Abstract

Synchronous primaries are uncommon in medical literature but pose many diagnostic and therapeutic challenges which require a multi-disciplinary approach. A 64-year-old female was presented with a vulva lesion and bilateral inguinal lymphadenopathy. She was also noted to have a palpable abdominal growth and an enlarged neck mass. Blood evaluation yielded elevated CA-125 and thyroid stimulating hormone levels. All other tests were unremarkable. Computed tomography of the neck showed a large thyroid mass with bilateral lobe involvement and abdominal scanning showed a large cystic lesion causing right hydronephrosis and hydroureter. She was diagnosed with a primary vulva squamous carcinoma in a background of lichen sclerosus which was managed surgically. The patient also underwent laparotomy and was found to have a borderline ovarian malignancy. A neck biopsy yielded a thyroid lymphoma which was treated with chemotherapy. Prophylactic radiation to the groin was performed after multi-disciplinary consult. There was an excellent clinical response to the treatment. This is the first report of concurrent vulva and ovarian pathology associated with a hematologic malignancy. Specific immunologic and genetic abnormalities have not yet been identified in literature which may link these supposedly random occurrences. However, the immune dysregulation associated with lichen sclerosus and its role in the pathogenesis of vulva squamous carcinoma is of interest in the background of a co-existing hematological malignancy, and an inherent compromised immune response. In addition, ongoing genetic research may yet elucidate a common link between these synchronous primaries.

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