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Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon mesenchymal tumor usually arising in the lower extremities and trunk. Only rare examples in the head and neck region have been reported. Fifteen cases of head and neck LGFMS were retrieved. MUC4 was performed on all cases. Results for smooth muscle actins, β-catenin, desmin, S100 protein, Epithelial membrane antigen (EMA) and STAT6 immunohistochemistry, as well as FUS rearrangement status, were recorded when available. Sites included neck (8), supraclavicular region (4) and orbit (1), parapharyngeal space (1) and lower lip (1). The age of the patients ranged from 3 to 97 years (median, 26 years). Tumors displayed classical morphologic features of LGFMS, as described. All cases (15/15) were positive for MUC4, and all cases tested (4/4) harbored FUS rearrangement. Variable positivity for EMA was identified in one case. Follow-up was available in 11 patients, ranging from 2 to 240 months (mean 71.4 months; median, 44 months). Three tumors recurred locally; none metastasized. In conclusion, although distinctly uncommon, LGFMS may arise in the head and neck region and should be distinguished from other more common spindle cell tumors in these locations. The morphologic, immunohistochemical and molecular genetic features of head/neck LGFMS are identical to those occurring elsewhere. The long-term metastatic risk of LGFMS in these locations remains to be fully elucidated.

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References

  1. Doyle LA, Mertens F. Low-grade fibromyxoid sarcoma. In World Health Organisation Classification of Soft Tissue and Bone Tumours, 5th edn; WHO Classification of Tumours Editorial Board, IARC Press, Lyon, France, 2019; pp. 127–29.

  2. Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJF, Fletcher CDM, Hornick JL. MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement. Am J Surg Pathol. 2012;36:1444–51.

    Article  Google Scholar 

  3. Mertens F, Fletcher CDM, Antonescu CR, Coindre JM, Colecchia M, Domanski HA, Downs-Kelly E, Fisher C, Goldblum JR, et al. Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene. Lab Invest. 2005;85:408–15.

    CAS  Article  Google Scholar 

  4. Ud Din N, Ahmad Z, Zreik R, Horvai A, Folpe AL, Fritchie F. Abdominopelvic and retroperitoneal low-grade fibromyxoid sarcoma: a clinicopathologic study of 13 cases. Am J Clin Pathol. 2018;29:128–34.

    Article  Google Scholar 

  5. Evans HL. Low-grade fibromyxoid sarcoma. A report of 12 cases. Am J Surg Pathol. 1993;17:595–600.

    CAS  Article  Google Scholar 

  6. Papadimitriou JC, Ord RA, Drachenberg CB. Head and neck fibromyxoid sarcoma: clinicopathological correlation with emphasis on peculiar ultrastructural features related to collagen processing. Ultrastruct Pathol. 1997;21:81–7.

    CAS  Article  Google Scholar 

  7. Lane KL, Shannon RJ, Weiss SW. Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low grade fibromyxoid sarcoma. Am J Surg Pathol. 1997;21:1481–8.

    CAS  Article  Google Scholar 

  8. Folpe AL, Lane KL, Paull G, Weiss SW. Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol. 2000;24:1353–60.

    CAS  Article  Google Scholar 

  9. Zamecnık M, Michal M. Low-grade fibromyxoid sarcoma: a report of eight cases with histologic, immunohistochemical, and ultrastructural study. Ann Diagn Pathol. 2000;4(4):207–17.

    Article  Google Scholar 

  10. Botev B, Casale M, Vincenzi B, D’Ascanio L, Santini D, Esposito V, Di Marino MP, Baldi A, Rinaldi V, Tonini G, Salvinelli F. A giant sarcoma of the parotid gland: a case report and review of the literature. Vivo. 2006;20:907–10.

    Google Scholar 

  11. Guillou L, Benhattar J, Gengler C, Gallagher G, Ranchère-Vince D, Collin F, Terrier P, Terrier-Lacombe MJ, Leroux A, et al. Translocation-positive low-grade fibromyxoid sarcoma: clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: a study from the French Sarcoma Gro. Am J Surg Pathol. 2007;31:1387–402.

    Article  Google Scholar 

  12. Marglani O, Commons S, Lamothe A. Radiation-induced low grade fibromyxoid sarcoma of the sternocleidomastoid muscle. J Otolaryngol. 2007;36:E73–5.

    PubMed  Google Scholar 

  13. Merchant SH. Low grade fibromyxoid sarcoma: a report of a case with epithelioid cell morphology, masquerading as a papillary thyroid carcinoma. Acta Cytol. 2009;53:689–92.

    Article  Google Scholar 

  14. Wu X, Petrovic V, Torode IP, Chow CW. Low grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance. Pathology. 2009;41:155–60.

    CAS  Article  Google Scholar 

  15. Tang Z, Zhou ZH, Lv CT, Qin LY, Wang Y, Tian G, Luo XL, Zhu Q, Xu XG. Low-grade fibromyxoid sarcoma: clinical study and case report. J Oral Maxillofac Surg. 2010;68:873–84.

    Article  Google Scholar 

  16. Rekhi B, Deshmukh M, Jambhekar NA. Low-grade fibromyxoid sarcoma: a clinicopathologic study of 18 cases, including histopathologic relationship with sclerosing epithelioid fibrosarcoma in a subset of cases. Ann Diagn Pathol. 2011;15:303–11.

    Article  Google Scholar 

  17. Evans HL. Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up. Am J Surg Pathol. 2011;35:1450–62.

    Article  Google Scholar 

  18. Abe Y, Hashimoto I, Nakanishi H. Recurring facial low-grade fibromyxoid sarcoma in an elderly patient: a case report. J Med Invest. 2012;59:266–9.

    Article  Google Scholar 

  19. He KF, Jia J, Zhao YF. Low-grade fibromyxoid sarcoma with cystic appearance and osseous metaplasia in the cheek: a case report and review of the literature. J Oral Maxillofac Surg. 2013;71:1143–50.

    Article  Google Scholar 

  20. Maretty-Nielsen K, Baerentzen S, Keller J, Dyrop HB, Safwat A. Low-grade fibromyxoid sarcoma: incidence, treatment strategy of metastases, and clinical significance of the FUS gene. Sarcoma. 2013. https://doi.org/10.1155/2013/256280.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Dong W, Zhang H. Low-grade fibromyxoid sarcoma of the thyroid: a case report. Ann Acad Med Singap. 2013;42:55–6.

    PubMed  Google Scholar 

  22. Lee EJ, Hwang HJ, Byeon HK, Park HS, Choi HS. A low grade fibromyxoid sarcoma masseter muscle: a case report. J Med Case Rep. 2015;9:176.

    Article  Google Scholar 

  23. Soma S, Bhat S, Shetty SK. Low grade fibromyxoid sarcoma of the palate: a case report. J Clin Diagn Res. 2015;9:XD01_2.

    Google Scholar 

  24. Mastoraki A, Strigkos T, Tatakis FP, Christophi A, Smyrniotis V. Recurrent low-grade fibromyxoid sarcoma of the neck: report of a case and review of the literature. Indian J Surg Oncol. 2015;6:296–9.

    Article  Google Scholar 

  25. Chaudhuri K, Kasimsetty CR, Lingappa A, Gujjar PV. Low-grade fibromyxoid sarcoma involving the mandible: a diagnostic dilemma. J Oral Maxillofac Pathol. 2016;20:33. https://doi.org/10.4103/0973-029X.185914.

    Article  Google Scholar 

  26. Spalthoff S, Bredt M, Gellrich NC, Jehn P. A rare pathology: low-grade fibromyxoid sarcoma of the maxilla. J Oral Maxillofac Surg. 2016;74(219):e1-10. https://doi.org/10.1016/j.joms.2015.09.018.

    Article  Google Scholar 

  27. Tatari MM, Elhariti L, Abou-elfadl M, Abada RL, Rouadi S, Roubal M, Mahtar M. Low grade fibromyxoid sarcoma of the neck: a case report. Ann Clin Case Rep. 2016;1:1056.

    Google Scholar 

  28. Cowan ML, Thompson LD, Leon ME, Bishop JA. Low-grade fibromyxoid sarcoma of the head and neck: a clinicopathologic series and review of the literature. Head Neck Pathol. 2016;10:161–6.

    Article  Google Scholar 

  29. Rao R, Honavar SG, Mulay K, Reddy VA. Primary orbital low-grade fibromyxoid sarcoma – A case report. Indian J Ophthalmol. 2019;67:568–70.

    Article  Google Scholar 

  30. Kumari K, Thota R, Chaudhary HL, Sharma MC, Thakar A, Singh G. Low-grade fibromyxoid sarcoma of the external auditory canal: a rare pathology and unusual location. Head Neck Pathol. 2019. https://doi.org/10.1007/s12105019010304.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Pellini R, De Virgilio A, Petruzzi G, Pichi B, Mercante G, Malvezzi L, Vidiri A, Spriano G. Low-grade fibromyxoid sarcoma of the tongue: a rare nosological entity. Otorinolaringologia. 2019;69:188–91.

    Article  Google Scholar 

  32. Kanato T, Kalyani S, Lailyang T, Santosh D, Rebecca T, Charai H. Low grade fibromyxoid sarcoma in oral cavity: a rare case report. Indian J Otolaryngol Head Neck Surg. 2019;71(Suppl1):25–6.

    CAS  Article  Google Scholar 

  33. Chetverikova E, Kasenõmm P. Low-grade fibromyxoid sarcoma of the lateral skull base: presentation of two cases. Case Rep Otolaryngol. 2019;2019:7917040. https://doi.org/10.1155/2019/7917040.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Park JM, Lim HR, Kim JH, Dong Lee DH. Giant low-grade fibromyxoid sarcoma in the neck. Korean J Otorhinolaryngol Head Neck Surg. 2020;63:432–5.

    Article  Google Scholar 

  35. Koucky V, Kalferta D, Novakovab DK, Plzaka J. Low-grade fibromyxoid sarcoma of the maxillary sinus. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2020. https://doi.org/10.5507/bp.2020.032.

    Article  PubMed  Google Scholar 

  36. Toro C, Costa P, Vecchio GM, Magro G. Low-grade fibromyxoid sarcoma of the parapharyngeal space: a case report and review of the literature. Oral Maxillofac Surg Cases. 2020;6:100152. https://doi.org/10.1016/j.omsc.2020.100152.

    Article  Google Scholar 

  37. Deewani H, Suhail A, Uddin N. Low grade fibromyxoid sarcoma of the parapharyngeal space- An unusual location. BMJ Case Rep. 2021;14(5):e237083. https://doi.org/10.1136/bcr-2020-237083.

    Article  PubMed  Google Scholar 

  38. Doblan A. Low-grade fibromyxoid sarcomas with the maxillary sinus localization: a case report and review of the literature. Indian J Otolaryngol Head Neck Surg. 2021. https://doi.org/10.1007/s12070-021-02562-4.

    Article  Google Scholar 

  39. Taverna C, Baněčková M, Lorenzon M, Palomba A, Franchi A, Skalova A, Agaimy A. MUC4 is a valuable marker for distinguishing secretory carcinoma of the salivary glands from its mimics. Histopathology. 2020. https://doi.org/10.1111/his.14251.

    Article  PubMed  Google Scholar 

  40. Weed DT, Gomez-Fernandez C, Bonfante E, Lee TD, Pacheco J, Carvajal ME, Goodwin WJ, Carraway KL. MUC4 (sialomucin complex) expression in salivary gland tumors and squamous cell carcinoma of the upper aerodigestive tract. Otolaryngol Head Neck Surg. 2001;124(2):127–41.

    CAS  Article  Google Scholar 

  41. Lacambra MD, Weinreb I, Demicco EG, Chow C, Sung YS, Swanson D, To KF, Wong KC, Antonescu CR, Dickson BC. PRRX-NCOA1/2 rearrangement characterizes a distinctive fibroblastic neoplasm. Genes Chromosomes Cancer. 2019;58:705–12.

    CAS  Article  Google Scholar 

  42. Dermawan JK, Azzato EM, Thangaiah JJ, Gjorgova-Gjeorgievski S, Rubin BP, Folpe AL, Agaimy A, Fritchi KJ. PRRX1-NCOA1 rearranged fibroblastic tumor: clinicopathologic, immunohistochemical and molecular genetic study of six cases of a potentially under-recognized, distinctive mesenchymal tumor. Histopathology. 2021. https://doi.org/10.1111/his.14454.

    Article  PubMed  Google Scholar 

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Gjorgova Gjeorgjievski, S., Fritchie, K., Thangaiah, J.J. et al. Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases. Head and Neck Pathol 16, 434–443 (2022). https://doi.org/10.1007/s12105-021-01380-y

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Keywords

  • Low-grade fibromyxoid sarcoma
  • Head and neck
  • Supraclavicular
  • Orbit
  • Lip
  • Parapharyngeal space
  • MUC4