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Related Concept Videos

Bone Formation by Endochondral Ossification01:24

Bone Formation by Endochondral Ossification

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Bone formation, or ossification, begins around the sixth to seventh week of embryonic development. Most bones develop from a cartilaginous template through the process of endochondral ossification. Cartilage formation begins when clusters of mesenchymal cells differentiate into chondrocytes. These chondrocytes proliferate rapidly and secrete an extracellular matrix that becomes encased in a membrane called the perichondrium. The resulting cartilage model provides a template that resembles the...
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Growth of Cartilage and Bone Tissue01:27

Growth of Cartilage and Bone Tissue

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Chondrocytes form a temporary cartilaginous model by dividing and secreting a thick gel-like extracellular matrix. Once the chondrocytes undergo programmed cell death, osteoblasts enter the site of the cartilaginous model. The process of replacing the temporary cartilaginous model with bone in an ordered manner is called endochondral ossification. In endochondral ossification, not all of the cartilage is replaced by bone tissue. Some cartilage that performs a protective and supportive function...
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Bone Disorders01:29

Bone Disorders

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Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
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Rous Sarcoma Virus (RSV) and Cancer01:03

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Rous Sarcoma virus or RSV was discovered by F. Peyton Rous in the year 1911 as a filterable transmissible agent that could cause tumors in chickens. He won a Nobel Prize for this discovery in 1966. His experiments clearly demonstrated that some cancers could be caused by infectious agents and led to the discovery of many more cancer-causing viruses in animals as well as humans.
RSV is a retrovirus that contains two copies of a plus-strand  RNA genome. Its genome consists of four main open...
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Bone Cells and Tissue01:30

Bone Cells and Tissue

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Bones contain a relatively small number of cells entrenched in a matrix of organic and inorganic components. Although bone cells compose only a small amount of the bone volume, they are crucial to its function. Four types of cells are found within the bone tissue— osteoblasts, osteocytes, osteogenic cells, and osteoclasts.
Osteoblasts and Osteocytes
The osteoblast is the bone cell responsible for forming new bone tissue. It is found in the growing portions of bone, including the...
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Spongy Bone01:09

Spongy Bone

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All bones comprise an outer layer of compact bone, and an interior made up of spongy bone tissue, also called cancellous or trabecular bone. In long bones, spongy bone tissue is mainly found in the interior of the epiphyses (broad ends of the bone).
Spongy bone is more porous, and less dense compared to compact bone. It is composed of concentric lamellae that are arranged irregularly to form the trabecular network. In some bones, the spaces between trabeculae contain red marrow, where...
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Osteosarcoma, Chondrosarcoma, and Chordoma.

Jeremy S Whelan1, Lara E Davis1

  • 1Jeremy S. Whelan, University College London Hospitals NHS Foundation Trust, London, United Kingdom; and Lara E. Davis, Knight Cancer Institute, Oregon Health & Science University, Portland, OR.

Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology
|December 9, 2017
PubMed
Summary
This summary is machine-generated.

Osteosarcoma, chondrosarcoma, and chordoma present management challenges due to rarity and heterogeneity. New treatments are needed, especially for advanced osteosarcoma, while improved therapies are emerging for chondrosarcoma and chordoma.

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Area of Science:

  • Orthopedic Oncology
  • Surgical Oncology
  • Medical Oncology

Background:

  • Osteosarcoma, chondrosarcoma, and chordoma are rare bone cancers with significant management challenges.
  • Their rarity and heterogeneity result in inadequate guidelines for diagnosis, treatment, and survivorship.
  • Current standard care for localized osteosarcoma (OS) offers good prognosis in younger patients, but outcomes are poor for metastatic or recurrent disease and older patients.

Purpose of the Study:

  • To review the current management challenges and therapeutic landscape for osteosarcoma, chondrosarcoma, and chordoma.
  • To highlight the need for improved treatment strategies and specialized care for these rare bone tumors.
  • To discuss the potential of new biologic insights for future therapeutic development.

Main Methods:

  • Review of existing literature and clinical guidelines for osteosarcoma, chondrosarcoma, and chordoma.
  • Analysis of current treatment standards, including chemotherapy, surgery, and radiotherapy.
  • Discussion of prognostic factors and outcomes based on disease stage and patient age.

Main Results:

  • Osteosarcoma shows high curability in localized disease for younger patients with standard neoadjuvant chemotherapy and surgery.
  • Outcomes for unresectable, recurrent, or older adult osteosarcoma patients remain poor, indicating a need for novel therapies.
  • Surgical resection is primary for chondrosarcoma and chordoma, with emerging biologic insights promising improved future treatments.

Conclusions:

  • Despite advances, significant unmet needs persist for osteosarcoma, chondrosarcoma, and chordoma management.
  • Multidisciplinary expert care in high-volume centers is crucial for optimizing outcomes in these rare bone tumors.
  • Continued research into biologic insights is essential for developing more effective treatments for chondrosarcoma and chordoma.