PVNS

色素性絨毛結節性滑膜炎 pigmented villonodular tenosynovitis

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/11/15 22:48:10」(JST)

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Pigmented villonodular synovitis
Classification and external resources
Micrograph of pigmented villonodular synovitis. H&E stain.
ICD-9	727.02
DiseasesDB	29902
eMedicine	article/394649
MeSH	D013586

Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth of the joint lining. It usually affects the hip or knee. It can also occur in the shoulder, ankle, elbow, hand or foot. In PVNS the lining of the joint, called the synovium, becomes swollen and grows. This growth harms the bone next to the joint. The lining also makes extra fluid that can cause swelling and make movement painful. PVNS is idiopathic, it doesn't seem to run in families or be caused by certain jobs or activities. Surgery can help but, even with treatment, PVNS comes back about half the time. If the pain remains then radiation therapy may help. In the worst cases the joint must be replaced.

Classification

Pigmented villonodular synovitis, described distinctly in 1941 by Charles J. Sutro, L. Lichtenstein, and H.L. Jafe, comes in two forms: localized and diffuse. Diffuse PVNS affects the entire synovium and typically occurs in large joints such as the knee or hip. Localized, or nodular, PVNS is less common than the diffuse form and typically occurs in smaller joints such as the hands and feet. Localized PVNS often arises in the form of a large benign tumour on the tendon sheaths of the joint. As the tumor grows in the joint, it damages the surrounding bone and tissues. Localized PVNS is predominantly found in females and is frequently found in the fingers. Although rare, localized PVNS may develop in large joints. In either case, the knee is the most commonly affected joint (80% of cases), followed by the hip, and less commonly the ankles and shoulders. PVNS is generally found more in men than women. 2 cases per million population; incidence of the localized form is 9 cases per million.

Clinical presentation

In general, pigmented villonodular synovitis often manifests initially as sudden onset, unexplained joint swelling and pain; the joint swelling is disproportionate to the amount of pain the patient feels at first. Decreased motion and increased pain occur as the disorder progresses as well as locking of the joint. The localized form often manifests initially as a painless, slow-growing mass and progresses to the other common symptoms of PVNS. The swelling often feels warm to the touch. Diffuse PVNS symptoms are often confused with those of Rheumatoid arthritis. While pigmented villonodular synovitis can occur in both pediatric and geriatric patients, it is more common with ages 20–50.

Causes

The exact cause is unknown. Some doctors believe it's caused by abnormal metabolism of fat. Others think it may be caused by repetitive inflammation. Some feel that blood within the joint may cause the inflammatory change. Risk factors for PVNS developing are not yet understood. Very little research has been carried out. However, a common theme in patients is a trauma experienced to the joint prior to the onset of symptoms.

Complications

PVNS is locally aggressive and can spread to surrounding tissues, causing bone erosion and tissue damage. If not treated early, it can spread to areas outside the joint. The disorder also has, on average, a 45% rate of recurrence.

Diagnosis

Micrograph of PVNS showing pigmented hemosiderin-laden macrophages (brown/red). H&E stain.

PVNS is radiologically diagnosed by magnetic resonance imaging (MRI). The disorder is difficult to identify and is often not diagnosed for four years or more after presentation due to nonspecific symptoms or a general paucity of symptoms.

Pathology

The synovial fluid of the joint is often grossly hemorrhagic.

PVNS, under the microscope, looks as the name of the condition suggests; it is composed on nodules and/or villi and has an abundant number of (pigmented) hemosiderin-laden macrophages.

Treatment

Once PVNS is confirmed by biopsy of the synovium of an affected joint, a synovectomy of the affected area is the most common treatment. Bone lesions caused by the disorder are removed and bone grafting is performed as needed. Because diffuse PVNS has a relatively high rate of recurrence, radiation therapy may be considered as a treatment option. In some cases, a total joint replacement is needed to relieve symptoms when PVNS causes significant joint destruction. However, not many Orthopedic Surgeons will risk trying a replacement, as the success rate with active PVNS is extremely low.

See also

• Giant cell tumor of the tendon sheath
• Fibroma of tendon sheath
• List of cutaneous conditions

References

UpToDate Contents

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• 1. 軟組織および骨に影響を与えるさまざまな良性疾患に対する抗腫瘍療法 antineoplastic therapy for miscellaneous benign diseases affecting soft tissue and bone
• 2. 成人における膝関節腫瘍のX線評価 radiologic evaluation of knee tumors in adults
• 3. 膝関節の炎症性関節炎に対する滑膜切除 synovectomy for inflammatory arthritis of the knee
• 4. 成人における股関節痛の放射線評価 radiologic evaluation of the painful hip in adults
• 5. 関節血腫 hemarthrosis

English Journal

• Osteoclast formation and function in pigmented villonodular synovitis.

• Taylor R, Kashima TG, Knowles H, Gibbons CL, Whitwell D, Athanasou NA.SourceNuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Nuffield Orthopaedic Centre, Oxford, UK.
• The Journal of pathology.J Pathol.2011 Sep;225(1):151-6. doi: 10.1002/path.2937. Epub 2011 Jun 27.
• Pigmented villonodular synovitis (PVNS) is a synovial tumour-like lesion that frequently causes osteolysis. PVNS contains numerous macrophages and osteoclast-like giant cells. In this study, we have analysed the cytochemical and functional characteristics of mononuclear and multinucleated cells in P
• PMID 21706481

• Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis.

• Ravi V, Wang WL, Lewis VO.SourceDepartment of Sarcoma Medical Oncology, UT MD Anderson Cancer Center, Houston, Texas 77030, USA. vravi@mdanderson.org
• Current opinion in oncology.Curr Opin Oncol.2011 Jul;23(4):361-6.
• PURPOSE OF REVIEW: To review recent developments in the molecular pathogenesis of tenosynovial giant cell tumor (TGCT) or pigmented villonodular synovitis (PVNS) and its therapeutic implications.RECENT FINDINGS: TGCT or PVNS is a benign clonal neoplastic proliferation arising from the synovium chara
• PMID 21577109

Japanese Journal

• 臨床室 水泳選手の肩甲上腕関節内に遊離体症状で発症したびまん型肩関節色素性絨毛結節性滑膜炎の1例

• 庭山 麻里絵,新福 栄治,内山 善康 [他]
• 整形外科 62(5), 436-438, 2011-05
• NAID 40018815589

• 側頭骨に発生した色素性絨毛結節性滑膜炎例

• 松下 友,松田 洋一,上田 祥久,中島 格
• 耳鼻咽喉科臨床 103(12), 1093-1099, 2010
• … Pigmented villonodular synovitis (PVNS) is benign, usually involving the knee, foot, etc. …
• NAID 130000805139

Related Links

• 症例5 40歳代 男性

限局性結節性滑膜炎 localized nodular synovitis（localized PVNSとも言われる） ... PVNSと限局性結節性滑膜炎の違い・びまん性のPVNSには滑膜の絨毛状や多結節状 の増殖が見られるが限局性結節性滑膜炎では滑膜所見はない。 ・ ヘモジデリン沈着が ...

• Pigmented villonodular synovitis - Wikipedia, the free encyclopedia

Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth of the joint lining. It usually affects the hip or knee. It can also occur in the shoulder, ankle, elbow, hand or foot. In PVNS the lining of the ...

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★リンクテーブル★

リンク元	「色素性絨毛結節性滑膜炎」
関連記事	「PV」

「色素性絨毛結節性滑膜炎」

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英

pigmented villonodular synovitis, PVS PVNS

関

関節血症

参考

• 1. wiki en

• http://en.wikipedia.org/wiki/Pigmented_villonodular_synovitis

• 2. 関節鏡写真

• http://radiographics.rsna.org/content/28/5/1519/F7.expansion

• 3. 英語 解説

• http://test2.aaos.org/oko/description.cfm?topic=ONC012&referringPage=menus/issues.cfm

「PV」

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