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Desmoplastic fibroma

DEFINITION AND PATHOGENESIS

  • (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

IMPORTANCE

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

CLINICAL FEATURES

  • 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

RADIOLOGIC FEATURES

  • >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

GROSS PATHOLOGY

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

HISTOLOGIC FEATURES

  • 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")

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • 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)

DISEASE COURSE AND TREATMENT

  • 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

SPECIAL CONSIDERATION

  • Infantile Desmoplastic Fibroma

    • Involvement of the maxilla reported

REFERENCES

Abdelwahab IF, Klein MJ, Hermann G, Steiner GC, Yang DC: Osteosarcoma arsing in a desmoplastic fibroma of the proximal tibia. AJR 178:613-5,2002.
Aboulafia A, Afatoon K, Levine AM: From the oncology department...Desmoplastic fibroma: an odd lesion in a strange place. Spine J 1:302-3,2001.

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.
Chan KW, Pun WK, Choi CH: Desmoplastic fibroma of bone. Pathology 19:201-3,1987.
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.
Daneyemez M, Akay KM, Izci Y: Desmoplastic fibroma of the cervical spine. Eur Spine J 14:799-802,2005.
Dehner LP, Risdall RJ, L'Heureux P: Giant-cell containing "fibrous" lesion of the sacrum. A roentgenographic, pathologic, and ultrastructural study of three cases. Am J Surg Pathol 2:55-70,1978.

Deniz K, Ceylan D: Desmoplastic fibroma of the skull. Acta Neurochir (Wien). 2008 Mar;150(3):285-90.
de Tomas-Cabrera AR, Marcial-Seoane RA, Marcial-Rojas RA: Desmoplastic fibroma of bone: report of one case and review of the literature. Bol Asoc Med PR 82:18-24,1990.
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.
Godinho FS, Chiconelli JR, Lemos C: Desmoplastic fibroma of bone. Report of a case. J Bone Joint Surg 49:560-1,1967.
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.
Jaffe HL: Tumors and Tumorous Conditions of the Bones and Joints. Philadelphia, PA, Lea & Febiger, 1958, 298-303.

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.
Kendi TK, Erakar A, Saglik Y, Yildiz HY, Erekul S: Desmoplastic fibroma of bone: case report. Clin Imaging 27:200-2,2003.
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.
Kong KY, Kang HS, Jung HW, Kim JJ, Lee CK: MR findings of desmoplastic fibroma of the spine. A case report. Acta Radiol 41:89-91,2000.
Krakovits GE, Julow J, Illyes G: Desmoplastic fibroma in the spine. A case report. Spine 16:481-2,1991.
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.
Marks KE: Fibrous tumor of bone. Ortho Clin North Am, 20:3:377-394,1989.

Min HS, Kang HG, Lee JH, Lee GK, Ro JY: Desmoplastic fibroma with malignant transformation. Ann Diagn Pathol. 2010 Feb;14(1):50-5.
Moorjani V, Stockton V: Desmoplastic fibroma with perineural extension. AJR 185:1498-9,2005.
Nag HL, Kumar R, Bhan S, Awasthy BS, Julka PK, Ray RR: Radiotherapy for desmoplastic fibroma of bone: a case report. J Orthop Surg (Hong Kong) 11:90-3,2003.
Nilsonne U, Gothlin G: Desmoplastic fibroma of bone. Acta Orthop Scand 40:205-15,1969.
Nishida J, Tajima K, Abe M, Honda M, Inomata Y, Shimamura T, Shiraishi H, Satoh T, Ehara S: Desmoplastic fibroma. Aggressive curettage as a surgical alternative for treatment. Clin Orthop 320:142-8,1995.
Obaro RO: Case report: desmoplastic fibroma of the sternum. Clin Radiol 46:359-60,1992.
Omojola MF, Cockshott WP, Beatty EG: Desmoplastic fibroma. J Can Assoc Radiol 31:273-5,1980.
Raatikainen TK, Kaarela OI, Holmstrom T, Kyllonen AP, Teerikangas HE, Waris TH: Desmoplastic fibroma of the calcaneus treated with a microvascular bone graft. Case report. Scand J Plast Reconstr Surg Hand Surg 33:111-6,1999.
Rabhan WN, Rosai J: Desmoplastic fibroma. Report of ten cases and review of the literature. J Bone Joint Surg 50:487-502,1968.

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.

Reid EN, Lawoyin DO, Suresh L, Longwe E: Desmoplastic fibroma of the anterior mandible. Case report and review of literature. N Y State Dent J. 2009 Apr;75(3):32-3.

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

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.
Sanfilippo NJ, Wang GJ, Larner JM: Desmoplastic fibroma: a role for radiotherapy? South Med J 88:1267-9,1995.
Savy JM, Laredo JD, Prier A, Jagueux M, Apoil A, Kaplan G: Case report 728: Desmoplastic fibroma. Skeletal Radiol 21:388-91,1992.
Scudese VA: Desmoplastic fibroma of the radius. Report of a case with segmental resection. Clin Orthop 79:141-4,1971.
Schachar N, DiFrancesco L, Temple W: Musculoskeletal images. Desmoplastic fibroma of the thigh. Can J Surg 44:255-7,2001.
Scheer GE, Kuhlman RE: Vertebral involvement by desmoplastic fibroma. Report of a case. JAMA 185:669-70,1963.
Schenkar DL, Kleinert HE: Desmoplastic fibroma of the hand: case report. Plast Reconstr Surg 59:128-33,1977.

Schneider M, Simmermann AC, Depprich RA, Kubler NR, Engers R, Naujoks CD, Handschel J: Desmoplastic fibroma of the mandible--review of the literature and presentation of a rare case. Head Face Med. 2009 Nov 24;5:25.
Schultz E, Hermann G, Irwin GA, Shih H: Case report 380. Desmoplastic fibroma of the mandible. Skeletal Radiol 15:560-4,1986.

Shi H, Wang P, Wang S, Yu Q: Desmoplastic fibroma of the mandible. Dentomaxillofac Radio. 2008 Oct;37(7):408-11.
Shinomiya K, Furuya K, Mutoh N: Desmoplastic fibroma in the thoracic spine. J Spinal Disord 4:229-33,1991.
Sim FH, Wold LE, Swee RG: Fibrous tumors of bone. In Murray JA: American Academy of Orthopaedic Surgeons Instructional Course Lectures, 1984, pp 40-59.
Smith SE, Kransdorf MJ: Primary musculoskeletal tumors of fibrous origin. Semin Musculoskeletal Radiol 4:73-88,2000.
Specchiulli F, Florio U: Desmoplastic fibroma of bone. (A study of three cases). Ital J Orthop Traumatol 2:141-50,1976.
Stadler HE, Williams HL: Benign lytic tumor of the ilium (desmoplastic fibroma):--a case report. J Indiana State Med Assoc 70:642-3,1977.
Sugiura I: Desmoplastic fibroma: case report and review of the literature. J Bone Joint Surg 58A:126-30,1976.
Takazawa K, Tsuchiya H, Yamamoto N, Nonomura A, Suzuki M, Taki J, Tomita K: Osteosarcoma arising from desmoplastic fibroma treated 16 years earlier: a case report. J Orthop Sci 8:864-8,2003.
Taconis WK, Schutte HE, van der Heul RO: Desmoplastic fibroma of bone: a report of 18 cases. Skeletal Radiol 23:282-8,1994.
Templeton K: Humerus mass in a 25-year-old woman. Clin Orthop 410:334-40,2003.
Thirupathi RG, Vuletin JC, Wadwa R, Ballah S: Desmoplastic fibroma of the ulna. A case report. Clin Orthop 179:231-8,1983.
Vanhoenacker FM, Hauben E, DeBeuckeleer LH, Willemen D, Van Marck E, DeSchepper AM: Desmoplastic fibroma of bone: MRI features. Skeletal Radiol 29:171-5,2000.
West R, Huvos AG, Lane JM: Desmoplastic fibroma of bone arising in fibrous dysplasia. Am J Clin Pathol 79:630-3,1983.

Wolfe SQ, Cervantes L, Olavarria G, Brathwaite C, Ragheb J, Morrison G: Desmoplastic fibroma of the pediatric skull. Report of three cases. J Neurosurg. 2005 Oct;103(4 Suppl):362-5.

Yoshida A, Ushiku T, Motoi T, Shibata T, Beppu Y, Fukayama M, Tsuda H: Immunohistochemical analysis of MDM2 and CDK4 distinguishes low-grade osteosarcoma from benign mimics. Mod Pathol. 2010 Jul 2.
Young JW, Aisner SC, Levine AM, Resnik CS, Dorfman HD: Computed tomography of desmoid tumors of bone: desmoplastic fibroma. Skeletal Radiol 17:333-7,1988.
Yu JS, Lawrence S, Pathria M, Resnick D, Haghighi P: Desmoplastic fibroma of the calcaneus. Skeletal Radiol 24:451-4,1995.

Zhang F, Ni B, Zhao L, Chen H, Li S, Zhou F, Yang J, Tsai N: Desmoplastic fibroma of the cervical spine: case report and review of the literature. Spine (Phila Pa 1976). 2010 Jun 15;35(14):E667-71.

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