Lugata John, Riley Kristen, Lyamuya Tecla, Shao Baraka, Mohamed Aisha, Skinner Elizabeth, Mremi Alex, Mchome Bariki
Department of Obstetrics and Gynecology Kilimanjaro Christian Medical Centre Moshi Tanzania.
School of Medicine KCMC University Moshi Tanzania.
Clin Case Rep. 2025 Aug 1;13(8):e70664. doi: 10.1002/ccr3.70664. eCollection 2025 Aug.
Primary vulvar lymphoma is an exceptionally rare malignancy, comprising less than 1% of all vulvar cancers and an even smaller fraction of extranodal non-Hodgkin lymphomas. Among these, high-grade T-cell lymphoma is exceedingly uncommon, with limited cases reported in the literature. Immunocompromised states, particularly HIV infection, are known to predispose individuals to aggressive and atypical lymphoid malignancies. This case report presents a rare instance of T-cell lymphoma of the vulva in a premenopausal woman, with the diagnosis confirmed through histopathological analysis of vulvar tissue. To the best of our knowledge, this is the first reported case of its kind in Tanzania. Case History: We report the rare case of a 46-year-old, P1L1, premenopausal woman living with HIV who presented with a rapidly enlarging, painful right vulvar mass with ulceration and foul discharge for 7 months. Pelvic examination revealed a large firm, friable mass with an ulcerated, erythematous surface involving the right labia majora, posterior fourchette, and perineum. She was also noted to have ipsilateral inguinal lymphadenopathy. A pelvic Magnetic Resonance Imaging (MRI) with and without IV contrast confirmed the presence of an ulcerated mass arising in the right labium measuring 6 × 2 cm. Prominent and enhancing bilateral inguinal-femoral lymph nodes were identified, the largest noted in the right inguinal region, measuring 4 × 2 cm. The radiological findings were suggestive of a right labial ulcerative malignant mass with inguinal-femoral lymphadenopathy. Staging investigations revealed localized disease without systemic involvement (Ann Arbor Stage II-E). Histopathological evaluation confirmed high-grade peripheral T-cell lymphoma, with immunohistochemistry (IHC) analysis results indicating that the tumor cells were positive for CD38, CD43, CD56, CD57, CD2, and CD3 but negative for CD4, CD5, CD20, BCL2, and PAX5. The findings favored the diagnosis of a high-grade T-cell lymphoma of the vulva, most likely extranodal NK/T-cell lymphoma. The patient was managed with combination chemotherapy Cyclophosphamide, Doxorubicin, Etoposide, Vincristine, and Prednisolone (CHOEP) protocol alongside continued antiretroviral therapy. She showed a favorable initial response with significant tumor regression and remains under close follow-up.
原发性外阴淋巴瘤是一种极为罕见的恶性肿瘤，占所有外阴癌的比例不到1%，在外周非霍奇金淋巴瘤中所占比例更小。其中，高级别T细胞淋巴瘤极为罕见，文献报道的病例有限。已知免疫功能低下状态，尤其是HIV感染，会使个体易患侵袭性和非典型淋巴恶性肿瘤。本病例报告呈现了一名绝经前女性患外阴T细胞淋巴瘤的罕见病例，通过对外阴组织的组织病理学分析确诊。据我们所知，这是坦桑尼亚首例此类报道病例。病例史：我们报告了一例罕见病例，一名46岁、孕1产1、患有HIV的绝经前女性，出现右侧外阴肿物迅速增大、疼痛，伴有溃疡和恶臭分泌物7个月。盆腔检查发现一个大的、质地硬且易碎的肿物，表面溃疡、红斑，累及右侧大阴唇、后阴唇系带和会阴。还发现她同侧腹股沟淋巴结肿大。盆腔磁共振成像（MRI）平扫及增强扫描证实右侧阴唇有一个6×2cm的溃疡肿物。双侧腹股沟股淋巴结肿大且强化，右侧腹股沟区最大的淋巴结大小为4×2cm。影像学检查结果提示右侧阴唇溃疡性恶性肿物伴腹股沟股淋巴结肿大。分期检查显示为局限性疾病，无全身受累（Ann Arbor分期II - E期）。组织病理学评估证实为高级别外周T细胞淋巴瘤，免疫组化（IHC）分析结果表明肿瘤细胞CD38、CD43、CD56、CD57、CD2和CD3呈阳性，但CD4、CD5、CD20、BCL2和PAX5呈阴性。这些结果支持外阴高级别T细胞淋巴瘤的诊断，最可能为外周NK/T细胞淋巴瘤。该患者接受了环磷酰胺、多柔比星、依托泊苷、长春新碱和泼尼松龙（CHOEP）方案的联合化疗以及持续的抗逆转录病毒治疗。她最初反应良好，肿瘤明显消退，目前仍在密切随访中。
