Epidemiology

Solitary fibrous tumours (SFTs) are exceedingly rare types of sarcomas (3.7% of soft-tissue sarcomas). These tumours can arise in the serous membranes, the meningeal dura, and the deep soft tissues.

Distribution of Disease

30% pleural, 30% abdominal, 20% head and neck including the meninges, and 20% in other sites (bones, deep soft truncal or limb tissues).

Pathophysiology

Characteristic genetic defect: 12q13 inversion resulting in fusion of NAB2 and STAT6

Clinical Presentation

Patients typically present with a palpable mass. These can be of variable growth, from indolent to highly aggressive and frankly malignant.

Less than 5% of patients can present with refractory paraneoplastic Hypoglycemia, called Doege-Potter syndrome, which was originally described in 1930 in the Annals of Surgery.

Management of SFT

  1. Surgical resection with wide negative margins
  2. Radiation therapy, either neoadjuvant or adjuvant or palliative
  3. Systemic chemotherapy in the setting of incurable metastatic SFT

References

  1. https://www-nejm-org.libaccess.lib.mcmaster.ca/doi/pdf/10.1056/NEJMcpc2300899