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Desmoplastic Fibroma of the Mandible: A Series of Three Cases and Review of Literature

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Abstract

The desmoplastic fibroma (DF) is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. We present three cases of DF in the mandible: the first in a 13 year old female involving the mandibular body in the region of teeth #s 27–#28, the second in a 57 year old female with a lesion apical to tooth #30, and the third in a 20-year-old female involving the left posterior mandible. Clinical, histologic, immunohistochemical (IHC) and radiographic features of this rare neoplasm are discussed. The challenges encountered in establishing an accurate diagnosis due to significant microscopic overlap with other spindle cell lesions are also detailed. Additionally, the findings of IHC stains including vimentin, smooth muscle actin, S-100 protein, β-catenin, HHF-35 and proliferation marker, Ki-67 on 3 cases are reported. The potential for misdiagnosis is high, especially in early lesions, since immunohistochemistry has been reported in literature to be inconsistent when differentiating DFs from other spindle cell lesions. A comparative review of DF and similar entities in the jaws with current considerations in treatment and prognosis is presented.

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References

  1. Jaffe HL. Desmoplastic fibroma and fibrosarcoma. In: Jaffe HL, editor. Tumors and tumorous conditions of the bones and joints. Philadelphia: Lea and Febiger; 1958. p. 298–303.

  2. Griffith JG, Irby WB. Desmoplastic fibroma: report of a rare tumor of the oral structures. Oral Surg Oral Med Oral Pathol. 1965;20:269–75.

    Article  CAS  PubMed  Google Scholar 

  3. Fletcher CDM, Uni KK, Mertens F. WHO classification of tumors. Pathology and genetics of tumors of soft tissue and bone. Lyon: IARC Press; 2002. p. 288.

  4. Said-Al-Naief N, Fernandes R, Louis P, Bell W, Siegal GP. Desmoplastic fibroma of the jaw: a case report and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;101(1):82–94.

    Article  PubMed  Google Scholar 

  5. Neville B, Damm D, Allen C, Bouquot J. Textbook of oral & maxillofacial pathology. 3rd ed. Philadelphia: WB Saunders Company; 2009. p. 658–60.

    Google Scholar 

  6. Montgomery E, Folpe AL. The Diagnostic value of B-catenin Immunohistochemistry. Adv Anat Pathol. 2005;12(6):350–6.

    Article  CAS  PubMed  Google Scholar 

  7. Bridge JA, Swarts SJ, Buresh C, Nelson M, Degenhardt JM, Spanier S, et al.   Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. Am J Pathol. 1999;154:729–33.

  8. Damm DD, Tomich CE, White DK, Drummond JF. Intraosseous fibrous lesions of the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999;87:334–40.

    Article  CAS  PubMed  Google Scholar 

  9. Tandon S, Garg RK. Intraoral desmoplastic fibroma: a manifestation of tuberous sclerosis. Fetal Pediatr Pathol. 2012;31:195–201.

    Article  PubMed  Google Scholar 

  10. Hauben EI, Jundt G, Cleton-Jansen AM, Yavas A, Kroon HM, Van Marck E, et al. Desmoplastic fibroma of bone: an immunohistochemical study including B-catenin expession and mutational analysis for B-catenin. Human Pathol. 2005;36:1025–30.

  11. Kang DM, Juhng SK, Sohn YJ, Kim HS. Imaging findings of desmoplastic fibroma rarely involving the clavicle: case report. Korean J Radiol. 2014;15(1):130–3.

  12. Frick MA, Sundaram M, Unni KK, Inwards CY, Fabbri N, Trentani F, et al. Imaging findings in desmoplastic fibroma of bone: distinctive T2 characteristics. AJR Am J Roentgenol. 2005;184:1762–7.

    Article  PubMed  Google Scholar 

  13. Wippold FJ 2nd, White FV, Jamroz G, Haughey B, Forsen J. Desmoplastic fibroma of the mandible in an infant. Pediatr Radiol. 2005;35:906–9.

  14. Ng TL, Gown AM, Barry TS, Cheang MC, Chan AK, Turbin DA, et al. Nuclear beta-catenin in mesenchymal tumors. Modern Pathol. 2005;18:68–74.

  15. Valenta T, Hausmann G, Basler K. The many faces and functions of B-catenin. EMBO J. 2012;31:2714–36.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  16. Bhattacharya B, Dilworth HP, Iacobuzio-Donahue C, Ricci F, Weber K, Furlong MA, et al. Nuclear B-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol. 2005;29(5):653–9.

  17. Azola AM, Wartmann CT, Fischer MK, Ambro BT, Pereira KD, et al. Desmoplastic fibroma arising from the anterior maxillary sinus in a child. Arch Otolaryngol Head Neck Surg. 2012;138(9):859–62.

  18. Taher LY, Saleem M, Velagapudi S, Dadado A. Fibromatosis arising in association with neuromuscular hamartoma of the mandible. Head Neck Pathol. 2013;7:280–4.

    Article  PubMed Central  PubMed  Google Scholar 

  19. Gondak RO, Corrêa MB, da Costa MV, Vargas PA, Lopes MA. Maxillary desmoplastic fibroma with initial symptoms suggestive of sinusitis. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;116(6):e510–3.

  20. Van Blarcom CW, Masson JK, Dahlin DC. Fibrosarcoma of the mandible: a clinicopathologic study. Oral Syrg. 1971;32:428–39.

    Article  Google Scholar 

  21. El-Mofty SK. Bone lesions. In: Gnepp DR, editor. Diagnostic surgical pathology of the head and neck. 2nd ed. Saunders-Elsevier; 2009. p. 736-42.

  22. Nishio J, Iwasaki H, Takagi S, Seo H, Aoki M, Nabeshima K, Naito M. Low-grade central osteosarcoma of the metatarsal bone: a clinicopathological, immunohistochemical, cytogenetic and molecular cytogenetic analysis. Anticancer Res. 2012;32:5429–36.

  23. Vargas-Gonzalez R, San Martin-Brieke W, Gil-Orduña C, Lara-Hernandez F. Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis. Pathol Oncol Res. 2004;10(4):237–9.

    Article  PubMed  Google Scholar 

  24. Iatrou IA, Lygidakis NT, Leventis MD. Case report: desmoplastic fibroma of the mandible in a child presenting with TMJ dysfunction. Eur Arch Paediatr Dent. 2008;9(2):105–8.

  25. Dhaif G, Satir AA, Sharif S. Desmoplastic fibroma of the mandible: a 5 year follow-up. Bahrain Med Bul. 2008;30(4):1–10.

    Google Scholar 

  26. Schneider M, Zimmermann AC, Depprich RA, Kübler NR, Engers R, Naujoks CD, et al. Desmoplastic fibroma of the mandible: review of the literature and presentation of a rare case. Head Face Med. 2009;5:25:1–5.

  27. Mir-Mari J, Aguirre-Urizar JM, Berini-Aytés L, Gay-Escoda C, et al. Giant desmoplastic fibroma in the anterior zone of the maxilla. J Craniofac Surg. 2001;22(6):2350–3.

  28. Joshua BZ, Silberstein E, Diomin V, Bodner L. Desmoplastic fibroma of the mandible associated with pathological fracture in a pediatric patient. Int J Pediatr Otorhinolaryngol Extra. 2014;9(2):60–3.

    Article  Google Scholar 

  29. Weiss SW, Goldblum JR. Enzinger and Weiss’s soft tissue tumors. In: Weiss SW, Goldblum JR, editors. 5th ed. Mosby-Elsevier; 2008. p. 303–324.

  30. Dehner LP. Tumors of the mandible and maxilla in children. I. Clinicopathologic study of 46 histologically benign lesions. Cancer. 1973;31:364–83.

    Article  CAS  PubMed  Google Scholar 

  31. Green MF, Sirikumara M. Desmoplastic fibroma of the mandible. Ann Plast Surg. 1987;19(3):284–90.

    Article  CAS  PubMed  Google Scholar 

  32. Moorjani V, Stockeon V. Desmoplastic fibroma with perineural extension. AJR. 2005;185:1498–9.

    Article  PubMed  Google Scholar 

  33. Shukul VK, Saxena S, Shankar BG. Desmoplastic fibroma: mandible. MJAFI. 2004;60(2):307–9.

    Google Scholar 

  34. Dalili-Kajan Z, Adham-Fumani G, Dalili-Kajan H. Desmoplastic fibroma with periosteal reaction. Acta Medica Iranica. 2007;45(4):325–8.

    Google Scholar 

  35. Sandrini FA, Vasconcelos BC, Bessa-nogueira RV. Desmoplastic fibroma of the mandible in a child: case report. Indian J Surg. 2007;69:254–6.

    Article  PubMed Central  PubMed  Google Scholar 

  36. Shi H, Wang P, Wang S, Yu Q. Desmoplastic fibroma of the mandible. Dentomaxillofacial Radiol. 2008;37:408–11.

    Article  CAS  Google Scholar 

  37. Salah MB, Mekni A, Azouz H, Blel A, Kharrat S, Kchir N, et al. Infantile desmoplastic fibroma of the maxilla. Tunis Med. 2008;86(8):749–51.

  38. Summa A, Cerasti D, Crisi G, Ormitti F, Ventura E, Sabato M. Desmoplastic fibroma of the mandible: usefulness of CT and MR imaging in diagnosis and treatment, a case report. Neuroradiol J. 2009;22:109–13.

  39. Reid EN, Lawoyin DO, Suresh L, Longwe E. Desmoplastic fibroma of the anterior mandible: case report and review of literature. NY State Dental J. 2009;75:32–3.

  40. Shekhar MG, Reddy RS, Ravikanth M, Lavanya R, Kumar M. Desmoplastic fibroma of the mandible: case report and review of literature. Prim Dent Care. 2011;18(3):115–8.

  41. Boedeker D, Kelsch R, Kraut R. Desmoplastic fibroma of the anterior maxillary alveolus. J Oral Maxillofac Surg. 2011;69(2):264–6.

    Google Scholar 

  42. Ferri A1, Leporati M, Corradi D, Ferri T, Sesenna E. Huge desmoplastic fibroma of the paediatric mandible: surgical considerations and follow-up in three cases. J Craniomaxillofac Surg. 2013;41(5):367–70.

  43. Averna R, De Filippo M, Ferrari S, Bacchini E, Rossi C. Desmoplastic fibroma of the mandible. Acta Biomed. 2011;82:69–73.

  44. Scott N, Pater M, Newlands C. Desmoplastic fibroma of the mandible: an unusual presentation and treatment. J Surg Case Rep. 2011;11:5.

    Google Scholar 

  45. Guru KN, Gupta MK, Pillai A, Moghe S, Satpathy S. Desmoplastic fibroma of the maxilla: report of a rare case. People’s J Sci Res. 2013;6(1):36–8.

  46. Tandon S, Garg RK. Intraoral desmoplastic fibroma: a manifestation of tuberous sclerosis. Fetal Pediatr Pathol. 2012;31:195–201.

    Article  PubMed  Google Scholar 

  47. Acosta-Feria M, Haro-Luna JJ, Ramos-Medina B, Gomez-Poveda A, Villar-Puchades R. Mandible reconstruction with fibular free flap after resection desmoplastic fibroma. Oral Oncol. 2013;49:S144.

  48. Vankeviciute RA, Neverauskiene A, Lukosevicius E. Fibro-osseous lesions of craniofacial bones in children: case reports. Acta Medica Litu. 2013;20(2):93–101.

    Google Scholar 

  49. Guruprasad Y. Desmoplastic fibroma of the mandible. J Cranio-Maxillary Dis. 2013;2(1):93–4.

    Article  Google Scholar 

  50. Sokhn A, Nasseh I, Karam F. Radiolucency in the mandible: a seventeen-year follow-up. Oral Surg Oral Med Oral Pathol Oral Radiol. 2014;117(2):153–8.

    Article  PubMed  Google Scholar 

  51. Burlini D, Conti G, Bardellini E, Amadori F. Rare case of desmoid-type fibromatosis of the mandibular region in a child: diagnosis and surgical management. Eur J Paediatr Dent. 2013;14(4):333–4.

    CAS  PubMed  Google Scholar 

  52. Lagace R, Delage C, Bouchard HL, Seemayer TA. Desmoplastic fibroma of bone: an ultrastructural study. Am J Surg Pathol. 1979;3(5):423–30.

    Article  CAS  PubMed  Google Scholar 

  53. Boon LC, Phaik KS, Khanijow V. Desmoplastic fibroma: report of a case with proliferative myositis. Ann Dent. 1991;50(2):28–32.

  54. Hopkins KM, Huttula CS, Kahn MA, Albright JE. Desmoplastic fibroma of the mandible: review and report of two cases. J Oral Maxillofac Surg. 1996;54(10):1249–54.

  55. Bakaeen G, Rajab LD. Desmoplastic fibroma of the mandible: report of a case. Int J Paediatr Dent. 1999;9(2):117–21.

  56. Kaplan KJ, Torske KR. Pathologic quiz case: a 3-year-old boy with swelling of the right mandible. Arch Pathol Lab Med. 2002;126(1):107–8.

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Acknowledgments

We would like to thank Dr. Barton Blumberg of The Villages, Florida for his contribution to Case 2.

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Correspondence to I. Bhattacharyya.

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Woods, T.R., Cohen, D.M., Islam, M.N. et al. Desmoplastic Fibroma of the Mandible: A Series of Three Cases and Review of Literature. Head and Neck Pathol 9, 196–204 (2015). https://doi.org/10.1007/s12105-014-0561-5

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