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Superficial low-grade fibromyxoid sarcoma.

Shira Ronen¹, Jennifer S Ko¹, Brian P Rubin¹

¹Department of Pathology, Cleveland Clinic, Cleveland, Ohio, USA.

Journal of Cutaneous Pathology

|September 8, 2022

Summary

Superficial low-grade fibromyxoid sarcoma (LGFMS) is more common in children and young adults, often affecting extremities. These superficial tumors appear to have a better prognosis than deep-seated LGFMS.

Keywords:

Evans tumor immunohistochemistry low-grade fibromyxoid sarcoma skin neoplasms superficial

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Area of Science:

Oncology
Pathology
Surgical Pathology

Background:

Low-grade fibromyxoid sarcoma (LGFMS) typically affects deep soft tissues, with a high rate of recurrence, metastasis, and mortality.
Previous studies suggest superficial LGFMS may be more prevalent in pediatric populations and potentially have a better prognosis.

Purpose of the Study:

To confirm that superficial LGFMS is more common in children and young adults.
To increase awareness of superficial LGFMS and its potential for a better clinical outcome.

Main Methods:

Retrospective review of superficial LGFMS cases diagnosed between 2008 and 2020.
Review of available slides and clinical data, including follow-up information.

Main Results:

The study included 23 patients (9 males, 14 females) with a median age of 29 years; 35% were children and 22% were young adults.
Most tumors (65%) involved the lower extremities and were primarily located in the subcutis (91%) and dermis (9%).
All tumors exhibited typical LGFMS features and were positive for MUC4 and/or FUS rearrangement. Follow-up in 14 cases showed no recurrences or metastases.

Conclusions:

Superficial LGFMS is more frequently observed in the extremities of children and young adults compared to deep-seated LGFMS.
Superficial LGFMS may present with a more favorable clinical outcome, though further long-term studies are warranted.