Lymphoepitelioma-like carcinoma of the skin: report of three cases

Abstract

Primary cutaneous lymphoepithelioma-like carcinoma (LELC) is an extremely rare cutaneous neoplasm with histopathological features similar to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. Microscopically, the tumor is well circumscribed and is composed of irregular nests of malignant epithelial cells in a background of reactive lymphoid cells including mature plasma cells. Its histogenesis remains unknown although an adnexal or epidermic origin has been proposed, and despite its poorly differentiated histology, the LELC prognosis is relatively good. We describe three new cases of this entity that support an epidermic origin.

Cutaneous Hematopathology

“…Unlike its extracutaneous counterparts and sinonasal lymphoepithelial carcinoma, however, lymphoepithelioma-like carcinoma of the skin has not shown an association with Epstein-Barr virus (EBV) (Arsenovic 2008 ;Carr et al 1992 ;Gillum et al 1996 ;Weiss et al 1989 ) with the exception of one rare case (Aoki et al 2010 ). Although its exact histogenesis remains unclear, the tumor is likely derived from epithelial or adnexal origin (Arsenovic 2008 ;Lopez et al 2011 ;Wick et al 1991 ).…”

“…It may be clinically mistaken for a basal cell carcinoma or Merkel cell carcinoma, and, in some cases with epidermal ulceration, it has been mistaken for squamous cell carcinoma (Lopez et al 2011 ).…”

“…Cutaneous LELC has a tendency for local recurrence when not completely excised (Hall et al 2006 ;Lopez et al 2011 ). While not often widely metastatic, some case reports document regional lymph node involvement and perineural invasion (Hall et al 2006 ;Robins and Perez 1995 ).…”

“…Schnebelen et al 557 57 months following diagnosis (Swanson et al 1988 ). Current treatment strategies include wide local excision or Mohs micrographic surgery (Jimenez et al 1995 ;Lopez et al 2011 ;Robins and Perez 1995 ).…”

“…The adrenal gland is the most common primary site for tumor development, but neuroblastoma may develop at any site along the sympathetic nervous system chain Klapman and Chun 1991 ;Van Nguyen and Argenyi 1993 ). Of soft tissue sarcomas, neuroblastoma is one of the most likely to metastasize to the skin; in fact, approximately one-third of patients with congenital neuroblastoma present with cutaneous metastases, and cutaneous metastases are particularly more prevalent in neonates than in any other patient population (Hawthorne et al 1970 ;Kao and Yu 1991 ;Lucky et al 1982 ;Wyatt and Hansen 2000 ).…”