Sarcomas and other rare vulval and vaginal cancers are an extremely heterogeneous group of tumours with diverse clinical behaviour and response to therapy. Due to their rarity and heterogeneity, the evidence base to support management decisions is limited. Treatment planning should prioritise fertility and organ preservation in patients of child bearing potential. Management is often extrapolated from recommendations developed for histologically similar cancers at other anatomic sites. The lack of standard guidelines underscores the need for multidisciplinary care in expert centres, registration in rare cancer networks and better genetic screening, all of which are essential for advancing management of these understudied neoplasms.

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Sarcomas and Other Rare Vulval and Vaginal Cancers

  • Monika A. Kucharczyk,
  • Emine Hatipoglu,
  • Christina Fotopoulou,
  • Robin L. Jones

摘要

Sarcomas and other rare vulval and vaginal cancers are an extremely heterogeneous group of tumours with diverse clinical behaviour and response to therapy. Due to their rarity and heterogeneity, the evidence base to support management decisions is limited. Treatment planning should prioritise fertility and organ preservation in patients of child bearing potential. Management is often extrapolated from recommendations developed for histologically similar cancers at other anatomic sites. The lack of standard guidelines underscores the need for multidisciplinary care in expert centres, registration in rare cancer networks and better genetic screening, all of which are essential for advancing management of these understudied neoplasms.