Radiotherapy in lymphoepithelioma-like carcinoma of the skin: review of the literature and report of a recurrent and metastatic case

Abstract

Primary lymphoepithelioma-like carcinoma of the skin (LELCS) is a very rare cutaneous neoplasm. Only about 70 cases have been documented in the literature. There are no prospective data regarding treatment methods. Surgical excision is sufficient therapy in the majority of cases. Radiation therapy is sometimes used in case of recurrence or positive margins after surgery. The metastatic potential of LELCS is extremely low and only five previously documented cases of lymph node spread have been reported. We present the case of an 80-year-old male with a tumor primarily located on the lower eyelid, with two regional recurrences and cervical lymph node spread after surgery, treated with concurrent chemoradiation. According to the available data, this is the first case of lymph node spread from an eyelid location and the first nodal recurrence after surgery.

Primary Lymphoepithelioma-Like Carcinoma of the Conjunctiva Metastatic to Regional Lymph Nodes and Parotid Gland in a Mexican Patient

“…Prior radiation dosing strategies specifically for conjunctival LELC include the administration of radiotherapy of 50 Gy in 25 fractions [ 12 ]. In our patient, adjuvant radiotherapy achieved remission of locally residual tumor and parotid and neck lymph node metastasis similar to the case reported by Mucha-Małecka et al [ 7 ]. Survival in ocular adnexal LELC varies by site and histology.…”

“…Like other lymphoid cell malignancies, this neoplasm has been associated in some tissues with the Epstein-Barr virus (EBV) in certain populations; nevertheless, this has only been reported in the Asian population for the orbit and ocular adnexa [ 3 – 5 ]. Treatment of this neoplasm may include surgery, radiotherapy, or chemotherapy [ 4 , 6 , 7 ]. This neoplasm represents a significant diagnostic challenge because it is an extremely rare presentation as a primary tumor in the orbit, eyelids, and ocular adnexa.…”

A rare mimic of basal cell carcinoma

“…3 Lymphatic metastases have been reported in six cases with treatment options including lymph node dissection and radiotherapy. 2 However, LELCS is usually managed with conventional excision, as for our patient. Other treatment options include margin-controlled excision and radiotherapy in the presence of perineural invasion or positive margins.…”

“…Histologically, LELCS is indistinguishable from undifferentiated nasopharyngeal carcinoma (lymphoepithelioma of the nasopharynx); however, whereas this is EBV‐positive, LELCS is EBV‐negative, and in situ hybridization is therefore vital in diagnosis. The origin of LELCS is still debated, with some clinicians considering it an inflamed, poorly differentiated variant of SCC, whereas others consider it likely to be of adnexal origin 1,2 …”

“…The origin of LELCS is still debated, with some clinicians considering it an inflamed, poorly differentiated variant of SCC, whereas others consider it likely to be of adnexal origin. 1,2 Although there are case reports of metastatic disease, LELCS is generally considered to be an indolent tumour with good disease-free survival. 3 Lymphatic metastases have been reported in six cases with treatment options including lymph node dissection and radiotherapy.…”