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Proximal epithelioid sarcoma of the vulva
  1. Sofia Alejandra Portillo and
  2. Erick Estuardo Estrada
  1. Gynecology and Obstetrics, Hospital General San Juan de Dios, Guatemala City, Guatemala, Guatemala
  1. Correspondence to Dr Erick Estuardo Estrada, Gynecology and Obstetrics, Hospital General San Juan de Dios, Guatemala City, Guatemala 01001, Guatemala; mderickestrada{at}

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A 15-year-old patient was seen by a general gynecologist reporting a rapidly eroding left vulvar mass in the previous 4 months. A biopsy was obtained, and the patient was referred to our institution. Computed tomography of the chest was negative for distant metastasis. Magnetic resonance imaging of the pelvis showed a solid tumor 12.4×10.5 cm, left inguinal adenopathy (largest measuring 1.7 cm). The patient was offered surgical resection and inguinal perineal flap reconstruction. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment.1 2

Figure 1

Grossly, the mass was 19×16×5 cm and revealed a solid white multi-lobed lesion with hemorrhagic areas. The deep surgical margin and the vulvar surgical margin were each 0.5 cm from the border of the mass.

Figure 2

Malignant neoplasm composed of large cells, with abundant eosinophilic cytoplasm, and nuclei with irregular borders displaced to the periphery. Necrosis and mitoses were observed. Immunohistochemistry was positive for vimentin, pancytokeratin, and epithelial membrane antigen (EMA), and negative for CD34, myogenin, desmin, and smooth muscle actin (SMA) antibodies.

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  • Collaborators Julio Lau de la Vega, Department of Obstetrics and Gynecology, Hospital General San Juan de Dios, Victor Argueta, Hessler Morales, William Alarcon, Department of Pathology, Hospital General San Juan de Dios, Jorge Pérez, Department of Plastic Surgery, Hospital General San Juan de Dios.

  • Contributors All authors contributed equally.

  • 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.

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