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Atypical presentation of intravascular leiomyomatosis mimicking advanced uterine sarcoma: modified laterally extended endopelvic resection with preservation of pelvic neural structures
  1. Philip Cowie,
  2. Ben Eastwood,
  3. Sarah Smyth and
  4. Hooman Soleymani majd
  1. Department of Gynaecological Oncology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  1. Correspondence to Hooman Soleymani majd; hooman.soleymani{at}ouh.nhs.uk

Abstract

Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.

  • gynecological cancer
  • vascular surgery

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Footnotes

  • PC and BE contributed equally.

  • Contributors PC and BE performed literature review and primary composition of manuscript. SS performed senior review and provided clinical patient care. HSM proposed the article, performed senior review and provided clinical patient care.

  • 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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