Tenosynovial giant cell tumor (TGCT)/pigmented villonodular synovitis (PVNS): Outcome of 313 patients before the era of kinase inhibitors.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10022-10022
Author(s):  
Emanuela Palmerini ◽  
Stefano Pengo ◽  
Robert G. Maki ◽  
Eric L. Staals ◽  
Angela Cioffi ◽  
...  

10022 Background: Tenosynovial giant cell tumor (TGCT) is a rare, usually benign neoplasm of synovium and tendon sheath. TGCT is classified as localized or diffuse according to the extent of synovial involvement. Surgery is the primary treatment, but the recurrence rate is high, with possible multiple recurrences, joint function deterioration and decline in quality of life. Recent data suggest a role for TKIs in advanced disease. In order to identify prognostic factors for recurrence, a retrospective pooled analysis was carried out in three institutions (Istituto Ortopedico Rizzoli, Bologna, Italy; Istituto Nazionale Tumori, Milano, Italy; Memorial Sloan Kettering Cancer Center, New York, USA). Methods: Clinical charts and pathology reports of patients (pts) treated in the period 1998-2008 were examined. Results: The study included 313 pts, 177 F and 136 M; median age: 36 years (range: 11-89 years). Most (64%) pts had tumors in the knee (15% ankle, 11% hip, 10% other). Tumor size was: <2 cm in 24% of pts, 2-5 cm in 44%, >5 cm in 32%. A diffuse pattern was reported in 69% of pts. The resection status was available in 289 pts: 51% had R0 surgery, 28% R1 and 21% R2. No metastases were documented. Local recurrence was reported in 76 pts (median time to recurrence: 15.7 months). With a median follow-up of 4.2 years, 5-year local recurrence-free survival (LRFS) was 66% (95% CI: 59 - 73). Size (< 2 cm 80% vs. 2-5 cm 67% vs. >5 cm 62%, p=0.04), gender (F 73% vs. M 56%, p=0.02), type (localized 78% vs. diffuse 61%, p=0.02), and resection status (R0 76% vs. R1 55%, vs. R2 57%, p=0.002) influenced 5-year LRFS, whereas age, tumor location and bone involvement did not. The 5-year 2nd LRFS was 43% (95% CI: 28 - 59). Multiple (2 to 5) local recurrences were observed in 39% of relapsed patients. Conclusions: The study confirms TGCT propensity to multiple local recurrences. Diffuse type, suboptimal surgery, male gender and larger tumors increase the recurrence risk. In order to improve the probability of local control, studies addressing the role of TKIs could be considered in subsets of patients.

2021 ◽  
Vol 24 (2) ◽  
pp. 100-107
Author(s):  
M. A. Khodorkovskiy

The review article outlines modern aspects of the nomenclature, etiopathogenesis, diagnosis and treatment of tenosynovial giant cell tumor of the tendon sheath. This is the second most common benign neoplasm of the hand. Non-radical surgical treatment of this disease leads to a large number of recurrence. To avoid tumor recurrence, surgeon must to have the basics of plastic reconstructive surgery, use delicate instruments and optical magnification. In some cases, adjuvant radiation therapy and administration of tyrosine kinase inhibitors may be required.


2020 ◽  
Vol 78 ◽  
pp. 413-415
Author(s):  
Rahul Singh ◽  
Martin N. Stienen ◽  
Kristen Ganjoo ◽  
Kevin S. Kolahi ◽  
Romain Cayrol ◽  
...  

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takehiro Ota ◽  
Yoshihiro Nishida ◽  
Kunihiro Ikuta ◽  
Satoshi Tsukushi ◽  
Kenji Yamada ◽  
...  

AbstractOsteochondral destruction and a high recurrence rate after surgery are major concerns that make difficult the treatment course of tenosynovial giant cell tumor. The aims of this study were to elucidate rates of postoperative local recurrence and osteochondral destruction, as correlated with various demographic factors. Eighty surgically treated patients with intra-articular tumors (knee: 49, ankle and foot: 12, hip: 10, others: 9) were included in this study. Factors including age, disease type (diffuse/localized), location, existence of osteochondral destruction were correlated with local recurrence or development/progression of osteochondral destruction. The 5-year local recurrence free survival rate was 71.4%. Diffuse type (n = 59, localized: n = 21) (P = 0.023) and knee location (P = 0.002) were independent risk factors for local recurrence. Diffuse type (P = 0.009) was a significant risk factor, and knee location (P = 0.001) was a negative factor for osteochondral destruction at the initial examination. Progression of osteochondral destruction was observed more often in cases with local recurrence (P = 0.040) and findings of osteochondral destruction at the initial examination (P = 0.029). Diffuse type is a factor that should be noted for both local recurrence and osteochondral destruction, while local recurrence occurs but osteochondral destruction is less observed in the knee.


2020 ◽  
Vol 26 (7) ◽  
pp. 822-827
Author(s):  
Shinji Tsukamoto ◽  
Riccardo Zucchini ◽  
Eric L. Staals ◽  
Andreas F. Mavrogenis ◽  
Manabu Akahane ◽  
...  

2016 ◽  
Vol 24 (2) ◽  
pp. 228-240 ◽  
Author(s):  
Stepan V. Domovitov ◽  
Chandhanarat Chandhanayingyong ◽  
Patrick J. Boland ◽  
David G. McKeown ◽  
John H. Healey

OBJECT There is no consensus regarding the appropriate treatment of sacral giant cell tumor (GCT). There are 3 main management problems: tumor control, neurological loss, and pelvic instability. The objective of this study was to examine oncological, neurological, and structural outcomes of sacral GCT after intralesional excision and local intraoperative adjunctive treatment. METHODS The authors retrospectively reviewed the records of 24 patients with sacral GCT who underwent conservative surgery (intralesional resection/curettage) at Memorial Sloan Kettering Cancer Center from 1973 through 2012. They analyzed patient demographic data, tumor characteristics, and operative techniques, and examined possible correlations with postoperative functional outcomes, complications, recurrence, and mortality. RESULTS There were 7 local recurrences (30%) and 3 distant recurrences (13%). Three of 24 patients (12.5%) had significant neurological loss after treatment—specifically, severe bowel and/or bladder dysfunction, but all regained function within 1–4 years. Larger tumor size (> 320 cm3) was associated with greater postoperative neurological loss. Radiation therapy and preoperative embolization were associated with prolonged disease-free survival. There were no local recurrences among the 11 patients who were treated with both modalities. Based on radiographic and clinical assessment, spinopelvic stability was present in 23 of 24 patients at final follow-up. CONCLUSIONS High local and distant recurrence rates associated with sacral GCT suggest the need for careful local and systemic follow-up in managing these patients. Intraoperative preservation of sacral roots was associated with better pain relief, improvement in ambulatory function, and retention of bowel/bladder function in most patients. Fusion and instrumentation of the sacroiliac joint successfully achieved spinopelvic stability in cases deemed clinically unstable. Despite improvement in the management of sacral GCT over 35 years, a need for novel therapies remains. The strategy of combining radiotherapy and embolization merits further study.


2019 ◽  
Vol 72 (12) ◽  
Author(s):  
Olena O Dyadyk ◽  
Anastasiia Hryhorovska

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.


2021 ◽  
pp. 100366
Author(s):  
Lenian Zhou ◽  
Shanyi Lin ◽  
Hanqiang Jin ◽  
Zhaoyuan Zhang ◽  
Changqing Zhang ◽  
...  

2021 ◽  
Vol 16 (4) ◽  
pp. 950-955
Author(s):  
Stephanie D. Zarate ◽  
David M. Joyce ◽  
Ana C. Belzarena

2021 ◽  
pp. 106689692110498
Author(s):  
Haider Mejbel ◽  
Gene P. Siegal ◽  
Shi Wei

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.


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