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Desmoplastic fibroma


  • (Desmoid tumor of bone, aggressive fibromatosis of bone)
  • The bony counterpart of the soft tissue desmoid tumor (aggressive fibromatosis)
  • Rare benign aggressive lesion occuring in metaphyseal location and the mandible


  • May originate from myofibroblasts, differentiating it from a low grade fibrosarcoma may be difficult


  • Usually presents in the first 3 decades of life; 90% between 15-40 years of age
  • 90% present with pain and the symptoms have been present on the average for 7 months
  • 15% present with pathologic fx
  • Incidence in Gardner's syndrome (± hereditary etiology)
  • May regress after menopause (± hormonal etiology)
  • TMJ dysfunction in a child with mandible location reported
  • Unusual location


  • >50% in femur, tibia, humerus, radius, 26% in mandible, 14% in pelvis
  • Average size is 7-10cm
  • Very similar to NOF except that they are central within the metaphysis
  • Can become more aggressive appearing with soft tissue masses (50%), cortical erosions, and pathological fx
  • Osteolytic with trabeculated, soap-bubble pattern
  • ± sequestrum (thick, course trabeculae within the lesion)
    • Other lesions associated with a bony sequestrum
      • Osteomyelitis
      • Skeletal tuberculosis
      • Radiation necrosis
      • Eosinophilic granuloma
      • Metastatic carcinoma
      • Primary lymphoma of bone
      • Fibrosarcoma of bone
  • No periosteal reaction unless there is a fx
  • Rarely will be parosteal (more aggressive)
  • Unusual location: rib
  • Bone scan shows increased central activity within the lesion
  • CT: more accurate assessment of extent of bone destruction including cortical breakthrough and articular invasion
  • MRI: demonstrates intramedullary tumor growth and soft tissue extension best
    • T1-weighted images: nonspecific low signal intensity
    • T2-weighted images: intermediate to high signal intensity with areas of low signal intensity, inhomogeneous enhancement
    • Intermediate to low signal intensity foci on T2-weighted images do not correspond to calcifications


  • Lobulated and firm lesion within the intramedullary canal, rarely juxtacortical
  • (If there is a soft tissue mass, rule out fibrosarcoma or MFH)


  • Identical to fibromatosis of soft parts
  • Hypocellular, well-formed collagenous matrix, spindled fibroblasts with monotonous, uniform nuclei
  • No trabeculae which helps to differentiate this from FD
  • Cellularity within a single specimen fairly constant
  • Extensive cartilaginous metaplasia reported
  • EM: "dense bodies" (as seen in fibroblastic and myofibroblastic cells), RER with dilated cisternae and other organelles for production of collagen, cell-to-cell connections ("microtendons")


  • If there is increased cellularity, increased pleomorphism, decreased collagen, soft tissue mass, think low-grade fibrosarcoma or MFH
  • Juxtacortical or fibromatosis (desmoid) of soft parts
  • Fibrous dysplasia
  • Chondromyxoid fibroma
  • GCT
  • NOF
  • Low-grade osteosarcoma (fibrous dysplasia-like variant)
  • Metastasis (thyroid or renal carcinoma)


  • Tx is wide surgical margins due to high rate of recurrence with intralesional resection (40%), although reports of aggressive curettage with excellent 3-5 yr follow-up
  • Slowly growing and locally aggressive without development of metastases
  • ± chemotx, XRT have been used in the past
  • Amputation seldomly indicated (lesion never metastasizes)
  • Extension to the neck, mediastinum, or abdomen may involve vital structures threatening survival
  • Malignant transformation reported


  • Infantile Desmoplastic Fibroma

    • Involvement of the maxilla reported


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Alemdaroglu KB, Iltar S, Pulat H, Atlihan D: Intermittent leg pain and swelling in a 30-year-old man. Clin Orthop Relat Res. 2007 Sep;462:248-54.

Apaydin M, Gelal F, Avci A, Adinas C, Sarsilmaz A, Varer M: Desmoplastic fibroma in humerus. J Med Imaging Radiat Oncol. 2008 Oct;52(5):489-90.
Barbashina V, Karabakhtsian R, Aisner S, Bolanowski P, Patterson F, Hameed M: Desmoplastic fibroma of the rib. Arch Pathol Lab Med 126:721-2,2002.
Bahk WJ, Kang YK, Lee AH, Mirra JM: Desmoid tumor of bone with enchondromatous nodules, mistaken for chondrosarcoma. Skeletal Radiol 32:223-6,2003.
Bertoni F, Calderoni P, Bacehini P, Campanacci M: Desmoplastic fibroma of bone. A report of six cases. J Bone Joint Surg 66:265-8,1984.
Beskin JL, Haddad RJ Jr: Desmoplastic fibroma of the first metatarsal. A case report. Clin Orthop 195:299-303,1985.
Bohm P, Krober S, Greschniok A, Laniado M, Kaiserling F: Desmoplastic fibroma of bone: a report of two patients, review of the literature, and therapeutic implications. Cancer 78:1011-23,1996.
Bridge JA, Swarts SJ, Buresh C, Nelson M, Degenhardt JM, Spanier S: Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. Am J Pathol 154:729-3,1999.
Bridge JA, Rosenthal H, Sanger WG, Neff JR: Desmoplastic fibroma arising in fibrous dysplasia. Chromosomal analysis and review of the literature. Clin Orthop 247:272-8,1989.
Butters M, Hamann H, Mohr W: Desmoplastic fibroma of the rib. Thorac Cardiovasc Surg 33:317-8,1985.

Callahan KS, Eberhardt SC, Fechner RE, Cerilli LA: Desmoplastic fibroma of bone with extensive cartilaginous metaplasia. Ann Diagn Pathol. 2006 Dec;10(6):343-6.
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Clayer M, Oakeshott R: Allograft bone in the treatment of desmoplastic fibroma. A case report. Clin Orthop 300:219-24,1994.
Cohen P, Goldenberg RR: Desmoplastic fibroma of bone; report of two cases. J Bone Joint Surg 47A:1620-5,1965.
Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW: Desmoplastic fibroma of bone: radiographic analysis. Radiology 172:827-32,1989.
Dahlin DC, Hoover NW: Desmoplastic fibroma of bone. Report of two cases. JAMA 188:685-7,1964.
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Deniz K, Ceylan D: Desmoplastic fibroma of the skull. Acta Neurochir (Wien). 2008 Mar;150(3):285-90.
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el-Tabbakh AO, Al-Arabi KM: Desmoplastic fibroma--a rare tumour in a rare site. Int Orthop 10:261-3,1986.
Frick MA, Sundaram M, Unni KK, Inwards CY, Fabbri N, Trentani F, Baccini P, Bertoni F: Imaging findings in desmoplastic fibroma of bone: distinctive T2 characteristics. AJR 184:1762-7,2005.
Fuji T, Hamada H, Masuda T, Yonenobu K, Fujiwara K, Yamashita K, Ono K: Desmoplastic fibroma of the axis. A case report. Clin Orthop 234:16-20,1988.
Gebhardt MC, Campbell CJ, Schiller AL, Mankin HJ: Desmoplastic fibroma of bone: a report of 8 cases and review of the literature. J Bone Joint Surg 67A:732-47,1985.
George A, Srivastava VM, Sundararaj GD: Desmoplastic fibroma of the proximal ulna. Australas Radiol 41:199-200,1997.
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Graudal N: Desmoplastic fibroma of bone. Case report and literature review. Acta Orthop Scand 55:215-9,1984.
Greenspan A, Unni KK: Case report 787: Desmoplastic fibroma. Skeletal Radiol 22:296-9,1993.
Grimer RJ, Carter SR, Sneath RS: Management of a huge tumour of the humerus by total replacement of the humerus: an 11-year follow-up. Arch Orthop Trauma Surg 117:298-9,1998.
Hadjipavlou A, Lander PH, Begin LR, Eibel P: Desmoplastic fibroma of a metatarsal. Case report. J Bone Joint Surg 68:459-61,1986.
Haney J, Olson PN, Griffiths HJ: Radiologic case study. The clinical and radiologic features of desmoplastic fibroma of bone. Orthopedics 17:77,80-5,88,1994.
Hardes J, Gosheger G, Halm H, Winkelmann W, Liljenqvist U: Three-level en bloc spondylectomy for desmoplastic fibroma of the thoracic spine: a case report. Spine 28:E169-72,2003.
Hardy R, Lehrer H: Desmoplastic fibroma vs. desmoid tumor of bone. Two cases illustrating a problem in differential diagnosis and classification. Radiology 88:899-901,1967.
Hauben EI, Jundt G, Cleton-Jansen AM, Yavas A, Kroon HM, Van Marck E, Hogendoorn PC: Desmoplastic fibroma of bone: an immunohistochemical study including beta-catenin expression and mutational analysis for beta-catenin. Hum Pathol 36:1025-30,2005.
Hayasaka K, Mineta M, Sautoh Y, Anioh K, Muraoka S, Miyatake Y: Desmoplastic fibroma of bone. Radiat Med 11:27-30,1993.
Hillman JS, Mesgarzadeh M, Tang CK, Bonakdarpour A, Reyes TG: Case report 481: Benign intraosseous fibroma (desmoplastic fibroma) associated with Paget disease of the iliac bone. Skeletal Radiol 17:356-9,1988.

Horiuchi T, Unoki T, Yokoh A: Desmoplastic fibroma of the calvarium. J Clin Neurosci. 1998 Jan;5(1):102-5.

Iatrou IA, Theologie-Lydidakis N, Leventis MD: Case report: desmoplastic fibroma of the mandible in a child presenting with TMJ dysfunction. Eur Arch Paediatr Dent. 2008 Jun;9(2):105-8.

Ikeshima A, Utsunomiya T: Case report of intra-osseous fibroma: a study on odontogenic and desmoplastic fibromas with a review of the literature. J Oral Sci. 2005 Sep;47(3):149-57.
Inwards CY, Unni KK, Beabout JW, Sim FH: Desmoplastic fibroma of bone. Cancer 68:1978-83,1991.
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Jennin F, Bousson V, Parlier C, Jomaah N, Khanine V, Laredo JD: Bony sequestrum: a radiologic review. Skeletal Radiol. 2010 Jun 23.
Juergens KU, Bullmann V, Link TM, Brinkschmidt C, Heindel W: Desmoplastic fibroma in the thoracic spine: an unusual localization of a rare primary bone tumor. Eur Radiol 11:273-5,2001.
Kamimura M, Itoh H, Kinoshita T, Yuzawa Y, Takahashi J, Takaoka K: Thoracic epidural desmoplastic fibroma. J Spinal Disord 13:267-70,2000.
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Kocher MS, Gebhardt MC, Mankin HJ: Reconstruction of the distal aspect of the radius with use of an osteoarticular allograft after excision of a skeletal tumor. J Bone Joint Surg 80:407-19,1998.
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Kuhlman RE, McNamee MJ: Quantitative microchemical studies of chondroblastoma, giant-cell tumor, chondromyxoid fibroma and desmoplastic fibroma. Clin Orthop 69:264-70,1970.
Lagace R, Delage C, Bouchard HL, Seemayer TA: Desmoplastic fibroma of bone. An ultrastructural study. Am J Surg Pathol 3:423-30,1979.

Lath R, Ranjan A, Ratnam BG, Gowrishankar S: Desmoplastic fibroma of the frontal bone. Neurol India. 2006 Sep;54(3):314-5.
Lichtman EA, Klein MJ: Case report 302. Desmoplastic fibroma of the proximal end of the left femur. Skeletal Radiol 13:160-3,1985.
Mahuken AH, Nolte-Ernsting CC, Wildberger JE, Wirtz DC, Gunther RW: Cross-sectional imaging patterns of desmoplastic fibroma. Eur Radiol 11:1105-10,2001.
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Min HS, Kang HG, Lee JH, Lee GK, Ro JY: Desmoplastic fibroma with malignant transformation. Ann Diagn Pathol. 2010 Feb;14(1):50-5.
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Rastogi S, Varshney MK, Trikha V, Khan SA, Mittal R: Desmoplastic fibroma: a report of three cases at unusual locations. Joint Bone Spine. 2008 Mar;75(2):222-5.

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Salah MB, Mekni A, Azouz H, Blel A, Kharrat S, Kchir N, Houet S, Zitouna M: Infantile desmoplastic fibroma of the maxilla. Tunis Med. 2008 Aug;86(8):749-51.
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