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Osteosarcoma

Osteogenic Sarcomas

Osteosarcoma (osteogenic sarcoma) is an aggressive malignant neoplasm, composed of anaplastic cells of mesenchymal origin. It is the most common primary bone cancer.


Presentation

Symptoms of osteosarcoma are dependent on the location of the tumor [7]. However, some of the most common presentations include limitation of motion of joints, limping, redness, swelling, bone fractures, bone pain following any form of movement or exertions on the extremities.

The pain experienced varies from one patient to the other. Some stop feeling pains when the joint is at rest while for others, the pains continue even when fully at rest leading to sleep deprivation in some cases. When any of these presentations are visible the muscle in the affected limb will feel smaller than those in the opposite limb.

Weight Loss
  • Systemic symptoms, such as fever and weight loss, occur rarely in the absence of very advanced disease. Epidemiology Osteosarcoma has a bimodal age distribution with the first peak in the second decade of life and the second among older adults.[curesarcoma.org]
  • loss Anemia Osteosarcomas located near the spinal cord may cause back pain that radiates out through the arms or legs.[chop.edu]
Localized Pain
  • We present the case of a 22-year-old girl with increasing local pain in the distal left thigh. The patient was initially diagnosed as bone cancer according to computed tomography (CT) and X-ray imaging.[ncbi.nlm.nih.gov]
  • Signs and Symptoms of Osteosarcoma Pain – the pain a child experiences is typically in the area of the tumor and is referred to as local pain. Swelling - there may be inflammation in the area of a tumor leading to swelling.[limbpreservation.org]
Developmental Delay
  • ATRX germline variations are responsible for the rare genetic disorder X-linked alpha-thalassemia mental retardation (ATR-X) syndrome characterized by severe developmental delay and alpha-thalassemia but no obvious increased risk of cancer.[ncbi.nlm.nih.gov]
Chest Discomfort
  • A 21-year-old man of African descent presented to our center complaining of shortness of breath, awareness of heart beats, easy fatigability, swelling of lower limbs, and left-side chest discomfort for the past 6 months getting worse for the last 3 months[ncbi.nlm.nih.gov]
Sputum
  • Sputum samples grew Mycoplasma avium complex and Mycobacterium fortuitum. Imaging showed a right-sided 7.6 cm mass with compression of the main bronchus. Subsequent biopsy with vimentin staining established the diagnosis of ESOS.[ncbi.nlm.nih.gov]
Bone Pain
  • Herein, we presented a 50-year-old man patient who visited hospital due to local bone pain in the left leg. He was initially diagnosed with osteoblastic osteosarcoma.[ncbi.nlm.nih.gov]
  • Common signs of this type of cancer include: bone pain (in motion, at rest, or when lifting objects) bone fractures swelling redness limping limitation of motion of joints How bone pain is experienced may vary.[healthline.com]
  • Symptoms may include bone pain or a mass on an arm or leg. Chemotherapy is the main treatment, along with surgery to remove the primary tumor. Most children and teens can fully recover from osteosarcoma.[spectrumhealth.org]
  • The first symptom is usually bone pain near a joint. This symptom may be overlooked because of other more common causes of joint pain.[medlineplus.gov]
Bone Disorder
  • (From Stedman, 25th ed) Synonyms: Osteosarcoma of bone (disorder), Bone Sarcoma, Osteochondrosarcoma, Osteosarcoma of bone, Sarcoma of the Bone, Osteogenic Sarcomas, Osteosarcoma - disorder, Sarcoma of Bone, Osteosarcoma, NOS, osteoid sarcoma, Osteogenic[ebi.ac.uk]
  • Previous treatment with radiation therapy Certain bone disorders. These include: Paget's disease of bone Fibrous dyplasia Certain inherited cancer syndromes. These include: Familial retinoblastoma LiFraumeni syndrome Bone infarct.[orthoinfo.aaos.org]
Leg Pain
  • Other symptoms may include any of the following: Bone fracture (may occur after a routine movement) Limitation of motion Limping (if the tumor is in the leg) Pain when lifting (if the tumor is in the arm) Tenderness, swelling, or redness at the site of[medlineplus.gov]
Cryptorchidism
  • We report a family in which two brothers had an undiagnosed genetic disorder comprised of dysmorphic features, microcephaly, severe intellectual disability (non-verbal), mild anemia, and cryptorchidism. Both developed osteosarcoma.[ncbi.nlm.nih.gov]
Foot Drop
  • The patient showed left-side foot drop only, without loss of sphincter function. In 1-year follow-up CT, excellent bony fusion was noticed.[ncbi.nlm.nih.gov]

Workup

There are different medical procedures that aid in the diagnosis of osteosarcoma [8]. Physical examination is needed to establish swelling and redness. Medical history is vital to see previous illnesses and medical treatments that may suggest the presence of an etiologic factor. A blood test may be used to check for the presence of tumors. Other tests that can be carried out include a CT scan, MRI, X-ray, PET scan, biopsy and bone scan. All of these will demonstrate the presence of osteosarcoma.

Mediastinal Mass
  • We suggest there is a possible link between mediastinal masses and nontuberculous mycobacteria infection.[ncbi.nlm.nih.gov]
Mycobacterium Fortuitum
  • Sputum samples grew Mycoplasma avium complex and Mycobacterium fortuitum. Imaging showed a right-sided 7.6 cm mass with compression of the main bronchus. Subsequent biopsy with vimentin staining established the diagnosis of ESOS.[ncbi.nlm.nih.gov]
Trophoblastic Cells
  • Abstract Human trophoblast cell surface antigen 2 (TROP2) has been noted to serve an important role in the proliferation and migration of various types of human cancers.[ncbi.nlm.nih.gov]

Treatment

Generally, osteosarcoma is treated with the aid of a combination of therapies [9]. This can include chemotherapy, radiation therapy and surgery. Before surgery, patients with high grade tumors are subjected to neo-adjuvant therapy.

Surgery involves the removal of tumor with a wide margin. The goal of this is to leave the area free of all disease.

Majority of tumors at the bones and joints can now be removed safely without losing the limb affected. To replace tissues removed during the surgery, a surgeon can use a metal implant, an allograft a combination of both or bone removed healthy areas of the body.

After a successful surgery, a pathologist should examine the necrosis rate of the osteosarcoma [10]. It is the indicator of how well the tumor is responding to chemotherapy. Drugs are chosen for additional chemotherapy treatment based on this necrosis rate. This additional chemotherapy lasts six months on average.

Prognosis

When the disease is localised and metastasis hasn’t ensued, there is a long term survival rate of 70 to 75% [6]. However as soon as metastasis sets in, prognosis is negative and survival rate drops to 30% especially if the metastasis affects the lungs.

Etiology

There have been many studies aimed at the etiologic factor responsible for osteosarcoma. However, it is still unclear, what actually leads to osteosarcoma. An etiologic factor is only seen in around 15% of cases [3].

Some of the etiologic factors that have been identified till date include:

  • Radiotherapy: Radiation treatment for cancer can lead to a damage of the DNA in bone cells. 
  • Underlying bone abnormalities: One good example of a disorder that can lead to the development of osteosarcoma is Paget disease. 
  • Genetic factors: Genes for osteosarcoma can be inherited from a parent and this increases the possibility of the condition developing. 

Epidemiology

Osteosarcoma is among the top 8 forms of childhood cancer. It makes up 2.4% of all malignancies in pediatric patients and around 20% of all cases of primary bone cancers [4]. In the United States, the incidence of the condition in patients aged 20 and below is placed at 5.0 cases per million each year in a general population. However the incidence varies slightly when ethnicity is considered. In blacks, incidence is 6.8/million, in Hispanics it is 6.5/million and in white ethnicities it is 4.6 per million each year.

The condition is seen more in males than in females with incidence placed at 5.4 per million and 4.0 per million respectively.
It originates more frequently in the metaphyseal region of tubular long bones. 42% of cases is seen in the femur, 19% is seen in the tibia and 10% in the humerus. Around 8% of all cases happen in the skull and jaw and another 8% is seen in the pelvis.

Sex distribution
Age distribution

Pathophysiology

Osteosarcoma arises when there is a malignant growth of the bone cells that are responsible for the production of the hard boney matrix [5]. This malignant growth is what forces the cell to grow into a cancerous tissue or tumor. The bone cells that are seen in the tumor retain their bone like functions by growth and division. This explains how they are able to spread to other areas of the body.

Prevention

Osteosarcoma cannot be prevented.

Summary

Osteosarcoma is the most common type of bone cancer [1]. It is seen mostly in children and teenagers as it generally involves the long bones near metaphyseal growth plates. It is possible for older adults to develop this condition but the vast majority of patients are aged 6 to 25. Teenagers develop this type of bone cancer the most and males develop it more than females. This phenomenon may be as a result of the fact that bones grow at a very fast rate for boys.

The cancer often develops in the bones of the legs and arms but most cases of it originate around the ends of the long bones which make up the knee [2]. When left untreated, osteosarcoma can spread to virtually any part of the body. The lungs and other bones are the most common metastasis sites for osteosarcoma.

Patient Information

Osteosarcoma is a cancer of the bone. It is mostly seen in the long bone in the legs in people younger than 20 years of age. However, it can arise in just about any bone in any part of the body. The cancer, if left untreated can spread to other organs of the body.

The chances of recovery from this type of cancer are high as long as the condition gets discovered early enough. Treatment is by surgery and use of chemotherapy primarily.

References

Article

  1. Marulanda GA, Henderson ER, Johnson DA, Letson GD, Cheong D. Orthopedic surgery options for the treatment of primary osteosarcoma. Cancer Control. Jan 2008;15(1):13-20. 
  2. Vander Griend RA. Osteosarcoma and its variants. Orthop Clin North Am. Jul 1996;27(3):575-81.
  3. Ottaviani G, Jaffe N. (2009). The epidemiology of osteosarcoma. In: Jaffe N. et al. "Pediatric and Adolescent Osteosarcoma". New York: Springer.
  4. Kim SY, Helman LJ. Strategies to Explore New Approaches in the Investigation and Treatment of Osteosarcoma. Cancer Treat Res. 2010;152:517-528.
  5. Peltier LF. Tumors of bone and soft tissues. Orthopedics: A History and Iconography. San Francisco, Calif: Norman Publishing; 1993:264-91.
  6. Rutkow IM. The nineteenth century. Surgery: An Illustrated History. St Louis, Mo: Mosby-Year Book; 1993:321-504.
  7. Mallon WJ. The registry of bone sarcoma. Ernest Amory Codman: The End Result of a Life in Medicine. Philadelphia, Pa: WB Saunders Co; 2000:107-21.
  8. Osuna D, de Alava E. Molecular pathology of sarcomas. Rev Recent Clin Trials 4 2009 (1): 12–26. 
  9. Dhaliwal J, Sumathi VP and Grimer RJ. Radiation-induced periosteal osteosarcoma. Grand Rounds 10: 13-18.
  10. Papalas JA, Balmer NN, Wallace C, Sangüeza OP. Ossifying dermatofibroma with osteoclast-like giant cells: report of a case and literature review. Am J Dermatopathol 2009 31 (4): 379–83. 

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Last updated: 2017-08-09 17:34