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.
Keywords: Oncology; Pathology.
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