Bone: Chondroma

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Bone: Chondroma

Identity

Note Chondroma is an uncommon benign tumour which characteristically forms mature cartilage. It is found mostly in the small bones of the hand and/or feet, although it can also occur in long, tubular bones, primarily the humerus, femur and ribs. Occasionally, focal areas of mixoid degeneration may result in a mistaken diagnosis of chondrosarcoma.

Classification

Chondromas are classified according to their location:

enchondroma: within the bone (within the medullary cavity),
periosteal chondroma: on the surface of the bone,
soft tissue chondroma in the soft tissue.

Clinics and Pathology

Disease Enchondroma

Note Fig: Enchondroma in the distal portion of the femur shaft. (courtesy of Dr Henry DeGroot at http://www.drdegroot.com)

Enchondroma is usually a solitary benign lesion in intramedullary bone. Usually asymptomatic, it is incidentally discovered as a palpable bony nodule. Rarely, causes soft tissue swelling and pain at the lesion site. Pain can be a sign of pathologic fracture. Both sex are equally affected, and any age group can be involved. It is thought to develop from epiphyseal cartilage rests that subsequently proliferate and slowly enlarge. Approximately 50% of solitary enchondromas are found in the hands, typically in the middle and distal portions of the metacarpals and the proximal portions of the phalanges, 10% in the feet, 20% in the proximal and distal parts of the femur and the proximal part of the humerus.
On gross examination, the lesion is well circumscribed and has the pale bluish-gray appearance typical of cartilage.
The nonerheditary syndrome of multiple enchondromas or enchondromatosis is known as Ollier's disease. Enchondromatosis associated with soft tissue hemangiomas is known as Maffucci's syndrome.

Pathology Microscopically, enchondroma is hypocellular with few double-nucleated cells without cytologic atypia, but cellularity may vary. There is no permeation of morrow. The matrix does not show any myxoid change. Calcification and ossification are common. Histologic appearance of enchondroma may recall that of a grade-1 chondrosarcoma. The permeation through the cortex into soft tissue must be identified before a diagnosis of chondrosarcoma is made.
The chondromas in Ollier disease and Maffucci syndrome may demonstrate a greater degree of cellularity and cytologic atypia, and may be difficult to distinguish from chondrosarcoma.

Fig: H&E 20x original magnification of an enchondroma: note lobules of benign cartilage cells and hyaline matrix. (courtesy of Dr Henry DeGroot at http://www.drdegroot.com).

Treatment No treatment is required for asymptomatic lesions. If fracture occurs it is usually treated with curretage and bone grafting.

Evolution A small percentage of enchondromas will undergo malignant transformation, usually throught a slow process, occurring over decades. It is more common in long bones than short.

Prognosis Prognosis for benign enchondroma is excellent. Solitary lesion in the hand rarely undergoes transformation. It has been suggested that Maffuccišs syndrome is associated with a very high incidence of malignancy, either in the skeleton or in visceral organs.

Disease Periosteal chondroma

Note
a) Fig: Ankle periosteal chondroma; {courtesy of Dr Nick Ordall http://www.xray2000.f9.co.uk/)
b) Fig: Chondroma of the right femur (courtesy of Dr Henry DeGroot at http://www.drdegroot.com/)
Periosteal chondroma is a painful cartilaginous lesion that arises from surface of cortex deep to the periosteum, producing broad based cartilaginous mass that may extend into soft tissues; often develops after adolescence. It does not infiltrate the adjacent soft tissue but may increase in size. It is similar in appearance and location to periosteal osteosarcoma. The potential for confusion with periosteal and parosteal osteosarcoma mandates a thorough investigation and biopsy of this lesion. The most common location is adjacent to the metaphysis. The cortex may be involved to a variable degree, but the lesions do not involve the medullary space.

Pathology It persists as mass of mature cartilage. Low power microscopy shows well circumscribed lobulated hyaline masses. Cellularity can vary, from hypo- to hyper-cellularity. The cartilage looks more active than enchondroma and the lesion may be confused with chondrosarcoma.

Fig: Bone tumor images (courtesy of Dr Henry DeGroot at http://www.drdegroot.com)

Treatment Periosteal chondromas are treated with conservative excision.

Prognosis Risk of recurrence after bloc marginal excision is less than 10%.

Disease Soft-tissue chondroma

Note Soft-tissue chondroma is a benign cartilage-forming tumor,usually arising from tenosynovial sheaths or the soft tissue adjacent to tendons in the hands and feet, usually without any connection to the underlying bone. Predominantly sited in the fingers, it is usually solitary, develops in adults, and may causes pain. It is composed entirely of mature hyaline cartilage. Infrequently, the tumor undergoes secondary changes and may exhibit morphologic features that result in diagnostic difficulty.

Pathology Microscopically, soft-tissue chondromas vary considerably in appearence. Most consist of hyaline cartilage arranged in lobular pattern, and may show focal fibrosis, ossification, or myxoid change. Diffuse calcification may occur, completely obscuring the cartilagineous nature of the lesion. In some variants, the cartilage matrix becomes extensively mineralized, often associated with necrosis of chondrocytes, causing the tumor to resemble tumoral calcinosis. Hyaline cartilage may also undergo enchondral ossification, mimicking an osteogenic neoplasm or a reactive lesion. Myxoid degeneration may create confusion with extraskeletal myxoid chondrosarcoma.

Treatment Local surgery is the treatment of choice.

Genetics

Note Cytogenetic studies of chondromas are scarse. A total of 16 cases with abnormal karyotypes have been reported: 6 enchondromas, 4 periosteal chondromas, and 6 soft part chondromas. No consistent abnormality has been detected, although chromosome or chromosomal region 4, 5, 6, 7 and 12q13-15 seems to be nonrandomly involved in changes.

Bibliography

Clonal karyotypic aberrationsin enchondromas.

Bridge JA, Persons DL, Neff JR, Bhatia P.

Cancer Detect Prev 1992; 16: 215-219.

PMID 1458512

Biologic and clinical significance of cytogenetic and molecular cytogenetic abnormalities in benign and malignant cartilaginous lesions.

Bridge JA, Bhatia PS, Anderson JR, Neff JR.

Cancer Genet Cytogenet 1993; 69: 79-90.

PMID 8402563

Rearrangement of band q13 on both chromosomes 12 in a periosteal chondroma.

Mandahl N, Willen H, Rydholm A, Heim S, Mitelman F.

Genes Chromosomes Cancer 1993; 6: 121-123.

PMID 7680888

Rearrangement of band q13 on both chromosomes 12 in a periosteal chondroma

Shadan FF, Mascarello JT, Newbury RO, Dennis T, Spallone P, Stock AD.

Cancer Genet Cytogenet. 2000; 118 :144-147.

PMID 10748295

The Cytogenetics of bone and soft tissue tumors.

Sandberg AA Bridge JA.

Austin: R.G. Landes Company; 1994

Involvement of chromosomes 6 and 11 in a soft tissue chondroma.

Dal Cin P, Qi H, Sciot R, Van den Bergh H.

Cancer Genet Cytogenet 1997; 93: 177-178.

PMID 9078305

Solitary enchondroma with clonal chromosomal abnormalities.

Gunawan B, Weber M, Bergmann F, Wildberger J, Fuzesi L.

Cancer Genet Cytogenet 1998; 104: 161-164.

PMID 9666812

Maffucci's syndrome: clinical and radiological features of a rare condition

McDermott AL, Dutt SN, Chavda SV, Morgan DW.

J Laryngol Otol. 2001; 115: 845-847.

PMID 11668006

Correlation between clinicopathological features and karyotype in 100 cartilaginous and chordoid tumours. A report from the Chromosomes and Morphology (CHAMP) Collaborative Study Group.

Tallini G, Dorfman H, Brys P, Dal Cin P, de Wever I, Fletcher CD, Jonson K, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Samson I, Sciot R, Van den Bergh H, Vanni R, Willen H.

J Pathol 2002; 196: 194-203.

PMID 11793371

Cytogenetic findings in benign cartilaginous neoplasms.

Buddingh EP, Naumann S, Nelson M, Neffa JR, Birch N, Bridge JA.

Cancer Genet Cytogenet 2003; 141: 164-168

PMID 12606137

Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: chondrosarcoma and other cartilaginous neoplasms.

Sandberg AA, Bridge JA.

Cancer Genet Cytogenet 2003;143: 1-31.

PMID 12742153

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Contributor(s)

Written 05-2003 Roberta Vanni

Citation

This paper should be referenced as such :
Vanni R . Bone: Chondroma. Atlas Genet Cytogenet Oncol Haematol. May 2003 .
URL : http://AtlasGeneticsOncology.org/Tumors/ChondromaID5147.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Thu Apr 17 14:14:28 2008

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Disease	Enchondroma
Note	Fig: Enchondroma in the distal portion of the femur shaft. (courtesy of Dr Henry DeGroot at http://www.drdegroot.com) Enchondroma is usually a solitary benign lesion in intramedullary bone. Usually asymptomatic, it is incidentally discovered as a palpable bony nodule. Rarely, causes soft tissue swelling and pain at the lesion site. Pain can be a sign of pathologic fracture. Both sex are equally affected, and any age group can be involved. It is thought to develop from epiphyseal cartilage rests that subsequently proliferate and slowly enlarge. Approximately 50% of solitary enchondromas are found in the hands, typically in the middle and distal portions of the metacarpals and the proximal portions of the phalanges, 10% in the feet, 20% in the proximal and distal parts of the femur and the proximal part of the humerus. On gross examination, the lesion is well circumscribed and has the pale bluish-gray appearance typical of cartilage. The nonerheditary syndrome of multiple enchondromas or enchondromatosis is known as Ollier's disease. Enchondromatosis associated with soft tissue hemangiomas is known as Maffucci's syndrome.
Pathology	Microscopically, enchondroma is hypocellular with few double-nucleated cells without cytologic atypia, but cellularity may vary. There is no permeation of morrow. The matrix does not show any myxoid change. Calcification and ossification are common. Histologic appearance of enchondroma may recall that of a grade-1 chondrosarcoma. The permeation through the cortex into soft tissue must be identified before a diagnosis of chondrosarcoma is made. The chondromas in Ollier disease and Maffucci syndrome may demonstrate a greater degree of cellularity and cytologic atypia, and may be difficult to distinguish from chondrosarcoma.
	Fig: H&E 20x original magnification of an enchondroma: note lobules of benign cartilage cells and hyaline matrix. (courtesy of Dr Henry DeGroot at http://www.drdegroot.com).

Treatment	No treatment is required for asymptomatic lesions. If fracture occurs it is usually treated with curretage and bone grafting.
Evolution	A small percentage of enchondromas will undergo malignant transformation, usually throught a slow process, occurring over decades. It is more common in long bones than short.
Prognosis	Prognosis for benign enchondroma is excellent. Solitary lesion in the hand rarely undergoes transformation. It has been suggested that Maffuccišs syndrome is associated with a very high incidence of malignancy, either in the skeleton or in visceral organs.

Disease	Periosteal chondroma
Note	a) Fig: Ankle periosteal chondroma; {courtesy of Dr Nick Ordall http://www.xray2000.f9.co.uk/) b) Fig: Chondroma of the right femur (courtesy of Dr Henry DeGroot at http://www.drdegroot.com/) Periosteal chondroma is a painful cartilaginous lesion that arises from surface of cortex deep to the periosteum, producing broad based cartilaginous mass that may extend into soft tissues; often develops after adolescence. It does not infiltrate the adjacent soft tissue but may increase in size. It is similar in appearance and location to periosteal osteosarcoma. The potential for confusion with periosteal and parosteal osteosarcoma mandates a thorough investigation and biopsy of this lesion. The most common location is adjacent to the metaphysis. The cortex may be involved to a variable degree, but the lesions do not involve the medullary space.
Pathology	It persists as mass of mature cartilage. Low power microscopy shows well circumscribed lobulated hyaline masses. Cellularity can vary, from hypo- to hyper-cellularity. The cartilage looks more active than enchondroma and the lesion may be confused with chondrosarcoma.
	Fig: Bone tumor images (courtesy of Dr Henry DeGroot at http://www.drdegroot.com)

Treatment	Periosteal chondromas are treated with conservative excision.
Prognosis	Risk of recurrence after bloc marginal excision is less than 10%.

Disease	Soft-tissue chondroma
Note	Soft-tissue chondroma is a benign cartilage-forming tumor,usually arising from tenosynovial sheaths or the soft tissue adjacent to tendons in the hands and feet, usually without any connection to the underlying bone. Predominantly sited in the fingers, it is usually solitary, develops in adults, and may causes pain. It is composed entirely of mature hyaline cartilage. Infrequently, the tumor undergoes secondary changes and may exhibit morphologic features that result in diagnostic difficulty.
Pathology	Microscopically, soft-tissue chondromas vary considerably in appearence. Most consist of hyaline cartilage arranged in lobular pattern, and may show focal fibrosis, ossification, or myxoid change. Diffuse calcification may occur, completely obscuring the cartilagineous nature of the lesion. In some variants, the cartilage matrix becomes extensively mineralized, often associated with necrosis of chondrocytes, causing the tumor to resemble tumoral calcinosis. Hyaline cartilage may also undergo enchondral ossification, mimicking an osteogenic neoplasm or a reactive lesion. Myxoid degeneration may create confusion with extraskeletal myxoid chondrosarcoma.
Treatment	Local surgery is the treatment of choice.

REVIEW articles	automatic search in PubMed
Last year publications	automatic search in PubMed