Benign Fibroblastic and Myofibroblastic Lesions

  • Cyril Fisher
Part of the Atlas of Anatomic Pathology book series (AAP)


A large group of relatively common soft tissue tumors are composed of a mixture of fibroblasts and myofibroblasts. Fibroblasts are slender cells with elongated tapered or serpiginous nuclei and little cytoplasm. Myofibroblasts tend to be plumper and have oval nuclei, each with a small distinct nucleolus, and variable amounts of amphophilic cytoplasm according to the functional state of the cells. Both have contractile and (stromal) secretory properties and can modulate into fibroblasts. Cell margins are poorly defined and tend to blend into adjacent collagen. Nuclear pleomorphism sometimes is a feature in both reactive and neoplastic conditions.


Sclerosing Cholangitis Autoimmune Pancreatitis Nuclear Pleomorphism Serum IgG4 Level Cell Margin 
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Suggested Reading

  1. Abraham SC, Montgomery EA, Giardiello FM, Wu TT. Frequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas. Am J Pathol. 2001;158:1073–8.PubMedCrossRefGoogle Scholar
  2. Al-Daraji WI, Ramsay HM, Ali RB. Storiform collagenoma as a clue for Cowden disease or PTEN hamartoma tumour syndrome. J Clin Pathol. 2007;60:840–2.PubMedCrossRefGoogle Scholar
  3. Antaya RJ, Cajaiba MM, Madri J, Lopez MA, Ramirez MC, Martignetti JA, et al. Juvenile hyaline fibromatosis and infantile systemic hyalinosis overlap associated with a novel mutation in capillary morphogenesis protein-2 gene. Am J Dermatopathol. 2007;29:99–103.PubMedCrossRefGoogle Scholar
  4. Balachandran K, Allen PW, MacCormac LB. Nuchal fibroma. A clinicopathological study of nine cases. Am J Surg Pathol. 1995;19:313–7.PubMedCrossRefGoogle Scholar
  5. Bernstein KE, Lattes R. Nodular (pseudosarcomatous) fasciitis, a nonrecurrent lesion: clinicopathologic study of 134 cases. Cancer. 1982;49:1668–78.PubMedCrossRefGoogle Scholar
  6. Chung EB, Enzinger FM. Proliferative fasciitis. Cancer. 1975;36:1450–8.PubMedCrossRefGoogle Scholar
  7. Coffin CM, Hornick JL, Zhou H, Fletcher CD. Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas. Am J Surg Pathol. 2007;31:410–6.PubMedCrossRefGoogle Scholar
  8. Dal Cin P, Sciot R, De Smet L, Van den Berghe H. Translocation 2;11 in a fibroma of tendon sheath. Histopathology. 1998;32:433–5.PubMedCrossRefGoogle Scholar
  9. Daum O, Vanecek T, Sima R, Curik R, Zamecnik M, Yamanaka S, et al. Reactive nodular fibrous pseudotumors of the gastrointestinal tract: report of 8 cases. Int J Surg Pathol. 2004;12:365–74.PubMedCrossRefGoogle Scholar
  10. Dehner LP, Coffin CM. Idiopathic fibrosclerotic disorders and other inflammatory pseudotumors. Semin Diagn Pathol. 1998;15:161–73.PubMedGoogle Scholar
  11. Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.PubMedCrossRefGoogle Scholar
  12. Dickey GE, Sotelo-Avila C. Fibrous hamartoma of infancy: current review. Pediatr Dev Pathol. 1999;2:236–43.PubMedCrossRefGoogle Scholar
  13. Enzinger FM, Dulcey F. Proliferative myositis. Report of thirty-three cases. Cancer. 1967;20:2213–23.PubMedCrossRefGoogle Scholar
  14. Erickson-Johnson MR, Chou MM, Evers BR, Roth CW, Seys AR, Jin L, et al. Nodular fasciitis: a novel model of transient neoplasia induced by MYH9-USP6 gene fusion. Lab Invest. 2011;91:1427–33.PubMedCrossRefGoogle Scholar
  15. Evans HL. Desmoplastic fibroblastoma. A report of seven cases. Am J Surg Pathol. 1995;19:1077–81.PubMedCrossRefGoogle Scholar
  16. Eyden B, Banerjee SS, Shenjere P, Fisher C. The myofibroblast and its tumours. J Clin Pathol. 2009;62:236–49.PubMedCrossRefGoogle Scholar
  17. Fetsch JF, Miettinen M. Calcifying aponeurotic fibroma: a clinicopathologic study of 22 cases arising in uncommon sites. Hum Pathol. 1998;29:1504–10.PubMedCrossRefGoogle Scholar
  18. Fletcher C, Unni K, Mertens F, editors. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2002.Google Scholar
  19. Giardiello FM, Hamilton SR, Krush AJ, Offerhaus JA, Booker SV, Petersen GM. Nasopharyngeal angiofibroma in patients with familial adenomatous polyposis. Gastroenterology. 1993;105:1550–2.PubMedCrossRefGoogle Scholar
  20. Glad H, Vainer B, Buchwald C, Petersen BL, Theilgaard SA, Bonvin P, et al. Juvenile nasopharyngeal angiofibromas in Denmark 1981–2003: diagnosis, incidence, and treatment. Acta Otolaryngol. 2007;127:292–9.PubMedCrossRefGoogle Scholar
  21. Hicks J, Mierau G. The spectrum of pediatric fibroblastic and myofibroblastic tumors. Ultrastruct Pathol. 2004;28:265–81.PubMedCrossRefGoogle Scholar
  22. Lauer DH, Enzinger FM. Cranial fasciitis of childhood. Cancer. 1980;45:401–6.PubMedCrossRefGoogle Scholar
  23. Liegl B, Fletcher CD. Ischemic fasciitis: analysis of 44 cases indicating an inconsistent association with immobility or debilitation. Am J Surg Pathol. 2008;32:1546–52.PubMedCrossRefGoogle Scholar
  24. Meis JM, Enzinger FM. Proliferative fasciitis and myositis of childhood. Am J Surg Pathol. 1992;16:364–72.PubMedCrossRefGoogle Scholar
  25. Meister P, Buckmann FW, Konrad E. Nodular fasciitis (analysis of 100 cases and review of the literature). Pathol Res Pract. 1978;162:133–65.PubMedCrossRefGoogle Scholar
  26. Mentzel T, Kutzner H. Dermatomyofibroma: clinicopathologic and immunohistochemical analysis of 56 cases and reappraisal of a rare and distinct cutaneous neoplasm. Am J Dermatopathol. 2009;31:44–9.PubMedCrossRefGoogle Scholar
  27. Metcalf JS, Maize JC, LeBoit PE. Circumscribed storiform collagenoma (sclerosing fibroma). Am J Dermatopathol. 1991;13:122–9.PubMedCrossRefGoogle Scholar
  28. Michal M, Fetsch JF, Hes O, Miettinen M. Nuchal-type fibroma: a clinicopathologic study of 52 cases. Cancer. 1999;85:156–63.PubMedCrossRefGoogle Scholar
  29. Miettinen M, Fetsch JF. Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a ­distinctive soft tissue lesion with stellate-shaped fibroblasts. Hum Pathol. 1998;29:676–82.PubMedCrossRefGoogle Scholar
  30. Montgomery EA, Meis JM, Mitchell MS, Enzinger FM. Atypical decubital fibroplasia. A distinctive fibroblastic pseudotumor occurring in debilitated patients. Am J Surg Pathol. 1992;16:708–15.PubMedCrossRefGoogle Scholar
  31. Pampel M, Maier S, Kreczy A, Weirich-Schwaiger H, Utermann G, Janecke AR. Refinement of the GINGF3 locus for hereditary gingival fibromatosis. Eur J Pediatr. 2010;169:327–32.PubMedCrossRefGoogle Scholar
  32. Patchefsky AS, Enzinger FM. Intravascular fasciitis: a report of 17 cases. Am J Surg Pathol. 1981;5:29–36.PubMedCrossRefGoogle Scholar
  33. Perosio PM, Weiss SW. Ischemic fasciitis: a juxta-skeletal fibroblastic proliferation with a predilection for elderly patients. Mod Pathol. 1993;6:69–72.PubMedGoogle Scholar
  34. Pettinato G, Manivel JC, Gould EW, Albores-Saavedra J. Inclusion body fibromatosis of the breast. Two cases with immunohistochemical and ultrastructural findings. Am J Clin Pathol. 1994; 101:714–8.PubMedGoogle Scholar
  35. Price SK, Kahn LB, Saxe N. Dermal and intravascular fasciitis. Unusual variants of nodular fasciitis. Am J Dermatopathol. 1993;15:539–43.PubMedCrossRefGoogle Scholar
  36. Sakamoto A, Yamamoto H, Yoshida T, Tanaka K, Matsuda S, Oda Y, et al. Desmoplastic fibroblastoma (collagenous fibroma) with a specific breakpoint of 11q12. Histopathology. 2007;51:859–60.PubMedCrossRefGoogle Scholar
  37. Shin SJ, Rosen PP. Bilateral presentation of fibroadenoma with digital fibroma-like inclusions in the male breast. Arch Pathol Lab Med. 2007;131:1126–9.PubMedGoogle Scholar
  38. Thomas JE, Moossavi M, Mehregan DR, McFalda WL, Mahon MJ. Juvenile hyaline fibromatosis: a case report and review of the literature. Int J Dermatol. 2004;43:785–9.PubMedCrossRefGoogle Scholar
  39. Thway K, Gibson S, Ramsay A, Sebire NJ. Beta-catenin expression in pediatric fibroblastic and myofibroblastic lesions: a study of 100 cases. Pediatr Dev Pathol. 2009;12:292–6.PubMedCrossRefGoogle Scholar
  40. Thway K, Fisher C, Sebire NJ. Pediatric fibroblastic and myofibroblastic lesions. Adv Anat Pathol. 2012;19:54–65.PubMedCrossRefGoogle Scholar
  41. Yantiss RK, Nielsen GP, Lauwers GY, Rosenberg AE. Reactive nodular fibrous pseudotumor of the gastrointestinal tract and mesentery: a clinicopathologic study of five cases. Am J Surg Pathol. 2003;27:532–40.PubMedCrossRefGoogle Scholar

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© Springer Science+Business Media, LLC 2013

Authors and Affiliations

  • Cyril Fisher
    • 1
  1. 1.Department of HistopathologyThe Royal Marsden HospitalLondonUK

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