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Fibroblastic/Myofibroblastic Tumors

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Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions

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Abstract

Fibroblastic/myofibroblastic sarcomas are mainly recognized and classified in the soft tissues while in the bone fewer entities were recognized.

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Selected Bibliography

  • Alaggio R, Bisogno G, Rosato A, Ninfo V, Coffin CM. Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature. Hum Pathol. 2009;40(11):1600–10, Review.

    Article  PubMed  Google Scholar 

  • Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma: between the past and the present. Arch Pathol Lab Med. 2008;132(6):1030–5, Review.

    PubMed  Google Scholar 

  • Anders JO, Aurich M, Lang T, Wagner A. Solitary fibrous tumor in the thigh: review of the literature. J Cancer Res Clin Oncol. 2006;132(2):69–75, Review.

    Article  CAS  PubMed  Google Scholar 

  • Antonescu CR, Baren A. Spectrum of low-grade fibrosarcomas: a comparative ultrastructural analysis of low-grade myxofibrosarcoma and fibromyxoid sarcoma. Ultrastruct Pathol. 2004;28(5–6):321–32.

    Article  PubMed  Google Scholar 

  • Archontaki M, Korkolis DP, Arnogiannaki N, Konstantinidou C, Georgopoulos S, Dendrinos P, Zarkadas G, Kokkalis G. Dermatofibrosarcoma protuberans: a case series of 16 patients treated in a single institution with literature review. Anticancer Res. 2010;30(9):3775–9, Review.

    PubMed  Google Scholar 

  • Arnaoutoglou C, Lykissas MG, Gelalis ID, Batistatou A, Goussia A, Doukas M, Xenakis TA. Low grade fibromyxoid sarcoma: a case report and review of the literature. J Orthop Surg Res. 2010;5:49.

    Article  PubMed  PubMed Central  Google Scholar 

  • Bahrami A, Folpe AL. Adult-type fibrosarcoma: A reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period. Am J Surg Pathol. 2010;34(10):1504–13.

    Article  PubMed  Google Scholar 

  • Bertoni F, Capanna R, Calderoni P, Patrizia B, Campanacci M. Primary central (medullary) fibrosarcoma of bone. Semin Diagn Pathol. 1984;1(3):185–98.

    CAS  PubMed  Google Scholar 

  • Bielack SS, Schroeders A, Fuchs N, Bacci G, Bauer HC, Mapeli S, Tomeno B, Winkler K. Malignant fibrous histiocytoma of bone: a retrospective EMSOS study of 125 cases. European Musculo-Skeletal Oncology Society. Acta Orthop Scand. 1999;70(4):353–60.

    Article  CAS  PubMed  Google Scholar 

  • Carlson JW, Fletcher CD. Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: analysis of a series and review of the literature. Histopathology. 2007;51(4):509–14, Review.

    Article  CAS  PubMed  Google Scholar 

  • Chung EB, Enzinger FM. Infantile fibrosarcoma. Cancer. 1976;38(2):729–39.

    Article  CAS  PubMed  Google Scholar 

  • Coffin CM, Sorensen PH. Infantile fibrosarcoma. In: World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2013. p. 89–90.

    Google Scholar 

  • Coffin CM, Jaszcz W, O’Shea PA, Dehner LP. So-called congenital-infantile fibrosarcoma: does it exist and what is it? Pediatr Pathol. 1994;14(1):133–50.

    Article  CAS  PubMed  Google Scholar 

  • Collini P, Sorensen PH, Patel S, Blay JY, Issels RD, Maki RG, Eriksson M, del Muro XG. Sarcomas with spindle cell morphology. Semin Oncol. 2009;36(4):324–37, Review.

    Article  PubMed  Google Scholar 

  • Demicco EG, Park MS, Araujo DM, et al. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. Mod Pathol. 2012;25(9):1298–306.

    Article  PubMed  Google Scholar 

  • Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJ, Fletcher CD, 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  PubMed  Google Scholar 

  • Erlandson RA, Antonescu CR, et al. Ultrastruct Pathol. 2004;28(5–6):283–9, Review. Erratum in: Ultrastruct Pathol. 2005;29(2):157.

    Article  PubMed  Google Scholar 

  • Eyden B, Banerjee SS, Shenjere P, Fisher C. The myofibroblast and its tumours. J Clin Pathol. 2009;62(3):236–49, Review.

    Article  CAS  PubMed  Google Scholar 

  • Fisher C. Myofibroblastic malignancies. Adv Anat Pathol. 2004;11(4):190–201, Review.

    Article  PubMed  Google Scholar 

  • Fletcher CD. Myofibroblastic tumours: an update. Verh Dtsch Ges Pathol. 1998;82:75–82, Review.

    CAS  PubMed  Google Scholar 

  • 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, Willén 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(6):1841–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. 4th ed. Lyon: International Agency for Research on Cancer; 2013.

    Google Scholar 

  • Gooskens SL, Oranje AP, van Adrichem LN, de Waard-van der Spek FB, den Hollander JC, van de Ven CP, van den Heuvel-Eibrink MM. Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP). Pediatr Blood Cancer. 2010;55(2):369–73, Review,

    Google Scholar 

  • Hattinger CM, Tarkkanen M, Benini S, Pasello M, Stoico G, Bacchini P, Knuutila S, Scotlandi K, Picci P, Serra M. Genetic analysis of fibrosarcoma of bone, a rare tumour entity closely related to osteosarcoma and malignant fibrous histiocytoma of bone. Eur J Cell Biol. 2004;83(9):483–91.

    Article  CAS  PubMed  Google Scholar 

  • Hoekstra HJ, Ham SJ, van der Graaf WT, Kamps WA, Molenaar WM, Schraffordt Koops H. Malignant fibrous histiocytoma of bone: a clinicopathologic study of 81 patients. Cancer. 1998;82(5):993–4.

    Article  CAS  PubMed  Google Scholar 

  • Kindblom LG, Mertens F, Coindre JM, Hornick JL, Meis JM. Sclerosing epithelioid fibrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2013. p. 97–8.

    Google Scholar 

  • Koplas MC, Lefkowitz RA, Bauer TW, Joyce MJ, Ilaslan H, Landa J, Sundaram M. Imaging findings, prevalence and outcome of de novo and secondary malignant fibrous histiocytoma of bone. Skeletal Radiol. 2010;39(8):791–8.

    Article  PubMed  Google Scholar 

  • Lombardi R, Jovine E, Zanini N, Salone MC, Gambarotti M, Righi A, Balladelli A, Colangeli M, Rocca M. A case of lung metastasis in myxoinflammatory fibroblastic sarcoma: analytical review of one hundred and thirty eight cases. Int Orthop. 2013;37(12):2429–36.

    Article  PubMed  PubMed Central  Google Scholar 

  • Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C. MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st century. Expert Rev Anticancer Ther. 2009;9(8):1135–44, Review.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • McArthur G. Dermatofibrosarcoma protuberans: recent clinical progress. Ann Surg Oncol. 2007;14(10):2876–86, Review.

    Article  PubMed  Google Scholar 

  • Meis JM, Kindblom LG, Mertens F. Myxoinflammtory fibroblastic sarcoma. In: World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2013. p. 87–8.

    Google Scholar 

  • Meis-Kindblom JM, Enzinger FM. Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19:979–93.

    Article  CAS  PubMed  Google Scholar 

  • Meis-Kindblom JM, Kindblom LG. Acral myxoinflammatory fibroblastic sarcoma: a low-grade tumor of the hands and feet. Am J Surg Pathol. 1998;22(8):911–24.

    Article  CAS  PubMed  Google Scholar 

  • 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;131(5):701–9.

    Article  PubMed  Google Scholar 

  • Mertens F, Fletcher CD, Antonescu CR, Coindre JM, Colecchia M, Domanski HA, Downs-Kelly E, Fisher C, Goldblum JR, Guillou L, Reid R, Rosai J, Sciot R, Mandahl N, Panagopoulos I. Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene. Lab Invest. 2005;85(3):408–15.

    Article  CAS  PubMed  Google Scholar 

  • Michal M, Kazakov DV, Hadravský L, Kinkor Z, Kuroda N, Michal M. High-grade myxoinflammatory fibroblastic sarcoma: a report of 23 cases. Ann Diagn Pathol. 2015;19(3):157–63.

    Article  PubMed  Google Scholar 

  • Michal M, Kazakov DV, Hadravský L, Agaimy A, Švajdler M, Kuroda N, Michal M. Pleomorphic hyalinizing angiectatic tumor revisited: all tumors manifest typical morphologic features of myxoinflammatory fibroblastic sarcoma, further suggesting 2 morphologic variants of a single entity. Ann Diagn Pathol. 2016;20:40–3.

    Article  PubMed  Google Scholar 

  • Miura K, Han G, Sano M, Tsutsui Y. Regression of congenital fibrosarcoma to hemangiomatous remnant with histological and genetic findings. Pathol Int. 2002;52(9):612–8.

    Article  PubMed  Google Scholar 

  • Mohajeri A, Tayebwa J, Collin A, Nilsson J, Magnusson L, von Steyern FV, Brosjö O, Domanski HA, Larsson O, Sciot R, Debiec-Rychter M, Hornick JL, Mandahl N, Nord KH, Mertens F. Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. Genes Chromosomes Cancer. 2013 Oct;52(10):873–86.

    Google Scholar 

  • Monson E, Vancourt R, Dawson J. Myxoinflammatory fibroblastic sarcoma: a case report and review of the literature. J Foot Ankle Surg. 2010;49(1):86.e1–3, Review.

    Article  Google Scholar 

  • Nascimento AF, Raut CP. Diagnosis and management of pleomorphic sarcomas (so-called “MFH”) in adults. J Surg Oncol. 2008;97(4):330–9, Review.

    Article  PubMed  Google Scholar 

  • Niini T, López-Guerrero JA, Ninomiya S, Guled M, Hattinger CM, Michelacci F, Böhling T, Llombart-Bosch A, Picci P, Serra M, Knuutila S. Frequent deletion of CDKN2A and recurrent coamplification of KIT, PDGFRA, and KDR in fibrosarcoma of bone--an array comparative genomic hybridization study. Genes Chromosomes Cancer. 2010;49(2):132–43.

    Article  CAS  PubMed  Google Scholar 

  • Niini T, Lahti L, Michelacci F, Ninomiya S, Hattinger CM, Guled M, Böhling T, Picci P, Serra M, Knuutila S. Array comparative genomic hybridization reveals frequent alterations of G1/S checkpoint genes in undifferentiated pleomorphic sarcoma of bone. Genes Chromosomes Cancer. 2011;50:291–306.

    Article  CAS  PubMed  Google Scholar 

  • Orbach D, Brennan B, De Paoli A, Gallego S, Mudry P, Francotte N, van Noesel M, Kelsey A, Alaggio R, Ranchère D, De Salvo GL, Casanova M, Bergeron C, Merks JH, Jenney M, Stevens MC, Bisogno G, Ferrari A. Conservative strategy in infantile fibrosarcoma is possible: the European Paediatric Soft Tissue Sarcoma Study Group experience. Eur J Cancer. 2016;57:1–9.

    Article  PubMed  Google Scholar 

  • Palmerini E, Gambarotti M, Staals EL, Zanella L, Sieberova G, Longhi A, Cesari M, Bonarelli S, Picci P, Ruggieri P, Alberghini M, Ferrari S. Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans. Clin Sarcoma Res. 2012;2(1):4. https://doi.org/10.1186/2045-3329-2-4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Papagelopoulos PJ, Galanis EC, Trantafyllidis P, Boscainos PJ, Sim FH, Unni KK. Clinicopathologic features, diagnosis, and treatment of fibrosarcoma of bone. Am J Orthop (Belle Mead NJ). 2002;31(5):253–7, Review.

    Google Scholar 

  • Park MS, Araujo DM. New insights into the hemangiopericytoma/solitary fibrous tumor spectrum of tumors. Curr Opin Oncol. 2009;21(4):327–31, Review.

    Article  PubMed  Google Scholar 

  • Perez-Montiel MD, Plaza JA, Dominguez-Malagon H, Suster S. Differential expression of smooth muscle myosin, smooth muscle actin, h-caldesmon, and calponin in the diagnosis of myofibroblastic and smooth muscle lesions of skin and soft tissue. Am J Dermatopathol. 2006;28(2):105–11.

    Article  PubMed  Google Scholar 

  • Randall RL, Albritton KH, Ferney BJ, Layfield L. Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop (Belle Mead NJ). 2004;33(12):602–8, Review.

    Google Scholar 

  • Righi A, Gambarotti M, Manfrini M, Benini S, Gamberi G, Cocchi S, Casadei R, Picci P, Vanel D, Dei Tos AP. Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases. Virchows Arch. 2015;467(3):339–44.

    Article  CAS  PubMed  Google Scholar 

  • Sanerkin NG. Definitions of osteosarcoma, chondrosarcoma, and fibrosarcoma of bone. Cancer. 1980;46(1):178–85.

    Article  CAS  PubMed  Google Scholar 

  • Sheng WQ, Hisaoka M, Okamoto S, Tanaka A, Meis-Kindblom JM, Kindblom LG, Ishida T, Nojima T, Hashimoto H. Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. Am J Clin Pathol. 2001;115(3):348–55.

    Article  CAS  PubMed  Google Scholar 

  • Shidham VB, Ayala GE, Lahaniatis JE, Garcia FU. Low-grade fibromyxoid sarcoma: clinicopathologic case report with review of the literature. Am J Clin Oncol. 1999;22(2):150–5, Review.

    Article  CAS  PubMed  Google Scholar 

  • Stadler FJ, Scott GA, Brown MD. Malignant fibrous tumors. Semin Cutan Med Surg. 1998;17(2):141–52.

    Article  CAS  PubMed  Google Scholar 

  • Sundram UN. Review: dermatofibrosarcoma protuberans: histologic approach and updated treatment recommendations. Clin Adv Hematol Oncol. 2009;7(6):406–8, Review.

    PubMed  Google Scholar 

  • Tarkkanen M, Larramendy ML, Böhling T, Serra M, Hattinger CM, Kivioja A, Elomaa I, Picci P, Knuutila S. Malignant fibrous histiocytoma of bone: analysis of genomic imbalances by comparative genomic hybridisation and C-MYC expression by immunohistochemistry. Eur J Cancer. 2006;42(8):1172–80.

    Article  CAS  PubMed  Google Scholar 

  • Wan S, Ning L, Hong R, Wu W, Fan S, Tsuchiya H, Tomita K. Clinicopathological features of solitary fibrous tumours in the extremities: four case reports and a literature review. J Int Med Res. 2010;38(2):694–704, Review.

    Article  CAS  PubMed  Google Scholar 

  • Wignall OJ, Moskovic EC, Thway K, Thomas JM. Solitary fibrous tumors of the soft tissues: review of the imaging and clinical features with histopathologic correlation. AJR Am J Roentgenol. 2010;195(1):W55–62, Review.

    Article  PubMed  Google Scholar 

  • Wojcik JB, Bellizzi AM, Dal Cin P, Bredella MA, Fletcher CD, Hornicek FJ, Deshpande V, Hornick JL, Nielsen GP. Primary sclerosing epithelioid fibrosarcoma of bone: analysis of a series. Am J Surg Pathol. 2014;38:1538–44.

    Article  PubMed  Google Scholar 

  • Wong SL. Diagnosis and management of desmoid tumors and fibrosarcoma. J Surg Oncol. 2008;97(6):554–8, Review.

    Article  PubMed  Google Scholar 

  • Zreik RT, Carter JM, Sukov WR, Ahrens WA, Fritchie KJ, Montgomery EA, Weiss SW, Folpe AL. TGFBR3 and MGEA5 rearrangements are much more common in “hybrid” hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcomas than in classical myxoinflammatory fibroblastic sarcomas: a morphological and fluorescence in situ hybridization study. Hum Pathol. 2016;53:14–24.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Laboratory of Experimental Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Piero Picci

  2. I.S.G. Italian Sarcoma Group, Bologna, Italy

    Piero Picci

  3. Department of Pathology, Azienda Ospedaliera di Padova, Padua, Italy

    Angelo Paolo Dei Tos

  4. Department of Medicine, University of Padua School of Medicine, Padua, Italy

    Angelo Paolo Dei Tos

  5. Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Marco Gambarotti & Alberto Righi

Authors
  1. Piero Picci
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  2. Angelo Paolo Dei Tos
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  3. Marco Gambarotti
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  4. Alberto Righi
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Corresponding author

Correspondence to Piero Picci .

Editor information

Editors and Affiliations

  1. I.S.G. Italian Sarcoma Group, Bologna, Italy

    Piero Picci

  2. IIIrd Clinic of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy

    Marco Manfrini

  3. Istituto Ortopedico Rizzoli, Bologna, Italy

    Davide Maria Donati

  4. Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Marco Gambarotti

  5. Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy

    Alberto Righi

  6. Istituto Rizzoli, Bologna, Italy

    Daniel Vanel

  7. Department of Medicine, University of Padua School of Medicine, Padua, Italy

    Angelo Paolo Dei Tos

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Picci, P., Dei Tos, A.P., Gambarotti, M., Righi, A. (2020). Fibroblastic/Myofibroblastic Tumors. In: Picci, P., et al. Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions. Springer, Cham. https://doi.org/10.1007/978-3-030-29676-6_42

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  • DOI: https://doi.org/10.1007/978-3-030-29676-6_42

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