We have
compiled some of our favorite articles in a handy print out so you can bring it to your
doctor or just keep it on hand as a reference.
You will
require Adobe Reader to read it. The free plug-in is available here.
The following are articles thqt have been chosen to answer the question : What is PVNS?We wish to give appropriate credit and gratitude to those who have made available this valuable information. If you feel we have not done so, please contact us right away.
Pigmented Villonodular Synovitis. A rare differential diagnosis of popliteal space-occupying lesion
[Original Article in German]
Markmiller M, Bohndorf K, von Schoenaich P, Wagner T, Ruter A.
Klinik fur Unfall- und Wiederherstellungschirurgie, Zentralklinikum Augsburg.
Pigmented villonodular synovitis (PVNS) is a disease that involves the lining of joints, bursae and tendon sheaths.
The incidence is low and estimated to be 1.8 patients per million population. The cause of PVNS is unclear and discussed to be either inflammatory or neoplastic.
PVNS has been described in 2 forms different for prognosis and treatment (nodular and diffuse). The articular form almost appears in the knee joint as we describe below in a 14 year-old patient. PVNS was first defined in 1941 by Jaffe e.a. [7]. Because of the uncommon occurrence of the disease it is difficult to amass patient series to allow confirmed statements on therapy and outcome. Larger patient series raise out of long periods of time. Differences in outcome and recurrence rates exist for the nodular and diffuse form. Clinical findings are moderate pain and swelling of joints due to effusion and synovial proliferation. Magnetic resonance imaging shows typical findings. Surgical procedures are recommended as open or arthroscopic synovectomy for the diffuse form of PVNS, local excision for the nodular form and arthrodesis or prosthetic replacement for joint destruction.
PMID: 10851961 [PubMed - indexed for MEDLINE]
Did you kow that the definition of Neoplastic is the new abnormal growth of tissue.
Localized pigmented villonodular synovitis: a rare cause of locking of the knee.
Williams AM, Myers PT.
Brisbane Orthopaedic and Sports Medicine Centre, Holy Spirit Hospital, Queensland, Australia.
A 34-year-old woman presented with a history of recurrent episodes of knee locking and swelling. Arthroscopy revealed characteristic brown colored pedunculated lesions in the intercondylar notch region. These were clearly causing interference to joint motion. The lesions were resected arthroscopically with prompt resolution in symptoms. Histological examination confirmed pigmented villonodular synovitis.
PMID: 9276062 [PubMed - indexed for MEDLINE]
This is a pretty intense article, non-medical persons may find this very difficult to understand. Give the info for your doctor to review.
Anatomic Pathology/Review Article Pathology of the Synovium John X. O'Connell, MB, FRCPC
Abstract
Synovium is specialized mesenchymal tissue that is essential for the appropriate function of the locomotor apparatus. It is the site for a series of pathologic processes that are characteristic, and in some cases specific, to this distinctive tissue. In this article, the normal microscopic anatomy of synovium is briefly reviewed. Synovial proliferative disorders, including pigmented villonodular synovitis, giant cell tumor of tendon sheath, hemosiderotic synovitis, and fatty infiltration of the synovial membrane are discussed. Additionally, the subjects of intrasynovial cartilaginous lesions (primary and secondary synovial chondromatosis) and crystal deposition diseases are reviewed. Finally, the response of synovial tissues to implanted foreign materials that are used in large and small joint arthroplasty are described.
Normal Anatomy
Synovium is composed of 2 to 3 layers of specialized cells termed synoviocytes. The synoviocytes overlie loose connective tissue consisting of fat, collagen, and blood vessels.
Synoviocytes, like their mesodermal first cousins, mesothelial cells, grow as layers that surround fluid-filled spaces. By light microscopy, synoviocytes are relatively indistinct, appearing only as flattened, oval, dark-staining nuclei that usually seem removed from the immediate luminal surface by a fine layer of eosinophilic tissue.
Most of the time, cytoplasmic borders are not visible, although when the cells are stimulated to divide or react to injury, they achieve rounded epithelioid morphologic features. Ultrastructurally, the cells lining the luminal aspect of the synovial cavity have ovoid nuclei, inconspicuous nucleoli, and long cytoplasmic processes that extend in a tendril-like arrangement circumferentially from the nucleus. The cytoplasmic processes contain flattened endoplasmic reticulum, ribosomes, a well-developed Golgi complex, and numerous subplasmalemmal pinocytotic vesicles.
The processes interdigitate extensively, but cell junctions are not present. The surface synovial cells traditionally have been termed B cells.
Deep beneath the tangled web of surface cell processes are oval to rounded cells that have nuclear features similar to those of B cells. These so-called A cells lack cytoplasmic processes, but they have short filopodia.
The A cells have cytoplasmic organelles similar to those of the B cells, as well as occasional lysosomes. Transitional forms between the A and B cells also are found. A and B cells are embedded in an extracellular matrix rich in collagen and pools of amorphous ground substance.
The supporting connective tissue beneath the surface synoviocytes varies in its composition depending on anatomic site. In large joints that contain folds of synovium, there typically is a well-developed vascular network and relatively abundant mature adipose tissue.
Deep beneath this loose connective tissue is the dense, supporting, fibrous joint capsule. In tendon sheaths, the supporting connective tissue network is scant, and the synovial cells lie in close apposition to the fibrous layer of the tendon sheath or the tendon.
Synovial Proliferative Disorders Pigmented Villonodular Synovitis and Giant Cell Tumor of Tendon Sheath
Pigmented villonodular synovitis (PVNS) is a proliferative disorder of the synovial lining of joints that produces localized or diffuse nodular thickening of the synovial membrane.
Any synovial joint may be affected, but the knee is affected the most frequently.
PVNS affects males and females with equal frequency and occurs in a wide age range, with greatest incidence in the second to fourth decades.
Patients typically complain of pain, swelling, or both of the affected joint. PVNS appears as a villous and nodular thickening of the synovial membrane Diffuse involvement of the synovium results in thin, finger-like excrescences admixed with 0.5- to 2.0-cm rounded nodules that cover the normally smooth synovial membrane. Localized nodular synovitis produces a single bulging mass within the synovium.
Microscopically, PVNS results in a papillary, villous, and nodular expansion of the synovial membrane. The surface synovial cells overlie a lobular and sheet-like arrangement of mononuclear rounded and epithelioid cells, multinucleated osteoclast-like giant cells, and lipid-rich cells.
The mononuclear cells frequently contain large hemosiderin granules and have central round nuclei, often with prominent nucleoli. Mild nuclear pleomorphism often is seen. Mitotic counts rarely exceed 5 per 10 high-power fields. Necrosis is rare. The sheets and nodules of mononuclear cells, which likely represent proliferated surface synovial cells, are embedded in a collagenous stroma. 5 In some cases, the nodular growth pattern is accentuated by broad bands of collagen. Most of the hemosiderin in PVNS lies beneath the surface synovial layer, and it often is found in aggregates in the rounded nodules.
A minority of cases of diffuse PVNS can result in bone erosion, sometimes involving adjacent bones of a joint.
PVNS is treated by surgical excision. Localized disease responds well to this approach; however, lesions that diffusely affect the synovium have a high rate of local recurrence (>50%).
PVNS that is refractory to repeated surgical excisions may be managed by low-dose radiation therapy.
Localized giant cell tumor of tendon sheath (GCTTS) is histologically virtually identical to PVNS and represents its extra-articular analogue....
If you want to read more you can find the entire article and images here: http://www.ajcp.com/oconnell1100ar.html
(This link is not active as of Aug 28/01-However, we will keep it on file just in case they bring it back up)
This article is of particular interest to me. I personally met these doctors in Toronto.
Outcome following radiation treatment for high-risk pigmented villonodular synovitis.
O'Sullivan B, Cummings B, Catton C, Bell R, Davis A, Fornasier V, Goldberg R.
Department of Radiation Oncology, Princess Margaret Hospital, Toronto, Canada.
PURPOSE: Pigmented villonodular synovitis (PVNS) is a rare proliferative process involving synovial membranes. It has a variable course, and while usually benign, may be destructive, resulting in major symptoms and loss of function leading to amputation. Optimum treatment is not always clear, and little information exists with respect to the role of radiotherapy. The purpose was to review our experience with radiotherapy in cases at high risk for recurrence with functional loss including instances where amputation was the sole alternative for symptomatic disease.
METHODS AND MATERIALS: The records of all patients registered between 1972 and 1992 with a diagnosis of PVNS were identified (21 cases). The records of 14 cases who received radiotherapy after referral were reviewed retrospectively for demographic information, radiotherapy treatment parameters, and tumor outcome.
RESULTS: All cases had confirmation of pathologic diagnosis. Six patients had primary and eight had recurrent disease (with a mean of 2.5 prior surgical procedures). All cases had both intra- and extraarticular disease and, without exception, the poorer prognosis diffuse subtype of the disease. The majority had one or more additional risk factors including skin, bone, tendon, neurovascular, or muscle group extension. With a mean follow-up time of 69 months (range 13-250 months), only one patient has shown persistence of disease. With the exception of that single case, all those with measurable disease had obvious disease until at least 12 months and, subsequently, manifested complete responses. The single case was lost from the clinic after 8 months from the initiation of radiotherapy to a dose of 30 Gy in 15 fractions and had a palpable mass at the time. He subsequently was noted to have a persisting mass and an excisional biopsy 9 years later showed PVNS. He remains well 21 years after treatment with good function. Eleven patients enjoyed excellent or good function from the affected limb and three had fair function. All patients had greater use of limb than at the time of treatment. No patient required amputation, and none had evidence of serious radiotherapy complications.
CONCLUSIONS: These results demonstrate that moderate dose radiotherapy is an effective modality in the treatment of a subset of cases with this rare condition. Its use has permitted avoidance of amputation in very advanced cases with acceptable function preservation. When treatment is indicated we currently recommend gross total removal of PVNS. This is followed by moderate dose radiotherapy (35 Gy in 15 fractions) for residual disease where salvage of subsequent recurrence may compromise function.
PMID: 7790264 [PubMed - indexed for MEDLINE]