Department of Obstetrics and Gynecology, Stanford University, Palo Alto, California, USA.
Department of Obstetrics and Gynecology, University of Colorado, Denver, Colorado, USA.
Int J Gynecol Cancer. 2020 Aug;30(8):1118-1123. doi: 10.1136/ijgc-2020-001516. Epub 2020 Jul 7.
Vulvar cancers account for 5% of all gynecologic malignancies; only 1%-3% of those vulvar cancers are primary vulvar sarcomas. Given the rarity of vulvar sarcomas, outcome data specific to histopathologic subtypes are sparse. The aim of this study was to identify clinical and pathologic factors of primary vulvar sarcomas that are associated with survival and may inform treatment decisions.
The Surveillance, Epidemiology, and End Results (SEER) database was searched for women diagnosed with vulvar sarcoma between 1973 and 2018. We identified 315 patients and reviewed their demographic, clinicopathologic, surgical, and survival information. Statistical analyses included χ and t-tests, Kaplan-Meier survival, and Cox regression analyses.
The most common histopathologies of vulvar sarcomas were dermatofibrosarcomas (85/315, 27%) and leiomyosarcomas (72/315, 22.9%). Rhabdomyosarcomas (18/315, 5.7%), liposarcomas (16/315, 5.1%), and malignant fibrous histiocytomas (16/315, 5.1%) were less frequent. The majority of patients underwent surgery (292/315, 92.7%), which included lymph node dissections in 21.6% (63/292). Survival and lymph node involvement varied significantly with histologic subtype. The 5-year disease-specific survival for dermatofibrosarcomas, liposarcomas, and fibrosarcomas was 100% and only 60.3% and 62.5% for malignant fibrous histiocytomas and rhabdomyosarcomas, respectively. None of the patients with (dermato)fibrosarcomas, liposarcomas, or leiomyosarcomas had positive lymph nodes, in contrast to rhabdomyosarcomas and malignant fibrous histiocytomas with 77.8% and 40% positive lymph nodes, respectively. The 5-year disease-specific survival for women with positive lymph nodes was 0%.
Vulvar sarcomas are heterogeneous with survival highly dependent on the histopathologic subtype. While surgical excision is the mainstay of treatment for all vulvar sarcomas, staging lymphadenectomy should be deferred for (dermato)fibrosarcomas, liposarcomas, and leiomyosarcomas as there were no cases of lymph nodes metastases.
外阴癌占妇科恶性肿瘤的 5%；其中只有 1%-3%为原发性外阴肉瘤。鉴于外阴肉瘤的罕见性，针对其组织病理学亚型的预后数据较为匮乏。本研究旨在确定与生存相关的原发性外阴肉瘤的临床和病理因素，为治疗决策提供信息。
检索 1973 年至 2018 年间在监测、流行病学和最终结果（SEER）数据库中诊断为外阴肉瘤的女性患者。我们共纳入 315 例患者，并回顾了她们的人口统计学、临床病理、手术和生存信息。统计学分析包括卡方检验和 t 检验、Kaplan-Meier 生存分析和 Cox 回归分析。
外阴肉瘤最常见的组织病理学类型为隆突性皮肤纤维肉瘤（85/315，27%）和平滑肌肉瘤（72/315，22.9%）。横纹肌肉瘤（18/315，5.7%）、脂肪肉瘤（16/315，5.1%）和恶性纤维组织细胞瘤（16/315，5.1%）较少见。大多数患者接受了手术（292/315，92.7%），其中 21.6%（63/292）进行了淋巴结清扫。生存和淋巴结受累情况与组织学亚型显著相关。隆突性皮肤纤维肉瘤、脂肪肉瘤和纤维肉瘤的 5 年疾病特异性生存率为 100%，而恶性纤维组织细胞瘤和横纹肌肉瘤分别为 60.3%和 62.5%。（皮）纤维肉瘤、脂肪肉瘤和平滑肌肉瘤患者无一例淋巴结阳性，而横纹肌肉瘤和恶性纤维组织细胞瘤的淋巴结阳性率分别为 77.8%和 40%。有淋巴结转移的女性患者 5 年疾病特异性生存率为 0%。
外阴肉瘤具有异质性，生存高度依赖于组织病理学亚型。虽然所有外阴肉瘤的主要治疗方法都是手术切除，但对于（皮）纤维肉瘤、脂肪肉瘤和平滑肌肉瘤，应避免进行分期性淋巴结切除术，因为这些肿瘤没有淋巴结转移的病例。
