ARTICLE
Auteur(s) : Torsten Hinz1, Andreas
Wiechert1, Thomas Bieber1, Ralf
Bauer1, Monika-H
Schmid-Wendtner1,2
1Department of Dermatology and Allergology,
University of Bonn, Sigmund-Freud-Str. 25, 53127 Bonn,
Germany
2Department of Dermatology and Allergology,
University of Munich, Frauenlobstr. 9-11; 80377 Munich,
Germany
A 91-year-old female presented with a five-year history of an
indolent, slowly enlarging lesion on the right temple. Physical
examination revealed an erythematous tumor, 15 mm in diameter
with a central erosion and telangiectasia in the periphery (figure 1A).
Clinically a basal cell carcinoma was assumed and the skin lesion
was excised in local anesthesia. The histopathologic features
showed a dermal infiltration by a dense nodular tumor consisting of
multiple lobules (figure
1B). They were built by syncytial epithelial cells in the
center surrounded by a dense lymphoplasmacellular infiltrate. These
lobules were not connected with the overlying epidermis. The tumor
cells presented a light eosinophilic cytoplasm and polymorphic
vesicular nuclei with prominent nucleoli (figure 1C). Mitotic
figures could also be found. Immunohistochemical examination showed
that the tumor cells were highly positive for pan-cytokeratin (figure 1D) and
epithelial membrane antigen, which confirmed the epithelial origin
of the tumor. The lymphocytic infiltration was positive for common
B- and T-cell markers. A squamous or glandular differentiation
was not present. No Epstein-Barr viral genomic sequences were
detected by in situ hybridization.
These findings conform to the diagnosis of a
lymphoepithelioma-like carcinoma (LELCS). The main differential
diagnosis, a metastasis of a lymphoepithelioma of the nasopharynx,
was excluded by a magnetic resonance imaging of the head. Because
of the advanced age of the patient, adjunctive radiotherapy was
rejected.
Discussion
Lymphoepithelial carcinoma is a very common malignancy of the
nasopharynx (Schmincke type). It can also be found in the palatine
tonsils, salivary glands, lung, thymus, gastrointestinal tract and
uterine cervix, but it rarely occurs on the skin [1]. LELCS was
first described by Swanson in 1988 and is mainly located in the
head and neck area [2]. There are only a few cases of a LELCS on
the trunk. Clinical features of the carcinoma are mostly nodules up
to 20 mm in diameter, increasing in size over months or years.
Most of the affected patients are elderly (> 50 years of
age) and there is no gender favoured [3]. Differential diagnosis
include squamous cell carcinoma, adnexal carcinoma, Merkel cell
tumors, lymphomas, skin metastasis and basal cell carcinoma, as in
our case.
Histologically the tumor affects the mid and reticular dermis
and occasionally the subcutis and skeletal muscle. Tumor cells are
mainly arranged in nodular structures surrounded by an inflammatory
infiltrate. There is no connection to the epidermis. LELCS
immunohistochemically present a positive staining for cytokeratins
and frequently a positive staining for epithelial membrane
antigens. The inflammatory cells stain with various T- and B-cell
markers [4].
The histogenesis is still controversial. Some authors have
suggested a link to skin appendages like sweat glands or hair
follicles [5]. In our case there was no evidence for a definite
adnexal differentiation. Our results confirm previous findings that
LELCS is not related to the Epstein-Barr virus, unlike
nasopharyngeal epithelial carcinoma [6].
LELCS seems to be a malignant, undifferentiated, epithelial
neoplasm with slow local growth and a moderate tendency to
metastasize. The prognosis is relatively good. Only one case of a
fatal distant metastasis has been reported. The management of LELCS
should include an imaging investigation for exclusion of metastases
of a nasopharynx carcinoma and a wide local excision, possibly
followed by an adjunctive radiotherapy. For the clinician, a biopsy
of a slowly growing tumor located on the head or neck of an elderly
patient is necessary, because LELCS can mimic more frequently
occurring tumors, including basal cell carcinoma as in our case,
and, therefore, the correct diagnosis would have been missed
without histopathological examination.
Acknowledgements
Financial support: none. Conflict of interest: none.
References
1 Glaich A, Behroozan D, Cohen J, Goldberg L.
Lymphoepithelioma-like carcinoma of the Skin: A report of two
cases treated with complete microscopic margin control and review
of the literature. Dermatol Surg 2006; 32: 316-9.
2 Swanson SA, Cooper PH, Mills SE, Wick MR.
Lymphoepithelioma-like carcinoma of the skin. Mod Pathol 1988; 1:
359.
3 Cavalieri S, Feliciani C, Massi G, et al.
Lymphoepithelioma-like carcinoma of the skin. Int J Immunopathol
Pharmacol 2007; 20: 851-4.
4 Petter G, Haustein UF. Rare and newly described
histological variants of cutaneous squamous epithelial carcinoma.
Classification by histopthology, cytomorphology and malignant
potential. Hautarzt 2001; 52: 288-97.
5 Ko T, Muramtsu T, Shirai T.
Lymphoepithelioma-like carcinoma of the skin. J Dermatol 1997; 24:
104-9.
6 Ferlicot S, Plantier F, Rethers L, Bui AD,
Wechsler J. Lymphoepithelioma-like carcinoma of the skin: a
report of 3 Epstein-Barr virus (EBV)-negative additional cases.
Immunohistochemial study of the stroma reaction. J Cutan Pathol
2000; 27: 306-11.
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