Τετάρτη 17 Ιουλίου 2019


Pigmented villonodular synovitis

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Localized pigmented villonodular synovitis; Diffuse pigmented villonodular synovitis; Tenosynovial giant cell tumors; See More


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Pigmented villonodular synovitis (PVNS) is a disease in which the tissue lining the joints and tendons in the body (synovium) grows abnormally. It is characterized by a noncancerous mass or tumor. There are two types of PVNS: the local or nodular form (where the tumor involves the tendons that support the joint, or in one area of the joint) and the diffuse form (where the entire lining of the joint is involved). Symptoms might include: pain, limitation of movement, and locking of the joint. In some cases, the normal joint structure can be destroyed. The knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, wrist, and rarely the jaw. The average age of diagnosis for this condition is 35 years. The cause of PVNS is unknown. Treatment involves surgery to remove the tumor and damaged portions of the synovium.[1][2][3]
Last updated: 4/4/2016
The exact cause of pigmented villonodular synovitis (PVNS) is unknown.  Some doctors believe that it is similar to arthritis, arising from swelling (inflammation) of the joint tissue. Others believe it develops like a tumor, caused by cells growing and multiplying more quickly than usual.[4] The association between a history of trauma and the development of  PVNS is unclear. One study found that 56% of individuals with PVNS had a history of previous trauma, while other studies have found a much lower incidence.[3] 

There have been studies suggesting that PVNS could be caused by specific genetic changes in the cells lining the joint. More studies are needed to research this association.[5]
Last updated: 4/5/2016
Pigmented villonodular synovitis (PVNS) is diagnosed via physician examination, imaging studies, and sometimes surgical procedures. Imaging studies commonly used include: X-ray, MRI, and CT scan. MRI findings are diagnostic in more than 95% of patients. CT scan findings are additionally often diagnostic, though they might not show the extent of the disease.[1] 

Other methods that might be utilized in the diagnostic process include joint aspiration, in which a needle is used to remove fluid from the joint and a biopsy, in which a small operation is completed to obtain a tissuesample.[2]
Last updated: 4/4/2016
Pigmented villonodular synovitis is first treated with surgery to remove as much of the abnormal tissue growth as possible.  The type of surgery depends on the location and extent of the disease within the joint.  Radiation therapy is sometimes used to treat this condition if surgery is not an option, or if the condition returns (recurs) after an initial surgery.[1][4]
Last updated: 4/5/2016
Pigmented villonodular synovitis (PVNS) is considered rare. Estimates of prevalence may vary depending on the type of PVNS (localized or diffuse). According to a recently published study based on registry data in Denmark, the prevalence in 2012 was 44 per 100,000 people for localized PVNS, and 11 per 100,000 people for diffuse PVNS.[6] This means that PVNS may occur in about 5-6 people out of 10,000 people. We are not aware of recent prevalence estimates of PVNS in the United States.
Last updated: 11/14/2017
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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

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In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
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  1. John R McGrath. Pigmented Villonodular Synovitis Imaging. Medscape. October 21, 2015; http://emedicine.medscape.com/article/394649-overview.
  2. American Academy of Orthopaedic Surgeons (AAOS). Pigmented Villonodular Synovitis. OrthoInfo. November 2014; http://orthoinfo.aaos.org/topic.cfm?topic=a00506.
  3. Guo-ping Xie Nan Jiang, Chang-xiang Liang, Jian-chun Zeng, Zhi-yuan Chen, Qian Xu, Rui-zhen Qi, Yi-rong Chen, Bin Yu. Pigmented Villonodular Synovitis: A Retrospective Multicenter Study of 237 Cases. PLoS One. March 23, 2015; 10(3):http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4370558/.
  4. Mendenhall WM, Mendenhall CM, Reith JD, Scarborough MT, Gibbs CP, Mendenhall NP. Pigmented Villonodular Synovitis. American Journal of Clinical Oncology. 2006; 29:548-550. http://www.ncbi.nlm.nih.gov/pubmed/17148989.
  5. Ravi V, Wang WL, Lewis VO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Current Opinion in Oncology. 2011; 23:361-366. http://www.ncbi.nlm.nih.gov/pubmed/21577109. Accessed 11/22/2011.
  6. Ehrenstein V, Andersen SL, Qazi I, Sankar N, Pedersen AB, Sikorski R, Acquavella JF. Tenosynovial Giant Cell Tumor: Incidence, Prevalence, Patient Characteristics, and Recurrence. A Registry-based Cohort Study in Denmark. J Rheumatol. October, 2017; 44(10):1476-1483. https://www.ncbi.nlm.nih.gov/pubmed/28765257.


Pigmented villonodular synovitis occurring in the temporomandibular joint
Publication date: August 2019
Source: Auris Nasus Larynx, Volume 46, Issue 4
Author(s): Fuminori Nomura, Yosuke Ariizumi, Yusuke Kiyokawa, Akihisa Tasaki, Yumiko Tateishi, Nobuaki Koide, Hiroaki Kawabe, Takashi Sugawara, Kentaro Tanaka, Takahiro Asakage

Abstract
Objective
Pigmented villonodular synovitis occurring in the region of the temporomandibular joint is a rare disease, requiring a review of the treatment method, follow-up period.
Method
Refer to the past literature, along with a retrospective search.
Results
An excision, including the skull base bone, was performed in all cases; however, recurrence was found in one case on which fractional excision was performed. Past reports have also indicated that en bloc resection was considered desirable.
Conclusion
It is necessary to perform en bloc resection on patients with pigmented villonodular synovitis occurring in the region of the temporomandibular joint. Furthermore, due to reported cases of recurrence after a long period of time, follow-up observations of about 10 years are considered necessary.

DISEASES & CONDITIONSPigmented Villonodular Synovitis Pigmented villonodular synovitis (PVNS) is a condition that causes the synovium—the thin layer of tissue that lines the joints and tendons—to thicken and overgrow. The mass or tumor that results from this overgrowth is not cancerous and does not spread (metastasize) to other areas of the body. PVNS is a progressive disease, however, that slowly worsens and can lead to bone damage and arthritis. PVNS usually affects the knee although it can affect other joints as well. In most cases, surgery is needed to remove the damaged joint lining and the mass. Description In a healthy joint, the synovium produces a small amount of fluid that lubricates the cartilage and aids in movement. In PVNS, the synovium produces extra fluid, causing swelling in the joint and making movement very painful. PVNS typically involves only one joint. In 80% of patients, the knee is involved, but PVNS can also affect the hip, ankle, shoulder, and elbow. The condition can affect people of all ages, but it occurs most often in young adults in their 30s and 40s. There are two forms of PVNS—localized and diffuse. Localized PVNS When the tumor involves the tendons that support the joint, or occurs in just one area of the joint, it is called localized PVNS. This type usually responds well to treatment. Diffuse PVNS When the condition is more widespread and involves an entire joint, it is called diffuse PVNS. It tends to be more destructive and is more difficult to treat. Related Articles TREATMENT Arthroscopy TREATMENT Total Joint Replacement DISEASES & CONDITIONS Rheumatoid Arthritis DISEASES & CONDITIONS Osteoarthritis Cause The cause of PVNS is not known. Genetic changes associated with PVNS have been identified, but the evidence for a genetic cause is not conclusive. Symptoms Localized PVNS causes pain and swelling in the affected joint. The swelling can be quite dramatic. Other symptoms may include locking, catching, and instability in the joint. In diffuse PVNS, there is often a gradual onset of symptoms including joint pain, swelling, and stiffness. In both types, the symptoms may come and go over time. Doctor Examination Your doctor will perform a physical examination and use imaging studies and other tests to diagnose PVNS. Tests X-rays. X-rays create clear pictures of dense structures such as bone. If PVNS has not damaged or caused changes in your bone, it may not appear in an x-ray. However, x-ray images may help your doctor rule out other causes for your pain. X-ray showing destruction of bone in an elbow with PVNS This x-ray of an elbow with diffuse PVNS shows holes in the bone caused by the tumor. Magnetic resonance imaging (MRI) scan. An MRI provides clear images of soft tissues and can be helpful in diagnosing PVNS. In localized PVNS, an MRI will show a nodular mass. In diffuse PVNS, it will show extensive thickening of the joint lining or an extensive mass, possibly with destructive bone changes and cartilage damage. MRI scans of an elbow with PVNS (Left) This MRI of an elbow shows a tumor caused by PVNS. (Right) In this MRI of the same elbow, an area where the tumor has eaten through the bone is visible. Joint aspiration. In this procedure, fluid is removed from the joint with a syringe and analyzed. In many cases of PVNS, the joint fluid is bloody. Biopsy. A biopsy may be necessary to confirm the diagnosis of PVNS. In a biopsy, a tissue sample of the tumor is taken and examined under a microscope. A small operation is required to obtain a tissue sample. Treatment Because PVNS destroys healthy bone and can grow to a large size, treatment involves surgery to remove the tumor and the damaged portions of the joint lining. When localized PVNS has also damaged a tendon, your surgeon will repair it during the procedure to remove the tumor. Your doctor will discuss the various surgical options with you. Surgical Procedures Arthroscopy. In many cases of both localized and diffuse PVNS, the tumor and damaged joint lining are removed arthroscopically. During arthroscopy the surgeon makes a few small incisions around the joint and inserts a small camera called an arthroscope. The camera displays pictures on a television screen, and the surgeon uses these images to guide miniature surgical instruments. Open surgery. Diffuse PVNS that affects both the front and back of the knee requires removal of the entire joint lining to reduce the chances of the tumor returning. In many cases, this is best achieved with traditional "open" surgery. A single larger incision provides the surgeon with full access to the joint, and facilitates removal of the mass and the entire joint lining. Combined arthroscopic and open surgery. When most of the mass is in the back of the knee, a combined surgical approach can be undertaken. The back of the knee is treated with open surgery to remove the mass and posterior joint lining, and the front of the knee is treated with arthroscopic removal of the anterior joint lining. This combined method decreases the scale of surgery, allowing for an easier recovery. Total joint replacement. In its end stages, diffuse PVNS can cause extensive joint destruction. Once the joint has been significantly damaged, the best option to relieve pain and improve function is a total joint replacement. Total joint replacement is a procedure in which parts of a damaged joint are removed and replaced with metal, plastic, or ceramic components. The tumor does not usually return after the joint has been replaced. Radiation Radiation therapy can shrink tumors and is sometimes used to treat widespread diffuse PVNS. It is usually reserved for patients in whom standard surgery has not been successful. In the past, radiation therapy has been given via an external beam that is directed from outside the skin to the inside of the affected joint. While often successful, this method can cause significant complications. A newer method called intra-articular radiation has also been used successfully. During this procedure, radioactive fluid is injected into the joint with a needle. Recovery After surgery, physical therapy will be extremely important in helping you return to your daily activities. Specific exercises will help you regain strength and range of motion in the affected joint. Recovery from arthroscopic surgery usually requires a short course of physical therapy, after which you may return to normal activity. Open surgery is more extensive, however, so there is an increased risk of postoperative stiffness. A more regimented and extensive physical therapy program is often required for patients recovering from open surgery to treat diffuse PVNS. In this case, the return to normal activity will take longer—possibly several months. Localized PVNS rarely recurs after surgery. The recurrence rate for diffuse PVNS is usually around 10%, but can be as high as 30%. Patients with diffuse PVNS will require physician follow-up for several years after surgery. During these visits your doctor may order tests such as an MRI to check for recurrence of PVNS. Statistical data in this article was reviewed by the AAOS Department of Research and Scientific Affairs.
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