Chondroma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor-in-Chief: Somal Khan Soujanya Thummathati, MBBS [2]

Overview

Chondroma is a benign tumor that arises from chondrocytes, which are cartilage cells involved in the synthesis and maintenance of the cartilaginous matrix (which consists mainly of collagen and proteoglycans). Enchondroma and juxtacortical chondroma are the common types of chondroma. Enchondroma arises from rests of growth plate cartilage or chondrocytes that are normally involved in the production and maintainence of the cartilaginous matrix, which consists mainly of collagen and proteoglycans. Juxta-cortical chondromas (also known as periosteal chondromas) arise from the periosteum of tubular bones. Juxtacortical chondroma was first discovered as periosteal chondroma by Lichtenstein and Hall, in 1952. The term Juxtacortical chondroma was first coined by Jaffe in 1956. On microscopic histopathological analysis, avascular lobules of hyaline cartilagenous matrix interspersed with chondrocytes, separated by normal marrow are characteristic findings of chondromas. On gross pathology, small (usually less than 3 cm), translucent, grey blue, nodular lesions with occasional calcifications are characteristic findings of chondromas. Genes involved in the pathogenesis of enchondroma and periosteal chondromas may include isocitrate dehydrogenase 1 (IDH1) and IDH2. Patients of all age groups may develop enchondromas. Enchondromas affect men and women equally. Periosteal chondromas commonly affect individuals in the 20-30 years age group. Males are more commonly affected with periosteal chondromas than females. Enchondroma must be differentiated from low-grade chondrosarcoma, fibrocartilaginous dysplasia, bone islands, and bone infarcts. Periosteal chondroma must be differentiated from periosteal chondrosarcoma and periosteal osteosarcoma. If left untreated, patients with chondroma may progress to develop pain and discomfort. Common complications of chondroma include malignant transformation into a low grade chondrosarcoma, pathologic fractures and skeletal deformities. The majority of patients with enchondroma are asymptomatic. Less common symptoms of enchondroma may include pain, enlargement of the affected digits, and slowing of the affected bone growth, which results in asymmetrical bone deformities. Symptoms of juxtacortical chondromas include dull aching pain and a visible swelling. On x rays, enchondroma is characterized by lytic lesions that contain calcified chondroid matrix. On x rays, periosteal chondroma is characterized by matrix calcification with ring and arc pattern and saucerisation of the adjacent bony cortex with a sclerotic periosteal reaction. Bone CT may be helpful in the diagnosis of chondroma. Findings on CT suggestive of chondroma include chondroid calcifications, mild endosteal scalloping and expansion of the overlying cortex with no cortical invasion, unless fractured. MRI of the affected bone is helpful in the diagnosis of chondroma. On MRI, enchondroma is characterized by well circumscribed, lobulated mass replacing the bone marrow. On MRI, periosteal chondroma is characterized by lobulated soft tissue lesion, abutting the cortex and may demonstrate evidence of pressure erosion on neighboring bone, with no evidence of medullary bone or soft tissue edema. The majority of cases of enchondroma are benign and only need observation. Surgical resection is indicated in cases where a malignant transformation of enchondroma is suspected and in the majority of cases of periosteal chondromas.

Historical Perspective

Juxtacortical chondroma was first discovered by Lichtenstein and Hall in 1952. The term juxtacortical chondroma was first coined by Jaffe in 1956.

Classification

Chondroma may be classified according to location of the outgrowth into 3 groups that include enchondrom, periosteal chondroma and synovial chondroma.

Pathophysiology

Enchondromas arise from rests of growth plate cartilage or chondrocytes that are normally involved in the production and maintainence of the cartilaginous matrix, which consists mainly of collagen and proteoglycans. Genes involved in the pathogenesis of enchondromas and periostal chondromas include isocitrate dehydrogenase 1 (IDH1) and IDH2 . On gross pathology, small (usually less than 3 cm), translucent, grey blue, nodular lesions with occasional calcifications are characteristic findings of chondromas. Enchondromas may be single or multiple. Multiple enchondromas are associated Maffucci syndrome, Ollier disease and metachondromatosis.

Causes

The cause of chondromas has not been identified. However, enchondroma is believed to occur either as an overgrowth of the cartilage that lines the ends of the bones, or as a persistent growth of original, embryonic cartilage.

Epidemiology and Demographics

The incidence of otesochondromas is approximately 30,000 per 100,000 of all cartilage tumors and 2,000 per 100,000 of all bone tumors.The prevalence of enchondromas is approximately 12,000-24,000 per 100,000 individuals of benign bone tumors and 3000-10,000 of all bone tumors. Patients of all age groups may develop enchondromas. The incidence of enchondroma peaks in the third and fourth decades of life. Enchondroma affects men and women equally.

Risk Factors

There are no established risk factors for chondromas.

Screening

There is insufficient evidence to recommend routine screening for chondromas.

Natural History, Complications and Prognosis

Chondroma is usually diagnosed incidentally. Common complications of chondroma include malignant transformation into a low grade growth disturbance, pathologic fracture, especially in short tubular and recurrence. Benign chondromas have a good prognosis with aprropriate treatment.

History and Symptoms

The majority of patients with enchondroma are asymptomatic. Less common symptoms of enchondroma may include pain, enlargement of the affected digits, and slowing of affected bone growth and asymmetrical bone deformities.

Physical Examination

Patients with chondroma usually appear well. Physical examination of patients with enchondroma is usually unremarkable. Physical examination of patients with periosteal chondroma is usually remarkable for tenderness and a palpable mass.

Laboratory Findings

There are no diagnostic lab findings associated with chondroma.

Electrocardiogram

There are no electrocardiogram findings associated with chondroma.

X Ray Findings

Findings on an x-ray suggestive of chondroma include well-defined or sclerotic border, sharp zone of transition, confinement by natural barriers, lack of destruction of the cortex and lack of extension into the soft tissue.

CT

Bone CT may be helpful in the diagnosis of chondroma. Findings on CT suggestive of chondroma include chondroid calcifications, mild endosteal scalloping and expansion of the overlying cortex with no cortical invasion, unless fractured.

MRI

MRI of the affected bone is helpful in the diagnosis of chondroma. On MRI, enchondroma is characterized by well circumscribed, lobulated mass replacing the bone marrow. On MRI, periosteal chondroma is characterized by lobulated soft tissue lesion, abutting the cortex and may demonstrate evidence of pressure erosion on neighboring bone, with no evidence of medullary bone or soft tissue edema.

Echocardiography or Ultrasound

There are no echocardiography or ultrasound findings associated with chondroma.

Other imaging studies

Other imaging studies for enchondroma includes radionuclide bone scan.

Medical Therapy

The majority of cases of enchondroma are benign and only need observation. Surgical resection is indicated in cases where a malignant transformation of enchondroma is suspected and in the majority of cases of periosteal chondromas.

Surgery

Surgery is not the first-line treatment option for patients with asymptomatic, benign chondromas. Surgical resection is usually reserved for patients at risk with either malignant transformation or pathological fractures.

Primary Prevention

There is no established method for prevention of chondroma.

Secondary Prevention

There is no established method for prevention of chondroma.

References

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