Advertisement
Review| Volume 65, ISSUE 5, P564-571, May 2012

Facial paediatric desmoid fibromatosis: A case series, literature review and management algorithm

Published:December 12, 2011DOI:https://doi.org/10.1016/j.bjps.2011.09.027

      Summary

      Desmoid fibromatosis (also known as infantile or aggressive fibromatosis) is a rare soft tissue tumour that is occasionally seen in children. Although histologically benign, its growth pattern is highly aggressive often showing invasion of surrounding musculature and bone.
      Frequently found in cosmetically sensitive areas, complete excision can present a challenging problem. However, incomplete surgical excision is associated with high recurrence rates and although the disease responds to chemo and radiotherapy, both carry significant risks in young children.
      The management of four paediatric desmoid fibromatoses occurring in the midface is discussed. The recent and pertinent literature is comprehensively reviewed and an algorithm for the management of paediatric desmoid fibromatoses is proposed.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Plastic, Reconstructive & Aesthetic Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

      1. Fibroblastic/myofibroblastic tumours.
        in: Fletcher C.D.M. Unni K.K. Lyon Mertens F. International Agency for Research on Cancer. Pathology and genetics of tumours of soft tissue and bone. IARC Press, 2002
        • Reitamo J.J.
        • Hayry P.
        • Nykyri E.
        • Saxen E.
        The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population.
        Am J Clin Pathol. 1982; 77: 665-673
        • Meazza C.
        • Bisogno G.
        • Gronchi A.
        • et al.
        Aggressive fibromatosis in children and adolescents. The Italian experience.
        Cancer. 2010; 116: 233-240
        • Enzinger F.M.
        • Weiss S.W.
        Soft tissue tumors.
        3rd ed. Mosby, St. Louis1995
        • Faulkner L.B.
        • Hajdu S.I.
        • Kher U.
        • et al.
        Pediatric desmoid tumor: retrospective analysis of 63 cases.
        J Clin Oncol. 1995; 13: 2813-2818
        • Huang K.
        • Fu H.
        • Shi Y.Q.
        • Zhou Y.
        • Du C.Y.
        Prognostic factors for extra-abdominal and abdominal wall desmoids: a 20-year experience at a single institution.
        J Surg Oncol. 2009; 100: 563-569
        • Carr R.J.
        • Zaki G.A.
        • Leader M.B.
        • Langdon J.D.
        Infantile fibromatosis with involvement of the mandible.
        Br J Oral Maxillofac Surg. 1992; 30: 257-262
        • Janahi W.M.
        • Darwish A.
        • Paulose K.O.
        • al-Khalifa S.
        Infantile fibromatosis.
        J Laryngol Otol. 1999; 113: 246-249
        • Sharma A.
        • Ngan B.Y.
        • Sándor G.K.
        • Campisi P.
        • Forte V.
        Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review.
        J Pediatr Surg. 2008; 43: 1596-1604
        • Lakhan S.E.
        • Eager R.M.
        • Harle L.
        Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review.
        J Hematol Oncol. 2008; 1: 3
        • Goldblum J.
        • Fletcher J.A.
        Desmoid-type fibromatosis.
        in: Fletcher C.D.M. Unni K.K. Mertens F. World Health Organization classification of tumors: Pathology and genetics of tumors of soft tissue and bone. International Agency for Research on Cancer Press, Lyon, France2002: 83-84
        • Chung K.H.
        • Charlton A.
        • Arbuckle S.
        • Chaseling R.
        • Owler B.K.
        Metachronous multifocal desmoid-type fibromatoses along the neuraxis with adenomatous polyposis syndrome.
        J Neurosurg Pediatr. 2010; 6: 372-376
        • Matsuo M.
        • Nakamura S.
        • Matsuo M.
        • Hamasaki Y.
        • Miyazaki S.
        • Watanabe T.
        A case of multiple infantile fibromatosis of unclassified type.
        Acta Paediatr Jpn. 1989; 31: 605-608
        • Buitendijk S.
        • van de Ven C.P.
        • Dumans T.G.
        • et al.
        Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature.
        Cancer. 2005; 104: 1090-1099
        • Mitchell G.
        • Thomas J.M.
        • Harmer C.L.
        Aggressive fibromatosis: evidence for a stable phase.
        Sarcoma. 1998; 2: 149-154
        • Hoffman C.D.
        • Levant B.A.
        • Hall R.K.
        Aggressive infantile fibromatosis: report of a case undergoing spontaneous regression.
        J Oral Maxillofac Surg. 1993; 51: 1043-1047
        • Allen P.W.
        The fibromatosis: a clinicopathological classification based on 140 cases.
        Am J Surg Pathol. 1977; 1: 255-260
        • Kadmon M.
        • Möslein G.
        • Buhr H.J.
        • Herfarth C.
        Desmoid tumors in patients with familial adenomatous polyposis (FAP). Clinical and therapeutic observations from the Heidelberg polyposis register.
        Chirurg. 1995; 66: 997-1005
        • Alman B.A.
        • Li C.
        • Pajerski M.E.
        • Diaz-Cano S.
        • Wolfe H.J.
        Increased β-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors).
        Am J Pathol. 1997; 151: 329-334
        • Ishizuka M.
        • Hatori M.
        • Dohi O.
        • et al.
        Expression profiles of sex steroid receptors in desmoid tumors.
        Tohoku J Exp Med. 2006; 210: 189-198
        • Reitamo J.J.
        • Scheinin T.M.
        • Hayry P.
        The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor.
        Am J Surg. 1986; 151: 230-237
        • Weiss S.W.
        Histological typing of soft tissue tumours.
        2nd ed. Springer-Verlag, London1994
        • Qi H.
        • Dal Cin P.
        • Hernández J.M.
        • et al.
        Trisomies 8 and 20 in desmoid tumors.
        Cancer Genet Cytogenet. 1996; 92: 147-149
        • Fletcher J.A.
        • Naeem R.
        • Xiao S.
        • Corson J.M.
        Chromosome aberrations in desmoid tumors. Trisomy 8 may be a predictor of recurrence.
        Cancer Genet Cytogenet. 1995; 79: 139-143
        • Breiner J.A.
        • Nelson M.
        • Bredthauer B.D.
        • Neff J.R.
        • Bridge J.A.
        Trisomy 8 and trisomy 14 in plantar fibromatosis.
        Cancer Genet Cytogenet. 1999; 108: 176-177
        • Dal Cin P.
        • DeSmet L.
        • Sciot R.
        • Van Damme B.
        • Van den Berghe H.
        Trisomy 7 and trisomy 8 in dividing and nondividing tumor cells in Dupuytren’s disease.
        Cancer Genet Cytogenet. 1999; 108: 137-140
        • Schofield D.E.
        • Fletcher J.A.
        • Grier H.E.
        • Yunis E.J.
        Fibrosarcoma in infants and children. Application of new techniques.
        Am J Surg Pathol. 1994; 18: 14-24
        • Bridge J.A.
        • Swarts S.J.
        • Buresh C.
        • 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: 729-733
        • Bhattacharya B.
        • Dilworth H.P.
        • Iacobuzio-Donahue C.
        • et al.
        Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.
        Am J Surg Pathol. 2005; 29: 653-659
        • Hoos A.
        • Lewis J.J.
        • Antonescu C.R.
        • et al.
        Characterization of molecular abnormalities in human fibroblastic neoplasms: a model for genotype-phenotype association in soft tissue tumors.
        Cancer Res. 2001; 61: 3171-3175
        • National Comprehensive Cancer Network Soft Tissue Sarcoma Panel
        Soft tissue sarcoma.
        J Natl Compr Canc Netw. 2010; 8: 630-674
        • Merchant N.B.
        • Lewis J.J.
        • Woodruff J.M.
        • et al.
        Extremity and trunk desmoid tumors: a multifactorial analysis of outcome.
        Cancer. 1999; 86: 2045-2052
        • Guadagnolo B.A.
        • Zagars G.K.
        • Ballo M.T.
        • et al.
        Long-term outcomes for desmoid tumors treated with radiation therapy.
        Int J Radiat Oncol Biol Phys. 2008; 71: 441-447
        • Jabbari S.
        • Andolino D.
        • Weinberg V.
        • et al.
        Successful treatment of high risk and recurrent pediatric desmoids using radiation as a component of multimodality therapy.
        Int J Radiat Oncol Biol Phys. 2009; 75: 177-182
        • Francis W.P.
        • Zippel D.
        • Mack L.A.
        • et al.
        Desmoids: a revelation in biology and treatment.
        Ann Surg Oncol. 2009; 16: 1650-1654
        • El-Haddad M.
        • El-Sebaie M.
        • Ahmad R.
        • et al.
        Treatment of aggressive fibromatosis: the experience of a single institution.
        Clin Oncol (R Coll Radiol). 2009; 21: 775-780
        • Schmidt D.
        • Klinge P.
        • Leuschner I.
        • Harms D.
        Infantile desmoid-type fibromatosis. Morphological features correlate with biological behaviour.
        J Pathol. 1991; 164: 315-319
        • Melis M.
        • Zager J.S.
        • Sondak V.K.
        Multimodality management of desmoid tumors: how important is a negative surgical margin?.
        J Surg Oncol. 2008 Dec 15; 98: 594-602
        • Ayala A.G.
        • Ro J.Y.
        • Goepfert H.
        • Cangir A.
        • Khorsand J.
        • Flake G.
        DF: a clinicopathologic study of 25 children.
        Semin Diagn Pathol. 1986; 3: 138-150
        • Spiegel D.A.
        • Dormans J.P.
        • Meyer J.S.
        • et al.
        Aggressive fibromatosis from infancy to adolescence.
        J Pediatr Orthop. 1999; 19: 776-784