eMedicine Specialties > Radiology > Musculoskeletal

Osteochondroma and Osteochondromatosis: Imaging

Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, Consultant Radiologist and Honorary Professor, North Manchester General Hospital Pennine Acute NHS Trust, UK
Coauthor(s): Mohammed Jassim Al-Salman, MBBS, Consulting Radiologist, King Abdul Aziz Medical City, National Guard Hospital; Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute
Contributor Information and Disclosures

Updated: Oct 19, 2009

Radiography

Findings


Plain radiograph of the cervical spine shows a so...

Plain radiograph of the cervical spine shows a solitary osteochondroma of the posterior elements of C6.

Plain radiograph of the cervical spine shows a so...

Plain radiograph of the cervical spine shows a solitary osteochondroma of the posterior elements of C6.


Radiographs of both hands of 36-year-old man show...

Radiographs of both hands of 36-year-old man show multiple osteochondromas involving the radii right distal fibula, metacarpals, and phalanges. Note the large osteochondroma involving the terminal phalanx of the right index finger.

Radiographs of both hands of 36-year-old man show...

Radiographs of both hands of 36-year-old man show multiple osteochondromas involving the radii right distal fibula, metacarpals, and phalanges. Note the large osteochondroma involving the terminal phalanx of the right index finger.


Plain radiograph of the pelvis in a 41-year-old w...

Plain radiograph of the pelvis in a 41-year-old woman shows multiple osteochondromas affecting the left transverse process of L5, the iliac blades, the superior pubic rami, the ischial spines, and the femoral necks. Note the modeling deformity of the femoral necks and the narrowing of the pelvic inlet as a result of osteochondromas. Several reports have described osteochondromas interfering with normal vaginal birth in pregnancy and leading to a higher rate of Cesarean deliveries.

Plain radiograph of the pelvis in a 41-year-old w...

Plain radiograph of the pelvis in a 41-year-old woman shows multiple osteochondromas affecting the left transverse process of L5, the iliac blades, the superior pubic rami, the ischial spines, and the femoral necks. Note the modeling deformity of the femoral necks and the narrowing of the pelvic inlet as a result of osteochondromas. Several reports have described osteochondromas interfering with normal vaginal birth in pregnancy and leading to a higher rate of Cesarean deliveries.


Solitary osteochondroma. Radiograph of 24-year-ol...

Solitary osteochondroma. Radiograph of 24-year-old woman who presented with a hard, palpable mass on the medial aspect of the upper calf. A bulbous, pedunculated osteochondroma arising from the medial side of the upper tibial diaphysis and pointing away from the metaphysis is noted. The patient reported no history of hereditary multiple exostoses.

Solitary osteochondroma. Radiograph of 24-year-ol...

Solitary osteochondroma. Radiograph of 24-year-old woman who presented with a hard, palpable mass on the medial aspect of the upper calf. A bulbous, pedunculated osteochondroma arising from the medial side of the upper tibial diaphysis and pointing away from the metaphysis is noted. The patient reported no history of hereditary multiple exostoses.


Multiple osteochondromatosis. Radiograph of the p...

Multiple osteochondromatosis. Radiograph of the pelvis in a 28-year-old woman known to have hereditary multiple exostoses who presented with a painful swelling of the left buttock. Note the multiple osteochondromas and fragmentation of the osteochondroma arising from the left anterior iliac crest (see Image 8 in Multimedia).

Multiple osteochondromatosis. Radiograph of the p...

Multiple osteochondromatosis. Radiograph of the pelvis in a 28-year-old woman known to have hereditary multiple exostoses who presented with a painful swelling of the left buttock. Note the multiple osteochondromas and fragmentation of the osteochondroma arising from the left anterior iliac crest (see Image 8 in Multimedia).


The plain radiographic appearances of an osteochondroma are those of a pedunculated or sessile bony excrescence with well-defined margins. In adults, the cartilage cap often contains flecks of calcification. Osteochondromas arising from the surface of a bone contain spongiosa and cortex that appear continuous with the parent bone; this is particularly obvious in long bones.

The most common site of origin for an osteochondroma is the metaphysis at bony sites of tendon and ligamentous attachments. Osteochondromas usually point away from its point of attachment toward the diaphysis. The metaphysis of the affected tubular bone may be widened. The long tubular bones are affected most frequently. In long bones, osteochondromas are typically located at the metaphysis. The sites of predilection include the distal femoral metaphysis, the proximal humeral metaphysis, the tibia, and the fibula.

The small bones of the hands and feet are affected in around 10% of patients. The innominate bone is involved in 5% of patients. The spine is less frequently involved (2%), but it can lead to cord compression. The scapula is affected in 1% of patients.

Osteochondromas arise less frequently from flat bones than from long bones. The spine, pelvis, ribs, and scapulae are the bones most commonly affected. A subungual osteochondroma is rare, but it is particularly prone to a painful bursa (not visible on plain radiographs) and fracture. An osteochondroma of the sesamoid bone of the hallux has been described, but it is extremely rare. Osteochondromas arising from the pelvis are commonly large and are typically associated with a soft-tissue mass that may grow outward or inward, displacing adjacent structures.

Radiologically differentiating a benign tumor from a sarcoma is problematic in the pelvis, particularly when the mass has a soft-tissue component. Planar tomography is still a cost-effective and useful procedure in depicting bone detail in complex skeletal areas. Typically, osteochondromas arising from the ribs are located at the costochondral junction, where they can cause a pneumothorax/hemothorax (rare) that may be evident on a plain radiograph.42,43 When the small bones of the hands and feet are affected, the appearances of the osteochondromas are identical to those found in the long bones.

Serial radiographs showing an enlarging osteochondroma with irregularity of its margin and accompanied by a soft-tissue mass should alert the clinician to sarcomatous transformation, particularly when the finding is accompanied by pain. Bone erosions and irregularity or scattered calcification are further clues that malignant transformation may have occurred.

Hereditary multiple exostoses (HME) is characterized by multiple osteochondromas that typically involve the proximal part of the humerus and the distal and proximal portions of the femur, tibia, and fibula. Often, there are associated defects of bone modeling and bony deformities — in particular, bilateral coxa valga and widening of the proximal femoral metaphysis. Bilateral, progressive changes in the forearm have been linked to the severity of the underlying disease.

Radial bowing may ensue as a result of disproportionate ulnar shortening with relative radial overgrowth. Radial-head subluxation or dislocation may be a sequel to the radial overgrowth, with a superficial resemblance to a Madelung anomaly, but the characteristic relative elongation or dorsal subluxation of the distal ulna seen in Madelung deformity is not present.

Plain radiographic findings of dysplasia epiphysealis hemimelica (DEH) include irregular ossification occurring to 1 side of the ossifying epiphysis or a carpal or tarsal bone. The adjacent metaphysis may be widened. With progression of disease, a lobulated bony mass protrudes from the epiphysis or the carpal or tarsal bone. Severe disease is associated with muscle wasting, growth disturbance, and joint deformities.

Degree of Confidence

Plain radiography remains the primary modality for imaging osseous pathology. Experience with bone radiography extends over 100 years. The normal variants are well defined. The diagnosis of osteochondromas is straightforward, particularly at the common sites in long bones. Plain radiographs are particularly good for diagnosing complications related to osteochondromas, such as fractures, osseous deformity, and growth disturbances.

Plain radiography is inexpensive, effective, and universally available. With the advent of digital radiography, the radiation dose can be better regulated, and digital images have the advantage of better sensitivity, better image manipulation, and better storage. In addition, the images can be transmitted to distant facilities.

Osteochondromas arising from complex areas can be clarified by means of planar tomography.

False Positives/Negatives

The list of differential diagnoses for osteochondromas is extensive. Osteomas, osteophytes, enthesophytes, heterotopic ossification, and parosteal osteosarcomas can all mimic osteochondromas. The list of systemic disorders and developmental anomalies that are accompanied by osteochondromas or osteochondroma-like abnormalities is long, and these may cause confusion with solitary osteochondromas or with hereditary multiple exostoses (HME).

False-negative or false-positive diagnosis may occur with malignant transformation. However, problems may arise with resected tumors that appear radiologically aggressive, even with the histologic confirmation of malignancy.

Computed Tomography

Findings


Multiple osteochondromatosis. Nonenhanced, axial ...

Multiple osteochondromatosis. Nonenhanced, axial computed tomography (CT) scan through the pelvis (same patient as in Image 7 in Multimedia). Note the fragmentation of the osteochondroma and the considerable soft-tissue mass. Histology of the resected specimen revealed a low-grade chondrosarcoma.

Multiple osteochondromatosis. Nonenhanced, axial ...

Multiple osteochondromatosis. Nonenhanced, axial computed tomography (CT) scan through the pelvis (same patient as in Image 7 in Multimedia). Note the fragmentation of the osteochondroma and the considerable soft-tissue mass. Histology of the resected specimen revealed a low-grade chondrosarcoma.


CT scanning can provide excellent bone detail of osteochondromas developing in the spine, shoulder, or pelvis, despite the complex nature of these bones. CT myelography is useful in evaluating the size and extent of spinal osteochondromas in patients presenting with compressive myelopathy.

Degree of Confidence

CT scanning is an excellent modality for depicting bone detail in skeletal lesions and calcification within surrounding cartilage and soft tissue. One major disadvantage of CT scanning, however, is that it provides no information about the metabolic activity of bone lesions.

False Positives/Negatives

An increase in the size of osteochondromas due to bursitis is a known complication, and a false-positive diagnosis of malignant transformation has been reported with CT scanning and MRI. Therefore, ultrasonographic evaluation is always recommended for the evaluation of enlarging solitary osteochondromas.

Magnetic Resonance Imaging

Findings

MRI is useful for assessing the continuity of the parent bone with the cortical and medullary bone in an osteochondroma. Cartilage in the cap has high signal intensity on T2-weighted, spin-echo MRI scans. This characteristic allows measurement of the cap, which is an important consideration in malignant transformation. MRI also provides information about inflammation in reactive bursa formation, impingement syndromes, and arterial and venous compromise. This study is the method of choice for evaluating compression of the spinal cord, nerve roots, and peripheral nerves.50

De Beuckleer and associates showed that MRI improves accuracy in the diagnosis of low-grade chondrosarcomas.51 MRI scans contribute only to the diagnostic workup of cases in which malignant change is suspected, because osteochondromas have a characteristic appearance on plain radiographs.

With chondrosarcomas, the chondroid origin of tumors may be identified with the lobular high signal intensity. Short-tau inversion recovery (STIR) images show peritumoral, soft-tissue edema in 83% of chondrosarcomas. Muscle impingement should be considered in the differential diagnosis of pain in association with osteochondromatosis. On T2-weighted MRI scans, muscle impingement is depicted as increased signal intensity within the muscle.

Degree of Confidence

MRI is useful because it allows the depiction of the continuity of the parent bone with the cortical and medullary bone in an osteochondroma. This is an important prerequisite in differentiating osteochondromas from other surface bone lesions.

MRI allows the distinction of muscle impingement, which may be radiographically occult and can be clinically confused with other complications, such as a fracture, bursitis, or malignant degeneration. MRI also improves accuracy in diagnosing low-grade chondrosarcomas. MRI contributes only in cases in which a malignant transformation is suspected.

False Positives/Negatives

CT scanning and MRI have variable success in differentiating benign osteochondromas from malignant osteochondromas, and false-positive and false-negative studies may result. A false-positive diagnosis can occur with bursal inflammation.

Ultrasonography

Findings

Ultrasonography can be applied to analyze the cartilaginous cap of an osteochondroma. The cap appears as a hypoechoic layer covering a hyperechoic underlying bone. Malghem and associates compared ultrasonographic measurements of cap thickness with measurement performed on pathologic specimens in 22 resected exostoses and 2 exostotic chondrosarcomas.52 The ultrasonographic measurements proved accurate, with a mean measurement error of less than 2 mm for cartilaginous caps thinner than 2 cm.

Ultrasonography is also valuable in the diagnosis of bursitis and other complications associated with osteochondromas, such as arterial or venous thrombosis, as well as aneurysm and pseudoaneurysm formation.

Degree of Confidence

The detection rate and measurement accuracy of ultrasonography in the search for and evaluation of cartilaginous caps are comparable to those of MRI and are higher than those of CT. The high sensitivity and specificity of ultrasonography in peripheral vascular pathology is well established.

False Positives/Negatives

Ultrasonography remains operator dependent and can be labor intensive. Ultrasonograms cannot depict the cartilage cap when it is inwardly orientated; however, this is relatively uncommon. False-positive and false-negative results can occur, particularly in cases of deep vein thrombosis in the lower calf.

Nuclear Imaging

Findings


Osteochondroma with malignant degeneration. Plain...

Osteochondroma with malignant degeneration. Plain radiograph of the pelvis in a 68-year-old woman who presented with a painful lump over the left greater trochanter. Note the sclerotic exostosis arising from the left greater trochanter. Technetium-99m (99mTc) diphosphonate scintigram (left) shows intense activity in the region of the left greater trochanter. At surgery (right), the lesion was diagnosed as a low-grade chondrosarcoma superimposed on an osteochondroma.

Osteochondroma with malignant degeneration. Plain...

Osteochondroma with malignant degeneration. Plain radiograph of the pelvis in a 68-year-old woman who presented with a painful lump over the left greater trochanter. Note the sclerotic exostosis arising from the left greater trochanter. Technetium-99m (99mTc) diphosphonate scintigram (left) shows intense activity in the region of the left greater trochanter. At surgery (right), the lesion was diagnosed as a low-grade chondrosarcoma superimposed on an osteochondroma.


Scintigraphy with bone-seeking isotopes is an effective method of imaging osteochondromas that are metabolically active. Thallium-201 (201 Tl) scintigraphy is useful in differentiating malignant transformation from benign osteochondroma in hereditary multiple exostoses (HME).

Aoki and associates showed that fluorodeoxyglucose positron emission tomography (FDG-PET) could be an objective and quantitative adjunct in the differential diagnosis and grading of chondrosarcomas.47

Degree of Confidence

A normal isotopic bone scan virtually excludes the diagnosis of malignant transformation of an osteochondroma.

False Positives/Negatives

A positive isotopic bone scan does not allow differentiation of the endochondral ossification occurring in a benign osteochondroma from the hyperemia and osteoblastic reaction occurring in a chondrosarcoma. Negative findings of201 Tl scintigraphy may not exclude the possibility of chondrosarcomas, and the utility of this method may be limited.

In their article about the role of radionuclide scintigraphy, Hendel and associates concluded that single, standing, planar bone scintigraphy has no value in distinguishing benign osteochondromas from malignant chondrosarcomas.53

Angiography

Findings

Vascular complications can occur as a result of osteochondromas, particularly when a bone lesion occurs around the knee. In this situation, arteriography is considered essential in planning surgical treatment. Effective diagnostic imaging is a key to the early operative removal of sarcomas. Angiography has also been used to assess malignant transformation; angiograms may depict neovascularity and the true extent of disease.

Degree of Confidence

Angiography remains the criterion standard in depicting vascular pathology and is an essential part of vascular intervention.

False Positives/Negatives

False-negative angiograms are possible in true aneurysms and in false ones, because a laminated thrombus may lie along the wall and partially fill the aneurysm. In such cases, an ultrasonogram may prove invaluable.

More on Osteochondroma and Osteochondromatosis

Overview: Osteochondroma and Osteochondromatosis
Imaging: Osteochondroma and Osteochondromatosis
Follow-up: Osteochondroma and Osteochondromatosis
Multimedia: Osteochondroma and Osteochondromatosis
References
Further Reading

References

  1. Hunter J. The Works of John Hunter. vol 1. London, England: Longman, Rees; 1835.

  2. Boyer, A. Traité des maladies chirurgicales. vol 3. Paris, France: Ve Migneret; 1814:594.

  3. Guy's hospital reports case of cartilaginous exostosis. Lancet. 1825;2:91.

  4. Purandare NC, Rangarajan V, Agarwal M, Sharma AR, Shah S, Arora A, et al. Integrated PET/CT in evaluating sarcomatous transformation in osteochondromas. Clin Nucl Med. Jun 2009;34(6):350-4. [Medline].

  5. El-Fiky TA, Chow W, Li YH, To M. Hereditary multiple exostoses of the hip. J Orthop Surg (Hong Kong). Aug 2009;17(2):161-5. [Medline].

  6. Alvarez CM, De Vera MA, Heslip TR, et al. Evaluation of the anatomic burden of patients with hereditary multiple exostoses. Clin Orthop Relat Res. Sep 2007;462:73-9. [Medline].

  7. Matsumoto Y, Matsuda S, Matono K, et al. Intra-articular osteochondroma of the knee joint in a patient with hereditary multiple osteochondromatosis. Fukuoka Igaku Zasshi. Dec 2007;98(12):425-30. [Medline].

  8. Müller E. Uber hereditare multiple cartilaginare exostosen und exchondrosen. Beitr Pathol Anat. 1914;57:232.

  9. Mansoor A, Beals RK. Multiple exostosis: a short study of abnormalities near the growth plate. J Pediatr Orthop B. Sep 2007;16(5):363-5. [Medline].

  10. Porter DE, Simpson AH. The neoplastic pathogenesis of solitary and multiple osteochondromas. J Pathol. Jun 1999;188(2):119-25. [Medline].

  11. Ahn J, Ludecke HJ, Lindow S, et al. Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1). Nat Genet. Oct 1995;11(2):137-43. [Medline].

  12. Bellaiche Y, The I, Perrimon N. Tout-velu is a Drosophila homologue of the putative tumour suppressor EXT-1 and is needed for Hh diffusion. Nature. Jul 2 1998;394(6688):85-8. [Medline].

  13. Bovée JV, Cleton-Jansen AM, Wuyts W, et al. EXT-mutation analysis and loss of heterozygosity in sporadic and hereditary osteochondromas and secondary chondrosarcomas. Am J Hum Genet. Sep 1999;65(3):689-98. [Medline][Full Text].

  14. Bridge JA, Nelson M, Orndal C. Clonal karyotypic abnormalities of the hereditary multiple exostoses chromosomal loci 8q24.1 (EXT1) and 11p11-12 (EXT2) in patients with sporadic and hereditary osteochondromas. Cancer. May 1 1998;82(9):1657-63. [Medline].

  15. Hall CR, Wu Y, Shaffer LG. Familial case of Potocki-Shaffer syndrome associated with microdeletion of EXT2 and ALX4. Clin Genet. Nov 2001;60(5):356-9. [Medline].

  16. Hecht JT, Hogue D, Strong LC, et al. Hereditary multiple exostosis and chondrosarcoma: linkage to chromosome II and loss of heterozygosity for EXT-linked markers on chromosomes II and 8. Am J Hum Genet. May 1995;56(5):1125-31. [Medline][Full Text].

  17. Hecht JT, Hogue D, Wang Y, et al. Hereditary multiple exostoses (EXT): mutational studies of familial EXT1 cases and EXT-associated malignancies. Am J Hum Genet. Jan 1997;60(1):80-6. [Medline].

  18. Li H, Yamagata T, Mori M. Association of autism in two patients with hereditary multiple exostoses caused by novel deletion mutations of EXT1. J Hum Genet. 2002;47(5):262-5. [Medline].

  19. McCormick C, Leduc Y, Martindale D, et al. The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate. Nat Genet. Jun 1998;19(2):158-61. [Medline].

  20. Philippe C, Porter DE, Emerton ME. Mutation screening of the EXT1 and EXT2 genes in patients with hereditary multiple exostoses. Am J Hum Genet. Sep 1997;61(3):520-8. [Medline][Full Text].

  21. Raskind WH, Conrad EU 3rd, Matsushita M. Evaluation of locus heterogeneity and EXT1 mutations in 34 families with hereditary multiple exostoses. Hum Mutat. 1998;11(3):231-9. [Medline].

  22. Stickens D, Evans GA. Isolation and characterization of the murine homolog of the human EXT2 multiple exostoses gene. Biochem Mol Med. Jun 1997;61(1):16-21. [Medline].

  23. Stickens D, Clines G, Burbee D, et al. The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes. Nat Genet. Sep 1996;14(1):25-32. [Medline].

  24. Wells DE, Hill A, Lin X. Identification of novel mutations in the human EXT1 tumor suppressor gene. Hum Genet. May 1997;99(5):612-5. [Medline].

  25. Hogue DA, Cole WG, Hecht J. Mutational steps leading to the development of bony exostoses. Am J Hum Genet. 1998;63:365.

  26. Jäger M, Westhoff B, Portier S, et al. Clinical outcome and genotype in patients with hereditary multiple exostoses. J Orthop Res. Dec 2007;25(12):1541-51. [Medline].

  27. Wuyts W, Van Hul W, Wauters J. Positional cloning of a gene involved in hereditary multiple exostoses. Hum Mol Genet. Oct 1996;5(10):1547-57. [Medline][Full Text].

  28. Sandell LJ. Multiple hereditary exostosis, EXT genes, and skeletal development. J Bone Joint Surg Am. Jul 2009;91 Suppl 4:58-62. [Medline].

  29. Alman BA. Multiple hereditary exostosis and hedgehog signaling: implications for novel therapies. J Bone Joint Surg Am. Jul 2009;91 Suppl 4:63-7. [Medline].

  30. Ligon AH, Potocki L, Shaffer LG, et al. Gene for multiple exostoses (EXT2) maps to 11(p11.2p12) and is deleted in patients with a contiguous gene syndrome. Am J Med Genet. Feb 17 1998;75(5):538-40. [Medline].

  31. Mertens F, Rydholm A, Kreicbergs A. Loss of chromosome band 8q24 in sporadic osteocartilaginous exostoses. Genes Chromosomes Cancer. Jan 1994;9(1):8-12. [Medline].

  32. Le Merrer M, Legeai-Mallet L, Jeannin PM. A gene for hereditary multiple exostoses maps to chromosome 19p. Hum Mol Genet. May 1994;3(5):717-22. [Medline].

  33. Ludecke HJ, Schmidt O, Nardmann J. Genes and chromosomal breakpoints in the Langer-Giedion syndrome region on human chromosome 8. Hum Genet. Dec 1999;105(6):619-28. [Medline].

  34. Ludecke HJ, Wagner MJ, Nardmann J. Molecular dissection of a contiguous gene syndrome: localization of the genes involved in the Langer-Giedion syndrome. Hum Mol Genet. Jan 1995;4(1):31-6. [Medline].

  35. Wu YQ, Heutink P, de Vries BB. Assignment of a second locus for multiple exostoses to the pericentromeric region of chromosome 11. Hum Mol Genet. Jan 1994;3(1):167-71. [Medline].

  36. Wise CA, Clines GA, Massa H. Identification and localization of the gene for EXTL, a third member of the multiple exostoses gene family. Genome Res. Jan 1997;7(1):10-6. [Medline].

  37. Kitagawa H, Shimakawa H, Sugahara K. The tumor suppressor EXT-like gene EXTL2 encodes an alpha1, 4-N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glycosaminoglycan-protein linkage region. The key enzyme for the chain initiation of heparan sulfate. J Biol Chem. May 14 1999;274(20):13933-7. [Medline][Full Text].

  38. Van Hul W, Wuyts W, Hendrickx J. Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family. Genomics. Jan 15 1998;47(2):230-7. [Medline].

  39. Wuyts W, Van Hul W, Hendrickx J, et al. Identification and characterization of a novel member of the EXT gene family, EXTL2. Eur J Hum Genet. Nov-Dec 1997;5(6):382-9. [Medline].

  40. Wuyts W, Di Gennaro G, Bianco F. Molecular and clinical examination of an Italian DEFECT11 family. Eur J Hum Genet. Jul 1999;7(5):579-84. [Medline].

  41. Florez B, Mönckeberg J, Castillo G, et al. Solitary osteochondroma long-term follow-up. J Pediatr Orthop B. Mar 2008;17(2):91-4. [Medline].

  42. Castells L, Comas P, Gonzalez A, et al. Case report: haemothorax in hereditary multiple exostosis. Br J Radiol. Mar 1993;66(783):269-70. [Medline].

  43. Uchida K, Kurihara Y, Sekiguchi S. Spontaneous haemothorax caused by costal exostosis. Eur Respir J. Mar 1997;10(3):735-6. [Medline].

  44. Taniguchi K. A practical classification system for multiple cartilaginous exostosis in children. J Pediatr Orthop. Sep-Oct 1995;15(5):585-91. [Medline].

  45. Leube B, Hardt K, Portier S, et al. Ulna/height ratio as clinical parameter separating EXT1 from EXT2 families?. Genet Test. Mar 2008;12(1):129-33. [Medline].

  46. Tanigawa N, Kariya S, Kojima H, et al. Lower limb ischaemia caused by fractured osteochondroma of the femur. Br J Radiol. Apr 2007;80(952):e78-80. [Medline].

  47. Aoki J, Watanabe H, Shinozaki T, et al. FDG-PET in differential diagnosis and grading of chondrosarcomas. J Comput Assist Tomogr. Jul-Aug 1999;23(4):603-8. [Medline].

  48. Abramovici L, Steiner GC. Bizarre parosteal osteochondromatous proliferation (Nora''s lesion): a retrospective study of 12 cases, 2 arising in long bones. Hum Pathol. Dec 2002;33(12):1205-10. [Medline].

  49. Harty JA, Kelly P, Niall D, et al. Bizarre parosteal osteochondromatous proliferation (Nora's lesion) of the sesamoid: a case report. Foot Ankle Int. May 2000;21(5):408-12. [Medline].

  50. Roach JW, Klatt JW, Faulkner ND. Involvement of the spine in patients with multiple hereditary exostoses. J Bone Joint Surg Am. Aug 2009;91(8):1942-8. [Medline].

  51. De Beuckeleer LH, De Schepper AM, Ramon F. Magnetic resonance imaging of cartilaginous tumors: is it useful or necessary?. Skeletal Radiol. Feb 1996;25(2):137-41. [Medline].

  52. Malghem J, Vande Berg B, Noel H, et al. Benign osteochondromas and exostotic chondrosarcomas: evaluation of cartilage cap thickness by ultrasound. Skeletal Radiol. 1992;21(1):33-7. [Medline].

  53. Hendel HW, Daugaard S, Kjaer A. Utility of planar bone scintigraphy to distinguish benign osteochondromas from malignant chondrosarcomas. Clin Nucl Med. Sep 2002;27(9):622-4. [Medline].

  54. Azouz EM. Magnetic resonance imaging of benign bone lesions: cysts and tumors. Top Magn Reson Imaging. Aug 2002;13(4):219-29. [Medline].

  55. Bessel-Hagen F. Deformity of the forearm. Arch Klin Chir. 1891;42:42-466.

  56. Black B, Dooley J, Pyper A, et al. Multiple hereditary exostoses. An epidemiologic study of an isolated community in Manitoba. Clin Orthop Relat Res. Feb 1993;212-7. [Medline].

  57. Bloch AM, Nevo Y, Ben-Sira L, et al. Winging of the scapula in a child with hereditary multiple exostoses. Pediatr Neurol. Jan 2002;26(1):74-6. [Medline].

  58. Bovée JV, Hogendoorn PC. Re. Review article entitled 'The neoplastic pathogenesis of solitary and multiple osteochondromas'. J Pathol. Mar 2000;190(4):516-7. [Medline].

  59. Cardelia JM, Dormans JP, Drummond DS, et al. Proximal fibular osteochondroma with associated peroneal nerve palsy: a review of six cases. J Pediatr Orthop. Sep-Oct 1995;15(5):574-7. [Medline].

  60. Cates HE, Burgess RC. Incidence of brachydactyly and hand exostosis in hereditary multiple exostosis. J Hand Surg [Am]. Jan 1991;16(1):127-32. [Medline].

  61. Cho KH, Park BH, Yeon KM. Ultrasound of the adult hip. Semin Ultrasound CT MR. Jun 2000;21(3):214-30. [Medline].

  62. Cirak B, Karabulut N, Palaoglu S. Cervical osteochondroma as a cause of spinal cord compression in a patient with hereditary multiple exostoses: computed tomography and magnetic resonance imaging findings. Australas Radiol. Sep 2002;46(3):309-11. [Medline].

  63. Cook A, Raskind W, Blanton SH, et al. Genetic heterogeneity in families with hereditary multiple exostoses. Am J Hum Genet. Jul 1993;53(1):71-9. [Medline].

  64. Copeland RL, Meehan PL, Morrissy RT. Spontaneous regression of osteochondromas. Two case reports. J Bone Joint Surg Am. Jul 1985;67(6):971-3. [Medline].

  65. Davidson MB. Status of research funded by the American Diabetes Association. Diabetes. Aug 2000;49(8):1394. [Medline][Full Text].

  66. Eder HG, Oberbauer RW, Ranner G. Cervical cord compression in hereditary multiple exostoses. J Neurosurg Sci. Mar 1993;37(1):53-6. [Medline].

  67. Ehrenfried A. Multiple cartilaginous exostoses--hereditary deforming chorodysplasia: a brief report on a little known disease. JAMA. 1915;64:1642-6.

  68. Ergun R, Okten AI, Beskonakli E, et al. Cervical laminar exostosis in multiple hereditary osteochondromatosis: anterior stabilization and fusion technique for preventing instability. Eur Spine J. 1997;6(4):267-9. [Medline].

  69. Fairbank HA. Diaphyseal aclasis. J Bone Joint Surg. 1949;31(B):105-13.

  70. Felix NA, Mazur JM, Loveless EA. Acetabular dysplasia associated with hereditary multiple exostoses. A case report. J Bone Joint Surg Br. May 2000;82(4):555-7. [Medline].

  71. Fogel GR, McElfresh EC, Peterson HA. Management of deformities of the forearm in multiple hereditary osteochondromas. J Bone Joint Surg Am. Jun 1984;66(5):670-80. [Medline].

  72. Garrison RC, Unni KK, McLeod RA, et al. Chondrosarcoma arising in osteochondroma. Cancer. May 1 1982;49(9):1890-7. [Medline].

  73. Glock Y, Nehme I, Delisle MB. Acute ischemia of a limb as a complication of multiple hereditary exostoses. Case report and literature review. J Cardiovasc Surg (Torino). Feb 2000;41(1):105-8. [Medline].

  74. Golfieri R, Baddeley H, Pringle JS. Primary bone tumors. MR morphologic appearance correlated with pathologic examinations. Acta Radiol. Jul 1991;32(4):290-8. [Medline].

  75. Gordon SL, Buchanan JR, Ladda RL. Hereditary multiple exostoses: report of a kindred. J Med Genet. Dec 1981;18(6):428-30. [Medline][Full Text].

  76. Griffiths HJ, Thompson RC Jr, Galloway HR. Bursitis in association with solitary osteochondromas presenting as mass lesions. Skeletal Radiol. 1991;20(7):513-6. [Medline].

  77. Hennekam RC. Hereditary multiple exostoses. J Med Genet. Apr 1991;28(4):262-6. [Medline].

  78. Jahss MH, Olives R. The foot and ankle in multiple hereditary exostoses. Foot Ankle. Nov 1980;1(3):128-42. [Medline].

  79. Kolar J, Matejovsky Z, Zidkova H, et al. Malignization of diaphyseal aclasis. Diagn Imaging. 1983;52(1):1-9. [Medline].

  80. Krooth RS, Macklin MT, Hilbish TF. Diaphyseal aclasis (multiple exostosis) on Guam. Am J Hum Genet. 1961;13:340-7.

  81. Langer LO Jr, Krassikoff N, Laxova R. The tricho-rhino-phalangeal syndrome with exostoses (or Langer-Giedion syndrome): four additional patients without mental retardation and review of the literature. Am J Med Genet. Sep 1984;19(1):81-112. [Medline].

  82. Levin KH, Wilbourn AJ, Jones HR Jr. Childhood peroneal neuropathy from bone tumors. Pediatr Neurol. Jul-Aug 1991;7(4):308-9. [Medline].

  83. McCornack EB. The surgical management of hereditary multiple exostosis. Ortho Rev. 1981;5:57-63.

  84. Mermer MJ, Gupta MC, Salamon PB, et al. Thoracic vertebral body exostosis as a cause of myelopathy in a patient with hereditary multiple exostoses. J Spinal Disord Tech. Apr 2002;15(2):144-8. [Medline].

  85. Meyer CA, White CS. Cartilaginous disorders of the chest. Radiographics. Sep-Oct 1998;18(5):1109-23; quiz 1241-2. [Medline].

  86. Murphey MD, Choi JJ, Kransdorf MJ, et al. Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation. Radiographics. Sep-Oct 2000;20(5):1407-34. [Medline][Full Text].

  87. Nawata K, Teshima R, Minamizaki T, et al. Knee deformities in multiple hereditary exostoses. A longitudinal radiographic study. Clin Orthop Relat Res. Apr 1995;194-9. [Medline].

  88. Ochsner PE. [Multiple cartilaginous exostoses and neoplastic degeneration: review of the literature (author's transl)]. Z Orthop Ihre Grenzgeb. 1978;116(3):369-78. [Medline].

  89. Oviedo A, Simmons T, Benya E. Bizarre parosteal osteochondromatous proliferation: case report and review of the literature. Pediatr Dev Pathol. Sep-Oct 2001;4(5):496-500. [Medline].

  90. Paik NJ, Han TR, Lim SJ. Multiple peripheral nerve compressions related to malignantly transformed hereditary multiple exostoses. Muscle Nerve. Aug 2000;23(8):1290-4. [Medline].

  91. Peterson HA. Multiple hereditary osteochondromata. Clin Orthop Relat Res. Feb 1989;222-30. [Medline].

  92. Pierz KA, Stieber JR, Kusumi K, et al. Hereditary multiple exostoses: one center's experience and review of etiology. Clin Orthop Relat Res. Aug 2002;49-59. [Medline].

  93. Porter DE, Benson MK, Hosney GA. The hip in hereditary multiple exostoses. J Bone Joint Surg Br. Sep 2001;83(7):988-95. [Medline].

  94. Quirini GE, Meyer JR, Herman M. Osteochondroma of the thoracic spine: an unusual cause of spinal cord compression. AJNR Am J Neuroradiol. May 1996;17(5):961-4. [Medline][Full Text].

  95. Raskind WH, Conrad EU, Chansky H. Loss of heterozygosity in chondrosarcomas for markers linked to hereditary multiple exostoses loci on chromosomes 8 and 11. Am J Hum Genet. May 1995;56(5):1132-9. [Medline].

  96. Sakai D, Mochida J, Toh E. Spinal osteochondromas in middle-aged to elderly patients. Spine. Dec 1 2002;27(23):E503-6. [Medline].

  97. Schmale GA, Conrad EU 3rd, Raskind WH. The natural history of hereditary multiple exostoses. J Bone Joint Surg Am. Jul 1994;76(7):986-92. [Medline].

  98. Shapiro SA, Javid T, Putty T. Osteochondroma with cervical cord compression in hereditary multiple exostoses. Spine. Jun 1990;15(6):600-2. [Medline].

  99. Sharma MC, Arora R, Deol PS. Osteochondroma of the spine: an enigmatic tumor of the spinal cord. A series of 10 cases. J Neurosurg Sci. Jun 2002;46(2):66-70; discussion 70. [Medline].

  100. Shin HT, Chang MW. Trichorhinophalangeal syndrome, type II (Langer-Giedion syndrome). Dermatol Online J. Dec 2001;7(2):8. [Medline].

  101. Silber JS, Mathur S, Ecker M. A solitary osteochondroma of the pediatric thoracic spine: a case report and review of the literature. Am J Orthop. Sep 2000;29(9):711-4. [Medline].

  102. Snearly WN, Peterson HA. Management of ankle deformities in multiple hereditary osteochondromata. J Pediatr Orthop. Jul-Aug 1989;9(4):427-32. [Medline].

  103. Solomon L. Bone growth in diaphysial aclasis. J Bone Joint Surg. 1961;43-B:700-16.

  104. Solomon L. Carpal and tarsal exostosis in hereditary multiple exostoses. Clin Radiol. Oct 1967;18(4):412-6. [Medline].

  105. Solomon L. Chondrosarcoma in hereditary multiple exostosis. S Afr Med J. Apr 6 1974;48(16):671-6. [Medline].

  106. Solomon L. Hereditary multiple exostosis. Am J Hum Genet. Sep 1964;16:351-63. [Medline][Full Text].

  107. Solomon L. Hereditary multiple exostosis. J Bone Joint Surg. 1963;45-B:292-304.

  108. Song KS. Spontaneous regression of osteochondromatosis of the radius after lengthening of the ulna: a case report. J Pediatr Orthop. Sep-Oct 2000;20(5):689-91. [Medline].

  109. Spatz DK, Guille JT, Kumar SJ. Distal tibiofibular diastasis secondary to osteochondroma in a child. Clin Orthop Relat Res. Dec 1997;195-7. [Medline].

  110. Stanton RP, Hansen MO. Function of the upper extremities in hereditary multiple exostoses. J Bone Joint Surg Am. Apr 1996;78(4):568-73. [Medline].

  111. Tani A, Tsuchimochi S, Nakabeppu Y. Bone and Tl-201 scintigraphy in a case of hereditary multiple exostoses. Clin Nucl Med. Dec 2001;26(12):1028-31. [Medline].

  112. Trevor D. Tarso-epiphysial aclasis: a congenital error of epiphysial development. J Bone Joint Surg. 1950;32-B:204-13.

  113. Uri DS, Dalinka MK, Kneeland JB. Muscle impingement: MR imaging of a painful complication of osteochondromas. Skeletal Radiol. Oct 1996;25(7):689-92. [Medline].

  114. Vallance R, Hamblen DL, Kelly IG. Vascular complications of osteochondroma. Clin Radiol. Nov 1985;36(6):639-42. [Medline].

  115. Vasseur MA, Fabre O. Vascular complications of osteochondromas. J Vasc Surg. Mar 2000;31(3):532-8. [Medline].

  116. Virchow R. Ueber th Entstenhung des Enchondroms und seine Besihungen zur Enchondrosis und Exostosis cartilaginea. Manatsberichte der Koniglichen Preussischen Akademie der Wissenshaften. 1876:760.

  117. Voutsinas S, Wynne-Davies R. The infrequency of malignant disease in diaphyseal aclasis and neurofibromatosis. J Med Genet. Oct 1983;20(5):345-9. [Medline][Full Text].

  118. Weiner DS, Hoyt WA Jr. The development of the upper end of the femur in multiple hereditary exostosis. Clin Orthop Relat Res. Nov-Dec 1978;187-90. [Medline].

  119. Wicklund CL, Pauli RM, Johnston D. Natural history study of hereditary multiple exostoses. Am J Med Genet. Jan 2 1995;55(1):43-6. [Medline].

  120. Woertler K, Lindner N, Gosheger G. Osteochondroma: MR imaging of tumor-related complications. Eur Radiol. 2000;10(5):832-40. [Medline].

Further Reading

Related eMedicine topics

Dysplasia Epiphysealis Hemimelica

Solitary Osteochondroma

Bone Metastases

Clinical guidelines

ACR Appropriateness Criteria® chronic hip pain
Taljanovic M, Daffner RH, Weissman BN, Bennett DL, Bleba JS, Jacobson JA, Morrison WB, Resnik CS, Roberts CC, Schweitzer ME, Seeger LL, Wise JN, Payne WK, Expert Panel on Musculoskeletal Imaging. ACR Appropriateness Criteria® chronic hip pain. [online publication]. Reston (VA): American College of Radiology (ACR); 2008. 8 p. [78 references]

Clinical trials

The Health Related Quality of Life in Patients With Hereditary Multiple Exostoses

Gene Mutations and Orthopaedic Symptoms Correlation of Multiple Hereditary Exostoses: Multicentre Project

Keywords

osteochondroma, osteochondromatosis, hereditary multiple exostoses, HME, multiple osteocartilaginous exostoses, diaphyseal achalasia, diaphysial achalasia, multiple hereditary osteochondromata, multiple congenital osteochondromata, diaphyseal aclasis, diaphysial aclasis, chondral osteogenic dysplasia of direction, chondral osteoma, deforming chondrodysplasia, dyschondroplasia exostosing disease, exostotic dysplasia, multiple osteomatoses osteogenic disease, familial bony spurs, metaphyseal spurs, multiple epiphyseal dysplasia, dysplasia epiphysealis hemimelica, Trevor disease, Trevor's disease

Contributor Information and Disclosures

Author

Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, Consultant Radiologist and Honorary Professor, North Manchester General Hospital Pennine Acute NHS Trust, UK
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR is a member of the following medical societies: American Association for the Advancement of Science, American Institute of Ultrasound in Medicine, British Medical Association, British Society of Interventional Radiology, Royal College of Physicians, Royal College of Physicians and Surgeons of the United States, Royal College of Radiologists, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.

Coauthor(s)

Mohammed Jassim Al-Salman, MBBS, Consulting Radiologist, King Abdul Aziz Medical City, National Guard Hospital
Disclosure: Nothing to disclose.

Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.

Medical Editor

Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Michael A Bruno, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Society of Nuclear Medicine, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Theodore E Keats, MD, Professor, Departments of Radiology and Orthopedics, University of Virginia School of Medicine
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.