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Case report
Isolated sporadic uterine lymphangioleiomyoma with unusual clinical and pathological features
  1. Sebastian Leathersich1,2,
  2. Mei Hui Eleanor Koay3,
  3. Alireza Khani3 and
  4. Sangeeta Malla Bhat2
  1. 1 Department of Obstetrics and Gynaecology, King Edward Memorial Hospital for Women, Subiaco, Western Australia, Australia
  2. 2 Department of Obstetrics and Gynaecology, Armadale Health Service, Armadale, Western Australia, Australia
  3. 3 Department of Anatomical Pathology, PathWest Laboratory Medicine Western Australia, Subiaco, Western Australia, Australia
  1. Correspondence to Dr Sebastian Leathersich; sebastian.leathersich{at}


We report a unique uterine neoplasm, favoured to represent an isolated extrapulmonary lymphangioleiomyoma with unusual pathological features, in a postmenopausal woman without tuberous sclerosis complex. The large neoplasm consisted of smooth muscle fascicles and cystic spaces lined by lymphatic cells, which were negative for the melanocytic staining that is characteristically positive in lymphangioleiomyomatosis (LAM). There are fewer than 30 cases of uterine LAM reported, none of which have demonstrated this morphology or these immunohistochemical findings. The origin of LAM cells in the more typical pulmonary LAM remains unclear; the unusual features in this case may represent a distinct pathological entity or a rare variant of typical extrapulmonary LAM, and may contribute to determining the cellular origin of these rare tumours. Conversely, this may represent a case of ‘prepulmonary’ LAM, providing supporting evidence for a possible gynaecological origin of these tumours in the broader affected (almost exclusively female) population.

  • obstetrics and gynaecology
  • respiratory medicine
  • pathology
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  • Contributors SL conceived and drafted the original version under the supervision of SMB. MHEK and AK reported the pathological findings and contributed to the final manuscript. The final version was reviewed and edited by all the authors.

  • 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 Obtained.

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

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