Superficial low-grade fibromyxoid sarcoma

Shira Ronen, Jennifer S. Ko, Brian P. Rubin, Scott E. Kilpatrick, Wei Lien Wang, Alexander J. Lazar, John R. Goldblum, Steven D. Billings

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Background: Low-grade fibromyxoid sarcoma (LGFMS) typically involves deep soft tissue (beneath the fascia) of the proximal extremities and trunk. Long-term follow-up has shown a high rate of local recurrence, metastasis, and death. To the best of our knowledge, there is only one previous large series focusing on superficial LGFMS suggesting superficial tumors are disproportionately more common in children and may have a better prognosis. Our study's primary goals are to confirm these findings and increase general awareness that LGFMS may arise in superficial soft tissue. Methods: We retrieved our cases of superficial LGFMS diagnosed between 2008 and 2020. Available slides were reviewed, and clinical data and follow-up information were obtained. Results: The patients included nine males and 14 females with a median age of 29 years; eight (35%) were children (<18 years) and five (22%) were young adults (18–30 years). The majority involved the lower extremities (65%). The tumors were primarily centered in the subcutis (91%) and dermis (9%). Microscopically, they had typical features of LGFMS with alternating fibrous and myxoid zones composed of bland, slightly hyperchromatic spindled cells. All were positive for MUC4 by immunohistochemistry and/or FUS rearrangement by FISH. Follow-up on 14 cases ranged from 11 to 148 months (median 61 months) with no evidence of recurrences or distant metastases. Conclusions: Compared to conventional deep-seated counterparts, superficial LGFMS is more likely to occur in the extremities of children and young adults and may have a better clinical outcome. Further studies with longer follow-up will likely help support these findings.

Original languageEnglish (US)
Pages (from-to)147-154
Number of pages8
JournalJournal of cutaneous pathology
Volume50
Issue number2
DOIs
StatePublished - Feb 2023

Keywords

  • Evans tumor
  • immunohistochemistry
  • low-grade fibromyxoid sarcoma
  • skin neoplasms
  • superficial

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

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