The effects of radiosynoviorthesis in pigmented villonodular synovitis of the knee

Dürr, Hans Roland; Capellen, Carl Ferdinand; Klein, Alexander; Baur‐Melnyk, Andrea; Birkenmaier, Christof; Jansson, Volkmar; Tiling, Reinhold

doi:10.1007/s00402-018-3097-4

Cited by 24 publications

(18 citation statements)

References 18 publications

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“…TOPP represents the largest prospective, international, multicenter disease registry for dt-TGCT, being able to include 166 patients in slightly more than 2 years and shows that conducting collaborative observational studies for a rare tumor is feasible. Current literature is largely focused on the oncological outcomes of this oftenchronic disease [16,18,20,[23][24][25][26][27]. Baseline data derived from this registry help to describe a preliminary understanding of the dt-TGCT patient journey and treatment decisions around disease onset and diagnosis of dt-TGCT patients.…”

Section: Discussionmentioning

confidence: 99%

“…Synovectomies are generally relatively invasive, with a high recurrence rate and repetitive surgery causing significant impairment [24]. Multimodality treatments (e.g., external beam radiotherapy and radiosynoviorthesis) have been performed in an attempt to reduce the recurrence rate in dt-TGCT, leading to varied reported outcomes [25][26][27]. In addition to surgery, several CSF1R inhibitors including TKIs showed promising results in tumor volume decrease and reduction of debilitating symptoms [16,18,20,21,34].…”

Section: Discussionmentioning

confidence: 99%

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The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study

Bernthal¹,

Spierenburg

Healey

et al. 2021

Orphanet J Rare Dis

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Background Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive neoplasm arising from the synovium of joints, bursae, and tendon sheaths affecting small and large joints. It represents a wide spectrum ranging from minimally symptomatic to massively debilitating. Most findings to date are mainly from small, retrospective case series, and thus the morbidity and actual impact of this rare disease remain to be elucidated. This study prospectively explores the management of TGCT in tertiary sarcoma centers. Methods The TGCT Observational Platform Project registry was a multinational, multicenter, prospective observational study involving 12 tertiary sarcoma centers in 7 European countries, and 2 US sites. This study enrolled for 2 years all consecutive ≥ 18 years old patients, with histologically diagnosed primary or recurrent cases of diffuse-type TGCT. Patient demographic and clinical characteristics were collected at baseline and every 6 months for 24 months. Quality of life questionnaires (PROMIS-PF and EQ-5D) were also administered at the same time-points. Here we report baseline patient characteristics. Results 166 patients were enrolled between November 2016 and March 2019. Baseline characteristics were: mean age 44 years (mean age at disease onset: 39 years), 139/166 (83.7%) had prior treatment, 71/166 patients (42.8%) had ≥ 1 recurrence after treatment of their primary tumor, 76/136 (55.9%) visited a medical specialist ≥ 5 times, 66/116 (56.9%) missed work in the 24 months prior to baseline, and 17/166 (11.6%) changed employment status or retired prematurely due to disease burden. Prior treatment consisted of surgery (i.e., arthroscopic, open synovectomy) (128/166; 77.1%) and systemic treatments (52/166; 31.3%) with imatinib (19/52; 36.5%) or pexidartinib (27/52; 51.9%). Treatment strategies at baseline visits consisted mainly of watchful waiting (81/166; 48.8%), surgery (41/166; 24.7%), or targeted systemic therapy (37/166; 22.3%). Patients indicated for treatment reported more impairment compared to patients indicated for watchful waiting: worst stiffness NRS 5.16/3.44, worst pain NRS 6.13/5.03, PROMIS-PF 39.48/43.85, and EQ-5D VAS 66.54/71.85. Conclusion This study confirms that diffuse-type TGCT can highly impact quality of life. A prospective observational registry in rare disease is feasible and can be a tool to collect curated-population reflective data in orphan diseases. Name of registry: Tenosynovial Giant Cell Tumors (TGCT) Observational Platform Project (TOPP). Trial registration number: NCT02948088. Date of registration: 10 October 2016. URL of Trial registry record: https://clinicaltrials.gov/ct2/show/NCT02948088?term=NCT02948088&draw=2.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study

Bernthal¹,

Spierenburg

Healey

et al. 2021

Orphanet J Rare Dis

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“…In general, the appropriate extent of excision should be determined on the basis of the type of PVNS, the presence of extraarticular extensions, and the experience of the surgeon. In addition, conventional radiotherapy, as well as radiosynoviorthesis, is considered for patients with large and even recurrent diffuse forms of the disease [34]. Preoperatively, although the patient had been recommended to receive radiosynoviorthesis 6-8 weeks after surgery, she declined to accept the adjuvant therapy because of the concerns about the potential side effects of the radiopharmacon, Fortunately, both intraoperative findings and the postoperative MRI ensured that no residual PVNS was found.…”

Section: Discussionmentioning

confidence: 99%

Open synovectomy treatment for intra- and extraarticular localized pigmented villonodular synovitis of the knee: a case report

Wen

Zhang

et al. 2021

BMC Musculoskelet Disord

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Background Pigmented villonodular synovitis (PVNS) is a rare, benign, proliferative neoplastic process that commonly affects synovial-lined anatomic spaces. The diffuse type (DPVNS) is characterized by invasion of the entire joint synovium, while the localized type (LPVNS) is characterized by a relatively normal synovial appearance. This report describes a unique case of massive intraarticular LPVNS with an extraarticular extension through the lateral patellar retinaculum. No similar cases have been found in the literature. Case presentation A 58-year-old woman had a history of hyperuricemia and knee trauma and presented with unilateral knee acute swelling and pain symptoms with sudden onset. Recent expansion of the LPVNS caused the development of a tender palpable soft tissue mass in the anterolateral aspect of the knee and acute reduced mobility. Preoperative magnetic resonance imaging of the knee revealed the presence of only the soft tissue mass and mild degenerative changes. Open synovectomy was performed successfully to excise the mass. Intraoperatively, macroscopic features of the bright brown inflamed synovium suggested LPVNS, which was confirmed histopathologically. Postoperatively, the symptoms of limited mobility and pain were appreciably relieved. Recurrence was not observed during the clinical follow-up at 1, 6 or 18 months after surgery. Conclusions Here, we report the unique case of localized pigmented villonodular synovitis of the knee in a misdiagnosed patient with intra- and extraarticular lesion, which might be attributed to the history of knee trauma and the focal defect of the lateral patellar retinaculum. Open synovectomy effectively relieved the symptoms of limited mobility and pain and no recurrence was observed prior to 18 months postoperatively. To reduce misdiagnosis, MRI examinations are recommended for all patients suspected of having PVNS, including those who have a history of hyperuricemia.

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“…TOPP represents the largest prospective, international, multi-center disease registry for dt-TGCT, being able to include 166 patients in slightly more than 2 years, and shows that conducting collaborative observational studies for rare tumor is feasible. Current literature is largely focused on the oncological outcomes of this often chronic disease [15,16,17,18,19,20,21,22]. Data derived from this registry made it possible for the rst time to describe the dt-TGCT patient journey and treatment decisions around disease onset and diagnosis of dt-TGCT patients.…”

Section: Discussionmentioning

confidence: 99%

“…Synovectomies are generally relatively invasive, with a high recurrence rate and repetitive surgery causing signi cant impairment [16]. Multimodality treatments (e.g., external beam radiotherapy and radiosynoviorthesis) have been performed in an attempt to reduce the recurrence rate in dt-TGCT, leading to varied reported outcomes [17,18,19]. In addition to surgery, several CSF1receptor inhibitors including TKIs showed promising results in tumor volume decrease and reduction of debilitating symptoms [20,21,22,30,31].…”

Section: Discussionmentioning

confidence: 99%

The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective real-world study

Bernthal¹,

Spierenburg²,

Healey³

et al. 2020

Preprint

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Background: Tenosynovial giant cell tumor (TGCT) is a rare locally aggressive neoplasm arising from the synovium of joints, bursae, and tendon sheaths affecting small and large joints. It represents a wide spectrum ranging from minimally symptomatic to massively debilitating. The majority of findings to date are mainly from small, retrospective case series, and thus the morbidity and actual impact of this rare disease remain to be elucidated. This study explores prospectively the real-world management of TGCT.Methods: The TGCT Observational Platform Project (TOPP) registry was a multinational, multicenter, prospective observational study involving 12 tertiary sarcoma centers in 7 European countries, and 2 US sites. This study enrolled for 2 years all consecutive ≥ 18 years old patients, with histologically diagnosed primary or recurrent cases of diffuse-type TGCT. Patient demographic and clinical characteristics were collected at baseline and every 6 months for 24 months. Quality of life questionnaires (PROMIS-PF and EQ-5D) were also administered at the same time-points. Here we report baseline patient characteristics.Results: 166 patients were enrolled between November 2016 and March 2019. Baseline characteristics were: mean age 44 years (mean age at disease onset: 39 years), 139/166 (83.7%) had prior treatment, 71/166 patients (42.8%) had ≥ 1 recurrence after treatment of their primary tumor, 76/136 (55.9%) visited a medical specialist ≥ 5 times, and 66/116 (56.9%) missed work in the 24 months prior to baseline, and 17/166 (11.6%) changed employment status or retired prematurely due to disease burden. Prior treatment consisted of surgery (i.e., arthroscopic, open synovectomy) (128/166; 77.1%), and systemic treatments (52/166; 31.3%) with imatinib (19/52; 36.5%) or pexidartinib (27/52; 51.9%). Treatment strategies at baseline visits consisted mainly of watchful waiting (81/166; 48.8%), surgery (41/166; 24.7%), or targeted systemic therapy (37/166; 22.3%). Patients indicated for treatment reported more impairment compared to patients indicated for watchful waiting: worst stiffness NRS 5.16/3.44, worst pain NRS 6.13/5.03, PROMIS-PF 39.48/43.85, and EQ-5D VAS 66.54/71.85.Conclusion: This study confirms that diffuse-type TGCT can highly impact quality of life. A prospective observational registry in rare disease is feasible and can be a tool to collect curated-population reflective data in orphan diseases.

show abstract

The effects of radiosynoviorthesis in pigmented villonodular synovitis of the knee

Cited by 24 publications

References 18 publications

The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study

The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study

Open synovectomy treatment for intra- and extraarticular localized pigmented villonodular synovitis of the knee: a case report

The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective real-world study

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