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Fibrous dysplasia: an unusual case of a very aggressive form with costo-vertebral joint destruction and invasion of the contralateral D7 vertebral body

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Abstract

Fibrous dysplasia (FD) is a benign fibro-osseous disease of the bone that may be solitary or multicentric. It is important to distinguish this type of lesion from low-grade osteosarcomas (LGOS) and from secondary sarcomas, because malignant transformation has rarely been reported. It is classically described as having a ground-glass appearance, endosteal scalloping, and thinning of the cortex. Cortical disruption is considered evidence of malignancy, but it can also be present in benign FD with aggressive behavior. We present an unusual case of aggressive FD of the 7th left rib, already diagnosed more than 22 years ago, where cortical and costo-vertebral joint disruption and 7th thoracic vertebral body involvement were not evidence of malignant behavior. From a histological perspective, FD and LGOS are similar; even if histology is of fundamental importance, the diagnosis has to be made based on the clinical and radiological aspects as well, although at imaging, differentiation between FD and LGOS can be difficult. In the present case, even though the histological examination suggested a benign lesion, the radiological examination instead consistently suggests malignancy. It is for this reason that there should be a high index of suspicion during follow-up and a new biopsy should be scheduled in case any changes occur during follow-up.

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References

  1. Campanacci M. Bone and soft tissue tumors: clinical features, imaging, pathology and treatment. 2nd ed. Austria: Vienna; 1999.

    Book  Google Scholar 

  2. Zoccali C, Teori G, Prencipe U, Erba F. Mazabraud’s syndrome: a new case and review of the literature. Int Orthop. 2009;33(3):605–10.

    Article  PubMed  Google Scholar 

  3. Steiner GC, Forest M, Vacher-Lavenu MC. Ultrastructure of low-grade intraosseous osteosarcoma of bone: a comparative study with fibrous dysplasia and parosteal osteosarcoma. Ultrastruct Pathol. 2006;30(4):293–9.

    Article  PubMed  Google Scholar 

  4. Bertoni F, Bacchini P, Fabbri N, Mercuri M, Picci P, Ruggieri P, et al. Osteosarcoma. Low-grade intraosseous-type osteosarcoma, histologically resembling parosteal osteosarcoma, fibrous dysplasia, and desmoplastic fibroma. Cancer. 1993;71(2):338–45.

    Article  CAS  PubMed  Google Scholar 

  5. Dujardin F, Binh MB, Bouvier C, Gomez-Brouchet A, Larousserie F, Muret A, et al. MDM2 and CDK4 immunohistochemistry is a valuable tool in the differential diagnosis of low-grade osteosarcomas and other primary fibro-osseous lesions of the bone. Mod Pathol. 2011;24(5):624–37.

    Article  CAS  PubMed  Google Scholar 

  6. Chen CY, Zhang HZ, Jiang ZM, Zhou J, Chen J, Liu L. Value of MDM2, CDK4 and SATB2 immunohistochemistry in histologic diagnosis of low-grade osteosarcoma. Zhonghua Bing Li Xue Za Zhi. 2016;45(6):387–92.

    CAS  PubMed  Google Scholar 

  7. Carter JM, Inwards CY, Jin L, Evers B, Wenger DE, Oliveira AM, Fritchie KJ. Activating GNAS mutations in parosteal osteosarcoma. Am J Surg Pathol. 2014 Mar;38(3):402-9

    Article  PubMed  Google Scholar 

  8. Kuznetsov SA, Cherman N, Riminucci M, et al. Age-dependent demise of GNAS-mutated skeletal stem cells and “normalization” of fibrous dysplasia of bone. J Bone Miner Res. 2008;23:1731–40.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Salinas-Souza C, De Andrea C, Bihl M, Kovac M, Pillay N, Forshew T, et al. GNAS mutations are not detected in parosteal and low-grade central osteosarcomas. Mod Pathol. 2015 Oct;28(10):1336–42.

    Article  CAS  PubMed  Google Scholar 

  10. Franceschina MJ, Hankin RC, Irwin RB. Low-grade central osteosarcoma resembling fibrous dysplasia. A report of two cases. Am J Orthop. 1997;26(6):432–40.

    CAS  PubMed  Google Scholar 

  11. Shah ZK, Peh WC, Koh WL, Shek TW. Magnetic resonance imaging appearances of fibrous dysplasia. Br J Radiol. 2005;78:1104–15.

    Article  CAS  PubMed  Google Scholar 

  12. Fitzpatrick KA, Taljanovic MS, Speer DP, Graham AR, Jacobson JA, Barnes GR, et al. Imaging findings of fibrous dysplasia with histopathologic and intraoperative correlation. AJR Am J Roentgenol. 2004;82(6):1389–98.

    Article  Google Scholar 

  13. Latham PD, Athanasou NA, Woods CG. Fibrous dysplasia with locally aggressive malignant change. Arch Orthop Trauma Surg. 1992;111(3):183–6.

    Article  CAS  PubMed  Google Scholar 

  14. Yao L, Eckardt JJ, Seeger LL. Fibrous dysplasia associated with cortical bony destruction: CT and MR findings. J Comput Assist Tomogr. 1994;18:91–4.

    Article  CAS  PubMed  Google Scholar 

  15. Dorfman HD, Ishida T, Tsuneyoshi M. Exophytic variant of fibrous dysplasia (fibrous dysplasia protuberans). Hum Pathol. 1994 Nov;25(11):1234–7.

    Article  CAS  PubMed  Google Scholar 

  16. Hermann G, Garcia R. Exophytic fibrous dysplasia of the rib. Osteologiai Kozlemenyek. 2013;1-2:9–12.

    Google Scholar 

  17. Muthusamy S, Subhawong T, Conway SA, Temple HT. Locally aggressive fibrous dysplasia mimicking malignancy: a report of four cases and review of the literature. Clin Orthop Relat Res. 2015;473(2):742–50.

    Article  PubMed  Google Scholar 

  18. Kashima TG, Gamage NM, Ye H, Amary MF, Flanagan AM, Ostlere SJ, et al. Locally aggressive fibrous dysplasia. Virchows Arch. 2013;463:79–84.

    Article  CAS  PubMed  Google Scholar 

  19. Vasiliadis HS, Arnaoutoglou C, Plakoutsis S, Doukas M, Batistatou A, Xenakis TA. Low-grade central osteosarcoma of distal femur, resembling fibrous dysplasia. World J Orthop. 2013;4(4):327–32.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Malhas AM, Sumathi VP, James SL, Menna C, Carter SR, Tillman RM, et al. Low-grade central osteosarcoma: a difficult condition to diagnose. Sarcoma. 2013;2012:764796.

    Google Scholar 

  21. Qu N, Yao W, Cui X, Zhang H. Malignant transformation in monostotic fibrous dysplasia: clinical features, imaging features, outcomes in 10 patients, and review. Medicine (Baltimore). 2015;94(3):e369.

    Article  Google Scholar 

  22. Zídková H, Kolár J, Matĕjovsky Z. Aggressive features of pelvic fibrous dysplasia. Rofo. 1994;161(2):183–5.

    Article  PubMed  Google Scholar 

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Correspondence to Giovanni Zoccali.

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Zoccali, C., Attala, D., Rossi, B. et al. Fibrous dysplasia: an unusual case of a very aggressive form with costo-vertebral joint destruction and invasion of the contralateral D7 vertebral body. Skeletal Radiol 47, 1571–1576 (2018). https://doi.org/10.1007/s00256-018-2961-1

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  • DOI: https://doi.org/10.1007/s00256-018-2961-1

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