Tenosynovial Giant Cell Tumor

P3, N=128, Not yet recruiting, SynOx Therapeutics Limited | Trial completion date: Sep 2025 --> Sep 2027 | Trial primary completion date: Dec 2023 --> Sep 2027

P3, N=40, Active, not recruiting, Daiichi Sankyo Co., Ltd. | Trial completion date: Mar 2024 --> Feb 2026

P3, N=90, Active, not recruiting, Abbisko Therapeutics Co, Ltd | Recruiting --> Active, not recruiting

P3, N=120, Active, not recruiting, Deciphera Pharmaceuticals LLC | Trial primary completion date: Mar 2024 --> Aug 2023

P2, N=17, Active, not recruiting, Andrew J. Wagner, MD, PhD | Trial completion date: Jul 2024 --> Jan 2025

P3, N=90, Recruiting, Abbisko Therapeutics Co, Ltd

The results of immunoblotting assays suggested that silybinin as well as compound glycyrrhizin inhibited the protein expression of CYP3A4 and CYP2C9 in rats. Therefore, the combination of pexidartinib with silybinin and compound glycyrrhizin should be monitored to avoid clinical adverse effects.

In conclusion, this study systematically explored the significance of COPS6 across different tumor types, providing a solid foundation for considering COPS6 as a novel biomarker in cancer research.

MMP9, SPP1, and TYROBP were identified as osteoclast-specific up-regulated genes of the tenosynovial giant cell tumour via bioinformatic analysis, which had a reasonable diagnostic efficiency and served as potential drug targets.

Our findings provide additional evidence that the CSF1/CSF1R pathway is involved in the pathogenesis of XGET/KPGCT. These findings suggest a possible role for CSF1R inhibition in the treatment of unresectable or metastatic XGET/KPGCT.

We identified the risk indicators and developed a nomogram in this study to forecast the likelihood of local recurrence-free survival in patients with diffused-tenosynovial giant cell tumor from temporomandibular joint.

P1/2, N=120, Active, not recruiting, Deciphera Pharmaceuticals LLC | Recruiting --> Active, not recruiting | Trial primary completion date: Jul 2022 --> Dec 2023

Careful consideration should be given to the differential diagnosis of pediatric patients presenting with monoarthritis. This report highlights the importance of early and accurate diagnosis and underscores the necessity for effective treatments for epithelioid sarcoma. Surgical resection or radical surgery is recommended, while novel treatment strategies targeting EZH2 show promise.

DOG1 is also useful in reinforcing a diagnosis of chondroblastoma in a clinicoradiological context, especially in laboratories lacking H3.3K36M immunostain. However, its staining pattern is variable.

TGCT can arise at the craniocervical junction and is easily misdiagnosed because of its rare occurrence. IHC examination of a tumor specimen should be done to confirm the diagnosis. GTR is the objective when treating these tumors, especially when they are the diffuse type, as they have a high recurrence rate. Radiation and small-molecule therapies are viable postoperative therapies if GTR cannot be achieved or in cases of recurrence.

For the first time, this paper was able to provide data on a large sample for Germany. Notably, the low sensitivity of the clinical diagnosis confirms the importance of histopathology for diagnosing L‑TSRZT.

In the current study, the metabolic activation of PEX was investigated in human/mouse liver microsomes (HLM/MLM) and primary human hepatocytes (PHH) using glutathione (GSH) and methoxyamine (NHOMe) as trapping reagents. CYP3A4 and CYP3A5 were identified as the primary enzymes responsible for the formation of these adducts using recombinant human P450s and CYP3A chemical inhibitor ketoconazole. Overall, our studies suggested that PEX metabolism can produce reactive metabolites mediated by CYP3A, and the association of the reactive metabolites with PEX hepatotoxicity needs to be further studied.

In Part 1, participants are randomized to receive vimseltinib 30 mg twice weekly or matching placebo for ≤24 weeks. Part 2 is a long-term treatment phase in which participants will receive open-label vimseltinib.

With the extension of treatment duration, there is an observed augmentation in the number of pts experiencing sustained tumor shrinkage and favorable safety of Pimicotinib with no apparent hepatotoxicity. Current data confers durable therapeutic benefits in TGCT pts, suggesting that prolonged exposure may represent an optimal treatment approach. In addition, a separate cohort with prior anti-CSF-1/CSF-1R therapies is underway to assess the safety and antitumor activity.

Pts with TGCT not amenable to surgery who received prior anti-CSF1/CSF1R therapy (including pexidartinib, cabiralizumab, or vimseltinib) were enrolled and treated with vimseltinib 30 mg twice weekly (recommended phase 2 dose). Longer follow-up demonstrated that vimseltinib continued to be well tolerated with a manageable safety profile in pts with TGCT not amenable to surgery who received prior anti-CSF1/CSF1R therapy. Antitumor activity continued to improve in this pretreated population, with an increased ORR. At week 25, all responders experienced ≥30% reductions in worst and average pain.

Pts with TGCT not amenable to surgery and no prior anti-CSF1/CSF1R therapy (previous treatment with imatinib or nilotinib was allowed) were enrolled and treated with vimseltinib 30 mg twice weekly (recommended phase 2 dose). Longer follow-up demonstrated that vimseltinib continued to be well tolerated with a manageable safety profile in pts with TGCT not amenable to surgery with no prior anti-CSF1/CSF1R therapy. Antitumor activity continued to improve with a promising overall ORR. At week 25, the majority of responders experienced ≥30% reductions in worst and average pain.

In Asian patients with symptomatic TGCT not amenable to improvement with surgery, pexidartinib demonstrated clinical benefit in tumor response and improvement in joint function. The overall safety profile of pexidartinib was comparable with previous data. Hepatotoxicity was manageable with frequent liver test monitoring and dose modifications as defined in the protocol, and no new safety signals were observed in this Asian population.

This review discusses the pathogenesis of TGCT, potential druggable targets and therapeutic approaches. It also evaluates the safety and efficacy of different systemic therapies.

In this review, we aim to discuss the biology, clinical management, and future treatment frontiers for three representative locally aggressive mesenchymal tumors: desmoid-type fibromatosis (DF), tenosynovial giant cell tumor (TSGCT) and giant cell tumor of bone (GCTB). These entities challenge clinicians with their unpredictable behavior and responses to treatment, and still lack a well-defined standard of care despite recent progress with newly approved or promising experimental drugs.

Hence, 3D-printed titanium prosthetic reconstruction of bone deficiency in the upper cervical spine was a safe and effective technique. Level IV.

TSGCT revealed high FDG uptake. Furthermore, SUVmax was higher in diffused TSGCT with extra-articular invasion than in intra-articular localized TSGCT; this may reflect its local aggressiveness.

Hence, high degree of suspicion and early en bloc resection is the key to its management. case reports; giant cell tumors; tendons; thumb.

P2, N=17, Active, not recruiting, Andrew J. Wagner, MD, PhD | Trial completion date: Dec 2023 --> Jul 2024

We found and summarized some surgical techniques that can help safeguard the important structures around massive TGCTs of temporal bone and middle skull base, and reconstruct the defects after tumor resection. The techniques are effective and feasible.

Low to intermediate intensity on T1- or T2-weighted images was useful to distinguish TSGCTs from subcutaneous tissue in the small joints. MRI using T2*-, as well as T1- and T2-weighted images, may be useful to detect lesions and assess the extent of TSGCTs in a tissue-specific manner, which is important for surgical planning.

P2, N=21, Recruiting, Daiichi Sankyo Co., Ltd. | Trial completion date: Jun 2024 --> Nov 2026 | Trial primary completion date: Mar 2023 --> Feb 2025

No abstract available

KEY MESSAGE: at the best of our knowledge, only few cases about drug-induce punctate anetoderma are already described in the literature. We report this case to underline the importance of this clinical condition for the dermatologists, in order to provide a proper diagnosis and a correct therapeutic management

Intra-articular tumor development, marginal resection and tendon involvement seem to contribute to recurrence. This tumor deserves to be known by dermatologists and evoked in front of any digital mass in order to carry out an imaging assessment and adequate surgical management.

Pexidartinib is the first CSF-1 receptor inhibitor approved for the treatment of TGCT. Here, we discuss various available treatment strategies and ongoing investigations and trials targeting diffuse TGCT, which include nilotinib, lacnotuzumab, cabiralizumab, vimseltinib, and emactuzumab.

No abstract available

Total knee arthroplasty represents a valid treatment for patients with PVNS end-stage osteoarthritis, with good clinical and functional results, even in longer follow-up. It would be advisable a multidisciplinary management and a meticulous rehabilitation and monitoring following the procedure, to reduce the emergence of recurrence and overall complications.

D-TSGCTs was associated with local recurrence and showed multinodularity infiltrative margin, and absent peripheral hypointensity. Disease severity including cartilage and tendon involvement was associated with local recurrence. Preoperative MRI evaluation by combining disease subtypes and severity can predict local recurrence sensitively.

No abstract available

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.