Pigmented villonodular synovitis and rheumatoid arthritis: diagnostic challenges and therapeutic considerations in a case of knee pain.

We present a case of a woman in her 30s who visited the rheumatology clinic due to her persistent knee pain for 5 years, which spread to multiple joints. She was diagnosed with seropositive rheumatoid arthritis (RA). While most joints responded well to methotrexate and subsequently etanercept, persistent unilateral knee pain prompted further investigation. Imaging revealed synovitis and joint effusion in her knee, prompting arthroscopy and synovial biopsy, revealing pigmented villonodular synovitis (PVNS). Despite initial success with a tricompartmental synovectomy, her disease recurred. The decision was made to pursue medical therapy, with pexidartinib initiated by the oncology team. Our case report highlights the importance of considering other underlying conditions in patients with RA who do not achieve full clinical improvement despite standard treatment. Physicians should remain vigilant for atypical presentations and imaging features in patients with RA, for early recognition of PVNS can significantly impact treatment decisions and patient outcomes.

Unusual presentation of talonavicular joint pigmented villonodular synovitis: a case report.

BACKGROUND: Pigmented villonodular synovitis is a rare yet locally invasive disorder impacting synovial tissues. This case report delineates the atypical manifestation of pigmented villonodular synovitis in the talonavicular joint, detailing its diagnostic complexity and successful management. CASE PRESENTATION: A 56-year-old Iranian patient with a 4-year history of chronic ankle pain, initially diagnosed with degenerative joint disease post-trauma based on imaging, underwent talonavicular fusion surgery. An unexpected pigmented villonodular synovitis mass was encountered during the procedure. Subsequent interventions encompassed tumor resection, talonavicular joint fusion, and allograft bone grafting. Despite the initial intervention, persistent pain and nonunion necessitated a secondary procedure, involving joint surface curettage and autograft bone grafting. At the 12-month follow-up, the patient remained pain-free without tumor recurrence. CONCLUSION: This case report highlights the significance of considering pigmented villonodular synovitis as a crucial differential diagnosis in chronic ankle pain, even when there is evidence of degenerative joint disease and a history of trauma. Magnetic resonance imaging serves a crucial role in accurate diagnosis. Treatment necessitates precise tumor removal, appropriate bone grafting techniques and secure fixation. LEVEL OF EVIDENCE: IV.

Active surveillance of diffuse-type tenosynovial giant cell tumors: A retrospective, multicenter cohort study.

BACKGROUND: Diffuse-type tenosynovial giant cell tumor (D-TGCT) is a mono-articular, soft-tissue tumor. Although it can behave locally aggressively, D-TGCT is a non-malignant disease. This is the first study describing the natural course of D-TGCT and evaluating active surveillance as possible treatment strategy. METHODS: This retrospective, multicenter study included therapy naïve patients with D-TGCT from eight sarcoma centers worldwide between 2000 and 2019. Patients initially managed by active surveillance following their first consultation were eligible. Data regarding the radiological and clinical course and subsequent treatments were collected. RESULTS: Sixty-one patients with primary D-TGCT were initially managed by active surveillance. Fifty-nine patients had an MRI performed around first consultation: D-TGCT was located intra-articular in most patients (n = 56; 95 %) and extra-articular in 14 cases (24 %). At baseline, osteoarthritis was observed in 13 patients (22 %) on MRI. Most of the patients' reported symptoms: pain (n = 43; 70 %), swelling (n = 33; 54 %). Eight patients (13 %) were asymptomatic. Follow-up data were available for 58 patients; the median follow-up was 28 months. Twenty-one patients (36 %) had radiological progression after 21 months (median). Eight of 45 patients (18 %) without osteoarthritis at baseline developed osteoarthritis during follow-up. Thirty-seven patients (64 %) did not clinically deteriorate during follow-up. Finally, eighteen patients (31 %) required a subsequent treatment. CONCLUSION: Active surveillance can be considered adequate for selected therapy naïve D-TGCT patients. Although follow-up data was limited, almost two-thirds of the patients remained progression-free, and 69 % did not need treatment during the follow-up period. However, one-fifth of patients developed secondary osteoarthritis. Prospective studies on active surveillance are warranted.

Pigmented villonodular synovitis/giant cell tumor in the knee.

PURPOSE OF REVIEW: Pigmented villonodular synovitis (PVNS) is a rare diagnosis in pediatric patients and commonly presents with symptoms of swelling and pain. Early diagnosis is important to prevent secondary degeneration into the subchondral bone. This review will analyze the etiology, clinical signs/symptoms, diagnosis, treatment, and recent literature on PVNS in the pediatric population. RECENT FINDINGS: Many theories of PVNS etiology have been described in the literature; however, an inflammatory response has been most widely accepted. PVNS can occur in any joint, but most commonly in the knee. The most common treatment for PVNS is synovectomy, and long-term follow-up is necessary to detect disease persistence or recurrence. SUMMARY: Although uncommon, PVNS does occur in the pediatric population and this diagnosis should be included in the differential of atraumatic joint swelling and pain.

Locally Invasive Pigmented Villonodular Synovitis of the Wrist Treated by Arthroscopic Resection and Bone Grafting.

Pigmented villonodular synovitis (PVNS) is a benign but locally aggressive neoplasm that can affect tendon sheath, bursae, or joint. The wrist joint however is uncommonly involved and here we present a case of chronic monoarticular joint pain and swelling in a healthcare professional that was later histologically verified to be PVNS of the radiocarpal joint. The patient had a magnetic resonance imaging (MRI) performed prior to surgery which showed a locally invasive bony tumor of the scaphoid. He subsequently underwent a wrist arthroscopic evaluation and resection with bone grafting as the index surgery and made an uneventful postoperative recovery. This is a novel technique to address PVNS of the wrist as these cases are usually managed using open procedures which can lead to additional scarring and disrupt the blood supply of the joint capsule.

What Are the Recurrence Rates, Complications, and Functional Outcomes After Multiportal Arthroscopic Synovectomy for Patients With Knee Diffuse-type Tenosynovial Giant-cell Tumors?

BACKGROUND: Diffuse-type tenosynovial giant-cell tumor (D-TGCT), formerly known as pigmented villonodular synovitis, is a rare, locally aggressive, invasive soft tissue tumor that primarily occurs in the knee. Surgical excision is the main treatment option, but there is a high recurrence rate. Arthroscopic surgical techniques are emphasized because they are less traumatic and offer faster postoperative recovery, but detailed reports on arthroscopic techniques and outcomes of D-TGCT in large cohorts are still lacking. QUESTIONS/PURPOSES: (1) What is the recurrence rate of knee D-TGCT after multiportal arthroscopic synovectomy? (2) What are the complications, knee ROM, pain score, and patient-reported outcomes for patients, and do they differ between patients with and without recurrence? (3) What factors are associated with recurrence after arthroscopic treatment in patients with D-TGCT? METHODS: In this single-center, retrospective study conducted between January 2010 and April 2021, we treated 295 patients with knee D-TGCTs. We considered patients undergoing initial surgical treatment with multiportal arthroscopic synovectomy as potentially eligible. Based on that, 27% (81 of 295) of patients were excluded because of recurrence after synovectomy performed at another institution. Of the 214 patients who met the inclusion criteria, 17% (36 of 214) were lost to follow-up, leaving 83% (178 of 214) of patients in the analysis. Twenty-eight percent (50 of 178) of patients were men and 72% (128 of 178) were women, with a median (range) age of 36 years (7 to 69). The median follow-up duration was 80 months (26 to 149). All patients underwent multiportal (anterior and posterior approaches) arthroscopic synovectomy, and all surgical protocols were determined by discussion among four surgeons after preoperative MRI. A combined open posterior incision was used for patients with lesions that invaded or surrounded the blood vessels and nerves or invaded the muscle space extraarticularly. Standard postoperative adjuvant radiotherapy was recommended for all patients with D-TGCT who had extraarticular and posterior compartment invasion; for patients with only anterior compartment invasion, radiotherapy was recommended for severe cases as assessed by the surgeons and radiologists based on preoperative MRI and intraoperative descriptions. Postoperative recurrence at 5 years was calculated using a Kaplan-Meier survivorship estimator. The WOMAC score (0 to 96, with higher scores representing a worse outcome; minimum clinically important difference [MCID] 8.5), the Lysholm knee score (0 to 100, with higher scores being better knee function; MCID 25.4), the VAS for pain (0 to 10, with higher scores representing more pain; MCID 2.46), and knee ROM were used to evaluate functional outcomes. Because we did not have preoperative patient-reported outcomes scores, we present data on the proportion of patients who achieved the patient-acceptable symptom state (PASS) for each of those outcome metrics, which were 14.6 of 96 points on the WOMAC, 52.5 of 100 points on the Lysholm, and 2.32 of 10 points on the VAS. RESULTS: The symptomatic or radiographically documented recurrence at 5 years was 12% (95% confidence interval [CI] 7% to 17%) using the Kaplan-Meier estimator, with a mean recurrence time of 33 ± 19 months. Of these, three were asymptomatic recurrences found during regular MRI reviews, and the remaining 19 underwent repeat surgery. There was one intraoperative complication (vascular injury) with no effect on postoperative limb function and eight patients with postoperative joint stiffness, seven of whom improved with prolonged rehabilitation and one with manipulation under anesthesia. No postradiotherapy complications were found. The proportion of patients who achieved the preestablished PASS was 99% (176 of 178) for the VAS pain score, 97% (173 of 178) for the WOMAC score, and 100% (178 of 178) for the Lysholm score. A lower percentage of patients with recurrence achieved the PASS for WOMAC score than patients without recurrence (86% [19] versus 99% [154], OR 0.08 [95% CI 0.01 to 0.52]; p = 0.01), whereas no difference was found in the percentage of VAS score (95% [21] versus 99% [155], OR 0.14 [95% CI 0.01 to 2.25]; p = 0.23) or Lysholm score (100% [22] versus 100% [156], OR 1 [95% CI 1 to 1]; p = 0.99). Moreover, patients in the recurrence group showed worse knee flexion (median 135° [100° to 135°] versus median 135° [80° to 135°]; difference of medians 0°; p = 0.03), worse WOMAC score (median 3.5 [0 to 19] versus median 1 [0 to 29]; difference of medians 2.5; p = 0.01), and higher VAS pain score (median 1 [0 to 4] versus median 0 [0 to 4]; difference of medians 1; p < 0.01) than those in the nonrecurrence group, although no differences reached the MCID. No factors were associated with D-TGCT recurrence, including the use of postoperative radiotherapy, surgical technique, and invasion extent. CONCLUSION: This single-center, large-cohort retrospective study confirmed that multiportal arthroscopic surgery can be used to treat knee D-TGCTs with a low recurrence rate, few complications, and satisfactory postoperative outcomes. Surgeons should conduct a thorough preoperative evaluation, meticulous arthroscopic synovectomy, and regular postoperative follow-up when treating patients with D-TGCT to reduce postoperative recurrence. Because the available evidence does not appear to fully support the use of postoperative adjuvant radiotherapy in all patients with D-TGCTs and our study design is inadequate to resolve this controversial issue, future studies should look for more appropriate indications for radiotherapy, such as planning based on a more precise classification of lesion invasion. LEVEL OF EVIDENCE: Level III, therapeutic study.

Oncological and functional outcomes of modified arthroscopic resection for intra-articular tenosynovial giant cell tumor of the knee using multiple portals.

BACKGROUND: Arthroscopic resection of tenosynovial giant cell tumor (TS-GCT) presents favorable outcomes. However, there are reportedly higher recurrence rates in patients who had incomplete resection. To minimize incomplete resection, we established a multiple portal approach depending on the location of the disease. In this study, we aimed to retrospectively evaluate the clinical outcomes of arthroscopic resection for both localized and diffuse types of TS-GCT of the knee. METHODS: From 2009 to 2019, 13 patients who underwent arthroscopic synovectomy of the knee and were histologically diagnosed with TS-GCT were included in this study. The pre- and postoperative range of motion (ROM) of the knee was measured. The Japanese Orthopaedic Association (JOA) score and the Knee Injury and Osteoarthritis Outcome Score (KOOS) were assessed at the final follow-up examination. Magnetic resonance imaging was performed to detect incomplete resection or local recurrence. RESULTS: Among the 13 patients, seven and six had localized and diffuse type TS-GCT, respectively. Regarding the knee ROM, preoperative knee flexion in patients with the localized type was limited compared with that in those with the diffuse type. However, the ROM was significantly improved in patients with both types postoperatively. The JOA score and KOOS of patients with both types at the final follow-up were favorable, and there were no significant differences between both types. There was neither recurrence nor incomplete resection in any patient for both types. CONCLUSION: All patients, regardless of the TS-GCT type, achieved favorable outcomes after arthroscopic surgery; especially, the failure rate was 0%.

Bone SPECT/CT in Advanced Diffuse Tenosynovial Giant Cell Tumor of the Wrist.

ABSTRACT: Tenosynovial giant cell tumor, previously known as pigmented villonodular synovitis, is a benign low-grade fibrohistiocytic proliferation with hemosiderin deposits in synovial joints. Mostly affecting the knee, it can also manifest in other synovial joints, infrequently also in the wrist. Tenosynovial giant cell tumor typically causes intense radionuclide uptake in all phases in planar bone scintigraphy, making a differentiation from other bone tumors or osteomyelitis difficult, especially in cases associated with extensive bone destruction. We present a case of an unusually advanced and extended tenosynovial giant cell tumor of the wrist in bone scintigraphy, SPECT/CT, radiograph, and MRI.

Tenosynovial Giant Cell Tumor (TSGCT) of the hip: MRI accuracy and results of surgical treatment.

Tenosynovial Giant Cell Tumor (TSGCT) or formerly pigmented villonodular synovitis (PVNS) is a rare nonmalignant tumor of the synovia seldom affecting the hip. MRI and surgical resection are the gold standards in its diagnosis and treatment. However, the accuracy of MRI is unknown, and only few reports on its surgical treatment results exist. The goal of the study was to investigate the MRI accuracy, results after surgical treatment, and natural history of untreated MRI-diagnosed hip TSGCT. Twenty-four consecutive patients with suspected TSGCT on hip MRI, between December 2006 and January 2018, were identified from our medical database. Six refused to participate. About 18 patients with a minimal follow-up of 18 months were enrolled. Charts were reviewed for histopathology results, specific treatment and recurrence. At the last follow-up, all patients had a clinical (Harris Hip Score [HHS]) and radiological examination (x-ray and MRI). Out of 18 patients with suspected TSGCT on MRI, with a mean age of 35y (range 17-52), 14 had surgi- cal resection and 4 refused surgery 1 of whom had a CT-guided biopsy. Out of 15 cases with biopsies, in 10 TSGCT was confirmed. Three surgically-treated patients showed recurrence on MRI after 24, 31 and 43 months. Two non-treated patients showed progression after 18 and 116 months. At the last follow-up (65 m; range 18-159), the mean HHS with or without recurrence was 90 and 80pts (ns). Operative vs. non-operative treatment showed HHS of 86 and 90pts (ns). In the conservatively-treated group, HHS with and without progression was 98 and 82pts (ns), respectively. MRI-suspected TSGCT of the hip was confirmed with biopsy in two-thirds of the cases. Surgical treatment showed recurrence in more than one-third of the patients. Two out of four untreated patients showed progression of the TSGCT-suspected lesion.

Arthroscopic Excision of Pigmented Villonodular Synovitis of the Trochanteric Bursa.

Trochanteric bursitis is a common disorder affecting middle-aged adults and usually presents with lateral-based hip pain and swelling. It usually responds to conservative measures, including adductor stretching, abductor strengthening, and select injections of corticosteroid or platelet-rich plasma. For refractory cases, excision, open or arthroscopic, is usually recommended. We observed a 55-year-old woman who had lateral hip pain and longstanding swelling consistent with refractory trochanteric bursitis. Her persistent symptoms, coupled with atypical findings on imaging, prompted an arthroscopic evaluation. Arthroscopic examination of the peritrochanteric space revealed a fulminant bursal inflammation that pierced through the iliotibial band. The bursal inflammation was excised arthroscopically and biopsy of the tissue revealed a diagnosis of pigmented villonodular synovitis (PVNS). The patient had an uneventful recovery and had a full resolution of symptoms with no recurrence noted at 3-year follow-up. This is the first reported case of arthroscopic excision of PVNS of the trochanteric bursa. Given that it may mimic trochanteric bursitis, it is important for clinicians to be aware of the possibility of this progressive condition for appropriate clinical intervention. [Orthopedics. 2023;46(6):e381-e383.].

Total Hip Arthroplasty or Arthroscopy for Pigmented Villonodular Synovitis of the Hip: A Retrospective Study with 3-Year Follow-Up at Minimum.

OBJECTIVE: Pigment Villonodular synovitis of the hip, a rare pain proliferation of the synovium, was treated successfully with total hip arthroplasty and arthroscopy. Most recent results come from small case series with no study comparing arthroscopy and arthroplasty. In this study, we aimed to show and compare the clinical outcomes of arthroscopy and total hip arthroplasty (THA) in pigment Villonodular synovitis of the hip. METHODS: This was a retrospective clinical trial with data from patients with pigment Villonodular synovitis of the hip between 2010 and 2019. The study included 17 patients in the THA group, and 20 patients in the arthroscopy group. The clinical outcomes were evaluated at 3, 6, and 12 months, at 1 and 2 years, and every 5 years afterward. The clinical efficacy was measured using the Harris hip scores (HHSs) and visual analogue scale (VAS) score. RESULTS: The mean HHS improved from 45.24 ± 10.36 to 78.94 ± 19.11 in the THA group (t = -6.394, P = 0.000) and 45.30 ± 11.08 to 71.60 ± 19.78 (t = -5.187, P = 0.000) in the arthroscopy group from pre-operation to the final follow-up. There is no significant difference between the two groups (t = 1.051, P = 0.301). The mean VAS improved from 3.65 ± 0.79 to 0.35 ± 0.70 (t = 12.890, P = 0.000) in the THA group and 4.05 ± 0.94 to 1.35 ± 1.79 (t = 5.979, P = 0.001) in the arthroscopy group postoperatively. There is no significant difference between the two groups (t = 1.329, P = 0.193). Recurrence of PVNS was diagnosed in four patients (20%) of the arthroscopy group and they underwent THA after arthroscopy, and the mean interval was 44.25 ± 6.98 months. All patients reached level 5 muscle strength by the final follow-up. All the patients' buckling ranges were over 105 degrees. Their internal and external hip rotation was over 15 degrees. Their hip adduction was over 20 degrees, and abduction over 30 degrees. CONCLUSION: Both THA and arthroscopy in the setting of PVNS can improve patients' function and lead to a low rate of local recurrence. By selecting patients well for each approach, one can expect a reasonable result.

Management of tenosynovial giant cell tumour of the temporomandibular joint: a systematic review.

Diffuse type tenosynovial giant cell tumour of the temporomandibular joint (D-TGCT-TMJ) is a rare proliferative disorder. The aim of this study was to perform a systematic review of the literature to summarize D-TGCT-TMJ management regimes and recurrence rates with at least 12 months of follow-up. Our secondary aim was to propose a minimum period of post-operative follow-up. A medline search for any D-TGCT-TMJ case detailing treatment, follow-up of at least 12 months, and presence of recurrence was undertaken. The following variables were extracted from the studies: patient's age and sex, presence of middle cranial fossa invasion, treatment undertaken, total length of follow-up, and presence of recurrence. All studies were assessed for bias as per the Joanna Briggs Institute systematic reviews appraisal tool. There were 63 cases reviewed and were predominantly managed with total resection (60.3%). Other modalities included: arthroplasty, subtotal resection with or without postoperative radiotherapy, medical therapy and surveillance. The recurrence rate was 9.52% and the longest follow-up period where recurrence was observed was at 60 months. Total resection and arthroplasty are common D-TGCT-TMJ management regimes. Patients with D-TGCT-TMJ should be followed up annually for at least 5 years postoperatively to assess for recurrence.

Rare Presentation of Giant Cell Tumors of the Tendon Sheath: Bilateral Involvement of Tendoachilles.

Giant cell tumor of tendon sheath arises from the synovium of tendon sheaths, joints, or bursa, mostly affects adults between 30 and 50 years of age, and is slightly more common in females. It corresponds to a localized form of pigmented villonodular synovitis (PVNS). Typically occur in the hand where they represent the second most common type of soft tissue tumors after synovial ganglions. Bilateral giant cell tumor of tendon sheath of tendoachilles is a rare presentation. We report the case of a 22-years-old female presenting with pain in both ankles without any history of trauma. On clinical examination, tenderness on both tendoachilles and local indurations were observed. Ultrasonography showed focal thickening of Achilles tendon bilaterally, and Doppler demonstrated increased flow in peritendinous area. MRI findings showed that most of the tumor had intermediate signal intensity and portions of the tumor had low signal intensity. Fine needle aspiration cytology confirmed the diagnosis of giant cell tumor of tendon sheath. Excision biopsy was done with no recurrence on subsequent follow-up.

Image findings of tendon sheaths affected by diffuse tenosynovial giant cell tumors of the skull base.

OBJECTIVE: This study investigated radiographic images and the differential diagnosis of intracranial diffuse tenosynovial giant cell tumor (D-TGCT) in order to better understand the disease and improve the rate of preoperative diagnosis. PATIENTS AND METHODS: Images and clinical data of patients with D-TGCT were retrospectively analyzed. Routine Computer Tomography (CT), routine Magnetic Resonance Imaging (MRI), and contrast-enhanced MRI were performed for nine cases. Susceptibility-weighted imaging (SWI) was also performed for one case. RESULTS: We reviewed nine patients (6 males and 3 females) aged between 24 and 64 years, with a mean age of 47.33 ± 14.92 years. The most frequent complaints were hearing loss (5/9, 55.6%), pain (4/9, 44%), masticatory symptoms (2/9, 22.2%), and mass (4/9, 44.4%), with a mean duration of 22 ± 21.43 months. All cases were centered on the base of the skull, and showed hyper-density soft-tissue mass with osteolytic bone destruction on CT. The tumor signal mainly showed iso-intensity or hypo-intensity on T1WI compared with that in the brain parenchyma in all patients. On T2WI, nine lesions mainly showed hypo-intensity. Among these nine lesions, three displayed cystic region showing hyper-intensity on T2WI and hypo-intensity on T1WI (Figure 2A, 2B) in the lesion. Nine lesions showed hypo-intensity on DWI sequences. SWI images presented low signal in two cases, showing the "flowering effect". Nine patients showed heterogeneous enhancement, and two patients had meningeal thickening. CONCLUSIONS: Intracranial D-TGCT is extremely rare, but must be differentiated from other tumors. Osteolytic bone destruction in the area of the skull base with hyper-density soft-tissue mass and hypo-intensity on T2WI images are indicative of D-TGCT.

Transglenoidal Approach in Pigmented Villonodular Synovitis of the Temporomandibular Joint.

Pigmented villonodular synovitis is a benign pathology with locally aggressive behavior caused by an uncontrolled proliferation of the articular synovial membranes. Here the authors present a case of pigmented villonodular synovitis of the temporomandibular joint with middle cranial fossa extension and review the different management options including surgery, which have been proposed to target this condition in the recent literature.