Is fibrous dysplasia malignant transformation?
Fibrous dysplasia (FD) is considered to be a benign pediatric disease that usually becomes dormant by adulthood. However, FD can undergo malignant transformation, which accounts for 0.4 ~ 1% of all FD cases . Coley and Stewart described the first diagnosed case of sarcoma arising from FD in 1945 .
Which malignant tumor develops from fibrous dysplasia?
The most prevalence type of malignancy developed from fibrous dysplasia is osteosarcoma (70% of cases), followed by fibrosarcoma (20% of cases), and chondrosarcoma (10% of cases), with malignant fibrous histiocytoma (4% of cases) , , .
What is a sarcomatous transformation?
Sarcomatoid transformation in a carcinoma is a rare event but frequently associated with advanced disease stage, aggressive clinical behavior and dismal prognosis. It’s likely a result of stepwise gene mutations in pluripotent stem cell and involves the epithelial to mesenchymal transition (EMT).
Is fibrous dysplasia an Expansile?
Fibrous dysplasia (FD) is a developmental benign medullary fibro-osseous process characterized by the failure to form mature lamellar bone and arrest as woven bone that can be multifocal. It can affect any bone and occur in a monostotic form involving only one bone or a polyostotic form involving multiple bones.
Is fibrous dysplasia a tumor?
Fibrous dysplasia is a benign fibroosseous bone tumor that accounts for 5% to 10% of benign bone tumors.
What is the cause of fibrous dysplasia?
What causes fibrous dysplasia? The exact cause of fibrous dysplasia is not known. It is believed to be due to a chemical defect in a specific bone protein. This defect may be due to a gene mutation present at birth, although the condition is not known to be passed down in families.
What does Sarcomatous mean?
(sar-KOH-muh) A type of cancer that begins in bone or in the soft tissues of the body, including cartilage, fat, muscle, blood vessels, fibrous tissue, or other connective or supportive tissue. Different types of sarcoma are based on where the cancer forms.
Which of the following terms refer to a malignant tumor of mesenchymal origin?
A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal (connective tissue) origin.
Does fibrous dysplasia need treatment?
Unfortunately, there is no cure for fibrous dysplasia; however, treatments may help to relieve pain, and supportive measures such as physical therapy may help strengthen muscle and improve range of motion.
How rare is polyostotic fibrous dysplasia?
Fibrous dysplasia is rare, accounting for just about 7% of all benign bone tumors. It can affect any bone in the body, but most often occurs in the: Femur (thighbone) Tibia (shinbone)
What is the prognosis of fibrous dysplasia?
Individuals with milder forms of FD often live normal, otherwise healthy lives. The prognosis is as widely variable as the disorder itself, and is based on the bones affected, whether other structures such as nerves are affected, and whether fractures occur.
Is malignant tumor curable?
However, malignant tumours will often eventually return after treatment. If this happens, or if you have a secondary tumour, a cure isn’t usually possible and treatment can instead be used to improve symptoms and prolong life. Read more about treating malignant brain tumours.
When is a tumor referred to as malignant?
Malignant tumors have cells that grow uncontrollably and spread locally and/or to distant sites. Malignant tumors are cancerous (ie, they invade other sites). They spread to distant sites via the bloodstream or the lymphatic system. This spread is called metastasis.
Does fibrous dysplasia affect life expectancy?
Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life.
What is the last stage of sarcoma?
Stage IV soft tissue sarcoma A sarcoma is considered stage IV when it has spread to distant parts of the body. Stage IV sarcomas are rarely curable. But some patients may be cured if the main (primary) tumor and all of the areas of cancer spread (metastases) can be removed by surgery.
Is 4 cm a large tumor?
A stage IIA cancer describes a tumor larger than 4 cm but 5 cm or less in size that has not spread to the nearby lymph nodes. Stage IIB lung cancer describes a tumor that is 5 cm or less in size that has spread to the lymph nodes within the lung, called the N1 lymph nodes.
What is the prevalence of malignant transformation of fibrous dysplasia?
Malignant transformation of fibrous dysplasia (FD) is rare ( 1 – 4 ). It can occur in monostotic and polyostotic FD, with a frequency of <1% among all FD ( 2 ).
What is fibrous dysplasia?
General Discussion. Fibrous dysplasia (FD) is a rare bone disorder. Bone affected by this disorder is replaced by abnormal scar-like (fibrous) connective tissue. This abnormal fibrous tissue weakens the bone, making it abnormally fragile and prone to fracture. Pain may occur in the affected areas.
What is the pathophysiology of osteosarcoma from fibrous dysplasia?
Secondary osteosarcoma from fibrous dysplasia (FD) is very rare. The etiology of FD is linked to activating missense mutations of the guanine nucleotide‑binding protein α‑subunit (GNAS) gene, which encodes the stimulatory α subunit of the G protein (Gsα) and is located at chromosome 20q13.
What is the difference between monostotic and polyostotic FD?
FD may only affect one solitary bone (monostotic disease) or the disorder can be widespread, affecting multiple bones throughout the body (polyostotic disease). The severity of the disorder can vary greatly from one person to another.