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Chondroblastoma

Updated : June 15, 2022





Background

Chondroblastoma is an uncommon and benign tumor which produces chondroids. It develops towards the ends of the long bones and represents under 1% of all bone cancers. Chondroblastoma ypically develops in the apophyses or epiphyses of individuals with underdeveloped skeletons.

The word chondroblastoma was coined due to the underdeveloped chondroid cells and the incompletely formed matrix associated with this condition.  Both malignant and benign etiologies need to be examined when diagnosing these neoplasms, which are typically found in the long bones.

Three sites which are most commonly affected by this condition are the proximal femur, distal femur, and the proximal humerus. Surgical treatment is the only preferred method of treatment for chondroblastomas. In general, the prognosis for chondroblastoma is favorable, and patients frequently experience complete remission after surgical therapy.

Epidemiology

Chrondroblastoma is a rare condition, merely responsible for just under 1% of all bone tumors. The mean age of diagnosis is between 19-23 years, and most cases are of individuals in their 20s or 30s. Males are twice as likely to be affected.

Chrondroblastomas always only affect one bone, and their incidence is much higher in long bones. This condition rarely affects the bones of the feet or hands, or any flat bones. Common regions affected include the distal and proximal femur, proximal humerus, and the proximal tibia.

Anatomy

Pathophysiology

Despite the presence of several studies, chondroblastoma histogenesis remains unresolved. Cartialge epiphyseal cartilage cells or germ cells could be the cells that this condition originates from.

Another hypothesized etiological site is a tendon sheath synovial cell or multipotent mesenchymal cell.

Etiology

Genetics

Analysing flow cytometric studies demonstrates that the majority of chondroblastomas have a diploid structure and have modest proliferation percentages. Clonal anomalies have been reported in fourteen chondroblastomas.

Unbalanced and balanced rearrangements of chromosomes 8 and 5 seem the most prevalent. Recent research has revealed that chondroblastomas contain unique genetic mutations that code for H3.3 histone.

In chondroblastomas, the H3F3A gene is not commonly mutated as H3F3A. Chondroblastoma-specific antibodies against H3F3 K36M mutation have been identified through research.

Prognostic Factors

Untreated chondroblastomas do not experience any regression. Its recurrence rates range between 8.3% to 21.4%. These percentages may be attributed to residual tumor material post-surgery. Inadequate surgery, hip and pelvic location, young age, and the presence of aneurysmal bone cysts are factors which enhance the likelihood of recurrence.

Chondroblastomas with a benign histopathology rarely causes pulmonary metastases. Such metastases are also clinically non-progressive and can be treated with surgical resection or simple observation. In very rare instances, a chondroblastoma can evolve into a malignant tumor. There are no consistent histological characteristics that can predict a more aggressive disposition.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://pubmed.ncbi.nlm.nih.gov/3978565/

https://www.ncbi.nlm.nih.gov/books/NBK536947/

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Chondroblastoma

Updated : June 15, 2022




Chondroblastoma is an uncommon and benign tumor which produces chondroids. It develops towards the ends of the long bones and represents under 1% of all bone cancers. Chondroblastoma ypically develops in the apophyses or epiphyses of individuals with underdeveloped skeletons.

The word chondroblastoma was coined due to the underdeveloped chondroid cells and the incompletely formed matrix associated with this condition.  Both malignant and benign etiologies need to be examined when diagnosing these neoplasms, which are typically found in the long bones.

Three sites which are most commonly affected by this condition are the proximal femur, distal femur, and the proximal humerus. Surgical treatment is the only preferred method of treatment for chondroblastomas. In general, the prognosis for chondroblastoma is favorable, and patients frequently experience complete remission after surgical therapy.

Chrondroblastoma is a rare condition, merely responsible for just under 1% of all bone tumors. The mean age of diagnosis is between 19-23 years, and most cases are of individuals in their 20s or 30s. Males are twice as likely to be affected.

Chrondroblastomas always only affect one bone, and their incidence is much higher in long bones. This condition rarely affects the bones of the feet or hands, or any flat bones. Common regions affected include the distal and proximal femur, proximal humerus, and the proximal tibia.

Despite the presence of several studies, chondroblastoma histogenesis remains unresolved. Cartialge epiphyseal cartilage cells or germ cells could be the cells that this condition originates from.

Another hypothesized etiological site is a tendon sheath synovial cell or multipotent mesenchymal cell.

Analysing flow cytometric studies demonstrates that the majority of chondroblastomas have a diploid structure and have modest proliferation percentages. Clonal anomalies have been reported in fourteen chondroblastomas.

Unbalanced and balanced rearrangements of chromosomes 8 and 5 seem the most prevalent. Recent research has revealed that chondroblastomas contain unique genetic mutations that code for H3.3 histone.

In chondroblastomas, the H3F3A gene is not commonly mutated as H3F3A. Chondroblastoma-specific antibodies against H3F3 K36M mutation have been identified through research.

Untreated chondroblastomas do not experience any regression. Its recurrence rates range between 8.3% to 21.4%. These percentages may be attributed to residual tumor material post-surgery. Inadequate surgery, hip and pelvic location, young age, and the presence of aneurysmal bone cysts are factors which enhance the likelihood of recurrence.

Chondroblastomas with a benign histopathology rarely causes pulmonary metastases. Such metastases are also clinically non-progressive and can be treated with surgical resection or simple observation. In very rare instances, a chondroblastoma can evolve into a malignant tumor. There are no consistent histological characteristics that can predict a more aggressive disposition.

https://pubmed.ncbi.nlm.nih.gov/3978565/

https://www.ncbi.nlm.nih.gov/books/NBK536947/

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