Paget’s disease of bone – wikipedia

Paget’s disease of bone (also termed osteitis deformans or ambiguously, just Paget’s disease) is caused by the excessive breakdown and formation of bone, followed by disorganized bone remodeling. Osteogenic sarcoma in dogs This causes affected bone to weaken, resulting in pain, misshapen bones, fractures and arthritis in the joints near the affected bones. Sarcoma metastasis Rarely, it can develop into a primary bone cancer known as Paget’s sarcoma.


Sarcoma leg symptoms Often Paget’s disease is localized to only a few bones in the body. Sarcoma arises from what type of tissue The pelvis, femur, and lower lumbar vertebrae are the most commonly affected bones. Synovial sarcoma symptoms foot Paget’s disease typically is localized, affecting just one or a few bones, as opposed to osteoporosis, for example, which usually affects all the bones in the body.

A later phase of the disease is characterized by the replacement of normal bone marrow with highly vascular fibrous tissue. Sarcoma cancer cells [1] Because early diagnosis and treatment is important, after age 40, siblings and children of someone with Paget’s disease should have an alkaline phosphatase blood test every two or three years. Ewing sarcoma pnet survival rate If the alkaline phosphatase level is above normal, other tests such as a bone-specific alkaline phosphatase test, bone scan, or X-ray can be performed.

Decisions about treating Paget’s disease may be complicated as the disease often affects people differently. Breast sarcoma survival rate In addition to this, it is sometimes difficult to predict whether a person with Paget’s disease, who shows no signs or symptoms of the disorder, will develop symptoms or complications (such as a bone fracture) in the future. Epithelioid sarcoma Although there is no cure for Paget’s disease, medications ( bisphosphonates and calcitonin) can help control the disorder and lessen pain and other symptoms. Types of cancer carcinoma sarcoma Medications are often successful in controlling the disorder, especially when started before complications begin.

Paget’s disease affects from 1.5 to 8.0 percent of the population, depending on age and country of residence. How rare is sarcoma Paget’s disease is rare in people less than 55 years of age. Sarcoma shoulder [2] Men are more commonly affected than women (3:2). Sarcoma stages [3] The disease is named after Sir James Paget.

Initially, there is a marked increase in the rate of bone resorption in localized areas, caused by large and numerous osteoclasts. Whats a sarcoma These localized areas of pathological destruction of bone tissue (osteolysis) are seen radiologically as an advancing lytic wedge in long bones or the skull. Sarcoma oncology When this occurs in the skull, it is called osteoporosis circumscripta. Undifferentiated sarcoma survival rate The osteolysis is followed by a compensatory increase in bone formation induced by the bone forming cells, called osteoblasts, that are recruited to the area. Sarcoma chemotherapy regimen This is associated with accelerated deposition of lamellar bone in a disorganized fashion. Sarcoma statistics This intense cellular activity produces a chaotic picture of trabecular bone (“mosaic” pattern), rather than the normal linear lamellar pattern. Sarcoma survival rate The resorbed bone is replaced and the marrow spaces are filled by an excess of fibrous connective tissue with a marked increase in blood vessels, causing the bone to become hypervascular. Ewing sarcoma cancer ribbon color The bone hypercellularity may then diminish, leaving a dense “pagetic bone,” also known as burned-out Paget’s disease.

Sir James Paget first suggested the disease was due to an inflammatory process. Granulocytic sarcoma Some evidence suggests that a paramyxovirus infection is the underlying cause of Paget’s disease, [2] which may support the possible role of inflammation in the pathogenesis. Sarcoma lung metastases prognosis However, no infectious virus has yet been isolated as a causative agent, and other evidence suggests an intrinsic hyperresponsive reaction to vitamin D and RANK ligand is the cause. Cancer sarcoma de kaposi [ citation needed] Further research is therefore necessary. Ewing sarcoma translocation [18] Diagnosis [ edit ]

The outlook is generally good, particularly if treatment is given before major changes in the affected bones have occurred. Types of sarcoma cancer in dogs Any bone or bones can be affected, but Paget’s disease occurs most frequently in the spine, skull, pelvis, femur, and lower legs. Cure sarcoma In general, symptoms progress slowly, and the disease does not spread to normal bones. Sarcoma institute Treatment can control Paget’s disease and lessen symptoms, but is not a cure.

Osteogenic sarcoma, a form of bone cancer, is a rare adult-onset complication of Paget’s disease that mainly occurs in children and adolescents between the ages of 10 and 19 years who do not have Paget’s disease. Granulocytic sarcoma prognosis The development of osteosarcoma may be suggested by the sudden onset or worsening of pain, and it occurs in less than one percent of patients with Paget’s disease. Pnet sarcoma Treatment [ edit ]

Although initially diagnosed by a primary care physician, Endocrinologists ( internal medicine physicians who specialize in hormonal and metabolic disorders), rheumatologists (internal medicine physicians who specialize in joint and muscle disorders), orthopedic surgeons, neurosurgeons, neurologists, oral and maxillofacial surgeons, podiatrists, and otolaryngologists are generally knowledgeable about treating Paget’s disease, and may be called upon to evaluate specialized symptoms. Synovial sarcoma foot Medication [ edit ]

The goal of treatment is to relieve bone pain and prevent the progression of the disease. Synovial cell sarcoma cancer These medications are usually recommended for people with Paget’s disease who:

• have bone pain, headache, back pain, or a nerve-related symptom (such as “shooting” pains in the leg) that is directly associated with the disease;

• develop hypercalcemia that occurs when a person with several bones affected by Paget’s disease and a high serum alkaline phosphatase level is immobilized.

Five bisphosphonates are currently available. Ewing sarcoma radiology In general, the most commonly prescribed are risedronic acid, alendronic acid , and pamidronic acid. Sarcoma breast cancer symptoms Etidronic acid and other bisphosphonates may be appropriate therapies for selected patients but are less commonly used. Sarcoma skin cancer pictures None of these drugs should be used by people with severe kidney disease.

• Etidronate disodium The approved regimen is once daily for six months; a higher dose is more commonly used. Synovial sarcoma images No food, beverage, or medications should be consumed for two hours before and after taking. Osteosarcoma symptoms The course should not exceed six months, but repeat courses can be given after rest periods, preferably of three to six months duration.

• Pamidronate disodium in intravenous form: the approved regimen uses an infusion over four hours on each of three consecutive days, but a more commonly used regimen is over two to four hours for two or more consecutive or nonconsecutive days.

• Alendronate sodium is given as tablets once daily for six months; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down (patient may sit).

• Tiludronate disodium are taken once daily for three months; they may be taken any time of day, as long as there is a period of two hours before and after resuming food, beverages, and medications.

• Risedronate sodium tablet taken once daily for 2 months is the prescribed regimen; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down (patient may sit).

• Zoledronic acid is given as an intravenous infusion; a single dose is effective for two years. Endometrial sarcoma staging This is recommended for most people at high risk with active disease. Sarcoma tumor [19]

Calcitonin, also called calcitonin-salmon, is a synthetic copy of a polypeptide hormone secreted by the ultimobranchial gland of salmon. Sarcoma tumor in lung Miacalcin is administered by injection, three times per week or daily, for 6–18 months. Signs and symptoms of sarcoma Repeat courses can be given after brief rest periods. Pleomorphic fibroblastic sarcoma Miacalcin may be appropriate for certain patients, but is seldom used. Epithelioid sarcoma like hemangioendothelioma Calcitonin is also linked to increased chance of cancer. Uterine sarcoma Due to the increased risk of cancer, the European Medicines Agency (EMA) recommended that calcitonin be used only on a short-term basis for 3 conditions for which it had previously been approved in the European Union: Paget’s disease, acute bone loss resulting from sudden immobilization, and hypercalcemia caused by cancer.

The EMA said it based its recommendations on a review of the benefits and risks of calcitonin-containing medicines. Sarcoma cancer centers Conducted by the agency’s Committee for Medicinal Products for Human Use (CHMP), the review encompassed available data from the companies that market these drugs, postmarketing safety data, randomized controlled studies, 2 studies of unlicensed oral calcitonin drugs, and experimental cancer studies, among other sources.

CHMP found that “a higher proportion of patients treated with calcitonin for long periods of time develop cancer of various types, compared with patients taking placebo.” The increase in cancer rates ranged from 0.7% for oral formulations to 2.4% for the nasal formulation. Ewing sarcoma stage 4 survival rate CHMP concluded that the benefits of calcitonin for osteoporosis did not exceed the risks. What is a sarcoma tumor The nasal spray’s only indication is for osteoporosis, thus justifying the drug’s removal from the market.

As a solution for injection or infusion, calcitonin should be administered for no more than 4 weeks to prevent acute bone loss resulting from sudden immobilization, and normally for no more than 3 months to treat Paget’s disease, the EMA said. Can sarcoma be cured The agency did not specify a time frame for the short-term use of calcitonin for treating hypercalcemia caused by cancer. Sarcoma lump on leg Surgery [ edit ]

Medical therapy prior to surgery helps to decrease bleeding and other complications. Connective tissue sarcoma Patients who are having surgery should discuss treatment with their physician. Angiosarcoma liver There are generally three major complications of Paget’s disease for which surgery may be recommended.

• Severe degenerative arthritis — If disability is severe and medication and physical therapy are no longer helpful, joint replacement of the hips and knees may be considered.

Complications resulting from enlargement of the skull or spine may injure the nervous system. Angiosarcoma of the breast However, most neurologic symptoms, even those that are moderately severe, can be treated with medication and do not require neurosurgery. Sarcoma breast cancer treatment Diet and exercise [ edit ]

In general, patients with Paget’s disease should receive 1000–1500 mg of calcium, adequate sunshine, and at least 400 units of vitamin D daily. Ewing sarcoma definition This is especially important in patients being treated with bisphosphonates; however, taking oral bisphosphonates should be separated from taking calcium by at least two hours, because the calcium can inhibit the absorption of the bisphosphonate. Sarcoma treatment guidelines Patients with a history of kidney stones should discuss calcium and vitamin D intake with their physicians.

Exercise is very important in maintaining skeletal health, avoiding weight gain, and maintaining joint mobility. Myxoid sarcoma symptoms Since undue stress on affected bones should be avoided, patients should discuss any exercise program with their physicians or physical therapists before beginning. Sarcoma signs Epidemiology [ edit ]

The overall prevalence and severity of Paget’s disease are decreasing; the cause for these changes is unclear. Sarcoma surgeon [20] Paget’s disease is rare in people less than 55 years of age, [2] and the prevalence increases with age. Stromal sarcoma in dogs [20] Evidence from studies of autopsy results have demonstrated Paget’s disease in about 3 percent of people older than 40 years of age. Retroperitoneal sarcoma symptoms [20] Paget’s disease is more common in males than females. Pediatric sarcoma prognosis [3] Rates of Paget’s disease are about 50 percent higher in men than in women.

About 15 percent of people with Paget’s disease also have a family member with the disease. Osteogenic sarcoma definition [2] In cases where the disease is familial, it is inherited in an autosomal dominant fashion, although not all people that inherit the affected version of the genes will express the disease ( incomplete penetrance). Ewing sarcoma medscape [2]

The incidence of Paget’s disease varies considerably with geographic location. Ewing sarcoma chemotherapy regimen [20] Paget’s predominantly affects people of European descent, whereas people of African, Asian, or Indian descent are less commonly affected. Sarcoma types of cancer [2] Paget’s disease is less common in Switzerland and Scandinavia than in the rest of Western Europe. Sarcoma bone cancer statistics [20] Paget’s disease is uncommon in the native populations of North and South America, Africa, Asia, and the Middle East. Sarcoma tumor size When an individual from these regions does develop Paget’s disease, there is typically some European ancestry present. Ewing sarcoma recurrence Etymology [ edit ]

Paget’s disease of bone was originally termed osteitis deformans, because it was thought to involve an inflammatory process, which is implied by the suffix -itis. Histiocytic sarcoma cytology Now, that term is considered technically incorrect, and the preferred term is osteodystrophia deformans. Basal cell carcinoma symptoms [21] Society and culture [ edit ]

• Retired Boston Red Sox center fielder Dom DiMaggio suffered from Paget’s disease and served as a member of the board of directors of the Paget Foundation. Spindle cell sarcoma stages [22]