Primary mycosis fungoides of the vulva: The first reported case

Introduction Cutaneous T-cell lymphomas (CTCLs) or B-cell lymphomas of the vulva are rare, aggressive, and often prone to either delayed or misdiag-nosis. Other cutaneous diseases, such as lichen sclerosis, sexually transmitted diseases and dermatopathologies (Citarella et al., 2003) can mimic the presentation of these cutaneous lymphomas. Furthermore, most cases of lymphoma of the vulva are secondary manifestations of other primary disease processes (Vang et al., 2000). In the following case, we present a primary mycosis fungoides (MF) of the vulva in a biopsy of redundant vulvar tissue. To our knowledge this is the first reported case of primary MF of the vulva, and underscores the need to include primary lymphoma in the differential diagnosis when patients present with vulvar lesions. A 50 year-old premenopausal gravida 0 presented to her gynecologist for evaluation of labial swelling and occasional pruritus. Initial evaluation of the labia demonstrated redundant labia and a random biopsy was collected. Labial biopsy was consistent with MF. Patient was then referred to gynecologic oncologist for further care. Initial exam showed labia to be redundant, five times normal size. The involved area included the right labia majora and portion of labia minora; it had a grayish white hue without ulceration or specific lesion. Her most recent Pap smear showed abnormal squamous cells of unknown significance (ASCUS); otherwise, her gynecological history was unremarkable. There were no palpable groin lymph nodes bilaterally. Extensive initial diagnostics included CT scans of the chest, abdomen , and pelvis, which were negative. Bilateral bone marrow biopsy results revealed a mild-to-moderate decreased cellularity of the bone marrow without evidence of metastatic carcinoma. Megakaryocytes were adequate in number and no evidence existed of granuloma or peritrabecular lymphoid infiltrates in the bone marrow biopsy. A core biopsy specimen was 20–30% cellular and therefore inconclusive for the presence of iron. Peripheral blood was unremarkable and the B-cell leukemia/lymphoma panel was negative. A thorough systematic examination of the patient's skin was unre-markable for ulcerations, scaling plaques, or microabscesses. The vulvar lesion was the only one observed. A right hemivulvectomy with bilateral groin lymph node dissection was performed. The redundant labium was excised in a deep-wise fashion to the perineal fascia. The surgical specimen was sent for regular histology, flow cytome-try, PCR and Southern Blot studies. Histological examination revealed an atypical lymphoid lesion made of predominantly small monotocous lymphocytes (Fig. 1) with round or convoluted nucleus and scanty clear cytoplasm. Those cells …