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- W2028124348 abstract "Purpose/Objective: Pigmented villonodular synovitis (PVNS) is a rare proliferative process originating from synovial membranes. Usually benign, it may be destructive, resulting in major symptoms and loss of function leading to amputation rarely. There is a paucity of outcome data following radiotherapy in high risk cases. Our purpose was to update a prior experience with extended follow-up and almost three times greater accrual of cases at high risk for recurrence with functional loss including instances where amputation was the sole alternative for symptomatic disease.Materials/Methods: The records of all patients (41 cases) who received radiotherapy for PVNS between 1972 and 2003 were reviewed retrospectively and, since 1986, prospectively. Eligibility included at least one year of follow-up following radiotherapy. Local control was defined as absence of clinical or imaging evidence of disease following treatment in either patients without overt disease at the time of radiotherapy, or in patients with stable disease on serial cross-sectional imaging of gross disease for at least one year following treatment.Results: All cases had pathologic confirmation of diagnosis at our center. 18 had primary and 23 recurrent disease (with a mean of 2 prior surgical procedures). 36 had origin from a joint (most commonly the knee in 37%) and 5 arose in periarticular tendon sheath. All but 1 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 and 17 of these lesion exceeded 10 cm in maximum dimension. 21 had gross residual disease at the time of radiotherapy. The mean radiotherapy dose was 38 Gy (range 28 Gy to 50 Gy), the majority receiving 35 Gy in 14 fractions. One patient had the malignant variant of PVNS and is the only patient with clear resection margins consistent with oncologic principles for malignant sarcomas. With a mean follow-up time of 77 months (range 13–337 months), only one patient has shown active disease by the criteria described. Two additional cases (both tendon sheath lesions of the wrist/hand) underwent subsequent biopsies of a palpable asymptomatic stable lesion (<1 cm in size). In one this took place 9 years following radiotherapy and he remained without treatment or progression 17 years later; the second had a biopsy 16 years following treatment, had no additional therapy and was well without progression 3 years later. No patient required amputation, and none had evidence of serious radiotherapy complications. Only one patient has developed a second malignancy, specifically a brain tumor but the site of PVNS was the ankle.Conclusions: Moderate dose radiotherapy is a very effective treatment in PVNS and permits local control and avoidance of amputation in very advanced and typically recurrent cases. When treatment is indicated we currently recommend gross total removal of PVNS followed by moderate dose radiotherapy for residual disease where salvage of subsequent recurrence may compromise function. Alternatively control of gross disease can also be expected following radiotherapy. Purpose/Objective: Pigmented villonodular synovitis (PVNS) is a rare proliferative process originating from synovial membranes. Usually benign, it may be destructive, resulting in major symptoms and loss of function leading to amputation rarely. There is a paucity of outcome data following radiotherapy in high risk cases. Our purpose was to update a prior experience with extended follow-up and almost three times greater accrual of cases at high risk for recurrence with functional loss including instances where amputation was the sole alternative for symptomatic disease. Materials/Methods: The records of all patients (41 cases) who received radiotherapy for PVNS between 1972 and 2003 were reviewed retrospectively and, since 1986, prospectively. Eligibility included at least one year of follow-up following radiotherapy. Local control was defined as absence of clinical or imaging evidence of disease following treatment in either patients without overt disease at the time of radiotherapy, or in patients with stable disease on serial cross-sectional imaging of gross disease for at least one year following treatment. Results: All cases had pathologic confirmation of diagnosis at our center. 18 had primary and 23 recurrent disease (with a mean of 2 prior surgical procedures). 36 had origin from a joint (most commonly the knee in 37%) and 5 arose in periarticular tendon sheath. All but 1 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 and 17 of these lesion exceeded 10 cm in maximum dimension. 21 had gross residual disease at the time of radiotherapy. The mean radiotherapy dose was 38 Gy (range 28 Gy to 50 Gy), the majority receiving 35 Gy in 14 fractions. One patient had the malignant variant of PVNS and is the only patient with clear resection margins consistent with oncologic principles for malignant sarcomas. With a mean follow-up time of 77 months (range 13–337 months), only one patient has shown active disease by the criteria described. Two additional cases (both tendon sheath lesions of the wrist/hand) underwent subsequent biopsies of a palpable asymptomatic stable lesion (<1 cm in size). In one this took place 9 years following radiotherapy and he remained without treatment or progression 17 years later; the second had a biopsy 16 years following treatment, had no additional therapy and was well without progression 3 years later. No patient required amputation, and none had evidence of serious radiotherapy complications. Only one patient has developed a second malignancy, specifically a brain tumor but the site of PVNS was the ankle. Conclusions: Moderate dose radiotherapy is a very effective treatment in PVNS and permits local control and avoidance of amputation in very advanced and typically recurrent cases. When treatment is indicated we currently recommend gross total removal of PVNS followed by moderate dose radiotherapy for residual disease where salvage of subsequent recurrence may compromise function. Alternatively control of gross disease can also be expected following radiotherapy." @default.
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- W2028124348 date "2005-10-01" @default.
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- W2028124348 title "Sustained Remission Following Radiation Treatment for High-Risk Pigmented Villonodular Synovitis" @default.
- W2028124348 doi "https://doi.org/10.1016/j.ijrobp.2005.07.088" @default.
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