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Lymphoepithelioma-like carcinoma of skin
Other Resources UpToDate PubMed

Lymphoepithelioma-like carcinoma of skin

Contributors: Nahlah Haddouch, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Lymphoepithelioma-like carcinoma of the skin (LELCS) is an exceptionally rare skin malignancy that typically arises on sun-exposed areas of the head and neck. It typically affects older adults and shows an approximately equal sex distribution.

LELCS usually presents as a firm, painless, slowly growing nodule, papule, or plaque, typically skin colored or erythematous. Although most lesions appear on the head or neck, they can also develop in other areas. The overlying skin may remain intact or become ulcerated. LELCS may rarely locally invade adjacent tissues or metastasize to regional lymph nodes. More distant metastases are exceedingly rare.

The precise cause of LELCS remains unclear. It is debated whether these tumors represent poorly differentiated squamous cell carcinomas or adnexal neoplasms with low differentiation. In contrast to undifferentiated nasopharyngeal carcinoma (lymphoepithelioma) and lymphoepithelioma-like carcinomas arising in other organs, including the lungs, salivary glands, stomach, and thymus, cutaneous LELCS does not appear to be linked to Epstein-Barr virus (EBV).

Codes

ICD10CM:
C44.99 – Other specified malignant neoplasm of skin, unspecified

SNOMEDCT:
403952004 – Lymphoepithelioma-like carcinoma of skin

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Differential Diagnosis & Pitfalls

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Best Tests

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Therapy

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References

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Last Reviewed:07/23/2025
Last Updated:08/04/2025
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Lymphoepithelioma-like carcinoma of skin
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