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Bone tumors may be classified as "primary tumors", which originate in bone or from bone-derived cells and tissues, and "secondary tumors" which originate in other sites and spread (metastasize) to the skeleton. Carcinomas of the prostate, breasts, lungs, thyroid, and kidneys are the carcinomas that most commonly metastasize to bone. Secondary malignant bone tumors are estimated to be 50 to 100 times as common as primary bone cancers.
A bone tumor (also spelled bone tumour) is a neoplastic growth of tissue in bone. Abnormal growths found in the bone can be either benign (noncancerous) or malignant (cancerous).
Average five-year survival in the United States after being diagnosed with bone and joint cancer is 67%.
Brown tumours consist of fibrous tissue, woven bone and supporting vasculature, but no matrix. The osteoclasts consume the trabecular bone that osteoblasts lay down and this front of reparative bone deposition followed by additional resorption can expand beyond the usual shape of the bone, involving the periosteum thus causing bone pain. The characteristic brown coloration results from hemosiderin deposition into the osteolytic cysts. Hemosiderin deposition is not a distinctive feature of brown tumors; it may also be seen in giant cell tumors of the bone.
Brown tumors may be rarely associated with ectopic parathyroid adenomas or end stage renal osteodystrophy.
Characterized by being less than 1.5 cm in diameter, osteoid osteomas most frequently occur in young men (Male:Female ratio 3:1) and may occur in any bone of the body, most frequently around the knee but often also seen in the vertebrae, in the long bones and less commonly in the mandible or other craniofacial bones.
Severe pain typically occurs at night, but can also be constant at any time of day. The chief complaint may only be of dull pain which is non radiating and persistent throughout 24 hours but increases significantly at night. Pain tends to be relieved with NSAIDs such as ibuprofen.
Ameloblastomas are often associated with the presence of unerupted teeth. Symptoms include painless swelling, facial deformity if severe enough, pain if the swelling impinges on other structures, loose teeth, ulcers, and periodontal (gum) disease. Lesions will occur in the mandible and maxilla, although 75% occur in the ascending ramus area and will result in extensive and grotesque deformities of the mandible and maxilla. In the maxilla it can extend into the maxillary sinus and floor of the nose. The lesion has a tendency to expand the bony cortices because slow growth rate of the lesion allows time for periosteum to develop thin shell of bone ahead of the expanding lesion. This shell of bone cracks when palpated and this phenomenon is referred to as "Egg Shell Cracking" or crepitus, an important diagnostic feature. Ameloblastoma is tentatively diagnosed through radiographic examination and must be confirmed by histological examination (e.g., biopsy).
An osteoid osteoma is a benign bone tumor that arises from osteoblasts and was originally thought to be a smaller version of an osteoblastoma. Osteoid osteomas tend to be less than 1.5 cm in size. The tumor can be in any bone in the body but are most common in long bones, such as the femur and tibia. They account for 10 to 12 percent of all benign bone tumors. "Osteoid osteomas may occur at any age, and are most common in patients between the ages of 4 and 25 years old. Males are affected approximately three times more commonly than females."
Many patients first complain of pain that may be worse at night, may be intermittent and of varying intensity and may have been occurring for some time. Teenagers who are active in sports often complain of pain in the lower femur, or immediately below the knee. If the tumor is large, it can present as overt localised swelling. Sometimes a sudden fracture is the first symptom, because affected bone is not as strong as normal bone and may fracture abnormally with minor trauma. In cases of more deep-seated tumors that are not as close to the skin, such as those originating in the pelvis, localised swelling may not be apparent.
There are three main clinical subtypes of ameloblastoma: unicystic, multicystic, peripheral. The peripheral subtype composes 2% of all ameloblastomas. Of all ameloblastomas in younger patients, unicystic ameloblastomas represent 6% of the cases. A fourth subtype, malignant, has been considered by some oncologic specialists, however, this form of the tumor is rare and may be simply a manifestation of one of the three main subtypes.
Ameloblastoma also occurs in long bones, and another variant is craniopharyngioma (Rathke's pouch tumour, pituitary ameloblastoma).
Patients typically present with swelling with or without pain. The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the tibia or fibula). Most commonly, patients are in their second or third decade, but adamantinoma can occur over a wide age range.
Benign osteofibrous dysplasia may be a precursor of adamantinoma or a regressive phase of adamantinoma.
Histologically, islands of epithelial cells are found in a fibrous stroma. The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles.
Adamantinoma (from the Greek word "adamantinos", meaning "very hard") is a rare bone cancer, making up less than 1% of all bone cancers. It almost always occurs in the bones of the lower leg and involves both epithelial and osteofibrous tissue.
The condition was first described by Fischer in 1913.
The brown tumor is a bone lesion that arises in settings of excess osteoclast activity, such as hyperparathyroidism. It is not a true neoplasm, as the term "tumor" suggests; however, it may mimic a true neoplasm. It most commonly affects the maxilla and mandible, though any bone may be affected. Brown tumours are radiolucent on x-ray.
An osteosarcoma (OS) or osteogenic sarcoma (OGS) is a cancerous tumor in a bone. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin (and thus a sarcoma) and that exhibits osteoblastic differentiation and produces malignant osteoid.
Osteosarcoma is the most common histological form of primary bone cancer. It is most prevalent in teenagers and young adults.
Individuals with an enchondroma often have no symptoms at all. The following are the most common symptoms of an enchondroma. However, each individual may experience symptoms differently. Symptoms may include:
- Pain that may occur at the site of the tumor if the tumor is very large, or if the affected bone has weakened causing a fracture of the affected bone
- Enlargement of the affected finger
- Slow bone growth in the affected area
The symptoms of enchondroma may resemble other medical conditions or problems. Always consult your physician for a diagnosis.
An odontogenic tumor is a neoplasm of the cells or tissues that initiate odontogenic processes.
Examples include:
- Adenomatoid odontogenic tumor
- Ameloblastoma, a type of odontogenic tumor involving ameloblasts
- Calcifying epithelial odontogenic tumor
- Keratocystic odontogenic tumor
- Odontogenic myxoma
- Odontoma
The afflicted may have relatively small amounts of pain that will quickly increase in severity over a time period of 6–12 weeks. The skin temperature around the bone may increase, a bony swelling may be evident, and movement may be restricted in adjacent joints.
Spinal lesions may cause quadriplegia and patients with skull lesions may have headaches.
Bone metastases are a major clinical concern that can cause severe pain, bone fractures, spinal cord compression, hypercalcemia, anemia, spinal instability, decreased mobility, and rapid degradation in the quality of life for patients. Patients have described the pain as a dull ache that grows worse over time, with intermittent periods of sharp, jagged pain. Even under controlled pain management, these periods of breakthrough pain can occur rapidly, without warning, several times a day. Pain may be worse at night and partially relieved by activity. Metastases to weightbearing bones may become symptomatic early in the course of disease as compared to metastases to the flat bones of the rib or sternum.
- Effects of bone metastasis
- severe pain
- bone fractures
- spinal cord compression
- hypercalcemia
- anemia
- spinal instability
- decreased mobility
Malignant transformation to squamous cell carcinoma may occur, but is unusual.
Swelling is the most common presenting complaint; however, OKCs may be asymptomatic and found incidentally on dental X-rays.
Aneurysmal bone cyst, abbreviated ABC, is an osteolytic bone neoplasm characterized by several sponge-like blood or serum filled, generally non-endothelialized spaces of various diameters.
The term is a misnomer, as the lesion is neither an aneurysm nor a cyst.
An enchondroma may occur as an individual tumor or several tumors. The conditions that involve multiple lesions include the following:
- Ollier disease (enchondromatosis) - when multiple sites in the body develop the tumors. Ollier disease is very rare.
- Maffucci's syndrome - a combination of multiple tumors and angiomas (benign tumors made up of blood vessels).
Bone metastases, or metastatic bone disease, is a class of cancer metastases that results from primary tumor invasion to bone. Bone-originating primary tumors such as osteosarcoma, chondrosarcoma, and Ewing's sarcoma are rare. Unlike hematological malignancies that originate in the blood and form non-solid tumors, bone metastases generally arise from epithelial tumors and form a solid mass inside the bone. Bone metastases cause severe pain, characterized by a dull, constant ache with periodic spikes of incident pain.
An osteolytic lesion (from the Greek words for "bone" (ὀστέον), and "to unbind" (λύειν)) is a softened section of a patient's bone formed as a symptom of specific diseases, including breast cancer and multiple myeloma. This softened area appears as a hole on X-ray scans due to decreased bone density. Osteolytic lesions can cause pain, increased risk of bone fracture, and spinal chord compression. These lesions can be treated using biophosphonates or radiation, though new solutions are being tested in clinical trials.
CGCG lesions are found more commonly in the anterior of the maxilla and the mandible in younger people (before age 20). They are characterized by large lesions that expand the cortical plate and can resorb roots and move teeth. They are composed of multi-nucleated giant cells. CGCG has a slight predilection for females.
Radiographically :
It appears as multilocular radiolucencies of bone.The margin of the lesion has scalloped appearance and is well demarcated. Resorption and divergence of roots is also seen.
There are two types of CGCG's, non-aggressive and aggressive. The former has a slow rate of growth and thus less likely to resorb roots and perforate the cortical plate. The aggressive form has rapid growth and thus is much more likely to resorb roots and perforate the cortical plate. It also has a high rate for recurrence and can be painful and cause paresthesia.
Differential diagnosis to include: odontogenic keratocyst (OKC), ameloblastoma, odontogenic myxoma, hemangioma, central odontogenic fibroma, hyperparathyroid tumor, and cherubism.
Central giant-cell granuloma (CGCG) is a benign condition of the jaws. It is twice as likely to affect women and is more likely to occur in 20- to 40-year-old people. Central giant-cell granulomas are more common in the mandible and often cross the midline.
Fibrous dysplasia is a mosaic disease that can involve any part or combination of the craniofacial, axillary, and/or appendicular skeleton. The type and severity of the complications therefore depend on the location and extent of the affected skeleton. The clinical spectrum is very broad, ranging from an isolated, asymptomatic monostotic lesion discovered incidentally, to severe disabling disease involving practically the entire skeleton and leading to loss of vision, hearing, and/or mobility.
Individual bone lesions typically manifest during the first few years of life and expand during childhood. The vast majority of clinically significant bone lesions are detectable by age 10 years, with few new and almost no clinically significant bone lesions appearing after age 15 years. Total body scintigraphy is useful to identify and determine the extent of bone lesions, and should be performed in all patients with suspected fibrous dysplasia.
Children with fibrous dysplasia in the appendicular skeleton typically present with limp, pain, and/or pathologic fractures. Frequent fractures and progressive deformity may lead to difficulties with ambulation and impaired mobility. In the craniofacial skeleton, fibrous dysplasia may present as a painless “lump” or facial asymmetry. Expansion of craniofacial lesions may lead to progressive facial deformity. In rare cases patients may develop vision and/or hearing loss due to compromise of the optic nerves and/or auditory canals, which is more common in patients with McCune-Albright syndrome associated growth hormone excess. Fibrous dysplasia commonly involves the spine, and may lead to scoliosis, which in rare instances may be severe. Untreated, progressive scoliosis is one of the few features of fibrous dysplasia that can lead to early fatality.
Bone pain is a common complication of fibrous dysplasia. It may present at any age, but most commonly develops during adolescence and progresses into adulthood.
Bone marrow stromal cells in fibrous dysplasia produce excess amounts of the phosphate-regulating hormone fibroblast growth factor-23 (FGF23), leading to loss of phosphate in the urine. Patients with hypophosphatemia may develop rickets/osteomalacia, increased fractures, and bone pain.