Faculty, Staff and Student Publications
Publication Date
2-1-2023
Journal
Journal of Cutaneous Pathology
DOI
10.1111/cup.14325
PMID
36074249
PMCID
PMC10091772
PubMedCentral® Posted Date
11-2-2022
PubMedCentral® Full Text Version
Post-print
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.
Keywords
Male, Female, Humans, Soft Tissue Neoplasms, Fibrosarcoma, Immunohistochemistry, Evans tumor, immunohistochemistry, low‐grade fibromyxoid sarcoma, skin neoplasms, superficial
Published Open-Access
yes
Recommended Citation
Ronen, Shira; Ko, Jennifer S; Rubin, Brian P; et al., "Superficial Low-Grade Fibromyxoid Sarcoma" (2023). Faculty, Staff and Student Publications. 3492.
https://digitalcommons.library.tmc.edu/uthgsbs_docs/3492
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