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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.

Images

WIKIDATA, CC BY-SA 3.0
WIKIDATA, CC BY-SA 3.0
WIKIDATA, CC BY-SA 3.0
WIKIDATA, CC BY-SA 3.0

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.

Entire Body System

  • Swelling

    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]

    Swelling in the area is another common symptom, although it might not occur until later. Depending on where the tumor is, it might be possible to feel a lump or mass. Limb pain and swelling are very common in normal, active children and teens. [cancer.org]

    He or she will examine the painful area, feeling for warmth, swelling, and tenderness, and looking for signs of joint swelling or fluid. You'll be asked about any difficulty moving the limb or using the joint. [drugs.com]

    Often, there is a firm swelling or lump in the area of the pain. This swelling is caused by the tumor growing inside the bone. If the cancer is in a leg bone, the person may limp. [familydoctor.org]

  • Weight Loss

    Unintended weight loss – some people experience weight loss due to the body’s reaction to the cancer. [limbpreservation.org]

    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]

  • 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 experienced with an osteosarcoma is typically in the area of the tumor, which is often called local pain. Swelling - there may be inflammation in the area of a tumor leading to swelling. [limbpreservation.org]

    The pain may be present for several weeks to several months.[1]The majority of patients with osteosarcoma present with localized pain at the primary tumor site.[3] Range of motion may also be limited as the tumor encroaches upon the joint space.[1] The [physio-pedia.com]

  • Lymphadenopathy

    […] seen in serous cystadenoma and other tumors frequently bilateral In the setting of suspected ovarian cystadenocarcinoma, features suggestive of extra-ovarian metastasis include: ascites - often of disproportionately large volume 3 peritoneal nodularity lymphadenopathy [radiopaedia.org]

    Night pain, “growing pains” History of minor trauma, injury, sprain or muscle strain Fever, night sweats, weight loss* Physical examination Local tenderness Swelling, mass, deformity Limp, muscle atrophy, decreased joint motion Pathologic fracture Lymphadenopathy [aafp.org]

Liver, Gall & Pancreas

  • Hepatomegaly

    In the current admission, a physical examination revealed bilateral lower limb swelling, pansystolic murmur on the left side of his sternum at fourth intercostal space (tricuspid area) grade three, hepatomegaly with a liver span of 17 cm, and a positive [ncbi.nlm.nih.gov]

Jaw & Teeth

  • Parotid Mass

    A 48-year-old woman presented with a right-sided infra-parotid mass encompassing neck regions II and III, measuring over 6 cm in craniocaudal diameter. She was initially treated by wide surgical resection. [ncbi.nlm.nih.gov]

Cardiovascular

  • Pansystolic Murmur

    In the current admission, a physical examination revealed bilateral lower limb swelling, pansystolic murmur on the left side of his sternum at fourth intercostal space (tricuspid area) grade three, hepatomegaly with a liver span of 17 cm, and a positive [ncbi.nlm.nih.gov]

Musculoskeletal

  • Bone Pain

    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]

    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]

    However, it can occur in any bone. The first symptom is usually bone pain near a joint. This symptom may be overlooked because of other more common causes of joint pain. [nlm.nih.gov]

  • Back Pain

    A 49-year-old woman presented with a 2-month history of lower back pain and a lumbar region mass. Magnetic resonance imaging demonstrated a heterogeneously enhanced mass in the L1 vertebral arch. [ncbi.nlm.nih.gov]

    Symptoms may include: Pain in the bone or joint that gets worse over time A painless swelling or a noticeable mass in the arm or leg A broken bone that occurs without injury or with minimal injury Stiffness or swelling of joints (uncommon) Back pain or [curesearch.org]

  • 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]

    Risk factors These factors increase the risk of osteosarcoma: Previous treatment with radiation therapy Other bone disorders, such as Paget's disease of bone and fibrous dysplasia Certain inherited or genetic conditions, including hereditary retinoblastoma [mayoclinic.org]

    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]

    Osteosarcoma is a rare disorder, but it is the most common cause of bone cancer in children and adolescents. [rarediseases.org]

  • Knee Pain

    Persistent knee pain in a recreational runner. Department of Family Medicine. University of Washington, Seattle. 2002. Sahin H, ,Ceylan N, Bayraktarogulu S, Savas R. Cardiac metastasis of osteosarcoma: A case report. [physio-pedia.com]

  • 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 [nlm.nih.gov]

    ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ12.175) A 13-year-old boy presents with left leg pain for 10 weeks. He reports a remote history of trauma while falling off of a trampoline 12 weeks ago. [orthobullets.com]

Urogenital

  • Scrotal Mass

    A 63-year-old man, who presented with a large painless left inguinal and scrotal mass, underwent radical orchiectomy. Pathological examination was suggestive of testiscular osteosarcoma. [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.

Serum

  • Alkaline Phosphatase Increased

    phosphatase Greatly elevated alkaline phosphatase High serum alkaline phosphatase Increased alkaline phosphatase Increased serum alkaline phosphatase [ more ] 0003155 Increased lactate dehydrogenase activity 0025435 Joint swelling 0001386 Pain 0012531 [rarediseases.info.nih.gov]

Other Pathologies

  • 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.

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

  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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