Abstract
This report describes an unusual EBV-negative lymphoepithelioma-like carcinoma of the vulva in a 73-year-old patient. The lesion was localised at the right minor labium and was resected by partial vulvectomy. A synchronous sentinel lymph node biopsy revealed a single micrometastasis in the right inguinal region, which prompted local radiotherapy. Follow-up nine months later showed only slight vulvar atrophy, without signs of local recurrence or distant metastases.Although lymphoepithelioma-like carcinomas of the skin and the female genital tract are presumed to have a better prognosis than their counterparts in the upper aerodigestive tract, possibly due to earlier detection and therapy, this case documents their potential for early metastasis.
Highlights
Lymphoepithelial carcinoma is an aggressive EBVassociated neoplasm of the nasopharyngeal region
Lymphoepithelioma-like carcinoma (LELC) of the skin, is a rare condition [1,2,3,4] and only three cases have been reported in the vulvar region [5,6,7]
The same is true for other sites of the female genital tract with some cases described in the uterine cervix [8,9,10,11,12,13,14], two reports each in the ovary [15], the endometrium [16,12], and the vagina [17,18], and only one in the Bartholin gland [19]
Summary
Lymphoepithelial carcinoma is an aggressive EBVassociated neoplasm of the nasopharyngeal region. LELC is said to be less aggressive in extranasal sites [1], regional lymph node metastases can occur [24,6]. In this case of a 73year-old woman, a small inguinal metastasis was detected by sentinel node technique. Due to the fact that the histological invasion depth of the tumor did not exceed 2 mm, the patient did not receive adjuvant treatment, but underwent radiaton of the right inguinal region with a focal dose of 5000cGy in 25 fractions because of the lymph node micrometastasis. Staining for p16 was evenly strong but HPV was not detected by immunohistochemistry, the in-situ-hybridisation of EBV was negative. IGH and T/gamma rearrangement of the lymphocytic tumor component proved polyclonality
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