O'Sullivan B, Cummings B, Catton C, Bell R, Davis A, Fornasier V, Goldberg R
Department of Radiation Oncology, Princess Margaret Hospital, Toronto, Canada.
Int J Radiat Oncol Biol Phys. 1995 Jun 15;32(3):777-86. doi: 10.1016/0360-3016(95)00514-Y.
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.
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.
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.
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.
色素沉着绒毛结节性滑膜炎（PVNS）是一种累及滑膜的罕见增殖性病变。其病程多变，通常为良性，但也可能具有破坏性，导致严重症状和功能丧失，甚至需要截肢。最佳治疗方案并不总是明确的，关于放疗的作用也知之甚少。本研究旨在回顾我们对放疗治疗高复发风险且可能导致功能丧失病例的经验，包括那些因症状性疾病而唯一选择是截肢的病例。
确定1972年至1992年间所有诊断为PVNS的患者记录（共21例）。回顾性分析14例转诊后接受放疗患者的记录，获取人口统计学信息、放疗治疗参数及肿瘤转归情况。
所有病例均经病理诊断证实。6例为原发性疾病，8例为复发性疾病（平均先前接受过2.5次外科手术）。所有病例均存在关节内和关节外病变，无一例外均为预后较差的弥漫型疾病。大多数病例有一个或多个其他危险因素，包括皮肤、骨骼、肌腱、神经血管或肌肉组织受累。平均随访时间为69个月（范围13 - 250个月），仅有1例患者疾病持续存在。除该病例外，所有可测量疾病的患者在至少12个月内均有明显疾病表现，随后均显示完全缓解。该病例在放疗开始8个月后，接受15次分割共30 Gy剂量放疗时失访，当时可触及肿块。9年后切除活检显示仍为PVNS。治疗21年后，其功能良好，状态良好。11例患者患侧肢体功能优良，3例功能尚可。所有患者肢体使用情况均较治疗时有所改善。无患者需要截肢，也无严重放疗并发症的证据。
这些结果表明，中等剂量放疗是治疗该罕见疾病部分病例的有效方法。在非常晚期的病例中，使用放疗可避免截肢并保留可接受的功能。当需要治疗时，我们目前建议彻底切除PVNS。随后，对于残留病变给予中等剂量放疗（15次分割共35 Gy），因为后续复发的挽救治疗可能会影响功能。
