Yordanov Angel, Ivanov Ivan, Kostov Stoyan, Hasan Ihsan, Dimitrova Vasilena
Department of Gynaecological Oncology, Medical University Pleven, 5800 Pleven, Bulgaria.
Department of General and Clinical Pathology, University Hospital "Dr. Georgi Stranski", 5800 Pleven, Bulgaria.
J Clin Med. 2025 Aug 26;14(17):6032. doi: 10.3390/jcm14176032.
Vulvar leiomyosarcoma (VLMS) is a rare and aggressive soft tissue malignancy arising from smooth muscle cells, comprising less than 3% of vulvar cancers. Its clinical resemblance to benign vulvar lesions often leads to delayed diagnosis. Despite surgical resection and adjuvant therapy, VLMS is associated with high recurrence rates and a poor prognosis, and due to its rarity, there is no standardized management or surveillance protocol. We present a case of high-grade VLMS in a postmenopausal woman, initially diagnosed in 2020 and managed with surgical excision and adjuvant radiotherapy. The primary tumor was a 10 cm solid, lobulated mass involving the mons pubis, with histology confirming high-grade leiomyosarcoma based on marked cellular atypia, high mitotic activity, and smooth muscle differentiation. Immunohistochemistry was positive for SMA, vimentin, and CD34, and negative for S100 and MyoD1. Five years later, the patient developed a local recurrence with an enlarged inguinal lymph node. She underwent complete tumor resection and bilateral inguinal lymphadenectomy. Histology of the recurrent lesion mirrored the initial findings, with no lymph node metastases. This case highlights the aggressive nature and potential for late recurrence in vulvar leiomyosarcoma, underscoring the importance of long-term surveillance. High-grade VLMS is a rare malignancy with a high recurrence risk. This case highlights the importance of early diagnosis, radical surgical treatment, and long-term surveillance. Although recurrence occurred five years after the initial treatment, timely surgical intervention led to a favorable postoperative course. Multidisciplinary management and individualized follow-up strategies remain key to improving outcomes in these rare gynecologic sarcomas.
外阴平滑肌肉瘤（VLMS）是一种罕见的侵袭性软组织恶性肿瘤，起源于平滑肌细胞，占外阴癌的比例不到3%。它在临床上与良性外阴病变相似，常导致诊断延迟。尽管进行了手术切除和辅助治疗，但VLMS的复发率高且预后不良，由于其罕见性，尚无标准化的管理或监测方案。我们报告一例绝经后女性的高级别VLMS病例，最初于2020年诊断，采用手术切除和辅助放疗进行治疗。原发肿瘤是一个10厘米的实性、分叶状肿块，累及耻骨联合，组织学检查根据明显的细胞异型性、高有丝分裂活性和平滑肌分化确诊为高级别平滑肌肉瘤。免疫组化显示平滑肌肌动蛋白（SMA）、波形蛋白和CD34呈阳性，S100和肌分化抗原1（MyoD1）呈阴性。五年后，患者出现局部复发，腹股沟淋巴结肿大。她接受了肿瘤完整切除和双侧腹股沟淋巴结清扫术。复发病变的组织学检查结果与最初发现相似，无淋巴结转移。该病例突出了外阴平滑肌肉瘤的侵袭性本质和晚期复发的可能性，强调了长期监测的重要性。高级别VLMS是一种罕见的恶性肿瘤，复发风险高。该病例突出了早期诊断、根治性手术治疗和长期监测的重要性。尽管在初始治疗五年后出现了复发，但及时的手术干预导致了良好的术后病程。多学科管理和个体化的随访策略仍然是改善这些罕见妇科肉瘤治疗效果的关键。
