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Oncology
Diffuse intra-abdominal sarcomatosis in myxoid pleomorphic liposarcoma
  1. William W Tseng1,
  2. Yu Liang2,
  3. Bao Nguyen3,
  4. Mark Agulnik4 and
  5. David Creytens5
  1. 1Surgery, City of Hope National Medical Center, Duarte, California, USA
  2. 2Pathology, City of Hope National Medical Center, Duarte, California, USA
  3. 3Advanced Imaging, City of Hope National Medical Center, Duarte, California, USA
  4. 4Medical Oncology and Therapeutics, City of Hope National Medical Center, Duarte, California, USA
  5. 5Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium
  1. Correspondence to Dr William W Tseng; wtseng{at}coh.org

Abstract

We present a case of an extremely rare type of soft-tissue sarcoma with an atypical clinical presentation. The patient, a female in her 20s with Li Fraumeni syndrome, had prior surgery for a large intra-abdominal tumour that was given the diagnosis of malignant myxoid spindle cell neoplasm. Her recurrence manifested as diffuse intra-abdominal sarcomatosis for which she ultimately underwent subtotal debulking with palliative intent. Final pathology rendered the diagnosis of myxoid pleomorphic liposarcoma, a newly described entity, distinct from the more common liposarcoma subtypes. The optimal treatment for this typically aggressive disease is currently unknown; until that is better defined, management should be carried out by sarcoma specialists.

  • Oncology
  • Pathology

https://doi.org/10.1136/bcr-2023-258407

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    Footnotes

    • Contributors All authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content. All authors gave final approval of the manuscript.

    • Funding Liposarcoma research by WWT is in part supported by the Wendy Walk Foundation

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

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