Low-grade fibromyxoid sarcoma (LGFMS; aka Evans tumor) is a very unusual low grade sarcoma with fibrous and myxoid stroma. It has several important clinical and histologic features that I discuss in detail in this video. Also discussed is hyalinizing spindle cell tumor with giant rosette, which is a unique morphologic subtype of LGFMS (click here: 27:55).
MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma (https://pubmed.ncbi.nlm.nih.gov/21415703/ Note that monophasic synovial sarcoma can be MUC4 positive, too).
Despite the similar name, low-grade fibromyxoid sarcoma is UNRELATED to myxofibrosarcoma. See my FIBROmyxo vs MYXOfibro Sarcoma Made Simple video: https://kikoxp.com/posts/3705
Additional relevant videos:
- WSI digital slide of LGFMS: https://kikoxp.com/posts/6806
- Another LGFMS video: https://kikoxp.com/posts/9852
- LGFMS Sample Pathology Report Template: https://kikoxp.com/posts/9926
- Myxofibrosarcoma (full video): https://kikoxp.com/posts/4190
- Perineurioma (vs LGFMS at 14:50): https://kikoxp.com/posts/3694
Additional suggested reading:
Evans HL. Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up.: https://www.ncbi.nlm.nih.gov/pubmed/21921785
Thway K, Fisher C, Debiec-Rychter M, Calonje E. Claudin-1 is expressed in perineurioma-like low-grade fibromyxoid sarcoma. https://www.ncbi.nlm.nih.gov/pubmed/19540561
Billings SD, Giblen G, Fanburg-Smith JC. Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population: https://www.ncbi.nlm.nih.gov/pubmed/15644777
Sedrak MP, Parker DC, Gardner JM. Low-grade fibromyxoid sarcoma with nuclear pleomorphism arising in the subcutis of a child. https://www.ncbi.nlm.nih.gov/pubmed/24188507
Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL. MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. https://www.ncbi.nlm.nih.gov/pubmed/21415703
A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: https://kikoxp.com/posts/5084 (dermpath) & https://kikoxp.com/posts/5083 (bone/soft tissue sarcoma pathology).
Please check out my Soft Tissue Pathology & Dermatopathology survival guide textbooks: http://bit.ly/2Te2haB
This video is geared towards medical students, pathology or dermatology residents, or practicing pathologists or dermatologists. Of course, this video is for educational purposes only and is not formal medical advice or consultation.
Presented by Jerad M. Gardner, MD. Please subscribe to my channel to be notified of new pathology teaching videos.
Follow me on:
Snapchat: JMGardnerMD
Twitter: @JMGardnerMD
Instagram: @JMGardnerMD
Kiko: https://kikoxp.com/profile/jerad_gardner1/content?filter=popular
Facebook: https://www.facebook.com/JMGardnerMD/
MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma (https://pubmed.ncbi.nlm.nih.gov/21415703/ Note that monophasic synovial sarcoma can be MUC4 positive, too).
Despite the similar name, low-grade fibromyxoid sarcoma is UNRELATED to myxofibrosarcoma. See my FIBROmyxo vs MYXOfibro Sarcoma Made Simple video: https://kikoxp.com/posts/3705
Additional relevant videos:
- WSI digital slide of LGFMS: https://kikoxp.com/posts/6806
- Another LGFMS video: https://kikoxp.com/posts/9852
- LGFMS Sample Pathology Report Template: https://kikoxp.com/posts/9926
- Myxofibrosarcoma (full video): https://kikoxp.com/posts/4190
- Perineurioma (vs LGFMS at 14:50): https://kikoxp.com/posts/3694
Additional suggested reading:
Evans HL. Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up.: https://www.ncbi.nlm.nih.gov/pubmed/21921785
Thway K, Fisher C, Debiec-Rychter M, Calonje E. Claudin-1 is expressed in perineurioma-like low-grade fibromyxoid sarcoma. https://www.ncbi.nlm.nih.gov/pubmed/19540561
Billings SD, Giblen G, Fanburg-Smith JC. Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population: https://www.ncbi.nlm.nih.gov/pubmed/15644777
Sedrak MP, Parker DC, Gardner JM. Low-grade fibromyxoid sarcoma with nuclear pleomorphism arising in the subcutis of a child. https://www.ncbi.nlm.nih.gov/pubmed/24188507
Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL. MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. https://www.ncbi.nlm.nih.gov/pubmed/21415703
A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: https://kikoxp.com/posts/5084 (dermpath) & https://kikoxp.com/posts/5083 (bone/soft tissue sarcoma pathology).
Please check out my Soft Tissue Pathology & Dermatopathology survival guide textbooks: http://bit.ly/2Te2haB
This video is geared towards medical students, pathology or dermatology residents, or practicing pathologists or dermatologists. Of course, this video is for educational purposes only and is not formal medical advice or consultation.
Presented by Jerad M. Gardner, MD. Please subscribe to my channel to be notified of new pathology teaching videos.
Follow me on:
Snapchat: JMGardnerMD
Twitter: @JMGardnerMD
Instagram: @JMGardnerMD
Kiko: https://kikoxp.com/profile/jerad_gardner1/content?filter=popular
Facebook: https://www.facebook.com/JMGardnerMD/
Level 1 Guru | Resident
November 25, 2021 at 4:49am