Hostname: page-component-78c5997874-mlc7c Total loading time: 0 Render date: 2024-11-19T12:24:18.816Z Has data issue: false hasContentIssue false
  • English
  • Français

Postoperative radiotherapy for pigmented villonodular synovitis (PVNS): case series and literature review

Published online by Cambridge University Press:  29 April 2021

T. Al-Hajri Open the ORCID record for T. Al-Hajri [Opens in a new window] ,
K. Al-Madailwi  and
J. Riromar
T. Al-Hajri*
Affiliation:
National Oncology Center, Royal Hospital, Muscat, Oman
K. Al-Madailwi
Affiliation:
National Oncology Center, Royal Hospital, Muscat, Oman
J. Riromar
Affiliation:
National Oncology Center, Royal Hospital, Muscat, Oman
*
Author for correspondence: Thuraya Al-Hajri, National Oncology Center, Royal Hospital, Royal Hospital Area, Al-Ghubra, P.O. Box 1331, Muscat, Oman. Tel: 0968 92854500. Fax: 096824627004. E-mail: [email protected]
Rights & Permissions [Opens in a new window]

Abstract

Purpose:

Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disease of the synovium with locally aggressive behaviour. We reviewed our experience using external beam radiotherapy (RT) in the treatment of PVNS.

Method:

We report five cases of PVNS who underwent Arthroscopic Synovectomy followed by postoperative RT in National Oncology centre in Oman. The total dose RT ranges between 30 and 36 Gray (Gy) Three-dimensional radiotherapy technique.

Conclusion:

Postoperative RT is effective in preventing disease recurrence and should be offered following maximal cytoreduction to enhance local control in PVNS.

Keywords

arthroscopic synovectomyexternal beam radiotherapypigmented villonodular synovitis (PVNS)
Type
Case Study
Information
Journal of Radiotherapy in Practice , Volume 22 , 2023 , e2
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Introduction

PVNS is a rare proliferative type of giant cell-rich tumours (GCTs). It commonly affects large joints such as knee, hip, ankle and elbow. MRI features of PVNS include different extent of synovial proliferation, joint effusion and erosion of bone, and in particular the deposit of hemosiderin within the synovial masses. There is no standard guideline or consensus on surgical procedure or postoperative therapy. The treatment is extrapolated from previous case series. Main stay of treatment is surgical intervention with arthroscopic synovectomy. However, rate of recurrence is high due to location and extent of the disease. Radiotherapy (RT) has a role in reducing risk of local recurrence.

Case Series

Five cases of PVNS were treated between 2012 and 2020 in our cancer centre. Patient’s baseline characteristics are shown in Table 1. The age at diagnosis was between 21 and 46 years. All patients have left knee PVNS, and all underwent Arthroscopic Synovectomy followed by postoperative RT. The total dose RT ranges between 30 and 36 Gray (Gy). Three-dimensional RT technique with two opposite portals was used. There were no reported significant side effects during RT treatment. Mean follow-up time is 40 months. All patients were on annual MRI follow-up which showed stable disease. The reported chronic side effects were minimal ipsilateral joint tenderness in one patient. All patients are doing well to date with no local recurrence or restrictions of movement.

Table 1.

F, female; M, male; RT, radiotherapy; Fr, fraction; NOM, no restriction of movement.

Discussion

GCTs are classified according to their site of origin, namely bone, soft tissue, synovium or tendon sheath. Those that arise from tendons and synovium are classified into two forms: localised (nodular tenosynovitis) mainly involves the digits and diffuse [pigmented villonodular synovitis (PVNS)] involving large joints such as knee, hip, ankle and elbow. Reference Gouin and Noailles1,Reference Heijden, Gibbons and Dijkstra2 The latter is rare with incidence of 2 cases per million per year. It is a benign proliferative tumour that develops in the synovium with high risk of recurrence. Reference Somerhausen and Fletcher3 The aetiology and pathogenesis of PVNS are unknown, but it may be due to chronic inflammation. Reference Oehler, Hans and Fassbender4 There is no difference between genders with younger patients seem predominantly affected. The most common complaint is intermittent joint pain. Reference Myers, Masi and Feigenbaum5

There is no standard guideline or consensus on surgical procedure or postoperative therapy. The treatment is extrapolated from previous case series. A multimodality approach including MRI, evaluation of the knee and complete synovectomy, either as a single or a staged procedure with postoperative therapy results in a lower recurrence rate and fewer complications. MRI features of PVNS include different extent of synovial proliferation, joint effusion and erosion of bone, and in particular the deposit of hemosiderin within the synovial masses. Reference Cheng, You, Liu, Zhao and Qu6

PVNS of the knee is associated with a higher recurrence rate than PVNS at other joints Reference Schwartz, Unni and Pritchard7 due to the anatomical limitation of the lesion; radical resection of diffuse PVNS is very difficult. Arthroscopic synovectomy is an appropriate treatment for knee PVNS. Extended synovectomy is recommended for diffuse PVNS. Reference Ponti, Sansone and Malchere8 This technique requires technical expertise, and complete excision is rarely achieved even by experienced arthroscopic surgeons.

The risk factors for recurrence include diffuse form of the disease, incomplete resection, location of the lesions, the experience and skills of the surgeon, and adjuvant therapy after surgery. Reference Fang and Zhang9

Postoperative RT has been used to achieve better local control in patients with primary or recurrent PVNS. Reference O’Sullivan, Cummings and Catton10 It should also be considered for patients receiving a radical synovectomy to treat inaccessible or hidden disease sites. Reference Blanco, Leon and Guthrie11 A large number of reports have confirmed that adjuvant postoperative external beam RT can further reduce the recurrence rate. De Carvalho et al reported that 8 patients with diffuse PVNS of the knee who had synovectomy with subsequent local external beam RT had a recurrence rate of 12.5% at 8.6 years of follow-up. Reference de Carvalho, Soares, Goncalves, Temponi and Melo Silva12 Griffin et al treated 49 patients with a mean follow-up of 94 months, with no recurrence of the disease in 94% of the patients and had better joint function in 41 patients. Reference Griffin, Ferguson and Catton13 A German review analysed data where RT was applied in 39 cases (95.1%) with excellent or good functional outcome noted in 34 cases (82.9%). The use of RT was not associated with early or late toxicity larger than Radiation Therapy Oncology Group toxicity grade II. Total doses in the range of 30–36 Gy are recommended. Reference Heyd, Seegenschmiedt and Micke14

There have been no studies on radiation dose-response relationships to date. At Asan Medical Center, patients have been given the conventional dose (32–34 Gy) or a lower dose (20 Gy) as postoperative treatment for diffuse PVNS of the knee, depending on the treating physicians who favoured different pathogenesis theories—neoplasia or chronic inflammation. Reference Park, Young and Jong15

Horoschak et al recommended that using 34 to 36 Gy radiation dose can achieve a better local control rate. Reference Horoschak, Tran and Bachireddy16 A retrospective study compared the clinical outcomes of patients who were treated with conventional or low-dose RT. It analysed the data of 23 patients who underwent Synovectomy followed by 4-MV or 6-MV external beam RT with a median dose of 20 (12–34) Gy in 10 fractions. At a median follow-up of 9 (0.8–12) years, 4 patients had recurrent disease, with a median disease-free interval of 5 years. Low-dose (20 Gy) RT appears to be as effective as moderate-dose treatment (around 35 Gy). Reference Park, Kim and Kim17

The recent development and investigation of systemic therapies targeting the colony-stimulating factor one (CSF1) pathway represent an important advancement in the treatment of GCT. These therapies may play a major role in the treatment of advanced, recurrent and recalcitrant diseases for which surgery carries more morbidity than expected benefit. The efficacy of four different tyrosine kinase inhibitors of the CSF1 receptor has recently been tested. This includes nilotinib, imatinib, emactuzumab and pexidartininb. Reference Tap, Wainberg and Anthony18

Conclusion

PVNS is a rare and locally aggressive disease which may involve any joints. Surgical resection plus adjuvant therapy is recommended for patients with risk factors of recurrence. Dose between 20 and 36 Gy is effective with minimal side effects.

References

Gouin, F, Noailles, T. Localized and diffuse forms of tenosynovial giant cell tumor (formerly giant cell tumor of the tendon sheath and pigmented villonodular synovitis). Orthop Traumatol Surg Res 2017; 103 (1): S91–S97.CrossRefGoogle ScholarPubMed
Heijden, L van der, Gibbons, C L M H, Dijkstra, P D S et al. The management of diffuse-type giant cell tumour (pigmented villonodular synovitis) and giant cell tumour of tendon sheath (nodular tenosynovitis). J Bone Jt Surg 2012; 94-B (7): 882–888.CrossRefGoogle ScholarPubMed
Somerhausen, N S, Fletcher, C D M. Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease. Am J Surg Pathol 2000; 24 (4): 479–492.CrossRefGoogle ScholarPubMed
Oehler, S, Hans, G, Fassbender, D et al. Cell populations involved in pigmented villonodular synovitis of the knee. The Journal of Rheumatology 2000; 27 (2): 463–470.Google ScholarPubMed
Myers, B W, Masi, A T, Feigenbaum, S. Pigmented villonodular synovitis and Tenosynovitis: a clinical epidemiologic study of 166 cases and literature review. Medicine (Baltimore) 1980; 59 (3): 223–238.CrossRefGoogle ScholarPubMed
Cheng, X G, You, Y H, Liu, W, Zhao, T, Qu, H. MRI features of pigmented villonodular synovitis (PVNS). Clin Rheumatol 2004; 23: 31–34.CrossRefGoogle ScholarPubMed
Schwartz, H S, Unni, K, Pritchard, D J. Pigmented villonodular synovitis. A retrospective review of affected large joints. Clin Orthop Relat Res 1989; 247: 243–255.CrossRefGoogle Scholar
Ponti, A De, Sansone, V, Malchere, M. Result of arthroscopic treatment of pigmented villonodular synovitis of the knee. Arthrosc J Arthrosc Relat Surg 2003; 19 (6): 602–607.CrossRefGoogle ScholarPubMed
Fang, Y, Zhang, Q. Recurrence of pigmented villonodular synovitis of the knee: a case report with review of literature on the risk factors causing recurrence. Medicine (Baltimore) [Internet]. 2020; 99 (16): e19856. https://journals.lww.com/mdjournal/fulltext/2020/04170/recurrence_of_pigmented_villonodular_synovitis_of.93.aspx CrossRefGoogle ScholarPubMed
O’Sullivan, B, Cummings, B, Catton, C et al. Outcome following radiation treatment for high-risk pigmented villonodular synovitis. Int J Radiat Oncol Biol Phys 1995; 32 (3): 777–786.CrossRefGoogle ScholarPubMed
Blanco, C E R, Leon, H O, Guthrie, T B. Combined partial arthroscopic synovectomy and radiation therapy for diffuse pigmented villonodular synovitis of the knee. Arthrosc J Arthrosc Relat Surg 2001; 17 (5): 527–531.CrossRefGoogle ScholarPubMed
de Carvalho, L H Jr, Soares, L F M, Goncalves, M B J, Temponi, E F, Melo Silva, O de Jr. Long-term success in the treatment of diffuse pigmented villonodular synovitis of the knee with subtotal synovectomy and radiotherapy. Arthrosc J Arthrosc Relat Surg 2012; 28 (9): 1271–1274.CrossRefGoogle ScholarPubMed
Griffin, A M, Ferguson, P C, Catton, C N et al. Long- term outcome of the treatment of high- risk tenosynovial giant cell tumor/pigmented villonodular synovitis with radiotherapy and surgery. Cancer 2012; 118 (19): 4901–4909.CrossRefGoogle ScholarPubMed
Heyd, R, Seegenschmiedt, M H, Micke, O. The role of external beam radiation therapy in the adjuvant treatment of pigmented villonodular synovitis. Z Orthop Unfall 2011; 149 (6): 677–682.CrossRefGoogle ScholarPubMed
Park, G, Young, S, Jong, H et al. Low-dose external beam radiotherapy as a postoperative treatment for patients with diffuse pigmented villonodular synovitis of the knee: 4 recurrences in 23 patients followed for mean 9 years. Acta Orthopaedica 2012; 83 (3): 256–260.CrossRefGoogle ScholarPubMed
Horoschak, M, Tran, P T, Bachireddy, P et al. External beam radiation therapy enhances local control in pigmented villonodular synovitis. Int J Radiat Oncol Biol Phys 2009; 75 (1): 183–187.CrossRefGoogle ScholarPubMed
Park, G, Kim, Y S, Kim, J H et al. Low-dose external beam radiotherapy as a postoperative treatment for patients with diffuse pigmented villonodular synovitis of the knee. Acta Orthop 2012; 83 (3): 256–260.CrossRefGoogle ScholarPubMed
Tap, W D, Wainberg, Z A, Anthony, S P et al. Structure-guided blockade of CSF1R kinase in tenosynovial giant-cell tumor. N Engl J Med 2015; 373: 428–437.CrossRefGoogle ScholarPubMed
Figure 0

Table 1.