Mesenchymal Chondrosarcoma is an extremely rare malignancy of bone or soft tissue, often an aggressive form of bone cancer. It is a variant type of the conventional Chondrosarcoma which is a form of bone cancer arising from cartilage cells. Cartilage is a specialized tissue and it serves as a buffer or cushion at joints.
The skeleton of an embryo consists of cartilage which slowly converts into bone. In Mesenchymal Chondrosarcoma, approximately two-thirds affect the bones, especially the spine, ribs and jaws. In other cases, it affects the areas of the body other than bone, in soft tissues such as muscle and fat. Mesenchymal Chondrosarcoma occurs with greater frequency in young adults (aged 10-30). It is present in 2% of all chondrosarcomas and it has no sexual or racial predilection, but females are slightly more affected than males.
Signs and symptoms
Symptoms could vary depending upon the exact location and progression of tumor. Mesenchymal Chondrosarcoma mostly arise in the bones of the jaw, spine or the ribs. Tumors can develop in arms and legs frequently as well and especially in the femur (thigh bone). Tumors appearing as soft tissue masses can affect the muscle or central nervous system.
Some symptoms of Mesenchymal Chondrosarcoma resemble that of many other illnesses and disorders and this can lead to a false diagnosis initially. This is seldom the sickness that doctors perceive when presented with symptoms that match this rare cancer. It can generally be diagnosed without much difficulty when pain and swelling in the specific part of the body is located though other possibilities are usually tested first.
Although specific symptoms associated with Mesenchymal Chondrosarcoma are vague and non specific; yet many develop pain and swelling in the affected area. Lumps and joint stiffness are other clinical symptoms of the tumor. Such symptoms can be present even long before a diagnosis is made. One third of patients are symptomatic for more than one year. A large tumor can compress nearby structures and cause additional symptoms, for instance, a spinal cord paralysis, or if in the eye socket, can cause pain, swelling, visual disturbances and protrusion of the eyeball. Since this is an aggressive form of cancer, it can spread to other areas of the body - lungs, liver, lymph nodes and other bones and can cause life threatening complications.
While the exact cause of Mesenchymal Chondrosarcoma is unknown and investigations are ongoing, there is no identifiable risk factor for the development of Mesenchymal Chondrosarcoma that has been discovered.
Whereas, due to abnormal changes in the structures and orientation of cells known as oncogenes or tumor suppressor genes, these malignancies can develop. Current research shows that abnormalities of DNA, carrier of the body's genetic code can cause cellular malignant transformation. These abnormal genetic changes appear to occur quite spontaneously for unknown reasons.
A diagnosis of Mesenchymal Chondrosarcoma may be difficult, but some seek attention due to pain and swelling. Diagnosis may be based upon clinical examination, and detailed patient history. A variety of specialized tests include X rays studies, a procedure in which a small sample of tissue is removed and studied under a microscope. A biopsy of a tumor or mass determines what type of cancer is present by identifying the cell type that makes up the tumor. In those suspected of Mesenchymal Chondrosarcoma, an open biopsy or a needle guided biopsy is recommended.
There are specialized imaging techniques that are used to evaluate the size, placement and extension of a tumor and to serve as an aid for future surgical procedures among those with Mesenchymal Chondrosarcoma. Such imaging techniques include CT scanning and MRI. Often CT scan of the lungs is performed as there is a risk of Mesenchymal Chondrosarcoma spreading to the lungs. Bone scans, tests that can detect areas of increased or decreased bone activity may be performed to determine whether Mesenchymal Chondrosarcoma has spread to affect other bones.
Like many other tumors, Mesenchymal Chondrosarcoma may metastasize thorough the blood and attack other organs within the body. It spreads to the lungs, though it may spread to other locations as well. When it appears near the spine, it is particularly troublesome. Depending upon the size of the tumor, it may place significant pressure on the spine and cause pain and partial paralysis. Should tumor be present in the skull, it can cause similar neurological problems.
Treating Mesenchymal Chondrosarcoma
To optimize treatment, a multidisciplinary group of health professionals at the time of suspicion of a bone sarcoma is highly recommended. Coordinated effort of a team of medical professionals is necessary to treat Mesenchymal Chondrosarcoma. While physicians specialize in the diagnosis and treatment of cancer, specialists in the use of radiation can treat cancer along with surgeons, oncology nurses and other specialists.
Specific therapeutic procedures and interventions vary depending upon numerous factors such as primary tumor location, stage of primary tumor and whether the tumor has spread to distant sites, individual's general health and other elements. Radiation may or may not be employed in the treatment of Mesenchymal Chondrosarcoma. Some medical oncologists advocate chemotherapy treatment plan. Radiation therapy post surgery is also typically standard practice for large tumors.
Since chances are that Mesenchymal Chondrosarcoma may recur after optimal therapy, patients should be routinely examined after surgery for follow up care. Surgical resection is the ideal method of treating Mesenchymal Chondrosarcoma assuming that the tumor can be removed without causing much damage to other organs or tissues.
Surgery is often followed up with chemotherapy and radiation to ensure that no cancerous cells are allowed to survive, grow and spread again. In case tumor is in an inoperable location, then radiation and chemotherapy can be used to shrink the tumor enough to allow for safe operation. Local sufficiently radical treatment for prevention of recurrence is vital. Patients have to avoid spicy food, smoking and drinking to avoid recurrence of the disease. Early detection and early diagnosis and treatment are the key to prevention and treatment of this disease.
Bibliography / Reference
Collection of Pages - Last revised Date: March 22, 2018