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Vulvar sarcoma outcomes by histologic subtype: a Surveillance, Epidemiology, and End Results (SEER) database review
  1. Sarah Johnson1,
  2. Malte Renz1,
  3. Lindsay Wheeler2,
  4. Elisabeth Diver1,
  5. Oliver Dorigo1,
  6. Babak Litkouhi1,
  7. Kian Behbakht2,
  8. Brooke Howitt3 and
  9. Amer Karam1
  1. 1Department of Obstetrics and Gynecology, Stanford University, Palo Alto, California, USA
  2. 2Department of Obstetrics and Gynecology, University of Colorado, Denver, Colorado, USA
  3. 3Deprtment of Pathology, Stanford University, Palo Alto, California, United States
  1. Correspondence to Dr Amer Karam, Stanford University School of Medicine, Stanford, USA; amkaram{at}


Objective 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.

Methods 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 χ2 and t-tests, Kaplan–Meier survival, and Cox regression analyses.

Results 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%.

Conclusions 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.

  • vulvar neoplasms

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  • SJ and MR contributed equally.

  • Contributors All listed authors fulfill authorship criteria. AK contributed to the conceptualization, data collection and writing of the manuscript, SJ, MR and LW contributed to the data collection and writing of the manuscript, OD, ED, BL, BH and KB contributed to the writing of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available upon reasonable request.