Pvns is rarely found in the temporomandibular joint tmj, with. A histopathologic and fluorescence in situ hybridization analysis of 2. Pigmented villonodular synovitis pvns is a rare, benign, idiopathic proliferative disorder of the synovium that results in villous and or nodular formations that have been reported to manifest. Histopathology of pigmented villonodular synovitis. Pigmented villonodular synovitis of knee radiology case. Localized and diffuse forms of synovial involvement were reported 2. Dec 19, 2008 pigmented villonodular synovitis pvns is a benign proliferative histiocytic disorder of the synovium. Pigmented villonodular synovitis of the temporomandibular joint. The pathologist and the hip diagnostic histopathology. This disease can be disabling and is characterised by a proliferation of synovial tissue ottoviani, 2011.
Pigmented villonodular synovitis, bursitis and tenosynovitis. I write with regard to the paper by zuber and colleagues that purports to demonstrate a case of pigmented villonodular synovitis pvns. Mri and thallium features of pigmented villonodular. It is characterized by the onset of monoarticular pain and swelling, the knee being the most frequently affected 80%. Histopathology reported to be pigmented villonodular synovitis. We report a 48 year followup of a case of recurrent pigmented villonodular synovitis of the knee. Hemosiderotic synovitis appears as a brown or rust color of the normal glistening translucent synovial membrane. Pigmented villonodular synovitis pvns is a disease where the tissue lining the joints and tendons grow abnormally to produce a noncancerous mass or tumor. Fineneedle aspiration of pigmented villonodular synovitis of the temporomandibular joint masquerading as a primary parotid gland lesion. When you have pvns, the lining of a joint becomes swollen and grows. Pigmented villonodular synovitis annals of the rheumatic. Pdf pigmented villonodular synovitis of thumba cytological.
Pigmented villonodular synovitis radiology reference. In early hemosiderotic synovitis, fine villous projections extend into the joint cavity, but the larger nodules and thickened fronds of pvns do not occur. Dec 22, 2018 what is the prognosis of pigmented villonodular synovitis. Pigmented villonodular synovitis genetic and rare diseases. Pigmented villonodular synovitis, am fam physician 1999. Despite the benign histological character, recurrent surgery need may be necessary because of frequent recurrence. Webpathology is a free educational resource with 10202 high quality pathology images of benign and malignant neoplasms and related entities. Thirtyfive presented data on the treatment of localized pigmented villonodular synovitis lpvns, 40 on diffuse pigmented villonodular synovitis dpvns, 1 on extraarticular lpvns, and 7 on dpvns with extraarticular involvement.
Background pigmented villonodular synovitis pvns belongs to a family of uncommon benign neoplastic diseases of uncertain etiology that. Pigmented villonodular synovitis of the hip and knee. Low dose radiotherapy and staged surgical resection for diffuse pigmented villonodular synovitis of the knee preserves normal knee function with minimal risk of recurrence felasfa m. Treatment of pigmented villonodular synovitis of the knee. Pigmented villonodular synovitis pvns is a benign, locally invasive synovial proliferation of unknown etiology affecting joints, bursae, and tendon sheaths. Pigmented villonodular synovitis is an uncommon disease that remains a diagnostic challenge. We present a case of pvns involving the lumbar spine. Although the use of more detailed molecular tools, as discussed above, does allow. Treatment of diffuse pigmented villonodular synovitis of. Although trauma, inflammation, neoplasia, and disorders of lipid metabolism have been implicated as causative factors of pvns, 4, 7, the etiology of this disease remains unclear. Diagnosis of chronic bacterial infection tuberculosis, lyme, fungal infection, sarcoidosis, leukaemic infiltration, pigmented villonodular synovitis, reticulohistocytosis, amyloidosis, haemochromatosis and ochronosis might require synovial tissue histology.
Treatment of diffuse pigmented villonodular synovitis of the. Pigmented villonodular synovitis is a benign lesion located in joints, tendon sheaths, or. Radiotherapy, pvns pigmented villonodular synovitis, surgery introduction pigmented villonodular synovitis pvns is a rare proliferative disorder of the synovium affecting any joint but frequently found in the knee. In 1941, jaffe described it as a group of synovial, tenosynovial, and bursal lesions 1. A patients guide to pigmented villonodular synovitis of the knee compliments of.
Pigmented villonodular synovitis pvns is an uncommon, benign, but locally aggressive lesion characterized by tumefactive synovial proliferation of unknown aetiology. Methods electronic medical records emr of four chinese institutes were queried for patients with histologically proven pvns between january 2005 and february 2014. It is characterized by inflammation and overgrowth of the synovium and it typically affects the hip or ankle. To illustrate the mri features of pigmented villonodular synovitis pvns, with special focus on the diffuse intraarticular form. Pigmented villonodular synovitis is most often painless inflammation or swelling, and overgrowth of the lining of a joint. Each case of pvns is usually characterized by its site of origin and by its form of growth. Pigmented villonodular synovitis pvns is a locally aggressive synovial proliferative.
Develops in synovial lining of joints, tendon sheaths and bursae, usually of knee 80%, ankle, hip, shoulder, elbow joint. Villonodular synovitis an overview sciencedirect topics. The tumor grew outside the dura and extended to the paravertebral connective tissue, causing sensory and motor disturbance indicative of spinal cord compression. Pigmented villonodular synovitis of joints histological and clinical. Pigmented villonodular synovitis of joints, on the other hand, is a rare complaint. Pigmented villonodular synovitis can affect any age group, but is most common between the ages of 30 to 40. It belongs to a family of synovial proliferative lesions, with two variants depending on the area involved. Pigmented villonodular synovitis of the temporomandibular. Pvns of the hip mainly occurs in young adults, requiring early diagnosis and adequate treatment to obtain good results. Pigmented villonodular synovitis of the knee the sports.
In the later stages, after chronic repeated hemorrhage, the synovium. Introduction pigmented villonodular synovitis pvns is a very rare disease. 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. Mar 23, 20 pigmented villonodular synovitis pvns is a disease of the joints characterized by inflammations and overgrowth of joint lining. This tendency to recur, the sometimes large mass presenting, and the cellularity, occasional mitoses and bone erosion, may even suggest malignancy. Pigmented villonodular synovitis pvns is a benign but locally aggressive proliferative disorder, commonly arising from the synovium of long bone joints, tendon sheaths and bursae. Pvns is an idiopathic monoarticular neoplastic synovial disease. Introduction tenosynovitis is a rare lesion, typically monoarthric, which primarily affects the major joints. This is not a primary inflammatory disease of joints, despite the name.
Pigmented villonodular synovitis pvns is a relatively rare, benign proliferation lesion of the synovium of large joints, but there is not much information available about the diseases aetiology, clinical history, differential diagnosis, treatment, and longterm effects. Outcomesresolutions the prognosis is generally excellent with surgical intervention which is on a complete removal of the lesion, even if they are deepseated. Pdf international clinical pathology journal pigmented. Fineneedle aspiration biopsy diagnosis of invasive. Pigmented villonodular synovitis pvns is a condition that causes the synoviumthe thin layer of tissue that lines the joints and tendonsto thicken and overgrow.
Pigmented villo nodular synovitis is most often painless inflammation or swelling, and overgrowth of the lining of a joint. Radiotherapy on kneerelated pigmented villonodular synovitis. Media in category histopathology of pigmented villonodular synovitis the following 2 files are in this category, out of 2 total. Pigmented villonodular synovitis pvns is a rare disease characterized by proliferation of synovial tissue in the joint, tendon sheath, and bursa. Pigmented villonodular synovitis pvns is a benign proliferative joint disease with an uncertain etiology that uncommonly involves the spine.
A case of avascular pigmented villonodular synovitis in the foot was encountered and surgically verified. It is also known as tenosynovial giantcell tumour, diffuse type. Pdf international clinical pathology journal pigmented villonodular synovitis pvns in human and animals. Physical therapy management of a patient with diffuse. Pathology outlines pigmented villonodular synovitis pvns. Pigmented villonodular synovitis pvns, also known as intraarticular giantcell tumor of the tendon sheath, is a joint disease characterized by inflammation and overgrowth of the joint lining, becoming benign tumors. Rare neoplasticlike villonodular hyperplasia of synovium and tendon sheaths in young adults composed of mononuclear cells and multinuclear giant cells with hemosiderin deposition bonetumor. Pigmented villonodular synovitis rsna publications online. Pigmented villonodular synovitis pvns represents an uncommon benign. Pigmented villonodular synovitis pvns is a rare, proliferative disease of synovium. Pvns is an extremely rare condition affecting only 1. Pigmented villonodular synovitis pvns is a disease of the joints characterized by inflammations and overgrowth of joint lining. Pigmented villonodular synovitis, also known as pvns, is a rare idiopathic proliferative disorder of the synovium that leads to villous and or nodular formations within joints, tendon sheaths and bursae 569.
This is because the tissue contains iron deposits called hemosiderin and the. Pigmented villonodular synovitis of thumba cytological diagnosis. He has no previous history of trauma, or any sports related injury he doesnt play any sport in fact. Is pigmented villonodular synovitis of the knee hereditary. It is characterized by a noncancerous mass or tumor.
The mass was golden brown in colour encircling the extensor tendon extensor hallucis longus tendon. Tenosynovial giant cell tumour tgct, also known as pigmented villonodular synovitis pvns, is a rare neoplasm affecting the synovium and tendon sheaths in young adults, with recurrent translocations involving col6a3 and csf1 genes 1. Their data were collected including gender, age at diagnosis, clinical presentation, affected site, symptom duration, comorbidities. The mass or tumor that results from this overgrowth is not cancerous and does not spread metastasize to other areas of the body. In 1941, jaffe described it as a group of synovial, tenosynovial, and bursal lesions. There is a localized and a diffuse form of the disorder with the localized or nodular version amenable to. It usually involves the knee joint in young adults and may be focal or diffuse. Localized and diffuse forms of synovial involvement were reported. In the case of pigmented villonodular synovitis pvns, mri findings are clear. Low dose radiotherapy and staged surgical resection for. Subcutaneous metastasis to the contralateral thigh was an unusual finding. A primary histopathological diagnosis of synovial sarcoma was made. The hip is less frequently affected than the knee, and hence is less discussed in scientific journals. Lowering the recurrence rate in pigmented villonodular.
Pigmented villonodular synovitis of the knee is a proliferative and inflammatory condition of the synovial tendon in the joint, tendon sheath or bursa. Comparative synovial histopathology during the disease course of arthritic diseases natural history of ra besides the heterogeneity within one disease, there might also be a variety of pathogenic mechanisms active during the disease course from initiation of joint inflammation to persistent chronic inflammation in a single patient. Pigmented villonodular synovitis pvns is a rare but disabling disease. Radiological features of pigmented villonodular synovitis. Histology of pvns revealed a hypertrophic synovial process characterized by villous, nodular, and villonodular proliferation and hemosiderin pigmentation. Presenting complaints commonly involve one joint, most often the knee or hip. The knee is a frequent target of this disease, and it is the joint most likely to be affected by special forms of synovitis such as pigmented villonodular synovitis and synovial chondromatosis. Pvns affects people in their third or fourth decades of life. Malignant pigmented villonodular synovitis archives of pathology. The 215 cases from our hospital are current 19722009 and those 62 from the jaffe collection from 1920 to 1968. Pigmented villonodular synovitis of joints mankin 2011. Pigmented villonodular synovitis pvns is a rare benign proliferative condition affecting synovial membranes of joints, bursae or tendons resulting from possibly neoplastic synovial proliferation with villous and nodular projections and hemoside. Pigmented villonodular synovitis, temporomandibular joint, giant cell granuloma, synovitis 1.
Pigmented villonodular synovitis is a nonmalignant condition that is best diagnosed by synovial biopsy. The overgrowth read more about pigmented villonodular synovitis pvns. Complex villous hypertrophy of the synovial lining a hematoxylin and eosin x 10. Pigmented villonodular synovitis, also known as pvns, is a rare idiopathic proliferative disorder of the synovium that leads to villous and or nodular formations within joints, tendon sheaths and. Graney, bs 1 daria brooks, md 1 james wittig, md 2 robert m. An executive in an it firm, presented with locking of knee, after getting up in morning.
The fusion protein is cleaved, resulting in the overproduction of csf1, which attracts csf1rexpressing nonneoplastic cells macrophages and monocytes. Histology demonstrated fibroblastic and histi ocytic proliferation, as well as increased mitotic activity in recurrent lesions. But lytic lesions of the bone can be caused by cancer so an mri and biopsy are required to make an accurate diagnosis. Pigmented villonodular synovitis pvns is a rare disease that can affect any joint, bursa or tendon sheath. Diffuse tenosynovial giantcell tumour is relatively common mostly benign chondroosseous tumour of the large joints. Patients with already recurring lesions of pigmented villonodular synovitis are especially at risk for further recurrence. Cytologic appearance of pigmented villonodular synovitis. Pigmented villonodular synovitis of the cervical spine. Coronal t 1 weighted a and axial short tau inversion recovery b mri sequences reveal sever e erosive disease because of the softtissue mass pvns.
A case of pigmented villonodular synovitis giant cell tumor of the synovium involving the vertebral column is presented. Pigmented villonodular synovitis occurs commonly in the third and fourth decades of life. Pigmented villonodular synovitis has a high risk of recurrence after synovectomy in the first 3 years. Pigmented villonodular synovitis giant cell tumor of the. A discussion of the synovial and bursal equivalents of the tenosynovial lesion commonly denoted as xanthoma, xanthogranuloma, giant cell tumor or myeloplaxoma of the tendon sheath, with some consideration of this tendon sheath lesion itself. Pigmented villonodular synovitis pvns is thought to be a neoplastic process related to tenosynovial giant cell tumor. Previously, it was known as pigmented villonodular synovitis, abbreviated pvns. Pigmented villonodular synovitis pvns facts written by charles p. Pigmented villonodular synovitis great toe springerlink. Pigmented villonodular synovitis pvns pathology outlines. Mar 19, 20 pigmented villonodular synovitis pvns is a relatively rare, benign proliferation lesion of the synovium of large joints, but there is not much information available about the diseases aetiology, clinical history, differential diagnosis, treatment, and longterm effects. Large joints, usually of the lower extremity, are affected. Pigmented villonodular synovitis recurrence risk is dependent on the effectiveness of the surgery.
Pigmented villonodular synovitis pvns is a rare benign proliferative condition affecting synovial membranes of joints, bursae or tendons resulting from possibly neoplastic synovial proliferation with villous and nodular projections and haemosiderin deposition. Apr 04, 2016 pigmented villonodular synovitis pvns is a disease in which the tissue lining the joints and tendons in the body synovium grows abnormally. Chromosomal analysis suggests that it is a true neoplasm and not an inflammatory process. Pigmented villonodular synovitis pvns is a unique benign proliferative. A patient s guide to pigmented villonodular synovitis of. It can also occur in the shoulder, ankle, elbow, hand or foot. Also known as diffuse tenosynovial giant cell tumor. Both pigmented villonodular synovitis as well as giant cell tumor of the tendon sheath are considered similar from the point of view of histological findings. Thus, it is a slow growing, albeit benign, locally invasive tumor of the synovium. The objective of this study is to present two series of cases of pigmented villonodular disease of joints pvns treated at different periods.
The term pigmented villonodular synovitis is generally used when diffuse intraarticular involvement is present, 2, 3. Pigmented villonodular synovitis pvns is a disease in which the tissue. Pigmented villonodular synovitis pvns is a joint problem that usually affects the hip or knee. Pigmented villonodular tenosynovitis of the temporomandibular. Pvns is most commonly monoarticular 70% knee joint but occasionally it can be oligoarticular. Pigmented villonodular synovitis pvns is a benign proliferative histiocytic disorder of the synovium. Diffuse tenosynovial giantcell tumour is relatively common mostly benign chondroosseous tumour of the large joints it is also known as tenosynovial giantcell tumour, diffuse type. Diagnosis we will confirm your diagnosis once we have carried out xrays, ultrasound and mri scans imaging, and we have the histology results from the biopsy taken from the disease site. Pdf pigmented villonodular synovitis pvns is a unique benign. Diffuse tenosynovial giantcell tumour libre pathology. Fnac suggestive of pvns which was finally confirmed by histopathology. Partial synovectomy is sufficient for the local type of pigmented villonodular synovitis.
The lesion is always monoarticular, the knee joint being by far the most common location. Sixty studies 1,019 patients met the inclusion criteria. Eighty per cent of the time pigmented villonodular synovitis affects just one joint of the body, primarily the knee joint. Pigmented villonodular synovitis has a variable histologic appearance, jaffe and others propose this reflects maturation of the process with the early cellular hypervascular phase giving way to subsequent hyalinization and fibrosis. The condition generally affects a persons knee or hip area, but can also be seen in other parts of the body as well, such as ankle, elbow, shoulder, hand or foot. Pigmented villonodular synovitis pvns dominic carreira. Microscopic examination reveals a characteristic histology, including marked synovial cell hyperplasia and subsynovial invasion by masses of polygonal cells, multinucleated giant cells. Histopathology in diffuse pigmented villonodular synovitis of the shoulder. Meticulous surgical resection reduces local recurrence rate in pigmented villonodular synovitis to less than 10%. From the department of pathology, cancer hospital and research institute, gwalior, m.
The objective was to describe the clinical presentation and outcomes of pvns. The lesion is composed of papillary, villous, and nodular areas covered by synovium. A case report submitted by christa schutte in partial fulfillment of the requirements for the doctor of physical therapy program primary advisor date 21615. Complete response to csf1r inhibitor in a translocation. Multifocal pigmented villonodular synovitis in the noonan. Pigmented villonodular synovitis pvns is a joint disease of the synovium, or joint lining. Pigmented villonodular synovitis pvns is a benign lesion that usually occurs in tendon sheaths, in burses or in the large joints synovia of the extremities. We conducted a study to analyse these aspects of pvns. Pigmented villonodular synovitis pathology orthobullets.
1520 920 474 341 943 765 1065 129 1339 763 1046 243 1000 395 840 725 1528 27 419 494 835 695 229 1260 312 647 1490 853 764 995