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Recurrent adult-type fibrosarcoma of the frontal bone in a child

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Abstract

Introduction

Primary, adult-type bone fibrosarcoma is an uncommon, malignant spindle-cell tumor of fibroblastic origin, rarely affecting children. Most frequently diagnosed among bone malignancies in the past, improved diagnostic techniques and further restrictive classification criteria have currently made the diagnosis of fibrosarcoma very unusual.

Case report

We hereby report the case of a 7-year-old child with a right frontal swelling mass. A computed tomography scan showed an osteolytic lesion of the right frontal bone, involving the diploe and the outer table of the skull. An en bloc surgical excision, followed by a thorough immunohistological evaluation, led to the diagnosis of fibroblastic proliferation, with low cellularity and minimal atypias. The patient had four recurrences during the 4-year follow-up. With an increasing histological grade at recurrences, a diagnosis of adult-type fibrosarcoma was made.

Conclusion

To the best of the authors’ knowledge, this is the first reported case of an adult-type fibrosarcoma arising in the frontal bone of a child.

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References

  1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (2013) WHO classification of tumours of soft tissue and bone. IARC Press, Lyon

    Google Scholar 

  2. Van Blarcom CW, Masson JK, Dahlin DC (1971) Fibrosarcoma of the mandible. A clinicopathologic study. Oral Surg Oral Med Oral Pathol 32:428–439

    Article  PubMed  Google Scholar 

  3. Dehner LP (1973) Tumors of the mandible and maxilla in children II. A study of 14 primary and secondary malignant tumors. Cancer 32:112–120

    Article  CAS  PubMed  Google Scholar 

  4. Slootweg PJ, Muller H (1984) Fibrosarcoma of the jaws. A study of 7 cases. J Maxillofac Surg 12:157–162

    Article  CAS  PubMed  Google Scholar 

  5. Bang G, Baardsen R, Gilhuus-Moe O (1989) Infantile fibrosarcoma in the mandible; case report. J Oral Pathol Med 18:339–343

    Article  CAS  PubMed  Google Scholar 

  6. Grundfast K, Healy G, Richardson M (1991) Fibrosarcoma of the infratemporal fossa in an 8-year-old girl. Head Neck 13:156–159

    Article  CAS  PubMed  Google Scholar 

  7. Lo Muzio L, Mignogna MD, Pannone G, Staibano S, Testa NF (1998) A rare case of fibrosarcoma of the jaws in a 4-year-old male. Oral Oncol 34:383–386

    Article  CAS  PubMed  Google Scholar 

  8. Daw NC, Mahmoud HH, Meyer WH, Jenkins JJ, Kaste SC, Poquette CA, Kun LE, Pratt CB, Rao BN (2000) Bone sarcomas of the head and neck in children. Cancer 88:2172–2180

    Article  CAS  PubMed  Google Scholar 

  9. Gosau M, Draenert FG, Winter WA, Mueller-Hoecker J, Driemel O (2008) Fibrosarcoma of the childhood mandible. Head Face Med 4:21. doi:10.1186/1746-160X-4-21

    Article  PubMed  PubMed Central  Google Scholar 

  10. Divya A, Patil R, Kannan N, Kesary SP (2009) Fibrosarcoma of the mandible: case report of a unique radiographic appearance. Oral Radiol 25:77–80

    Article  Google Scholar 

  11. Nanda KD, Mehta A, Nanda J (2013) Fibrosarcoma of the mandible: a diagnostic dilemma. J Clin Diagn Res 7:1804–1805

    PubMed  PubMed Central  Google Scholar 

  12. Swain N, Kumar S, Dhariwal R, Routray S (2013) Primary fibrosarcoma of maxilla in an 8-year-old child: a rare entity. J Oral Maxillofac Pathol 17:478. doi:10.4103/0973-029X.125226

    Article  PubMed  PubMed Central  Google Scholar 

  13. Dahlin DC, Ivins JC (1969) Fibrosarcoma of bone. A study of 114 cases. Cancer 23:35–41

    Article  CAS  PubMed  Google Scholar 

  14. Huvos AG, Higinbotham NL (1975) Primary fibrosarcoma of bone. Cancer 35:837–847

    Article  CAS  PubMed  Google Scholar 

  15. Larsson S-E, Lorentzon R, Boquist L (1976) Fibrosarcoma of bone. A demographic, clinical and histopathological study of all cases recorded in the Swedish cancer registry from 1958 to 1968. J Bone Joint Surg Br 58:412–417

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

    CAS  PubMed  Google Scholar 

  17. Papagelopoulos PJ, Galanis E, Frassica FJ, Sim FH, Larson DR, Wold LE (2000) Primary fibrosarcoma of bone. Clin Orthopaedics Relat Res 373:88–103

    Article  Google Scholar 

  18. Kuhn FA, Javer AR (2003) Low-grade fibrosarcoma of the anterior skull base: endoscopic resection and repair. Am J Rhinol 17:347–350

    PubMed  Google Scholar 

  19. Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, Hoffer FA, Speights R, Gebhardt MC, Dahl GV, Grier HE, Pediatric Oncology Group (2007) Vinblastine and methotrexate for desmoid fibromatosis in children: results of a pediatric oncology group phase II trial. J Clin Oncol 25:501–506

    Article  CAS  PubMed  Google Scholar 

  20. Cecchetto G, Alaggio R, Dall’Igna P, Bisogno G, Ferrari A, Gigante C, Casanova M, Sotti G, Zanetti I, Carli M (2005) Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer 104:2006–2012

    Article  PubMed  Google Scholar 

  21. Paulus W, Slowik F, Jellinger K (1991) Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18:395–402

    Article  CAS  PubMed  Google Scholar 

  22. Oliveira AM, Scheithauer BW, Salomao DR, Parisi JE, Burger PC, Nascimento AG (2002) Primary sarcomas of the brain and spinal cord. Am J Surg Pathol 26:1056–1063

    Article  PubMed  Google Scholar 

  23. Bahrami A, Folpe AL (2010) Adult-type fibrosarcoma: a reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period. Am J Surg Pathol 34:1504–1513

    Article  PubMed  Google Scholar 

  24. Romeo S, Bovée JVMG, Kroon HM, Tirabosco R, Natali C, Zanatta L, Sciot R, Mertens F, Athanasou N, Alberghini M, Szuhai K, Hogendoorn PC, Dei Tos AP (2012) Malignant fibrous histiocytoma and fibrosarcoma of bone: a re-assessment in the light of currently employed morphological, immunohistochemical and molecular approaches. Virchows Arch 461:561–570

    Article  CAS  PubMed  Google Scholar 

  25. Edge SB, Byrd DR, Compton CC, Greene FL, Trotti A (eds) (2010) American Joint Committee on Cancer (AJCC) cancer staging manual, 7th edn. Springer, New York

    Google Scholar 

  26. Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C (1984) Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 33:37–42

  27. Bourgeois JM, Knezevich SR, Mathers JA, Sorensen PH (2000) Molecular detection of the ETV6-NTRK3 gene fusion differentiates congenital fibrosarcoma from other childhood spindle cell tumors. Am J Surg Pathol 24:937–946

    Article  CAS  PubMed  Google Scholar 

  28. Ladanyi M, Antonescu CR, Leung DH, Leung DH, Woodruff JM, Kawai A, Healey JH, Brennan MF, Bridge JA, Neff JR, Barr FG, Goldsmith JD, Brooks JS, Goldblum JR, Ali SZ, Shipley J, Cooper CS, Fisher C, Skytting B, Larsson O (2002) Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res 62:135–140

    CAS  PubMed  Google Scholar 

  29. Bertoni F, Calderoni P, Bacchini P, Campanacci M (1984) Desmoplastic fibroma of bone. J Bone Joint Surg Br 66:265–268

  30. O’Neill JP, Bilsky MH, Kraus D (2013) Head and neck sarcomas: epidemiology, pathology, and management. Neurosurg Clin N Am 24:67–78

    Article  PubMed  Google Scholar 

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Correspondence to Antonella Coli.

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The authors declare that they have no conflict of interest.

Ethical statement

The present report was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Novello, M., Di Rocco, C., Tamburrini, G. et al. Recurrent adult-type fibrosarcoma of the frontal bone in a child. Childs Nerv Syst 32, 1169–1173 (2016). https://doi.org/10.1007/s00381-015-2998-6

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  • DOI: https://doi.org/10.1007/s00381-015-2998-6

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