Access Keys:
Skip to content (Access Key - 0)

Myofibroblastic Sarcoma



  •  Propensity for local recurrence and associated with a low risk of metastatic spread



  • Radiographs may reveal osseous erosion
  • MRI
    • Hypointense on T1
    • Hyperintense on T2
    • SUV reported 9.8
      • Found incidentally using FDG-PET as a cancer screening tool


  •  Firm pale white mass with ill-defined margins



  • Nodular fasciitis
  • Fibrosarcoma
  • Aggressive fibromatosis
  • Calcifying fibrous pseudotumor
  • Inflammatory myofibroblastic tumor
  • Epithelioid sarcoma
  • Myofibroblastic differentiation of other sarcomas



  • Fibroblastic and Myofibroblastic Sarcoma

    • Spindle-cell sarcoma showing fibroblastic-myofibroblastic differentiation with a predominance of fibroblasts has been reported in the skin
  • High-grade Myofibroblastic Sarcoma

    • Has been reported arising from the pericardium
      • LR treated with chemotherapy (doxorubicin/ifosfamide) and XRT with 6 month disease free survival reported
    • Has been reported associated with XRT (postradiation high-grade myofibroblastic sarcoma)
  • Epithelioid Inflammatory Myofibroblastic Sarcoma

    • See Inflammatory Myofibroblastic Tumor (IMT)
  • Vaccination Associated Myofibroblastic Sarcoma

    • Reported 4 months after Rabies vaccination with multiple LRs
  • Animal Model

    • Has been reported in the maxillary region in a dog
    • Has been reported in the orbit of a cat
    • Has been reported at the second site of an equine influenza vaccination in a quarterhorse shortly after the vaccination
      • Associated with multifocal osseous metaplasia
    • Has been reported in the pleura of a pig


Agaimy A, Wünsch PH, Schroeder J, Gaumann A, Dietmaier W, Hartmann A, Hofstaedter F, Mentzel T. Low-grade abdominopelvic sarcoma with myofibroblastic features (low-grade myofibroblastic sarcoma): clinicopathological, immunohistochemical, molecular genetic and ultrastructural study of two cases with literature review. J Clin Pathol. 2008 Mar;61(3):301-6. Epub 2007 May 18.

Agostini T, Mori A, Leporatti G, Dini M, Franchi A. Cutaneous carcinosarcoma: report of a case with mylfibroblastic sarcomatous component. Dermatol Surg. 2008 Mar;34(3):418-22; discussion 422.

Artopoulou II, Lemon JC, Clayman GL, Chambers MS. Stent fabrication for graft immobilization following wide surgical excision of myofibroblastic sarcoma of the buccal mucosa: a clinical report. J Prosthet Dent. 2006 Apr;95(4):280-5.
Bell CM, Schwarz T, Dubielzig RR. Diagnostic features of feline restrictive orbital myofibroblastic sarcoma. Vet Pathol. 2011 May;48(3):742-50.

Bisceglia M, Magro G. Low-grade myofibroblastic sarcoma of the salivary gland. 1999;23:1435-6.

Biselli R, Boldrini R, Ferlini C, Boglino C, Inserra A, Bosman C. Myofibroblastic tumours: neoplasias with divergent behaviour. Ultrastructural and flow cytometric analysis. Pathol Res Pract. 1999;195(9):619-32. Pubmed

Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK, Lee TJ, Ro JY, Silverman JS. A case of cutaneous low-grade myofibroblastic sarcoma. J Dermatol. 2001 Jul;28(7):383-7.
Chiller K, Parker D, Washington C. Myofibrosarcoma treated with Mohs micrographic surgery. Dermatol Surg. 2004 Dec;30(12 Pt 2):1565-7.
Covello R, Licci S, Pichi B, Spriano G, Vidiri A, Morelli L, Rosenberg AE. Low-grade myofibroblastic sarcoma of the larynx. Int J Surg Pathol. 2011 Jan 12.

Coyne JD. Low-grade myofibroblastic sarcoma of the piriform fossa: a case report with a literature review of a tumour with a predilection for the head and neck. Br J Oral Maxillofac Surg. 2007 Jun;45(4):335-7.

Demarosi F, Bay A, Moneghini L, Carrassi A. Low-grade myofibroblastic sarcoma of the oral cavity. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009 Aug;108(2):248-54.

Dhillon G, Rodríguez-Cruz E, Kathawala M, Alqassem N. Primary cardiac myofibroblastic sarcoma. Case report and review of diagnosis and treatment of cardiac tumors. Bol Asoc Med P R. 1998 Jul-Dec;90(7-12):130-3.
Diaz-Cascajo C, Borghi S, Weyers W, Metze D. Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases. J Cutan Pathol. 2003 Feb;30(2):128-34.
Eisenstat J, Gilson T, Reimann J, Sampson B. Low-grade myofibroblastic sarcoma of the heart causing sudden death. Cardiovasc Pathol. 2008 Jan-Feb;17(1):55-9.

Dobashi Y, Noguchi T, Nasuno S, Jiang SX, Kameya T. Hyalinizing spindle cell tumor with giant rosettes: report of a case showing remarkable myofibroblastic differentiation. Pathol Res Pract. 2001;197(10):691-7.

Donner LR, Trompler RA, White RR 4th. Progression of inflammatory myofibroblastic tumor (inflammatory pseudotumor) of soft tissue into sarcoma after several recurrences. Hum Pathol. 1996 Oct;27(10):1095-8.
Dubielzig RR, Hawkins KL, Miller PE. Myofibroblastic sarcoma originating at the site of rabies vaccination in a cat. J Vet Diagn Invest. 1993 Oct;5(4):637-8. Pubmed

Eyden B. The myofibroblast: a study of normal, reactive and neoplastic tissues, with an emphasis on ultrastructure. Part 2 - tumours and tumour-like lesions. J Submicrosc Cytol Pathol. 2005 Nov;37(3-4):231-96.

Fisher C. Low-grade sarcomas with CD34-positive fibroblasts and low-grade myofibroblastic sarcomas. Ultrastruct Pathol. 2004 Sep-Dec;28(5-6):291-305.

Fisher C. Myofibroblastic malignancies. Adv Anat Pathol. 2004 Jul;11(4):190-201. Pubmed

Fisher C. Myofibrosarcoma. Virchows Arch. 2004 Sep;445(3):215-23. Pubmed

Fletcher CD. Myofibroblastic tumours: an update. Verh Dtsch Ges Pathol. 1998;82:75-82. Pubmed

Fletcher CD, Dal Cin P, de Wever I, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Sciot R, Tallini G, van den Berghe H, Vanni R, Willen H. Correlation between clinicopathological features and karyotype in spindle cell sarcomas. A report of 130 cases from the CHAMP study group. Am J Pathol 1999;154:1841-7.

González-Cámpora R, Escudero AG, Ríos Martín JJ, Armas Padrón JR, Vázquez AH, Vázquez Ramírez FJ. Myofibrosarcoma (low-grade myofibroblastic sarcoma) with intracytoplasmic hyaline (fibroma-like) inclusion bodies. Ultrastruct Pathol. 2003 Jan-Feb;27(1):7-11.

Goodlad JR, Mentzel T, Fletcher CD. Low grade fibromyxoid sarcoma: clinicopathological analysis of eleven new cases in support of a distinct entity. Histopathology. 1995 Mar;26(3):229-37. Pubmed

Grisaru D, Peyser MR, Bernstein-Lipschitz L, Yaron Y, Lessing JB. Malignant fibrous histiocytoma with myofibroblastic differentiation of the vulva in an adolescent female. Eur J Obstet Gynecol Reprod Biol. 1998 Aug;79(2):219-21.
Jay A, Piper K, Farthing PM, Carter J, Diwakar A. Low-grade myofibroblastic sarcoma of the tongue. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Nov;104(5):e52-8.

Kacerovska D, Michal M, Kutzner H, Rychnovsky J, Kazakov DV. Metastatic desmoplastic malignant melanoma associated with low-grade myofibroblastic sarcoma. Am J Dermatopathol. 2009 Jul;31(5):490-4.

Köktener A, Dilmen G. Endobronchial myofibroblastic sarcoma presenting with hemoptysis in a patient with a normal chest x-ray. South Med J. 2004 Mar;97(3):314-5.
Kolli S, Ziaee A, Lee R, Lim MJ, Levy B, Labovitz AJ. Myofibroblastic sarcoma of mitral valve: a case report. J Am Soc Echocardiogr. 2005 Mar;18(3):285-6.
Kondo S, Yoshizaki T, Minato H, Horikawa I, Tatsumi A, Furukawa M. Myofibrosarcoma of the nasal cavity and paranasal sinus. Histopathology 2001;39:216-7.

Kubota Y, Ishikawa Y, Shibahara T, Kadota K. Myofibroblastic sarcoma of the pleura in a pig. J Vet Med Sci. 2000 Aug;62(8):913-6.
Lach B, Benoit BG. Myofibroblastic sarcoma in meningioma: a new variant of "metaplastic" meningioma. Ultrastruct Pathol. 2007 Sep-Oct;31(5):357-63. Erratum in: Ultrastruct Pathol. 2007 Nov-Dec;31(6):409.

Laco J, Simáková E, Slezák R, Tucek L, Mottl R, Spacek J, Ryska A. Low grade myofibroblastic sarcoma of tongue: a case report. Cesk Patol. 2006 Jul;42(3):150-3.

Lagacé R, Seemayer TA, Gabbiani G, Schürch W. Myofibroblastic sarcoma. Am J Surg Pathol. 1999 Nov;23(11):1432-5. Pubmed

Lioulias A, Misthos P, Neofotistos K, Legaki S. Primary myofibrosarcoma of the lung. A rare case with challenging diagnosis. J BUON. 2009 Jan-Mar;14(1):143-5.

Lucin K, Musta? E, Jonji? N. Breast sarcoma showing myofibroblastic differentiation. Virchows Arch. 2003 Aug;443(2):222-4.

Mariño-Enríquez A, Wang WL, Roy A, Lopez-Terrada D, Lazar AJ, Fletcher CD, Coffin CM, Hornick JL. Epithelioid inflammatory myofibroblastic sarcoma: An aggressive intr-abdominal variant of inflammatory myofibroblastic tumor with nuclear membrane or perinuclear ALK. Am J Surg Pathol. 2011 Jan;35(1):135-44. Pubmed

Meng GZ, Zhang HY, Bu H, Yang GH, Zhang XL, Yang G. Myofibroblastic sarcoma of the nasal cavity and paranasal sinus: a clinicopathologic study of 6 cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Oct;104(4):530-9.

Meng GZ, Zhang HY, Bu H, Zhang XL, Pang ZG, Ke Q, Liu X, Yang G. Myofibroblastic sarcomas: a clinicopathological study of 20 cases. Chin Med J (Engl). 2007 Mar 5;120(5):363-9.
Meng GZ, Zhang HY, Zhang Z, Wei B, Bu H. Myofibroblastic sarcoma vs. nodular fasciitis: a comparative study of chromosomal imbalances. Am J Clin Pathol. 2009 May;131(5):701-9.
Mentzel T. Myofibroblastic sarcomas: a brief review of sarcomas showing a myofibroblastic line of differentiation and discussion of the differential diagnosis. Curr Diagn Pathol 2001;7:17-24.

Mentzel T, Dry S, Katenkamp D, Fletcher CD. Low-grade myofibroblastic sarcoma: analysis of 18 cases in the spectrum of myofibrloblastic tumors. Am J Surg Pathol 1998;22:1228-38. Pubmed

Mentzel T, Fletcher JA. Low grade myofibroblastic sarcoma. In Pathology and Genetics of Tumours of Soft Tissue and Bone. Fletcher CDM, Unni, KK, Mertens F (eds). IARC Press, Lyon, 2002, pp. 94-5.
Miyazawa M, Naritaka Y, Miyaki A, Asaka S, Isohata N, Yamaguchi K, Murayama M, Shimakawa T, Katsube T, Ogawa K, Fujibayashi M. A low-grade myofibroblastic sarcoma in the abdominal cavity. Anticancer Res. 2011 Sep;31(9):2989-94.
Montgromery E, Goldblum JR, Fisher C. Myofibrosarcoma: a clinicopathologic study. Am J Surg Pathol 2001;25:219-28.

Morgan PB, Chundru S, Hatch SS, Hawkins HK, Adegboyega PA, Eltorky MA. Uncommon malignancies: case 1. Low-grade myofibroblastic sarcoma of the breast. J Clin Oncol. 2005 Sep 1;23(25):6249-51.

Morii T, Mochizuki K, Sano H, Fujino T, Harasawa A, Satomi K. Occult myofibroblastic sarcoma detected on FDG-PET performed for cancer screening. Ann Nucl Med. 2008 Nov;22(9):811-5.

Nagata Y, Matsuno T, Hamada N, Shimose S, Arihiro K, Ochi M. Low-grade myofibroblastic sarcoma of the palm. Scand J Plast Reconstr Surg Hand Surg. 2008;42(3):164-7.
Ni C, Xu YY, Zhou SH, Wang SQ. Differential diagnosis of inflammatory myofibroblastic tumour and low-grade myofibroblastic sarcoma: two case reports with a literature review. J Int Med Res. 2011;39(1):311-20.

Qiu X, Montgomery E, Sun B. Inflammatory myofibroblastic tumor and low-grade myofibroblastic sarcoma: a comparative study of clinicopathologic features and further observations on the immunohistochemical profile of myofibroblasts. Hum Pathol. 2008 Jun;39(6):846-56.

Roth TM, Fratkin J, Woodring TC, McGehee RP. Low-grade myofibroblastic sarcoma of the vulva. Gynecol Oncol. 2004 Jan;92(1):361-4.
San Miguel P, Fernández G, Ortiz-Rey JA, Larrauri P. Low-grade myofibroblastic sarcoma of the distal phalanx. J Hand Surg Am. 2004 Nov;29(6):1160-3.

Sirvent N, Hawkins AL, Moeglin D, Coindre JM, Kurzenne JY, Michiels JF, Barcelo G, Turc-Carel C, Griffin CA, Pedeutour F. ALK probe rearrangement in a t(2;11;2)(p23;p15;q31) translocation found in a prenatal myofibroblastic fibrous lesion: toward a molecular definition of an inflammatory myofibroblastic tumor family? Genes Chromosomes Cancer. 2001 May;31(1):85-90.
Smith DM, Mahmoud HH, Jenkins JJ, III, Rao B, Hopkins KP, Parham DM. Myofibrosarcoma of the head and neck in children. Pediatr Pathol Lab Med 1995;15:402-18. Pubmed

Taccagni G, Rovere E, Masullo M, Christensen L, Eyden B. Myofibrosarcoma of the breast: review of the literature on myofibroblastic tumors and criteria for defining myofibroblastic differentiation. Am J Surg Pathol. 1997 Apr;21(4):489-96. Review. Erratum in: Am J Surg Pathol 1997 Jun;21(6):742.

Tajima Y, Sudoh K, Matsumoto A, Kikuchi S, Sasaki H. Femoral neuropathy induced by a low-grade myofibroblastic sarcoma of the groin. J Neurol. 2005 Nov;252(11):1416-7.

Takácsi-Nagy Z, Muraközy G, Pogány P, Fodor J, Orosz Z. Myofibroblastic sarcoma of the base of tongue. Case report and review of the literature. Strahlenther Onkol. 2009 Mar;185(3):198-201.

Takahama A Jr, Nascimento AG, Brum MC, Vargas PA, Lopes MA. Low-grade myofibroblastic sarcoma of the parapharyngeal space. Int J Oral Maxillofac Surg. 2006 Oct;35(10):965-8.

Tavora F, Miettinen M, Fanburg-Smith J, Franks TJ, Burke A. Pulmonary artery sarcoma: a histologic and follow-up study with emphasis on a subset of low-grade myofibroblastic sarcomas with a good long-term follow-up. Am J Surg Pathol. 2008 Dec;32(12):1751-61.
Tsuchiya T, Suzuki K, Hojo Y, Shiraki A, Imaoka M, Shibutani M, Mitsumori K. Low-grade myofibroblastic sarcoma of the maxillary region in a dog. J Comp Pathol. 2011 Oct 11.

Watanabe K, Tanaka M, Takashi K, Yamada H, Tajino T. Fibronexus in low-grade myofibrosarcoma: a case report. Ultrastruct Pathol. 2008 May-Jun;32(3):97-100.


Download: PDF | EPUB

Famous Quote
"What lies behind us and what lies before us are tiny matters compared to what lies within us." Oliver Wendell Holmes
Related Content

Resources for Myofibroblastic Sarcoma and related topics on OrthopaedicsOne.