Prospects of Treating Tenosynovial Giant Cell Tumor through Pexidar-tinib: A Review

Author(s):  
Yunes M.M.A. Alsayadi ◽  
Pooja A. Chawla

Background: Tenosynovial giant cell tumor refers to a group of rarely occurring tumors that are formed in the joints, which are characterized by pain, swelling, and limitation of movement of the joint. Surgery is the main treatment strategy, but the tumor is likely to recur, especially in pigmented villonodular synovitis, which is the diffuse-type of giant cell tumor. Pexidartinib was approved in August 2019 by the Food and Drug Administration (FDA) with a brand name TURALIO as the first systemic approved therapy for patients having Tenosynovial Giant Cell Tumors (TGCT). Objective: In this review, different aspects pertaining to pexidartinib have been summarized including pathophysiology of TGCT, chemistry, pharmacokinetics and pharmacodynamics of pexidartinib. Special attention is given to various reported clinical trials of pexidartinib. Methods: A comprehensive literature search was conducted in the relevant databases to identify studies published in this field during recent years Conclusion: Pexidartinib acts by inhibiting the colony-stimulating factor (CSF1)/CSF1 receptor pathway which leads to inhibition of the cell lines proliferation and promotes the autophosphorylation process of ligand-induced CSF1 receptor. Pexidartinib emerged as a potential drug candidate for the treatment of TGCT.

2012 ◽  
Vol 136 (8) ◽  
pp. 901-906 ◽  
Author(s):  
David R. Lucas

Tenosynovial giant cell tumors are a group of generally benign intra-articular and soft tissue tumors with common histologic features. They can be roughly divided into localized and diffuse types. Localized types include giant cell tumors of tendon sheath and localized pigmented villonodular synovitis, whereas diffuse types encompass conventional pigmented villonodular synovitis and diffuse-type giant cell tumor. Localized tumors are generally indolent, whereas diffuse tumors are locally aggressive. Recent developments indicate that tenosynovial giant cell tumors are clonal neoplastic tumors driven by overexpression of CSF1. Herein, I report a case of intra-articular, localized tenosynovial giant cell tumor (or localized pigmented villonodular synovitis) and review the classification, histopathology, and recent developments regarding its pathogenesis.


2021 ◽  
Vol 23 (5) ◽  
pp. 656-663
Author(s):  
S. I. Herasymenko ◽  
O. A. Kostogryz ◽  
Yu. O. Kostogryz ◽  
A. M. Babko ◽  
V. M. Mayko

The aim. To develop a treatment algorithm with the aim of improving the treatment results of diffuse pigmented villonodular synovitis of the knee. To study and analyze the results of surgical treatment of diffuse pigmented villonodular synovitis of the knee. Materials and methods. The study was based on the results of treatment of 48 patients with pathohistologically verified diagnosis of diffuse pigmented villonodular synovitis (tenosynovial giant cell tumor) of the knee undergoing treatment at the State Institution “Institute of Traumatology and Orthopedics of the National Academy of Medical Sciences of Ukraine” from 2010 to 2019. Results. The best functional results can be achieved in patients with diffuse pigmented villonodular synovitis of the knee in stage I of the disease. Using the method of mathematical statistics to evaluate the results obtained, we see that scores obtained through the Lysholm Scoring Scale were statistically significantly different in stages before a surgery (P = 0.000782), 3 months (P = 0.00005) and 6 months after the surgery (P = 0.04); but over time, these differences diminished and became actually insignificant 12 months after the surgery (P = 0.89). Conclusions. Applying our therapeutic algorithm for the treatment of patients with diffuse pigmented villonodular synovitis of the knee, the outcome of the Lysholm score was excellent in 22 % of patients, good – in 45 %, satisfactory – in 21 % and poor – in 12 % of patients.


10.3823/2360 ◽  
2017 ◽  
Vol 10 ◽  
Author(s):  
Hasna Salhi ◽  
Olfa Jaidane ◽  
Jamel Ben Hassouna ◽  
Tarek Ben Dhieb ◽  
Monia Hechiche ◽  
...  

Tenosynovial giant cell tumors (TGCT) are a group of generally benign intra-articular and soft tissue tumors with common histological features. TGCT is also known as pigmented villonodular synovitis. There are localized and diffuse forms. Localized types include giant cell tumors of tendon sheath and localized pigmented villonodular synovitis, whereas diffuse types encompass conventional pigmented villonodular synovitis and diffuse type giant cell tumor. Localized tumors are generally indolent, whereas diffuse tumors are locally aggressive. In this article, we report the case of a diffuse-type extra-articular TGCT found in the left thigh of a 73-year-old woman who presented with a painless but gradually progressive swelling in the left thigh since eighteen months. On examination, there was a soft cystic swelling measuring 22 cm. The swelling was fixed to the underlying soft tissues. She had a Computed Tomography scan of the left thigh showing a mass of fluid density, well encapsulated between the muscles of the thigh, measuring 20x10 cm of major axes, compressing the femoral vessels without invading them. The patient had a complete marginal resection of the tumor. The immune-histopathological findings were consistent with those of a diffuse type of Giant Cell Tenosynovial Tumor. At 18 months follow-up, the patient is asymptomatic with no evidence of disease recurrence.  The extra-articular diffuse type TGCT is more aggressive than the localized type. Although these tumors are benign in the majority of cases, malignant transformation has been reported. Therefore, close follow-up is recommended after tumor excision.


Sign in / Sign up

Export Citation Format

Share Document