Skip to main content
SpringerLink
Log in

Fibrous, Fibrohistiocytic, and Myofibroblastic Tumors of Intermediate Malignancy

  • Chapter
  • First Online:
Book cover Soft Tissue Tumors of the Skin

  • 1950 Accesses

Abstract

Fibrous, fibrohistiocytic, and myofibroblastic tumors of intermediate malignancy comprise a group of lesions that has a relatively high risk of local recurrence, but a limited risk of metastasis. Most of these tumors present in young adults as superficial masses. Members of this group include dermatofibrosarcoma protuberans and its numerous variants, plexiform fibrohistiocytic tumor, angiomatoid fibrous histiocytoma, giant cell tumor of soft tissue, and inflammatory myofibroblastic tumor.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Selected References

  1. Bohman SL, Goldblum JR, Rubin BP, Tanas MR, Billings SD. Angiomatoid fibrous histiocytoma: an expansion of the clinical and histological spectrum. Pathology. 2014;46(3):199–204.

    Article  Google Scholar 

  2. Carter JM, Weiss SW, Linos K, DiCaudo DJ, Folpe AL. Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy. Mod Pathol. 2014;27(2):294–302.

    Article  CAS  Google Scholar 

  3. Chen G, Folpe AL, Colby TV, Sittampalam K, Patey M, Chen MG, Chan JK. Angiomatoid fibrous histiocytoma: unusual sites and unusual morphology. Mod Pathol. 2011;24(12):1560–70.

    Article  CAS  Google Scholar 

  4. Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol. 1995;19(8):859–72.

    Article  CAS  Google Scholar 

  5. Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol. 2007;31(4):509–20.

    Article  Google Scholar 

  6. de Menezes S, Mar A, Dowling J, Wright A. A case of cutaneous inflammatory Myofibroblastic tumor. Pediatr Dermatol. 2016;33(6):e377–80.

    Article  Google Scholar 

  7. Folpe AL, Morris RJ, Weiss SW. Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts. Mod Pathol. 1999;12(9):894–902.

    CAS  PubMed  Google Scholar 

  8. Gleason BC, Hornick JL. Inflammatory myofibroblastic tumours: where are we now? J Clin Pathol. 2008;61(4):428–37.

    Article  CAS  Google Scholar 

  9. Guo H, Garcia RA, Perle MA, Amodio J, Greco MA. Giant cell tumor of soft tissue with pulmonary metastases: pathologic and cytogenetic study. Pediatr Dev Pathol. 2005;8(6):718–24. Epub 2005 Nov 21.

    Article  Google Scholar 

  10. Inoue T, Misago N, Okawa T, Narisawa Y. Inflammatory myofibroblastic tumor of the skin grossly mimicking squamous cell carcinoma: a case report. J Dermatol. 2012;39(1):107–10.

    Article  Google Scholar 

  11. Jacobson-Dunlop E, White CR Jr, Mansoor A. Features of plexiform fibrohistiocytic tumor in skin punch biopsies: a retrospective study of 6 cases. Am J Dermatopathol. 2011;33(6):551–6.

    Article  Google Scholar 

  12. Jaffer S, Ambrosini-Spaltro A, Mancini AM, Eusebi V, Rosai J. Neurothekeoma and plexiform fibrohistiocytic tumor: mere histologic resemblance or histogenetic relationship? Am J Surg Pathol. 2009;33(6):905–13.

    Article  Google Scholar 

  13. Jha P, Moosavi C, Fanburg-Smith JC. Giant cell fibroblastoma: an update and addition of 86 new cases from the Armed Forces Institute of Pathology, in honor of Dr. Franz M Enzinger. Ann Diagn Pathol. 2007;11(2):81–8.

    Article  Google Scholar 

  14. Kuzel P, Mahmood MN, Metelitsa AI, Salopek TG. A clinicopathologic review of a case series of dermatofibrosarcoma protuberans with fibrosarcomatous differentiation. J Cutan Med Surg. 2015;19(1):28–34.

    Article  Google Scholar 

  15. Lee JC, Liang CW, Fletcher CDM. Giant cell tumor of soft tissue is genetically distinct from its bone counterpart. Mod Pathol. 2017;30(5):728–33.

    Article  CAS  Google Scholar 

  16. Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71(4):781–6.

    Article  Google Scholar 

  17. Llombart B, Serra-Guillén C, Monteagudo C, López Guerrero JA, Sanmartín O. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30(1):13–28.

    Article  Google Scholar 

  18. Maher OM, Prieto VG, Stewart J, Herzog CE. Characterization of metastatic angiomatoid fibrous histiocytoma. J Pediatr Hematol Oncol. 2015;37(4):e268–71.

    Article  Google Scholar 

  19. Mancini I, Righi A, Gambarotti M, Picci P, Dei Tos AP, Billings SD, Simi L, Franchi A. Phenotypic and molecular differences between giant-cell tumour of soft tissue and its bone counterpart. Histopathology. 2017;71(3):453–60.

    Article  Google Scholar 

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

    Article  Google Scholar 

  21. Mentzel T, Schärer L, Kazakov DV, Michal M. Myxoid dermatofibrosarcoma protuberans: clinicopathologic, immunohistochemical, and molecular analysis of eight cases. Am J Dermatopathol. 2007;29(5):443–8.

    Article  Google Scholar 

  22. O’Connell JX, Wehrli BM, Nielsen GP, Rosenberg AE. Giant cell tumors of soft tissue: a clinicopathologic study of 18 benign and malignant tumors. Am J Surg Pathol. 2000;24(3):386–95.

    Article  Google Scholar 

  23. Oliveira AM, Dei Tos AP, Fletcher CD, Nascimento AG. Primary giant cell tumor of soft tissues: a study of 22 cases. Am J Surg Pathol. 2000;24(2):248–56.

    Article  CAS  Google Scholar 

  24. Remstein ED, Arndt CA, Nascimento AG. Plexiform fibrohistiocytic tumor: clinicopathologic analysis of 22 cases. Am J Surg Pathol. 1999;23(6):662–70.

    Article  CAS  Google Scholar 

  25. Salomao DR, Nascimento AG. Plexiform fibrohistiocytic tumor with systemic metastases: a case report. Am J Surg Pathol. 1997;21(4):469–76.

    Article  CAS  Google Scholar 

  26. Schaefer IM, Fletcher CD. Myxoid variant of so-called angiomatoid “malignant fibrous histiocytoma”: clinicopathologic characterization in a series of 21 cases. Am J Surg Pathol. 2014;38(6):816–23.

    Article  Google Scholar 

  27. Shah KK, McHugh JB, Folpe AL, Patel RM. Dermatofibrosarcoma protuberans of distal extremities and Acral sites: a Clinicopathologic analysis of 27 cases. Am J Surg Pathol. 2018;42(3):413–9.

    Article  Google Scholar 

  28. Shmookler BM, Enzinger FM, Weiss SW. Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans. Cancer. 1989;64(10):2154–61.

    Article  CAS  Google Scholar 

  29. Tanas MR, Rubin BP, Montgomery EA, Turner SL, Cook JR, Tubbs RR, Billings SD, Goldblum JR. Utility of FISH in the diagnosis of angiomatoid fibrous histiocytoma: a series of 18 cases. Mod Pathol. 2010;23(1):93–7.

    Article  CAS  Google Scholar 

  30. Thway K, Fisher C. Angiomatoid fibrous histiocytoma: the current status of pathology and genetics. Arch Pathol Lab Med. 2015;139(5):674–82.

    Article  CAS  Google Scholar 

  31. Thway K, Noujaim J, Jones RL, Fisher C. Dermatofibrosarcoma protuberans: pathology, genetics, and potential therapeutic strategies. Ann Diagn Pathol. 2016;25:64–71.

    Article  Google Scholar 

  32. Wada N, Ito T, Uchi H, Nakahara T, Tsuji G, Yamada Y, Oda Y, Furue M. Superficial CD34-positive fibroblastic tumor: a new case from Japan. J Dermatol. 2016;43(8):934–6.

    Article  Google Scholar 

  33. Zelger B, Weinlich G, Steiner H, Zelger BG, Egarter-Vigl E. Dermal and subcutaneous variants of plexiform fibrohistiocytic tumor. Am J Surg Pathol. 1997;21(2):235–41.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, Oregon Health and Science University School of Medicine, Portland, OR, USA

    Reena Singh

  2. Department of Pathology, University of Michigan Medical School, Ann Arbor, MI, USA

    Aaron M. Udager & Rajiv M. Patel

  3. Department of Pathology, Cleveland Clinic, Cleveland, OH, USA

    Steven D. Billings

Authors
  1. Reena Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Aaron M. Udager
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Steven D. Billings
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rajiv M. Patel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rajiv M. Patel .

Editor information

Editors and Affiliations

  1. Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA

    Steven D. Billings

  2. Departments of Pathology and Dermatology, University of Michigan Medical Center, Ann Arbor, MI, USA

    Rajiv M. Patel

  3. Department of Pathology and Laboratory Medicine, University of Wisconsin–Madison, Madison, WI, USA

    Darya Buehler

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Science+Business Media, LLC, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Singh, R., Udager, A.M., Billings, S.D., Patel, R.M. (2019). Fibrous, Fibrohistiocytic, and Myofibroblastic Tumors of Intermediate Malignancy. In: Billings, S., Patel, R., Buehler, D. (eds) Soft Tissue Tumors of the Skin. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-8812-9_5

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-8812-9_5

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-8810-5

  • Online ISBN: 978-1-4939-8812-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation