the eyelids.

Endocrine mucin-producing sweat gland carcinoma: twelve new cases suggest that it  is a precursor of some invasive mucinous carcinomas.
 

Treatment for primary mucinous carcinoma of the skin is wide local excision. Recurrence is common. 

A few cases show metastatic spread to local lymph nodes.

Mucinous carcinoma of the axilla is often associated with regional lymph node involvement.

 

Further reading:

Primary Cutaneous Mucinous Carcinoma: A Systematic Review and Meta-Analysis of Outcomes After Surgery.

Primary mucinous carcinoma of the skin.

Small primary cutaneous mucinous carcinoma mimicking an early basal cell carcinoma.

Neuroendocrine differentiation of primary mucinous carcinoma of the cheek skin.

Primary mucinous carcinoma of the periocular region: successful management with local resections over 30 years.

Primary mucinous carcinoma of the skin arising from an in situ component.

Mucinous eccrine carcinoma: a rare case of recurrence with lacrimal gland extension.

Cutaneous mucinous carcinoma arising in extramammary Paget disease of the perineum.

Primary cutaneous mucinous carcinoma of the cheek. Case report.

Primary cutaneous mucinous carcinoma: a rare entity.

 


 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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