Pigmented Villonodular Synovitis Research Articles

Surgical management of 144 diffuse-type TGCT patients in a single institution: A 20-year cohort study.

Surgery is the mainstay of treatment for tenosynovial giant cell tumors (TGCTs). However, achieving a cure through surgery alone remains challenging, especially for the diffuse-type (D-TGCT). Our goal was to describe the surgical management of patients with D-TGCT related to large joints, treated between 2000 and 2020. We analyzed the effect of (in)complete resections and the presence of postoperative tumor (POT) on magnetic resonance imaging(MRI) on radiological and clinical outcomes. A total of 144 patients underwent open surgery for D-TGCT, of which 58 (40%) had treatment before. The median follow-up was 65 months. One hundred twenty-five patients underwent isolated open surgeries, in which 25 (20%) patients' D-TGCT was intentionally removed incompletely. POT presence on the first postoperative MRI was observed in 64%. Both incomplete resections and POT presence were associated with higher rates of radiological progression (73% vs. 44%; Kaplan-Meier[KM]analysis p = 0.021) and 59% versus 7%; KManalysis p < 0.001), respectively. Furthermore, patients with POT presence clinically worsened more often than patients without having POT (49% vs. 24%; KManalysis p = 0.003). D-TGCT is often resected incompletely and tumor presence is commonly observed on the first postoperative MRI, resulting in worse radiological and clinical outcomes. Therefore, surgeons should try to remove D-TGCT in toto and consider other multimodal therapeutic strategies.

Pigmented villonodular synovitis: literature review and case report

Pigmented villonodular synovitis (PVNS) is a rare disease, its diagnosis has certain difficulties. This is due to the absence of characteristic etiological factors and clinical manifestations of PVNS, as well as the insufficient level of knowledge among doctors. The article presents a review of the literature on the diagnosis and treatment of PVNS, as well as a clinical case, which peculiarity is the late diagnosis of this disease, despite the presence of its certain clinical and morphological manifestations.

A Case of Pigmented Villonodular Synovitis

Tenosynovial giant cell tumors (TGCT) are a rare group of generally non-malignant tumors that involves the synovium and tendon sheath. A young female patient presented to the outpatient department with a complaint of unilateral knee swelling and pain. She was evaluated as such and based on a provisional diagnosis of benign synovial proliferation, she was treated with an arthroscopic resection. We discuss our case and discuss the possible medical therapies to prevent recurrence.

Contribution of the Ultrasound Techniques in the Evaluation of Knee Joint Damage in the Case of Pigmented Villonodular Synovitis and Rheumatoid Arthritis.

This is a prospective study to evaluate the clinical value of high-frequency ultrasound (HFUS), superb microvascular imaging (SMI), and contrast-enhanced ultrasound (CEUS) in differentiation of pigmented villonodular synovitis (PVNS) and highly active rheumatoid arthritis (RA). Twenty PVNS patients and 24 active RA patients were selected to undergo HFUS, SMI, and CEUS examinations. The characteristics of HFUS, SMI, and CEUS in PVNS and RA were compared, and the differential diagnosis performances of HFUS, SMI, and CEUS in PVNS and RA were evaluated by receiver operating characteristic (ROC) analysis. There were statistically significant in joint effusion, synovial thickness, synovial morphology, synovial echo, synovial vessel shape, synovial enhanced direction, and enhanced pattern between PVNS and RA (P < .05). However, no statistically significant were found in bone erosion, synovial boundary, blood signal grading of synovium, synovial enhanced strength, and CEUS quantitative parameters (including PI, TTP, S, MTT, and AUC) (P > .05). The AUC of HFUS, SMI, and CEUS for differential diagnosis PVNS and RA were 0.832, 0.675, and 0.817, respectively. The AUC of HFUS + SMI, HFUS + CEUS, SMI + CEUS, HFUS + SMI + CEUS were 0.923, 0.940, 0.817, and 0.940, respectively. The AUC of HFUS + SMI and HFUS + CEUS was higher than that of each alone (P < .05). HFUS, SMI, and CEUS can be used as supplementary methods for diagnosis and differential diagnosis in PVNS and active RA. What is more, the combination of HFUS + SMI and HFUS + CEUS was suggested.

Randomized, placebo-controlled, double blind, phase II study of zaltoprofen for patients with diffuse-type and unresectable localized tenosynovial giant cell tumors.

11568 Background:A tenosynovial giant cell tumor (TGCT) is a locally aggressive benign neoplasm arising from intra- or extra-articular tissue, and categorized localized (L-TGCT) or diffuse-type (D-TGCT). D-TGCT most commonly develops in the knee or ankle. We revealed that zaltoprofen, a nonsteroidal anti-inflammatory drug possessing the ability to activate peroxisome proliferator-activated receptor gamma (PPARγ), can inhibit the proliferation of TGCT stromal cells via PPARγ. PPARγ is a ligand-activated transcription factor that belongs to the nuclear hormone receptor superfamily. Therefore, we conducted a randomized trial to evaluate whether zaltoprofen was effective for patients with TGCT. Methods:This study was a randomized, placebo-controlled, double-blind, multicenter trial to evaluate the safety and efficacy of zaltoprofen for patients with L-TGCT or D-TGCT. For the treatment group, zaltoprofen 480 mg/day was administered for 48 weeks. The primary outcome was the progression-free rate (PFR) at week 48 after treatment administration. Disease progression was defined as follows requiring surgical interventions: 1) repetitive joint swelling due to hemorrhage; 2) limited range of joint motion; 3) invasion of adjacent cartilage or bone; 4) severe joint space narrowing; or 5) increase in tumor size (target lesion). This study was registered at the University Hospital Medical Information Network (UMIN) Clinical Trials Registry (UMIN000025901). Results:We allocated forty-one patients to zaltoprofen (n = 21; Z) or placebo (n = 20; P) groups. The mean age was 44 years (range, 26 to 66) in the group Z and 47 years (range, 24 to 69) in the group P. TGCTs located at a knee in 26 patients and at an ankle in 15 patients. The primary outcome of PFR for the group Z was not higher than that for the group P at week 48 (84.0% vs. 90.0%; p = 0.619). The objective tumor response was graded as partial response (PR) in 1 patient, stable disease (SD) in 19, and progressive disease (PD) in 1 for the group Z, and SD in 17 and PD in 3 for the group P. The mean musculoskeletal tumor society scoring system for group Z was significantly improved from baseline to week 48 (83.8% vs. 93.0%, p = 0.02), whereas that for the group P was not significantly improved (82.2% vs. 86.8%; p = 0.167). During the study period, one severe adverse event was observed (grade 3 hypertension) in the group Z. Conclusions:To our knowledge, this was the first study to evaluate the efficacy of zaltoprofen in patients with TGCT. The TGCT patients in zaltoprofen group did not have higher PER compared with those in the placebo group at week 48. Zaltoprofen appeared to improve the limb-function. Both groups presented with a stable disease course for 48 weeks, therefore, the long-term clinical course of TGCT should be clarified. Further analysis with long-term administration of zaltoprofen is considered for the future study. Clinical trial information: UMIN000025901.

MOTION: A randomized, phase 3, placebo-controlled, double-blind study of vimseltinib (DCC-3014) for the treatment of tenosynovial giant cell tumor.

TPS11590 Background: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive neoplasm that occurs in the synovium of joints, bursae, or tendon sheaths. TGCT is caused by upregulation of the colony-stimulating factor 1 (CSF1) gene, resulting in aberrant CSF1 expression and the recruitment of CSF1 receptor (R)-dependent inflammatory cells. Resection is the primary treatment, but nonsurgical treatment options are necessary for patients with symptomatic TGCT not amenable to surgical resection. Vimseltinib is an oral switch control TKI specifically designed to selectively and potently inhibit CSF1R. In a Phase 1/2 study in patients with TGCT, vimseltinib showed encouraging antitumor activity with an overall objective response rate (ORR) of 42% in the cohort receiving 30 mg twice weekly (recommended phase 2 dose; Gelderblom et al, ESMO 2021 Poster). Vimseltinib was also well tolerated, and the majority of the common (≥15%) treatment-emergent adverse events (TEAEs) were Grades 1–2. Among these common TEAEs, the only Grade 3–4 event in the Phase 2, twice-weekly, 30-mg cohort was increased blood creatine phosphokinase (CPK); however, this elevated CPK was not associated with any symptoms (Gelderblom et al, ESMO 2021 Poster). Phase 1/2 efficacy and safety data support further development of vimseltinib; here, we describe the ongoing Phase 3 study for patients with TGCT not amenable to surgical resection. Methods: MOTION (NCT05059262) is a Phase 3, randomized, placebo-controlled, double-blind study that aims to evaluate the efficacy and safety of vimseltinib for the treatment of TGCT not amenable to surgical resection. Participants must be at least 18 years of age and have histologically confirmed and symptomatic TGCT for which surgical resection will potentially cause worsening functional limitation or severe morbidity. Prior CSF1R therapy is not permitted (previous imatinib and nilotinib is allowed). In Part 1 of the study, eligible participants will be randomized 2:1 to receive either vimseltinib 30 mg twice a week or matched placebo for 24 weeks. The primary outcome measure is ORR assessed by central read using Response Evaluation Criteria in Solid Tumors version 1.1 at 25 weeks. Secondary outcome measures include ORR per tumor volume score, range of motion, and patient-reported outcomes. Participants assigned to placebo in Part 1 will have the option to receive vimseltinib in Part 2, a long-term treatment phase in which participants will receive open-label vimseltinib. This international study plans to randomize 120 participants and is currently enrolling. Clinical trial information: NCT05059262.

Surufatinib in U.S. patients with soft tissue sarcoma.

11557 Background: Surufatinib is an inhibitor of VEGFR1, 2, &amp; 3, FGFR1, and CSF-1R. A manageable safety profile, and statistically significant improvement in progression-free survival (PFS) in patients (pts) treated with surufatinib have previously been demonstrated in pts with advanced NETs of extrapancreatic (epNET) and pancreatic (pNET) origin in 2 phase 3 randomized trials conducted in China (SANET-ep; NCT02588170 &amp; SANET-p; NCT02589821). Similar safety and efficacy were demonstrated in this phase 1 study (NCT02549937) in pts with NETs. Here we report the results of surufatinib in pts with soft tissue sarcoma. Methods: This study is a phase 1, dose escalation (ESC)/expansion (EXP) study to evaluate the safety and efficacy of surufatinib in the US and Europe (EXP only). ESC previously reported the recommended phase 2 dose as 300mg once daily. Enrollment into advanced, adult, soft tissue sarcoma EXP cohorts is ongoing. The primary endpoint is PFS rate at 4 months (mo). Soft tissue sarcoma histological subtypes include: angiosarcoma (AS), epithelioid sarcoma (ES), leiomyosarcoma (LMS), pigmented villonodular synovitis (PVNS), synovial sarcoma (SS) and undifferentiated pleomorphic sarcoma (UPS). Results: 32 adults with soft tissue sarcoma were enrolled (2 each AS and ES; 10 LMS; 1 PVNS; 9 SS; 8 UPS). The median age across all subtypes was 56.5 years (range 26-77), and the majority of pts were female (68.8%). 59.3% of pts received ≥ 3 prior lines of therapy (Tx) (median lines of Tx: 3 [range 1-6]). As of 15 Nov 2021, 1 pt (PVNS) remained on Tx. The PFS rate at 4 mo was 17.5%. The median PFS was 2.56 mo (95% CI; 0.92-2.92). The median duration on treatment (mDoT) for all pts was 11.2 weeks (wks) (0.4-39.0). The mDoT for LMS pts was 11.6 wks (3.1-36.0), mDoT for SS pts was 11.9 wks (0.4-27.9), and mDoT for UPS pts was 8.2 wks (2.7-27.9). Of 29 pts with ≥ 1 post baseline assessment, 6 pts (20.7%) achieved stable disease of ≥ 8.0 wks from start of Tx. No pts achieved a partial or complete response. The safety profile of surufatinib remains consistent with previously completed trials. All pts (n = 32) reported ≥ 1 adverse event (AE), and 21 pts (65.6%) reported AEs ≥ grade 3. The most common AEs of any grade were fatigue (53.1%), hypertension (43.8%), diarrhea (40.6%), anemia (25.0%), blood bilirubin increase (25.0%), headache (21.9%), and proteinuria (21.9%). The most commonly reported AEs ≥ grade 3 in &gt; 1 pt were hypertension (21.9%), fatigue (12.5%), and anemia (9.4%). AEs leading to Tx discontinuation occurred in 9.4% of pts. Conclusions: Surufatinib demonstrated minimal antitumor activity as a single agent in heavily pretreated pts across various types of soft tissue sarcoma. The safety profile in pts with soft tissue sarcoma remains consistent with previously reported and ongoing studies with surufatinib. Clinical trials are ongoing with surufatinib globally, with active recruitment in PD-L1 combination studies. Clinical trial information: NCT02549937.

Unusual Uptake of [131I] in a Tenosynovial Giant Cell Tumour Relapse in a Patient with Differentiated Thyroid Cancer.

A 77-year-old woman with follicular thyroid cancer underwent total thyroidectomy and subsequent Iodine-131 remnant ablation. She had a history of a wide tenosynovial giant cell tumor (TGCT) of the right wrist and hand that had been resected thirteen years ago. Post-therapeutic scintigraphy and single photon emission computed tomography showed mild uptake on the distal right forearm, wrist and hand. Magnetic resonance imaging and posterior histopathology confirmed a relapse of TGCT. No radioiodine adverse effects were reported after a one-year follow-up. As far as we know, this report is the first in the literature to a TGCT visualized on post-therapy radioiodine scan.

The future of radiosynoviorthesis: bright or bleak?

Radiosynoviorthesis (RSO) is a decades known, effective intra-articular nuclear medicine local therapy, with few rare side-effects, in which inflamed synovial membrane is treated by means of colloidal beta-emitters. There are major variations worldwide in terms of acceptance, frequency of use and approved indications for this procedure. Thus, reliable figures that reflect reality are only available for a few countries. A Europe-wide survey revealed that RSO is carried out most frequently in Germany, where RSO is the most common nuclear medicine therapy with about 70,000 joints treated per year. The main indications include synovitis due to rheumatoid arthritis, hemophilia and pigmented villonodular synovitis (PVNS), and depending on national approvals, osteoarthritis. Despite the many indications, there are very few published scientific studies and therefore, RSO evidence is lacking. Reliable data on the clinical usage of RSO and demographics of RSO specialists are only available in Germany, thus we discuss the future challenges of RSO mainly from a German perspective. In the German healthcare system, RSO is performed primarily on an outpatient basis and plays only a minor role in the university setting. The necessary expertise for RSO is therefore lacking, for the most part, at university training centers. Currently, nearly more than three quarters of the German RSO experts are over fifty years old, illustrating a shortage of young talent. In the future, RSO providers from the non-university or private sector will have to cooperate with universities through networks and will have to intensify their cooperation with referring physicians, such as rheumatologist and orthopedic surgeons, and patients in order to maintain a timely and beneficial exchange of information. In networks of RSO experts, the participants must jointly develop and establish training concepts and facilities for future talents, elaborate on guidelines, if clinically useful expand the range of indications, initiate studies to generate further evidence and finally make the procedure more public. In addition, it is worthwhile to apply this process beyond human medicine to other fields, such as medical physics and veterinary medicine. If these points are implemented, the future of RSO will be bright, if it fails, it looks bleak.

Phase 1 study of C019199, an oral CSF-1R/DDRs/VEGFR2 multiple kinase inhibitor, to assess the safety, tolerability, pharmacokinetics, and pharmacodynamics in patients with advanced solid tumors, including tenosynovial giant cell tumor.

TPS3177 Background: C019199 was designed to be a tumor immune microenvironment (TME) modulator in order to improve the efficacy against tumor growth when combined with immune checkpoint inhibitors, like anti-PD1 or anti-PD-L1. It was shown to have a specific inhibition profile against CSF-1R, DDR1 and VEGFR2 with IC50 of 14nM, 40nM and 79nM, respectively. CSF-1R’s inhibition may deplete Tumor-associated macrophages (TAMs) in TME, help the infiltration of T cells and enhance T cell responses. But a single modulating mechanism may not be strong enough or may be vulnerable and easily overcome by tumors. C019199 can reshape the TME by additionally inhibiting DDRs and VEGFR2, which may remove the "physical barrier" of tumor extracellular matrix and further increase T cell infiltration on top of inducing tumor blood vessels normalization. As a single agent with the appropriate combination of multiple modulation effects, it will potentially turn "cold" tumors into "hot" tumors. Preclinical studies have shown that C019199 inhibits tumor growth in multiple animal tumor models and has better antitumor efficacy when combined with immune checkpoint blockades. Methods: A first-in-human, open label, multicenter, dose-escalation/expansion study of C019199 is currently enrolling. Eligible subjects (age ≥ 18 years and &lt;76 years) with histologically or cytologically confirmed relapsed, refractory, or progressive metastatic solid tumors will be enrolled in. In Part A, the safety and tolerability of C019199 will be assessed in about 25 subjects to identify the maximum tolerated dose and recommended phase Ⅱ dose (PR2D). In Part B, the safety and antitumor activity of the RP2D will be assessed in about 60 subjects in disease-specific expansion cohorts. Primary endpoints are adverse events, laboratory abnormalities, dose-limiting toxicities. Secondary endpoints will include pharmacokinetics, objective response rate, progression-free survival, and disease control rate. Exploratory biomarker analyses include CSF-1 and VEGF. Clinical trial information: CTR20202045.

Unusual Presentation and Surgical Treatment of a Phosphaturic Mesenchymal Tumor in a Knee.

A 30-year-old woman presented to our hospital with an 11-year history of gradually enlarging masses around the left knee and 2-year history of progressively worsening bone pain. Tumor-induced osteomalacia (TIO), a rare paraneoplastic syndrome caused by phosphaturic mesenchymal tumors (PMTs) was suspected, but the postoperative pathology of her two operations was both reported as tenosynovial giant cell tumor (TGCT), making its diagnosis confusing. The possibility of hypophosphatemia, insufficient blood supply, innervation of the left lower limbs, as well as the unclear pathology, make it unreasonable to perform tumor-type knee prosthesis replacement directly. Finally, we placed static polymethylmethacrylate (PMMA) spacer at first, then when the concentration of blood phosphorus level rose to the normal range, the pathology was confirmed to be TIO, the blood supply and innervation was satisfying, tumor-type knee prosthesis replacement was performed. She was discharged post operative day 15 after the prothesis implantation without incident. One and a half years after her surgery, the concentration of blood phosphorus was still in the normal range, the symptom of systemic bone pain had improved significantly, the prosthesis was still in a good position and no recurrence was caught.

Midterm outcomes of 18 patients with primary intra-articular diffuse tenosynovial giant cell tumor (TGCT) of the knee treated with complete arthroscopic synovectomy and postoperative low-dose radiotherapy at a mean follow-up of 68months.

To evaluate the long-term clinical outcome of the treatment of complete arthroscopic synovectomy combined with low-dose external radiotherapy in the knee affected by primary intra-articular diffuse tenosynovial giant cell tumor (TGCT). From May 2009 to January 2016, 18 patients with intra-articular diffuse TGCT underwent complete arthroscopic synovectomy and low-dose external-beam radiotherapy in Zhongnan Hospital were enrolled in this retrospective study. The preoperative symptoms of patients, the complications during or after the arthroscopic procedure and the recurrence were collected and recorded. Each patient was evaluated before treatment and at the follow-up visit. Efficacy evaluation criteria were based on Ogilvie-Harris score, and the evaluation of the functional ability of knee was based on the International Knee Documentation Committee (IKDC) Score. The mean time from symptom onset to surgery was 9.1 ± 5.8months (range from 3 to 29months). The most frequent preoperative symptoms were joint effusion (100%), diffuse non-specific knee pain (66.7%), and a decreased range of motion (83.3%). Mean follow-up time was 68.0 ± 18.1months (range from 35 to 120months). The mean evaluation score according to the Ogilvie-Harris criteria before treatment was 3.19 ± 0.74, which corresponded to a rating of "poor". The mean score after treatment was 8.79 ± 1.57, rated as "good". The Ogilvie-Harris score was significantly increased after treatment (P = 0.003). The IKDC score of all patients increased significantly from 37.1 ± 3.8 before treatment to 83.9 ± 11.3 after treatment. The IKDC score was obviously increased after treatment (P = 0.001). No recurrence was noted at final follow-up. There were no complications during or after the arthroscopic procedure. The outcome of this study proved that complete arthroscopic synovectomy combined with low-dose external radiotherapy was appropriate for treating primary intra-articular diffuse TGCT of the knee joint. It could be safely and reliably used with minimal complications, fast postoperative recovery and satisfactory control of recurrence rates.

Пигментный ворсинчато-узловой синовит субарокмиального пространства (случай из клинической практики)

Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disorder affecting synovial membranes, bursae, tendons, skin and bone. Materials The article presents a rare clinical case treated for PVNS of the subacromial space. The patient complained of pain in the shoulder joint at the largest possible points, and a deformity developed 7 months prior to the visit could be visualized at the anterior aspect of the shoulder joint. The patient denied any history of trauma. Arthroscopy of the shoulder joint was performed to rule out the intra-articular nature of the lesion with an exploratory puncture performed to establish the diagnosis with instrumental methods. Delto-pectoral approach was used to remove the neoplasm that was histologically examined to confirm the diagnosis. The upper limb was postoperatively immobilized with a Gilchrist-bandage for 2 weeks. The sutures were removed 2 weeks after the operation. Result The patient underwent a course of rehabilitation to restore the function of the upper limb. The upper limb function was preoperatively and postoperatively evaluated with the ASES questionnaire. The patient showed the shoulder function completely regained at two years with ASES scored 100. MRI scan of the shoulder joint showed no signs of recurrence. Extra-articular location of PVNS is very rare and is described in few case reports. Open procedure or arthroscopic removal of the affected synovial membrane are used to treat the condition. Conclusion Removal of the affected synovial membrane allowed us to obtain a good clinical result.

Divergent Signaling Pathways May Lead to Convergence in Cancer Therapy - A Review.

Cancer is a chaos of uncontrolled cell proliferation that has consistently invented new circuitry programs to operate inside the cell machinery. Globally, cancer statistics account for 65% of mortality worldwide, mainly due to the adoption of lifestyle behaviours. In 2020, FDA approved 40 new drugs, out of which 16 (40%) were approved as cancer drugs. Overall, the risk of dying from cancer decreased, but further reductions in cancer death rates can be accelerated by applying existing cancer control knowledge across all the population segments, emphasising those in the lowest socio-economic and other disadvantaged population. Various therapeutic regimes, including low-molecular-weight inhibitors, targeting oncogenic signaling pathways are under development. However, the pitfall of targeted therapies is the quick emergence of acquired drug resistance encumbered with toxic side effects. Several FDA acclaimed therapeutic legacies or biosimilars earmarked signaling pathways of rare diseases (cystic fibrosis, erythropoietic protoporphyria, neuromyelitis optica spectrum disorder, tenosynovial giant cell tumor, sickle cell disease, systemic sclerosis-associated interstitial lung disease, muscular dystrophy), neurological and psychiatric disorders, infectious diseases, heart, lung, circulatory, endocrine diseases, autoimmune conditions, cancers and blood disorders. When cancer progresses, these signals develop specific characteristics that can be targeted for anti-cancer therapy. The designer inhibitors have emerged as novel pharmaceutical interventions that aim to block the pathways in an effort to reverse the abnormal phenotype of the cancer cells. Numerous cell-signaling channels have evolved and invigorated to make off three-dimensional feedback networks. The magnitude of accessible information by pathways occupies curated information as a consortium. To fully appreciate the pivotal roles that signaling cascades play in tumor development, it is necessary to understand the involved signaling cascades in the interaction between cancer cells. The prime endeavour is to canonically curate all signaling pathways involving cell cycle, EGFR, MAPK, GPCR, PI3K/ AKT/mTOR, immune checkpoints, nuclear receptors, janus kinase, transcription activators etc., involving the manipulation of genetic and nuclear receptors. Here, we will summarize the vast amount of information describing the signals that mediate crosstalk between cancer cells and the targets related to this crosstalk.

The relationship of preoperative hematologic parameters to invasion of anatomic structures and recurrence in tenosynovial giant-cell tumor of the digits

Although the pathogenesis of Teno-Synovial Giant Cell Tumor (TSGCT) is not known, inflammation is thought to play a role in the etiology beside some other factors. Many researchers have found a close relationship between hematological parameters such as Mean Platelet Volume (MPV), Platelet/MPV Ratio (Plt/MPV), Monocyte/Neutrophil Ratio (MNR), Neutrophil/Lymphocyte Ratio (NLR), Platelet/Lymphocyte Ratio (PLR) and certain inflammatory, autoimmune and neoplastic diseases. The aim of the present study was to assess the relationship of hematological parameters (NLR, MNR and Plt/MPV) to tumor recurrence and spread after TSGCT surgery. Data from 102 patients who matched the inclusion criteria comprised preoperative hematological parameters, tumor size, anatomic region, side, presence of bone invasion, proximity to joints or neurovascular structures, and postoperative recurrence and complications. The mean follow-up was 54.2 months (±26.5). Recurrence was observed in 12 (11.8%) of the 102 cases. No significant correlation was found between recurrence and hematologic parameter, age, laterality (right-left) or zone (volar-dorsal). There were no significant differences in NLR, Plt/MPV or MNR between patients with and without bone, capsular, nerve or total involvement. The study thus showed that the various inflammatory parameters (NLR, Plt/MPV and MNR) calculated from blood count were not predictive of tumor invasion into anatomical structures or of digital TSGCT recurrence.

Anterior Capsule Reconstruction in the Setting of PVNS.

Pigmented villonodular synovitis (PVNS) is a benign proliferative disease affecting tendon sheaths and synovial tissue. Pigmented villonodular synovitis in the foot and ankle has a high rate of recurrence, which can be destructive if incompletely removed. This case series analyzes functional outcomes after PVNS operative resection with an anterior ankle capsular reconstruction, using a novel technique. Three patients with PVNS underwent surgery between 2010 and 2020. The operative technique involved a posterior, midline approach for PVNS resection of the affected ankle joint, followed by a standard anterior approach for capsular excision. Subsequent anterior capsular reconstruction was performed with a regenerative tissue matrix and a bioresorbable anchoring system. Preoperative and postoperative range of motion for the ankle and subtalar joints, visual analog scale (VAS) for pain, and return to daily activities was assessed along with appropriate radiographs and magnetic resonance imaging (MRI) imaging. All 3 surgeries had a mean follow-up period of 52.3 (range, 4-123) months and resulted in successful recovery as assessed by the VAS and self-reported activity. Preoperative ankle dorsiflexion and plantarflexion along with subtalar inversion and eversion range of motion were all normal. Postoperative ankle motion and subtalar motion were not statistically different. No complications or recurrence of PVNS was observed. Further investigation is warranted to better understand the clinical outcomes of this technique designed to successfully eliminate PVNS recurrence. IV- Retrospective case series.

Pigmented villonodular synovitis of the temporomandibular joint with skull base extension: a retrospective case series

Most studies on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) with skull base extension mostly are case report. Here, we summarize the clinical features, treatments, and outcomes of PVNS of the TMJ with skull base extension in a large case series. We reviewed the clinical information relating to patients diagnosed with PVNS of the TMJ with skull base extension information of patients in our center between 2011 and 2020. We reviewed 10 patients (4 males and 6 females). All cases had presented with a unilateral lesion extending the middle skull base. PVNS of the TMJ with skull base extension occurred on the left side in 6 patients (60%) and on the right side in 4 patients (40%). Of the 10 patients, pain and mass were the most prevalent symptoms. All patients received surgery and no recurrence was seen after 35.90 ± 25.35 months follow-up. Despite destructive biological behavior, surgery can achieve an excellent outcome for patients with PVNS of the TMJ with skull base extension. An en bloc resection may prevent recurrence and provide long-term relief. Radiotherapy may be reserved for subtotal excision and recurrent lesions but require further investigation.

Dumbbell-type tenosynovial giant cell tumor of the buttocks: a report of two cases

BackgroundTenosynovial giant cell tumor (TSGCT) is a benign tumor derived from the synovium of the joints, bursa, and tendon sheaths, which is mainly located around the tendon sheath of hand and foot. Extra-articular TSGCT are relatively rare and are mainly found in the soft tissue around the large joint. They are rarer when located purely intramuscular or subcutaneous, and mostly in the lower extremities.Case DescriptionWe report two rare cases of completely extra-articular TSGCT located at the buttocks. One case was a 23-year-old young male presenting with left buttock swelling and pain for 1 year. Magnetic resonance imaging (MRI) examination revealed a dumbbell-type cystic solid mass in the left buttock, growing anteriorly from the deep surface of the gluteus muscle along the medial of the lesser trochanter. The lesion showed a heterogeneous mixed signal and was well-defined. MRI presentation needs to be differentiated with neurogenic or mesenchymal tumors, and radical resection of left gluteal tumor + neurovascular exploration surgery was performed. Another case of TSGCT we present here was diagnosed in a 55-year-old male elderly patient. Computed tomography angiography (CTA) revealed an irregular soft tissue mass in the left buttock involving the sacroiliac joint. T1-weighted imaging (T1WI) on MRI showed a mixed signal with predominantly isosignal, well-defined, and seemingly enveloped. A left buttock tumor resection with the scraping of the sacroiliac joint lesion was performed.ConclusionsBased on histopathological examination, the diagnosis was diffuse-type TSGCT for both cases. Both patients were periodically monitored after surgery, and one of them showed no imaging findings of recurrence or metastases seven years after surgery; the other case showed recurrence one year after surgery, which was resected and treated with radiotherapy, and there has been no recurrence so far. TSGCT occurring completely intramuscular is rare, with atypical clinical symptoms and imaging presentation, requiring differentiation with mesenchymal and giant cell-rich tumors.

HSR22-152: Healthcare Resource Utilization, Dosing and Time on Treatment in Patients on Pexidartinib Tenosynovial Giant Cell Tumors: Real-World Evidence from a Claims-Based Dataset

"HSR22-152: Healthcare Resource Utilization, Dosing and Time on Treatment in Patients on Pexidartinib Tenosynovial Giant Cell Tumors: Real-World Evidence from a Claims-Based Dataset" published on 31 Mar 2022 by National Comprehensive Cancer Network.

Author Correction: Surgical Treatment Patterns, Healthcare Resource Utilization, and Economic Burden in Patients with Tenosynovial Giant Cell Tumor Who Underwent Joint Surgery in the United States

Author Correction: Surgical Treatment Patterns, Healthcare Resource Utilization, and Economic Burden in Patients with Tenosynovial Giant Cell Tumor Who Underwent Joint Surgery in the United States