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Extrarenal rhabdoid tumour of the vulva: diagnostic and management challenges
  1. Sarita Kumari1,
  2. Jayashree Natarajan1,
  3. Lalit Kumar2 and
  4. Neerja Bhatla1
  1. 1Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, Delhi, India
  2. 2Medical Oncology, All India Institute of Medical Sciences, New Delhi, Delhi, India
  1. Correspondence to Dr Sarita Kumari; sarita2325{at}


Extrarenal rhabdoid tumour (ERT) of vulva is a rare gynaecological neoplasm with an aggressive course and no clear management guidelines. We present the case of a 25-year-old woman with a rapidly increasing mass in right vulva suggestive of sarcoma. Wide local excision was done. Histopathology examination revealed ERT of vulva. Six weeks later she manifested lung metastases. Despite adjuvant chemotherapy, the disease progressed and she died 8 months later. We review the literature and briefly discuss the epidemiology, treatment approaches, prognostic factors and expected outcomes of this rare disease.

  • cancer intervention
  • cancer - see oncology

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  • Contributors The four authors provided significant contribution to the case report. SK and JN came up with the concept of the manuscript. SK and JN conducted the literature review and drafted the manuscript. LK and NB provided supervision and critical revisions of the manuscript. SK obtained informed consent.

  • 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.

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