Pancras C.W. Hogendoorn, MD, PhD Principle Researcher MHERF research registry Professor of Pathology,
Department of Pathology, Leiden University Medical Center, Molecular tumor pathology and tumor genetics,
Netherlands. Head of EuroBoneT consortium, a European Commission granted Network of Excellence for
studying the pathology and genetics of bone tumors. In 2002 the World Health Organization (WHO) has
redefined the definition of Multiple Hereditary Exostoses (MHE) into Multiple Osteochondromas (MO)(Bovee and
Hogendoorn, 2002).
This was done because of the very wide and a-specific use both inside the medical community as well as by
patients of the term "exostosis". It ranges in use from osteochondroma(Khurana et al., 2002), the benign
cartilage tumor involved in MHE, to a perversion in the direction of growth (eg Nora's lesion or Trevor disease)
and even to a reactive process (eg subungual exostosis). Therefore the WHO decided to redefine the
terminology for the bony outgrowths into different terms reflecting the different nature of the diseases involved
aiming at a better defined diagnosis. "Exostoses" involved in MHE are now specified as osteochondromas and
defined as benign cartilaginous tumors.
The current WHO definition of an osteochondroma is "a cartilage capped bony projection arising on the
external surface of bone containing a marrow cavity that is continuous with that of the underlying bone" (WHO
2002)(Khurana et al., 2002). With this changein terminology, Multiple Hereditary Exostoses is no longer
applicable as name for this disorder . That is why the WHO also changed the name of MHE to Multiple
Osteochondromas (MO) and stated that: "A diagnosis of multiple exostoses can be made when radiologically at
least two osteochondromas of the juxta-epiphyseal region of long bones are observed. MO is diagnosed in
case of a positive family history and/or a proven germline mutation in one of the EXT genes" (WHO 2002)
(Bovee and Hogendoorn, 2002).
It is very important to correctly diagnosis Multiple Osteochondromas. Especially good monitoring of the patient
is important after Multiple Osteochondromas is established.This scheme was taken from "Multiple
Osteochondromas: clinicopathological and genetic spectrum and suggestions for clinical management." by L.
Hameetman, J.V.M.G. Bovee, A.H.M. Taminiau, H.M. Kroon and P.C.W. Hogendoorn published Hereditary
Cancer in Clinical Practice (Volume 2(4) pp. 161-173)(Hameetman et al., 2004).
Department of Pathology, Leiden University Medical Center, Molecular tumor pathology and tumor genetics,
Netherlands. Head of EuroBoneT consortium, a European Commission granted Network of Excellence for
studying the pathology and genetics of bone tumors. In 2002 the World Health Organization (WHO) has
redefined the definition of Multiple Hereditary Exostoses (MHE) into Multiple Osteochondromas (MO)(Bovee and
Hogendoorn, 2002).
This was done because of the very wide and a-specific use both inside the medical community as well as by
patients of the term "exostosis". It ranges in use from osteochondroma(Khurana et al., 2002), the benign
cartilage tumor involved in MHE, to a perversion in the direction of growth (eg Nora's lesion or Trevor disease)
and even to a reactive process (eg subungual exostosis). Therefore the WHO decided to redefine the
terminology for the bony outgrowths into different terms reflecting the different nature of the diseases involved
aiming at a better defined diagnosis. "Exostoses" involved in MHE are now specified as osteochondromas and
defined as benign cartilaginous tumors.
The current WHO definition of an osteochondroma is "a cartilage capped bony projection arising on the
external surface of bone containing a marrow cavity that is continuous with that of the underlying bone" (WHO
2002)(Khurana et al., 2002). With this changein terminology, Multiple Hereditary Exostoses is no longer
applicable as name for this disorder . That is why the WHO also changed the name of MHE to Multiple
Osteochondromas (MO) and stated that: "A diagnosis of multiple exostoses can be made when radiologically at
least two osteochondromas of the juxta-epiphyseal region of long bones are observed. MO is diagnosed in
case of a positive family history and/or a proven germline mutation in one of the EXT genes" (WHO 2002)
(Bovee and Hogendoorn, 2002).
It is very important to correctly diagnosis Multiple Osteochondromas. Especially good monitoring of the patient
is important after Multiple Osteochondromas is established.This scheme was taken from "Multiple
Osteochondromas: clinicopathological and genetic spectrum and suggestions for clinical management." by L.
Hameetman, J.V.M.G. Bovee, A.H.M. Taminiau, H.M. Kroon and P.C.W. Hogendoorn published Hereditary
Cancer in Clinical Practice (Volume 2(4) pp. 161-173)(Hameetman et al., 2004).
Literature:
References
1. Bovee, J.V.M.G. and P.C.W.Hogendoorn. 2002. Multiple osteochondromas. In World Health Organization
classification of tumours. Pathology and genetics of tumours of soft tissue and bone. C.D.M.Fletcher, K.K.Unni,
and F.Mertens, editors. IARC Press, Lyon. 360-362.
2. Hameetman, L., J.V.M.G.Bovee, A.H.M.Taminiau, H.M.Kroon, and P.C.W.Hogendoorn. 2004. Multiple
Osteochondromas: Clinicopathological and Genetic Spectrum and Suggestions for Clinical Management.
Hereditary Cancer in Clinical Practice 2:161-173.
3. Khurana, J., F.Abdul-Karim, and J.V.M.G.Bovee. 2002. Osteochondroma. In World Health Organization
classification of tumours. Pathology and genetics of tumours of soft tissue and bone. C.D.M.Fletcher,
K.K.Unni,and F.Mertens, editors. IARC Press, Lyon. 234-236.
References
1. Bovee, J.V.M.G. and P.C.W.Hogendoorn. 2002. Multiple osteochondromas. In World Health Organization
classification of tumours. Pathology and genetics of tumours of soft tissue and bone. C.D.M.Fletcher, K.K.Unni,
and F.Mertens, editors. IARC Press, Lyon. 360-362.
2. Hameetman, L., J.V.M.G.Bovee, A.H.M.Taminiau, H.M.Kroon, and P.C.W.Hogendoorn. 2004. Multiple
Osteochondromas: Clinicopathological and Genetic Spectrum and Suggestions for Clinical Management.
Hereditary Cancer in Clinical Practice 2:161-173.
3. Khurana, J., F.Abdul-Karim, and J.V.M.G.Bovee. 2002. Osteochondroma. In World Health Organization
classification of tumours. Pathology and genetics of tumours of soft tissue and bone. C.D.M.Fletcher,
K.K.Unni,and F.Mertens, editors. IARC Press, Lyon. 234-236.
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trust worthy information:By the Health On the Net Foundation. Click here to verify.#
HON Conduct 282463 and is linked on the NIH National Library of Medicine,
Directory of Health Organizations (SIS) website,as well as the link for Patient
Information on The Diseases Database a cross-referenced index of human disease,
and the Intute: health & life sciences a free online service providing access to the
very best Web resources for education and research located in the UK
The MHE Research Foundation is proud to be working with the EuroBoNeT consortium,
a European Commission granted Network of Excellence for studying the pathology
and genetics of bone tumors.
a European Commission granted Network of Excellence for studying the pathology
and genetics of bone tumors.