Intramuscular Tenosynovial Giant Cell Tumor Harboring a Novel CSF1-CD96 Fusion Transcript

2021 ◽  
pp. 106689692110498
Author(s):  
Haider Mejbel ◽  
Gene P. Siegal ◽  
Shi Wei
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Gene Rearrangement ◽  
Fusion Transcript ◽  
Cell Tumor ◽  
Molecular Profile ◽  
Small Subset ◽  
Fusion Partner ◽  
Giant Cell Tumors ◽  
Tenosynovial Giant Cell Tumor

Tenosynovial giant cell tumors typically arise in the synovium of joints, bursae, or tendon sheaths. They may occur in an intra- or extra-articular location and can be divided into localized and diffuse types. The neoplastic nature of the lesion has been supported by a recurrent CSF1 gene rearrangement in a small subset of lesional cells, of which the most common fusion partner is COL6A3. Herein, we report a case of intramuscular localized tenosynovial giant cell tumor harboring a novel CSF1-CD96 fusion transcript, thus expanding the molecular profile of this tumor.

scholarly journals Management of a Huge Tenosynovial Giant Cell Tumor of the Wrist

2021 ◽  
Author(s):  
Woo Jong Kim ◽  
Sang Mi Lee ◽  
Byungsung Kim ◽  
Jae-Hwi Nho ◽  
Jun Bum Kim ◽  
...  
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Rare Case ◽  
Tendon Sheath ◽  
Cell Tumor ◽  
Benign Neoplasms ◽  
Diffuse Type ◽  
Giant Cell Tumors ◽  
Tenosynovial Giant Cell Tumor

Tenosynovial giant cell tumors (TGCTs) are typically benign neoplasms of the joint, bursa, and tendon sheath. Usually, TGCT presents as a small localized tumor on the hand and wrist. The diffuse-type TGCT is more aggressive and mainly affects large joints such as knees, hips, ankles, and elbows. Diffuse-type TGCT of small joints is rare. To our knowledge, this is a very rare case of a huge diffuse-type TGCT on the wrist. We report a huge TGCT, which grew gradually over 10 years.

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

2020 ◽  
Vol 20 ◽  
Author(s):  
Yunes M.M.A. Alsayadi ◽  
Pooja A. Chawla
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Pigmented Villonodular Synovitis ◽  
Cell Tumor ◽  
Drug Candidate ◽  
Brand Name ◽  
Giant Cell Tumors ◽  
Tenosynovial Giant Cell Tumor ◽  
Pigmented Villonodular ◽  
Stimulating Factor

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.

scholarly journals Tenosynovial Giant Cell Tumor: Case Report and Review

2012 ◽  
Vol 136 (8) ◽  
pp. 901-906 ◽  
Author(s):  
David R. Lucas
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Pigmented Villonodular Synovitis ◽  
Cell Tumor ◽  
Villonodular Synovitis ◽  
Giant Cell Tumors ◽  
Tenosynovial Giant Cell Tumor ◽  
Recent Developments ◽  
Localized Pigmented Villonodular Synovitis ◽  
Pigmented Villonodular

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.

CLINICAL AND MORPHOLOGICAL CORRELATIONS AND HISTOPATHOLOGY OF JOINT DAMAGE IN PATIENTS WITH DIFFUSE-TYPE TENOSYNOVIAL GIANT CELL TUMOR

2019 ◽  
Vol 72 (12) ◽  
Author(s):  
Olena O Dyadyk ◽  
Anastasiia Hryhorovska
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Cell Size ◽  
Giant Cells ◽  
Cell Tumor ◽  
Multinucleated Giant Cells ◽  
Diffuse Type ◽  
Association Coefficient ◽  
Mean Values ◽  
Tenosynovial Giant Cell Tumor

Introduction: Tenosynovial giant cell tumor (TSGCT) (synonym – pigmented villonodular synovitis) – is a rare benign proliferative lesion of the synovial sheath, localized in the joint capsule, bursa or tendon sheath and characterized by locally destructive growth. Depending on the prevalence within the joint elements, the presence of a capsule around the tumor, histophotographic features of cell structure and clinical behavior TSGCT can be divided to localized or diffuse type. The aim of the study was researching of histopathological properties of diffuse-type TSGCT, determine the parameters its morphological indicators and to find out the correlation between these morphological and clinical parameters. Materials and methods: The research material was used biopsy (resect) of pathological lesions from 50 patients who were diagnosed and histologically verified diffuse-type TSGCT. Microscopic examinations of the stained sections and their photo archiving were carried out with use of a Olympus-CX 41 light optical microscope. Group measurable parameters (mean values and Pearson tetrachoric index (association coefficient) were calculated in groups of comparison for morphological and clinical indices of TSGCT. The mean values were compared by Student’s test, P value of ≤0.1 was considered statistically significant. Results:Correlation analysis of indicators that accounted for the pairs of cases «clinic – morphology» revealed the relationships, that had the highest parameters of the association coefficient between such indicators: «presence of villous growths» - «severity of hemosiderosis» (if hypertrophied synovial villi available, with vascular injection and pronounced proliferation of synovial cells, there is also a significant accumulation of hemosiderin pigment); «presence of villous growths» - «type of predominant cellular proliferates» (if cells of TSGCT diffuse type consists of monotonous sheets of stromal cells, with uniform, oval to reniform nuclei, the proliferation of villi in synovial layer is non-distinctive); «presence of nodes» - «kind of stroma» (if nodes predominate, their histological structure is mainly represented by polymorphic clusters of synovitis cells in the form of cells, strands, chains, solid formations, among immature connective tissue with low hyalinosis); «cell size (area, cm²)» - «severity of haemosiderosis» and «cell size (area, cm²)» - «the number of multinucleated giant cells» (there is a pronounced deposition of pigment and accumulation of osteoclast-like multinucleated giant cells type, although usually their number is relatively small compared to the localized type of TSGCT). Conclusions: Morphological parameters, that we have identified, characterize pathological changes in the tissues of TSGCT; careful analysis of the frequency of their occurrence in the different comparison groups made it possible to establish intergroup differences and correlations between individual indicators, which were previously unknown or not obvious. Our study was determine to analyze of incidence rates and correlation relationships, revealed some previously unknown differences and dependencies that are important for understanding the pathogenesis, improvement of diagnosis and prognosis of diffuse-type TSGCT.

Giant Cell Tumor of the Talar Neck

2007 ◽  
Vol 97 (3) ◽  
pp. 225-228 ◽  
Author(s):  
Hakan Selek ◽  
Hamza Özer ◽  
Sacit Turanli ◽  
Özlem Erdem
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Clinical Signs ◽  
Cell Tumor ◽  
Giant Cell Tumors ◽  
Radiographic Appearance ◽  
Aneurysmal Bone Cysts ◽  
Tumors Of Bone ◽  
Brown Tumors ◽  
Small Bones

We describe a patient with a giant cell tumor in the talar head and neck of the left foot who was diagnosed as having osteochondritis dissecans and treated with arthroscopic drilling in this same location 3 years earlier. Giant cell tumors can be confused with several conditions, including giant cell reparative granulomas, brown tumors, and aneurysmal bone cysts. Giant cell tumors of bone typically occur in the epiphysis of long bones, including the distal femur and proximal tibia. They are uncommonly found in the small bones of the foot or ankle, and talar involvement is rare. Despite this rarity, the radiographic appearance and clinical signs of talar lesions should be considered in the differential diagnosis of nontraumatic conditions in the foot. (J Am Podiatr Med Assoc 97(3): 225–228, 2007)

scholarly journals Tenosynovial giant cell tumor of the distal tibiofibular joint

2021 ◽  
Vol 16 (4) ◽  
pp. 950-955
Author(s):  
Stephanie D. Zarate ◽  
David M. Joyce ◽  
Ana C. Belzarena
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Cell Tumor ◽  
Tenosynovial Giant Cell Tumor ◽  
Tibiofibular Joint

scholarly journals Tenosynovial Giant Cell Tumor in the Midfoot Treated With Femoral Head Allograft Reconstruction

2018 ◽  
Vol 57 (1) ◽  
pp. 172-178 ◽  
Author(s):  
Daniel J. Fuchs ◽  
Paul J. Switaj ◽  
Terrance D. Peabody ◽  
Anish R. Kadakia
Keyword(s):  
Femoral Head ◽  
Giant Cell Tumor ◽  
Giant Cell ◽  
Cell Tumor ◽  
Allograft Reconstruction ◽  
Tenosynovial Giant Cell Tumor

scholarly journals Correlation between Campanacci’s radiological classification of giant cell tumor of bone and expression of Cyclin D1 and PCNA

2017 ◽  
Vol 7 (1) ◽  
pp. 47
Author(s):  
Eréndira G. Estrada-Villaseñor ◽  
Hidalgo Bravo Alberto ◽  
C. Bandala ◽  
P. De la Garza-Montano ◽  
Reyes Medina Naxieli ◽  
...  
Keyword(s):  
Cyclin D1 ◽  
Giant Cell Tumor ◽  
Giant Cell ◽  
Descriptive Study ◽  
Cell Tumor ◽  
Giant Cell Tumors ◽  
Radiological Classification ◽  
Tumors Of Bone ◽  
Made In

Giant cell tumor of bone is considered by his behavior a benign but aggressive neoplasm. The objective of our study was to determine if there is a correlation between the Campanacci’s radiological classification of giant cell tumors of bone and the expression by immunohistochemistry of Cyclin D1 and proliferation cell nuclear antibody (PCNA). A retrospective and descriptive study was made. In total, there were 27 cases. All cases showed Cyclin D1 and PCNA positivity. Rho Spearman for Campanacci and Cyclin D1 expression was 0.06 and for Campanacci and PCNA was 0.418. We conclude that there is a positive correlation between PCNA expression in giant cell tumors of Bone and the Campanacci’s radiological classification II and III, butCyclin D1 expression was no related with radiologic features.

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