Raras
Buscar doenças, sintomas, genes...
Tumor tenossinovial de células gigantes
ORPHA:66627CID-10 · M12.2DOENÇA RARA

É um tumor que cresce de forma agressiva e se espalha pelos tecidos próximos na região onde surge, especificamente na bainha do tendão (uma espécie de capa que envolve os tendões). Ele é formado por diferentes tipos de células, como as que lembram as das articulações, células de defesa do corpo (macrófagos) que contêm um pigmento de ferro (hemossiderina), células espumosas e células inflamatórias. Na maioria das vezes, também estão presentes células gigantes com muitos núcleos, parecidas com as que "comem" o osso (osteoclastos), embora em poucos casos elas possam estar ausentes ou ser raras. Atinge principalmente adultos jovens. Os sintomas incluem inchaço, dor e acúmulo de líquido na articulação.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

É um tumor que cresce de forma agressiva e se espalha pelos tecidos próximos na região onde surge, especificamente na bainha do tendão (uma espécie de capa que envolve os tendões). Ele é formado por diferentes tipos de células, como as que lembram as das articulações, células de defesa do corpo (macrófagos) que contêm um pigmento de ferro (hemossiderina), células espumosas e células inflamatórias. Na maioria das vezes, também estão presentes células gigantes com muitos núcleos, parecidas com as que "comem" o osso (osteoclastos), embora em poucos casos elas possam estar ausentes ou ser raras. Atinge principalmente adultos jovens. Os sintomas incluem inchaço, dor e acúmulo de líquido na articulação.

Pesquisas ativas
10 ensaios
32 total registrados no ClinicalTrials.gov
Publicações científicas
531 artigos
Último publicado: 2026 Apr 7

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
20.0
Europe
Início
Adolescent
+ adult, childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: M12.2
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🦴
Ossos e articulações
9 sintomas
👂
Ouvidos
2 sintomas
🧠
Neurológico
1 sintomas
🩸
Sangue
1 sintomas

+ 11 sintomas em outras categorias

Características mais comuns

90%prev.
Artralgia
Muito frequente (99-80%)
55%prev.
Hemorragia articular
Frequente (79-30%)
55%prev.
Osteólise
Frequente (79-30%)
55%prev.
Inchaço articular
Frequente (79-30%)
55%prev.
Rigidez articular
Frequente (79-30%)
55%prev.
Artrite
Frequente (79-30%)
24sintomas
Muito frequente (1)
Frequente (7)
Ocasional (12)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

ArtralgiaArthralgia
Muito frequente (99-80%)90%
Hemorragia articularJoint hemorrhage
Frequente (79-30%)55%
OsteóliseOsteolysis
Frequente (79-30%)55%
Inchaço articularJoint swelling
Frequente (79-30%)55%
Rigidez articularJoint stiffness
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico531PubMed
Últimos 10 anos200publicações
Pico202565 papers
Linha do tempo
2026Hoje · 2026🧪 2010Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado2
3Fase 34
2Fase 29
1Fase 12
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Tumor tenossinovial de células gigantes

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

32 ensaios clínicos encontrados, 10 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
444 papers (10 anos)
#1

Pediatric patients with tenosynovial giant cell tumor: real-world evidence from an observational registry.

Orphanet journal of rare diseases2026 Jan 30

Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive tumor originating in the synovial lining of the joint, bursa, and tendon sheath. TGCT typically affects individuals between 20 and 50 years of age and pediatric cases are considered ultra-rare. Research and clinical trials thus far have been largely focused on the adult TGCT population. Therefore, data are needed to understand the impact of TGCT on pediatric patients' quality of life and any differences between adults. Here we report the result of the pediatric TGCT population enrolled in an observational patient registry. A total of 122 pediatric patients (9.5%) were included in this exploratory, cross-sectional analysis of a longitudinal 1,278-patient registry from October 06, 2022 to November 26, 2024. Among pediatric patients, 73.0% had diffuse TGCT (D-TGCT; n = 89), 16.4% had localized TGCT (L-TGCT; n = 20), and 10.6% had unspecified TGCT (n = 13). Pediatric patients had a median age at diagnosis of 14.5 years. More than half of pediatric patients were initially misdiagnosed and were more likely to be misdiagnosed than adults (62.3% vs. 49.9%, p < 0.01) and received joint aspirations significantly more frequently than adult patients (47.5% [n = 58] vs. 22.5% [n = 112], p < 0.05). Most pediatric patients were diagnosed by orthopedic surgeons (n = 79, 64.8%), and 52.5% of pediatric patients were diagnosed ≥ 1 years after symptom onset. Pediatric patients with D-TGCT underwent an average of 3.4 surgeries, compared to 1.8 surgeries for those with L-TGCT. Recurrence rates were similar among adults and pediatric patients with 66.3% of pediatric patients with D-TGCT having ≥ 1 post-operative recurrence compared to 15.0% of L-TGCT pediatric patients, respectively. Despite no approved systemic therapies for pediatric use, pediatric and adult patients consulted medical oncologists in similar rates and systemic therapies were prescribed similarly but infrequently overall (n = 21, 17.2% in pediatric patients and n = 95, 19.1% in adults). Pediatric patients had significant disease burden, as compared to adults with TGCT, which severely affected their quality of life. The reliance on surgical treatment and underuse of multidisciplinary care emphasizes the unmet need for provider education and treatment advancements tailored to this population. This study was an analysis of an observational patient registry and therefore was not registered as a clinical trial; no trial registration number is available. The study protocol was approved by Advarra (protocol reference number: Pro00077310). Patient enrollment for this analysis occurred from October 6, 2022, to November 26, 2024.

#2

TGCT of the shoulder - a case series and literature review.

Archives of orthopaedic and trauma surgery2026 Mar 09

Tenosynovial giant cell tumor (TGCT), also known as pigmented villonodular synovitis (PVNS), involving the shoulder is extremely rare and can present with a challenging clinical course. Due to the complex anatomy of the shoulder, both diagnosis and treatment are demanding. This study aims to evaluate the diagnostic and therapeutic management of shoulder TGCT based on a case series and a review of the literature. Between 2005 and 2021, four patients (2 females, 2 males) with shoulder TGCT (1 localized, 3 diffuse) underwent surgical treatment at our institution. The minimum follow-up was 39 months (range: 39–233 months). Functional outcomes were assessed using the Disabilities of the Arm, Shoulder and Hand Questionnaire (DASH), the Oxford Shoulder Score (OSS), and the Short Form 36 Health Survey (SF-36). Additionally, a literature review of 65 studies comprising 108 patients was performed. Treatments consisted of arthroscopic and open resections, tendon repair, adjuvant radiosynoviorthesis, and radiotherapy, as appropriate. All patients were recurrence-free at the last follow-up, except for one with stable residual disease after radiotherapy. The mean interval from symptom onset to diagnosis was 23.6 ± 28.1 months. In the literature cohort, the mean patient age was 50.3 ± 19.7 years, with a nearly equal gender distribution. Diffuse TGCT was more common (66.1%) than nodular TGCT (33.8%). Treatment was primarily surgery, arthroscopic (53.2%) or open (42.9%), with adjuvant therapies applied in 9.3% of cases. After a mean follow-up of 37.6 ± 36.2 months in 67 cases, diffuse in 44.8%, nodular in 19.4% (no data regarding TGCT-type in 35.8%) the reported recurrence rate was 10.6% and 4.5% remained with residual disease. TGCT of the shoulder remains a rare and complex condition requiring individualized treatment strategies. Arthroscopic and open resections are the mainstays of therapy, while the role of adjuvant treatments should be carefully considered. Given the risk of recurrence, follow-up is essential. Further studies are needed to establish standardized treatment protocols and evaluate long-term outcomes. Level IV (Case series with no comparison group).

#3

Tenosynovial Giant Cell Tumor With Bone Erosion of the Tibial Midshaft: A Case Report.

JBJS case connector2026 Jan 01

A 37-year-old woman presented with a 2-week history of a palpable leg mass. Radiographs revealed a 7-mm focal cortical lytic lesion in the midmedial tibial diaphysis with adjacent soft-tissue component. MRI confirmed soft-tissue mass with cortical erosion. Biopsy established a localized type tenosynovial giant cell tumor (TGCT). En bloc excision of the 3-cm mass was performed. Histopathology confirmed localized type TGCT with cortical erosion. The patient remained asymptomatic at the 1-year follow-up without recurrence. This rare presentation with midshaft tibial erosion highlights that TGCT should remain in the differential diagnosis even for apparent primary bone lesions in unusual nonperiarticular locations.

#4

Soft tissue and juxtaarticular tumors of the knee.

Skeletal radiology2026 Feb 18

While the wide spectrum of soft tissue neoplasms found throughout the body may occur at the knee, this review focuses on the more commonly encountered intraarticular or juxtaarticular solid soft tissue lesions that may be encountered in this location. The benign intraarticular synovial-based tumors or tumor-like processes of tenosynovial giant cell tumor, synovial chondromatosis, and lipoma arborescens are reviewed, as are the solid juxtaarticular neoplasms of nerve sheath tumors, synovial sarcoma, and myxoid liposarcoma. Several uncommon lesions that occur at the knee while relatively rare elsewhere are also discussed and include synovial vascular malformations, the Hoffa fat pad chondroma, and popliteal arterial cystic adventitial disease. Finally, clear cell sarcoma, a rare malignancy which originates in ligaments and tendons, has the knee as the second most common site of occurrence and may be misinterpreted as a benign entity or sequelae of injury, is reviewed.

#5

Bilateral Localized-Tenosynovial Giant Cell Tumor of the Knee: Case Report and Review.

Journal of clinical medicine2026 Jan 27

Background/Objectives: Tenosynovial giant cell tumor (TGCT), formerly known as pigmented villonodular tenosynovitis (PVNS), is a rare, benign, inflammatory mesenchymal neoplasm originating from the synovium of joints, bursae, or tendon sheaths. Although TGCT can affect any joint, the knee is the most commonly involved site, particularly in cases of diffuse-type TGCT. Bifocal or multifocal involvement is exceedingly uncommon. Methods: Herein, we present a case of localized TGCT with bilateral knee involvement in a 48-year-old female. Results: The patient underwent open arthrotomy with marginal excision of the localized lesions in both knees. Histology and immunohistochemistry staining conformed the diagnosis. At the five-year follow-up, the patient remains asymptomatic and free of recurrence. Conclusions: Given the rarity of bilateral TGCT, clinicians should maintain a high index of suspicion when evaluating patients presenting with bilateral knee pain and swelling and include TGCT in the differential diagnosis. To our knowledge, this represents the fifteenth reported case of bilateral knee TGCT in the literature.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC321 artigos no totalmostrando 198

2026

TGCT of the shoulder - a case series and literature review.

Archives of orthopaedic and trauma surgery
2026

[Vimseltinib in non-resecable tenosynovial giant cell tumor].

Bulletin du cancer
2026

Tenosynovial Giant Cell Tumor With Bone Erosion of the Tibial Midshaft: A Case Report.

JBJS case connector
2026

Soft tissue and juxtaarticular tumors of the knee.

Skeletal radiology
2026

Bilateral Localized-Tenosynovial Giant Cell Tumor of the Knee: Case Report and Review.

Journal of clinical medicine
2026

Posterior Knee TGCT (Formerly PVNS) Masquerading as a Popliteal Lesion: Combined Arthroscopic Synovectomy and Posterior Open Excision With 8-Year Disease-Free Follow-Up.

Sports health
2026

Mixed-Methods to Define Meaningful Change using Exit Interviewand Clinical Trial Data in Patients with Tenosynovial Giant Cell Tumor (TGCT).

Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation
2026

Pediatric patients with tenosynovial giant cell tumor: real-world evidence from an observational registry.

Orphanet journal of rare diseases
2026

High Risk of Drug-Drug Interactions Caused by Pexidartinib via UDP-Glucuronosyltransferases Inhibition.

Chemical research in toxicology
2026

Open synovectomy for treatment of tenosynovial giant cell tumors of the knee in children.

Journal of surgical case reports
2026

A rare case report of chondroblastoma like soft tissue chondroma and discussion of its benign mimics.

Indian journal of pathology &amp; microbiology
2026

Management of tenosynovial giant cell tumor: approved and investigational therapies.

Expert review of anticancer therapy
2026

Tenosynovial Giant Cell Tumor of the Thumb with Bony Erosion - A Case Report on Surgical Excision with Adjuvant Chemical Cauterization.

Journal of orthopaedic case reports
2026

Current Treatment Strategies for Diffuse Tenosynovial Giant Cell Tumor: A Review of the Literature.

JB &amp; JS open access
2025

Imaging findings of intra-articular tumor/tumor-like lesions based on pathologic correlation.

Japanese journal of radiology
2026

Calcified chondroid mesenchymal neoplasm.

Seminars in diagnostic pathology
2025

Mechanistic Study of CD90-Positive Synovial Fibroblasts in the Invasion and Recurrence of Pigmented Villonodular Synovitis.

Journal of inflammation research
2025

Cystic Lesions of the Hand Skeleton.

Seminars in musculoskeletal radiology
2025

The Impact of Osseous Erosions on Intraarticular Tenosynovial Giant Cell Tumors of the Knee and Local Recurrence.

Orthopedics
2026

Multifocal tenosynovial giant cell tumor: A case report.

Radiology case reports
2025

[Localized tenosynovial giant cell tumor of the knee. Case report].

Acta ortopedica mexicana
2025

Knowledge map of recurrence in tenosynovial giant cell tumor: a bibliometric analysis.

Frontiers in oncology
2026

Genicular Embolization as a Therapeutic Alternative in Tenosynovial Giant Cell Tumor: A Case Report.

Journal of vascular and interventional radiology : JVIR
2025

Uncommon Localization of Tenosynovial Giant Cell Tumor in the Coracoclavicular Ligament: Diagnostic Challenges.

The American journal of case reports
2025

Tenosynovial Giant Cell Tumors: A Musculoskeletal Case Series.

Cureus
2026

Giant Cell-Rich Tumors of Bone and Soft Tissue.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2025

PIGMENTED VILLONODULAR SYNOVITIS IN THE ANKLE OF A PEDIATRIC PATIENT: A CASE REPORT.

Georgian medical news
2026

Temporomandibular Joint Lesions with Intracranial Extension: Illustrative Cases from a Systematic Review of the Literature and Our Institution.

AJNR. American journal of neuroradiology
2025

MRI Radiomics for Predicting the Diffuse Type of Tenosynovial Giant Cell Tumor: An Exploratory Study.

Diagnostics (Basel, Switzerland)
2025

Tenosynovial giant cell tumour in children. Our experience with 24 patients in a third level Children's Hospital, an observational study.

European journal of pediatrics
2025

Tenosynovial giant cell tumor (TGCT): Mechanisms and advances in targeted treatments.

Critical reviews in oncology/hematology
2025

Pharmacokinetics and pharmacodynamics of an anti-CSF1 receptor antibody in the intra-articular treatment of tenosynovial giant cell tumor.

European journal of cancer (Oxford, England : 1990)
2025

Adverse events of pexidartinib for the treatment of TGCT: a real-world disproportionality analysis using FDA Adverse Event Reporting System database.

Frontiers in oncology
2025

Proteomics and metabolomics studies in pigmented villonodular synovitis uncover the regulation of monocyte differentiation by the ADGRE5-NF-κB pathway.

BMC medicine
2025

[MRI in tenosynovial giant cell tumor (TGCT) - (formerly pigmented villonodular synovitis - PVNS)].

RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin
2025

A disproportionality analysis of adverse events caused by pexidartinib from the FDA adverse event reporting system.

Scientific reports
2025

Diagnosis and Treatment Options in Pigmented Villonodular Synovitis of the Knee: A Narrative Review.

Journal of clinical medicine
2025

A Phase 3 Study of the Efficacy and Safety of Pexidartinib in East Asian Patients with Tenosynovial Giant Cell Tumor.

Oncology and therapy
2025

Primary tenosynovial giant cell tumor arising in the nasal cavity with molecular confirmation: the first reported case.

Virchows Archiv : an international journal of pathology
2025

Don't Dismiss the Swelling: A Rare Case of Tenosynovial Giant Cell Tumor in a Young Woman's Finger - A Case Report.

Journal of orthopaedic case reports
2025

Tenosynovial giant cell tumor and its differential diagnosis in children.

Pediatric radiology
2025

Giant Cell Tumor of Hoffa's Fat Pad: A Case Report.

JNMA; journal of the Nepal Medical Association
2025

Real-world experience of patients newly initiated on pexidartinib for tenosynovial giant cell tumor.

Future oncology (London, England)
2025

Long-term efficacy and safety of pexidartinib in patients with tenosynovial giant cell tumor: final results of the ENLIVEN study.

The oncologist
2025

Tenosynovial Giant Cell Tumors of the Hand: Analysis of Risk Factors for Surgical Margin and Recurrence.

Medicina (Kaunas, Lithuania)
2025

Diffuse-Type Tenosynovial Giant Cell Tumor of the Knee: Clinical Course After Anterior Open Synovectomy.

Current oncology (Toronto, Ont.)
2025

Characterizing the metabolites of the tyrosine-kinase inhibitor pexidartinib in mouse feces, urine, plasma, and liver.

Journal of pharmaceutical and biomedical analysis
2025

The economic and humanistic burden of tenosynovial giant cell tumor: a targeted literature review.

Future oncology (London, England)
2025

Chondroid Diffuse Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Case of a Rare Maxillofacial Tumor With Unique Pathophysiology and Therapeutic Considerations.

Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons
2025

Microsurgical resection of tenosynovial giant cell tumor in the digits microsurgery for digital GCT.

Journal of hand and microsurgery
2025

Single-Cell RNA Sequencing Study on Two Synovial Derived Tumors in the Temporomandibular Joint.

Oral diseases
2025

Vimseltinib: A novel colony stimulating factor 1 receptor (CSF1R) inhibitor approved for treatment of tenosynovial giant cell tumors (TGCTs).

Intractable &amp; rare diseases research
2025

TURALIO® Risk Evaluation and Mitigation Strategy (tREMS) program: 5-year retrospective hepatic safety assessment.

Future oncology (London, England)
2025

Pigmented villonodular synovitis of the temporomandibular joint. evaluation of 12 patients from 2017 to 2023.

Oral and maxillofacial surgery
2025

Medical Management of Tenosynovial Giant Cell Tumor.

Current oncology reports
2025

A Novel Case of Tenosynovial Giant Cell Tumor at 4 Years After Total Knee Arthroplasty for Medial Osteoarthritis With Synovial Osteochondromatosis.

Case reports in orthopedics
2025

Case Report: Giant cell lesions in the Maxillofacial region: diagnostic points and treatment strategies.

Frontiers in oncology
2025

MR Imaging Features of Malignant Tenosynovial Giant Cell Tumors.

Academic radiology
2025

Large Tenosynovial Giant Cell Tumor Treated with Hip Arthroscopy in the Setting of Osteoarthritis: 13-Year Outcomes with Annual MRI Surveillance: A Case Report.

JBJS case connector
2025

A Case Report on an Uncommon Presentation of Giant Cell Tumor of the Tendon Sheath in the Infrapatellar Region.

Cureus
2025

Vimseltinib (Romvimza) for tenosynovial giant cell tumor.

The Medical letter on drugs and therapeutics
2025

Tenosynovial Giant Cell Tumor Involving Quadriceps Muscle - A Case Report of Two Cases.

Journal of orthopaedic case reports
2025

Localized and diffuse tenosynovial giant cell tumor: real-world results from a patient observational registry.

Future oncology (London, England)
2025

Patient-reported continued benefits of pexidartinib for tenosynovial giant cell tumor based on a real-world study in the United States.

The oncologist
2025

Advancing Preoperative Diagnosis: Case Report and Imaging Analysis of Cervical Spine Tenosynovial Giant Cell Tumor.

The American journal of case reports
2025

Tenosynovial giant cell tumor as an uncommon cause of persistent hip pain.

Pain practice : the official journal of World Institute of Pain
2025

Nosologic Reappraisal of the Recently Proposed Calcified Chondroid Mesenchymal Neoplasm Concept in a Series of 20 Cases.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2025

Tenosynovial Giant Cell Tumor and Pigmented Villonodular Synovitis.

The Journal of the American Academy of Orthopaedic Surgeons
2025

Single-Cell Analysis Clarifies Pathological Heterogeneity in Tenosynovial Giant Cell Tumor and Identifies Biomarkers for Predicting Disease Recurrence.

Advanced science (Weinheim, Baden-Wurttemberg, Germany)
2025

Recurrent diffuse tenosynovial giant cell tumor of the posterior knee: a rare presentation with synovial herniation (Baker's cyst) in a young patient.

BMC musculoskeletal disorders
2025

Necroptotic Suppression of Lung Cancer Cell Proliferation and Migration: A Comprehensive In Vitro and In Silico Study to Determine New Molecular Targets for Pexidartinib.

Cell biochemistry and function
2025

Mismatch Between Preoperative MRI Findings and Postoperative Histological Results in the Treatment of Tenosynovial Giant Cell Tumor.

Anticancer research
2025

Therapeutic advances in Tenosynovial giant cell Tumor: Targeting the CSF1/CSF1R axis.

Cancer treatment reviews
2025

An evaluation of vimseltinib for treatment of tenosynovial giant cell tumors.

Expert review of anticancer therapy
2025

Fibroma of Tendon Sheath Revisited.

In vivo (Athens, Greece)
2025

A Rare Cutaneous Presentation of Malignant Tenosynovial Giant Cell Tumor.

The American Journal of dermatopathology
2025

Diagnostic Challenge of Localized Tenosynovial Giant Cell Tumor in Children.

Diagnostics (Basel, Switzerland)
2025

Giant Cell Tumor of Flexor Tendon Sheath: An Interesting Clinical Presentation as Subungual Growth.

Skin appendage disorders
2024

Recent advances in immunohistochemical and molecular profiling for differential diagnosis between giant cell-rich lesions and tenosynovial giant cell tumors.

Frontiers in oncology
2025

Peficitinib suppresses diffuse-type tenosynovial giant cell tumor by targeting TYK2 and JAK/STAT signaling.

Science China. Life sciences
2025

Recurrent Diffuse Tenosynovial Giant Cell Tumor of the Temporomandibular Joint.

Head and neck pathology
2024

Treatment of Tenosynovial Giant Cell Tumor of the Cervical Spine with Postoperative Anti-RANKL Antibody (Denosumab) Administration.

Acta medica Okayama
2024

Emerging therapeutics in the management of tenosynovial giant cell tumor (TGCT).

Expert review of anticancer therapy
2025

Tenosynovitis with psammomatous calcifications: Series of 18 cases and review of the literature emphasizing a common source of expert consultation and updated differential diagnosis.

Virchows Archiv : an international journal of pathology
2025

A phase 4, multicenter, global clinical study to evaluate discontinuation and rechallenge of pexidartinib in patients with tenosynovial giant cell tumor previously treated with pexidartinib.

Cancer
2024

Is intercalary frozen autograft augmented with intramedullary cement and bridging plates fixation a durable reconstruction?

Journal of orthopaedic surgery and research
2024

Treatment Modalities for Refractory-Recurrent Tenosynovial Giant Cell Tumor (TGCT): An Update.

Medicina (Kaunas, Lithuania)
2025

Chronic hearing loss turns out being a calcified chondroid mesenchymal neoplasm with FN1::FGFR2 fusion.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2024

Case report: Tenosynovial giant cell tumor.

Frontiers in oncology
2024

Vimseltinib versus a placebo in patients with tenosynovial giant cell tumor: a plain language summary of the MOTION phase 3 trial.

Future oncology (London, England)
2024

Patient Outcomes After Resection of Pigmented Villonodular Synovitis in Patients in King Abdulaziz Medical City.

Cureus
2024

Localized type tenosynovial giant cell tumor with metastases to lungs and pleura: a case report and literature review.

Journal of medical case reports
2024

[Synovial chondromatosis : Results from the histopathological arthritis register of the German Society for Orthopedic Rheumatology].

Zeitschrift fur Rheumatologie
2026

MANEUVER: A Phase III study of pimicotinib to assess efficacy and safety in tenosynovial giant cell tumor patients.

Future oncology (London, England)
2024

Atypical Presentation of Tenosynovial Giant Cell Tumor on the Hallucal Flexor Tendon Sheath: A Case Report.

Journal of the American Podiatric Medical Association
2024

Chondroid Synoviocytic Neoplasm: A Clinicopathologic, Immunohistochemical, and Molecular Genetic Study of a Distinctive Tumor of Synoviocytes.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2024

Malignant tenosynovial giant cell tumor: A case report.

Asian journal of surgery
2024

Pexidartinib Upfront in a Case of Tenosynovial Giant Cell Tumor: Proof of Concept for a Treatment Paradigm Shift.

Oncology and therapy
2024

Diffuse-Type Tenosynovial Giant Cell Tumor An Update of Current Management Strategies.

Bulletin of the Hospital for Joint Disease (2013)
2024

Tenosynovial Giant Cell Tumor in the Gruberi Bursa.

Balkan medical journal
2024

Multiportal Arthroscopy-assisted Surgical Resection of Diffuse-type Tenosynovial Giant Cell Tumors in the Ankle Joint Yields Favorable Clinical Outcomes: A Retrospective Study.

Orthopaedic surgery
2024

Localized Tenosynovial Giant Cell Tumor After Total Knee Arthroplasty.

Arthroplasty today
2024

Peripheral Vascular Compression in a Patient With Diffuse Tenosynovial Giant Cell Tumor of the Knee: A Case Report Focusing on the Role of the Ultrasound.

Cureus
2024

TURALIO® Risk Evaluation and Mitigation Strategy Program (tREMS): 3-year retrospective hepatic safety assessment.

Future oncology (London, England)
2024

ISLET-1 expression in soft tissue neoplasms reveals high sensitivity but moderate specificity for desmoplastic small round cell tumors and potential utility as a diagnostic biomarker.

Pathology, research and practice
2024

Comprehensive Molecular Characterization of a Large Series of Calcified Chondroid Mesenchymal Neoplasms Widening Their Morphologic Spectrum.

The American journal of surgical pathology
2024

CSF1R Inhibition in Patients with Advanced Solid Tumors or Tenosynovial Giant Cell Tumor: A Phase I Study of Vimseltinib.

Clinical cancer research : an official journal of the American Association for Cancer Research
2024

Fine-needle aspiration cytology of diffuse type tenosynovial giant cell tumor with malignant trasformation and review of literature.

Diagnostic cytopathology
2024

Cytoplasmic PPARγ Significantly Correlates With P53 Immunohistochemical Expression and Tumor Size in Localized Tenosynovial Giant Cell Tumor.

Cureus
2024

Incidental Abdominal Wall Endometriosis in Plastic Surgery: Two Cases and Review of the Literature.

Plastic and reconstructive surgery. Global open
2024

Giant Cell Tumor of Soft Tissue: An Updated Review.

Journal of clinical medicine
2024

Tenosynovial giant cell tumor: case report and molecular investigation.

Oral surgery, oral medicine, oral pathology and oral radiology
2024

Giant Cell Tumor in Tarsal Midfoot Bones: A Case Report.

Cureus
2024

Case report: Primary sarcoma of the mandible with a novel SLMAP-BRAF fusion.

Frontiers in oncology
2024

Recurrent Localized Tenosynovial Giant Cell Tumor of the Left Ring Finger: A Case Report and Literature Review.

Cureus
2024

MRgFUS ablation of a recurrent tenosynovial giant cell tumor in the foot using ExAblate 2100 system in combination with patient immobilization device.

Radiography (London, England : 1995)
2024

Thighplasty at the Time of Stage-1 Bone-Anchored Osseointegration Surgery.

JBJS essential surgical techniques
2024

Intra-Articular Nodular Fasciitis of the Knee, A Rare Clinical Presentation and Histological Diagnosis: A Case Report.

Orthopedic reviews
2024

Invasion Patterns and Long-Term Clinical Outcomes of Diffuse Tenosynovial Giant Cell Tumor of the Ankle Joint.

Cureus
2024

A Case Report of Diffuse-type Tenosynovial Giant Cell Tumor as a Calcaneus Mass: A Diagnostic Challenge.

Current medical imaging
2024

Physiologically Based Pharmacokinetic Absorption Model for Pexidartinib to Evaluate the Impact of Meal Contents and Intake Timing on Drug Exposure.

Clinical pharmacology in drug development
2024

Real-world drug utilization and treatment patterns in patients with tenosynovial giant cell tumors in the USA.

Future oncology (London, England)
2024

Calcified Chondroid Mesenchymal Neoplasms.

Surgical pathology clinics
2024

Histiocytic sarcoma mimicking localized tenosynovial giant cell tumor in the pediatric foot: A rare case report with MRI Findings.

Radiology case reports
2024

Diffuse-Type Tenosynovial Giant Cell Tumor: What Are the Important Findings on the Initial and Follow-Up MRI?

Cancers
2024

Arthroscopic Excision of a Localized Tenosynovial Giant Cell Tumor of the Knee: A Case Report.

Current rheumatology reviews
2024

Active surveillance of diffuse-type tenosynovial giant cell tumors: A retrospective, multicenter cohort study.

European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
2023

A Rare Case of Giant Cell Tumour of Tendon Sheath and Its Arthroscopic In Toto Excision.

Cureus
2024

Unmasking Coincident Hodgkin Lymphoma and Giant Cell Tumor: Insights from [18F] FDG PET/CT.

Asia Oceania journal of nuclear medicine &amp; biology
2024

Medium-term Outcomes of Excision Using Surgical Microscope of Tenosynovial Giant Cell Tumors of the Hand.

Anticancer research
2024

What Are the Recurrence Rates, Complications, and Functional Outcomes After Multiportal Arthroscopic Synovectomy for Patients With Knee Diffuse-type Tenosynovial Giant-cell Tumors?

Clinical orthopaedics and related research
2023

[Diagnosis and management of tenosynovial giant cell tumor].

Revue medicale suisse
2023

Exploring the Effect of Compound Glycyrrhizin and Silybinin on the Metabolism of Pexidartinib in Rats Based on CYP3A4 and CYP2C9.

Advances in pharmacological and pharmaceutical sciences
2023

Computational exploration of the significance of COPS6 in cancer: Functional and clinical relevance across tumor types.

World journal of clinical oncology
2023

Oncological and functional outcomes of modified arthroscopic resection for intra-articular tenosynovial giant cell tumor of the knee using multiple portals.

Journal of orthopaedic surgery (Hong Kong)
2023

Unveiling the Enigma: Uncommon Hand Giant Cell Tumor within the Tendon Sheath.

Journal of orthopaedic case reports
2023

Uncommon Presentation of a Giant Cell Tumor of the Tendon Sheath of the Hand: A Case Report.

Cureus
2024

A Rare Clinical Presentation With a Difficult Imaging Diagnosis of an Intra-articular Clear Cell Sarcoma of the Knee.

Clinical nuclear medicine
2024

CSF1 expression in xanthogranulomatous epithelial tumor/keratin-positive giant cell-rich tumor.

Human pathology
2023

Outcomes of arthroscopic elbow synovectomy and neurolysis of the ulnar nerve for tenosynovial giant cell tumor in a young athlete: a case report and literature review.

JSES international
2024

Synovial chondromatosis: Rare involvement of metacarpophalangeal joint and diagnostic challenges in MR imaging.

Skeletal radiology
2023

Clinical retrospective analysis with a predictive model for diffused-tenosynovial giant cell tumors of the temporomandibular joint.

BMC cancer
2024

Real-World Patient Experience of Pexidartinib for Tenosynovial Giant-Cell Tumor.

The oncologist
2023

Supporting patients in the transition to the revised pexidartinib dosing regimen: perspectives from the multidisciplinary clinical and allied health professional team.

Orphanet journal of rare diseases
2023

Localized tenosynovial giant cell tumor in children.

Journal of children's orthopaedics
2023

Tenosynovial giant cell tumor: a case report.

Journal of medical case reports
2023

Bone SPECT/CT in Advanced Diffuse Tenosynovial Giant Cell Tumor of the Wrist.

Clinical nuclear medicine
2023

1HNMR-based serum metabolomic profiling for patients with diffuse-type tenosynovial giant cell tumor.

Asian journal of surgery
2023

Diffuse-type tenosynovial giant cell tumor between the suboccipital bone and posterior C1 arch: illustrative case.

Journal of neurosurgery. Case lessons
2023

Outcomes of Tenosynovial Giant Cell Tumor of the Foot and Ankle.

Foot &amp; ankle international
2024

[Localized tenosynovial giant cell tumor : Results from the Histopathological Arthritis Register of the German Society for Orthopedic Rheumatology].

Zeitschrift fur Rheumatologie
2024

The MOTION study: a randomized, phase III study of vimseltinib for the treatment of tenosynovial giant cell tumor.

Future oncology (London, England)
2023

Recurrent diffuse tenosynovial giant cell tumor of the knee treated with adjuvant radiotherapy: A case report.

International journal of surgery case reports
2023

Distal Dorsal Thumb Mass: Giant Cell Tumor of the Tendon Sheath in an Unusual Location.

Cureus
2023

Current therapies and future prospective for locally aggressive mesenchymal tumors.

Frontiers in oncology
2023

Multimodal management of tenosynovial giant cell tumors (TGCT) in the landscape of new druggable targets.

Journal of surgical oncology
2023

Identifying the Reactive Metabolites of Tyrosine Kinase Inhibitor Pexidartinib In Vitro Using LC-MS-Based Metabolomic Approaches.

Chemical research in toxicology
2023

Imaging characteristics of tenosynovial giant cell tumors on 18F-fluorodeoxyglucose positron emission tomography/computed tomography: a retrospective observational study.

BMC musculoskeletal disorders
2026

Surgical Techniques and Functional Reconstruction for Complex Tenosynovial Giant Cell Tumor of Temporal Bone and Middle Skull Base.

Ear, nose, &amp; throat journal
2023

T2-star (T2*)-weighted magnetic resonance imaging of tenosynovial giant cell tumors.

European journal of radiology open
2023

Diffuse Tenosynovial Giant Cell Tumor Encircling the First Metatarsal Bone Remodeling after Resection. A Case Report.

Journal of orthopaedic case reports
2023

Updates on the Treatment of Tenosynovial Giant Cell Tumor.

Hematology/oncology and stem cell therapy
2023

Prediction of local recurrence in tenosynovial giant cell tumor of the knee: Based on preoperative MRI evaluation into disease subtypes and severity.

PloS one
2023

Tenosynovial Giant Cell Tumor (TSGCT) of the hip: MRI accuracy and results of surgical treatment.

Acta orthopaedica Belgica
2023

Total Synovectomy and Bone Grafting/Cementation after Curettage of the Bone Lesion in Diffuse‏ Type ‏of Tenosynovial‏ Giant Cell Tumor‎: A Retrospective Cohort Study.

The archives of bone and joint surgery
2024

T2 Black Synovitis in Musculoskeletal MRI: Disease Spectrum and Imaging Characteristics of Joint Diseases.

Current medical imaging
2023

A rare case of long-term joint swelling caused by pigmented villonodular synovitis in a 3-year-old girl: a case report.

Frontiers in surgery
2023

Understanding the Effect of Osteoarthritis on Surgical Treatment Patterns, Healthcare Resource Utilization, and Costs Among Patients With Tenosynovial Giant Cell Tumors.

Journal of the American Academy of Orthopaedic Surgeons. Global research &amp; reviews
2023

Arthroscopic resection of localized tenosynovial giant cell tumor in the deep infrapatellar bursa: a case report.

Croatian medical journal
2023

18F-FDG PET/CT image of tenosynovial giant cell tumor in the thoracic vertebra.

Hellenic journal of nuclear medicine
2023

Image findings of tendon sheaths affected by diffuse tenosynovial giant cell tumors of the skull base.

European review for medical and pharmacological sciences
2023

Dosing Recommendation Based on the Effects of Different Meal Types on Pexidartinib Pharmacokinetics in Healthy Subjects: Implementation of Model-informed Drug Development Strategy.

Clinical pharmacology in drug development
2023

Tenosynovial Giant Cell Tumor of Foot in a Patient with Radioiodine-Refractory Thyroid Cancer: Imaging Findings on FDG-PET/CT, MRI, and Radioiodine Scan.

World journal of nuclear medicine
2023

Tenosynovial Giant Cell Tumor Observational Platform Project (TOPP) Registry: A 2-Year Analysis of Patient-Reported Outcomes and Treatment Strategies.

The oncologist
2023

Effectiveness of arthroscopic excision based on the distribution of the tenosynovial giant cell tumor around knee joint.

The Knee
2022

Presurgical Perspective and Posttreatment Evaluation of Soft Tissue Tumors of the Ankle and Foot in Adults.

Seminars in musculoskeletal radiology
2022

Presurgical Perspective and Postsurgical Evaluation of Morton's Neuroma and Other Nerve Lesions.

Seminars in musculoskeletal radiology
2023

One-Stage Synovectomies Result in Improved Short-Term Outcomes Compared to Two-Stage Synovectomies of Diffuse-Type Tenosynovial Giant Cell Tumor (D-TGCT) of the Knee: A Multicenter, Retrospective, Cohort Study.

Cancers
2023

MRI Prediction Model for Tenosynovial Giant Cell Tumor with Risk of Diffuse-type.

Academic radiology
2022

Extensile Anterior and Posterior Knee Exposure for Complete Synovectomy of Diffuse Tenosynovial Giant Cell Tumor (Pigmented Villonodular Synovitis).

JBJS essential surgical techniques
2022

Surgical treatment of spinal tenosynovial giant cell tumor: Experience from a single center and literature review.

Frontiers in oncology
2023

Incidental Detection of a Tenosynovial Giant Cell Tumor of the Thigh on [68Ga]Ga-FAPI PET/CT: Presentation of an Unusual Case.

Journal of nuclear medicine : official publication, Society of Nuclear Medicine
2022

Exploration of comorbidity mechanisms and potential therapeutic targets of rheumatoid arthritis and pigmented villonodular synovitis using machine learning and bioinformatics analysis.

Frontiers in genetics
2022

Intra-Articular Tenosynovial Giant Cell Tumor Mimicking Septic Arthritis: A Report of Two Cases.

Journal of orthopaedic case reports
2023

Outcomes of Surgical Treatment for Localized Tenosynovial Giant-Cell Tumor of the Foot and Ankle: A Case Series.

Acta medica Lituanica
2023

Radiological and pathological characteristics of synovial hemangioma of the knee.

Experimental and therapeutic medicine
2022

Complementary Effects of Surgery and Pexidartinib in the Management of Patients with Complex Diffuse-Tenosynovial Giant Cell Tumor.

Case reports in orthopedics
2023

A novel colony-stimulating factor 1 (CSF1) translocation  involving human endogenous retroviral element in a tenosynovial giant cell tumor.

Genes, chromosomes &amp; cancer
2022

Role of colony-stimulating factor 1 in the neoplastic process of tenosynovial giant cell tumor.

Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine
2023

Long-term Outcomes of Diffuse or Recurrent Tenosynovial Giant Cell Tumor Treated with Postoperative External Beam Radiation Therapy.

Practical radiation oncology
2022

Lateral T-Capsulotomy with Hip Arthroscopy for Access and Excision of Posterior Intra-Articular Mass.

Arthroscopy techniques
2023

Novel CSF1R-positive tenosynovial giant cell tumor cell lines and their pexidartinib (PLX3397) and sotuletinib (BLZ945)-induced apoptosis.

Human cell
2022

Expanding the molecular spectrum of tenosynovial giant cell tumors.

Frontiers in oncology
2024

Use of neoadjuvant pexidartinib with limb salvage surgery for diffuse tenosynovial giant cell tumor: A case report.

Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association
2022

Localized Pigmented Villonodular Synovitis of the Knee Joint: A Case Report.

Journal of orthopaedic case reports
2023

CSF1R inhibitors are emerging immunotherapeutic drugs for cancer treatment.

European journal of medicinal chemistry
2022

Diagnostic utility of CSF1 immunohistochemistry in tenosynovial giant cell tumor for differentiating from giant cell-rich tumors and tumor-like lesions of bone and soft tissue.

Diagnostic pathology
2022

FNAC study of giant cell tumor of tendon sheath (localized tenosynovial giant cell tumor): Clinico-radiological correlation and cytopathological features.

Diagnostic cytopathology
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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Pediatric patients with tenosynovial giant cell tumor: real-world evidence from an observational registry.
    Orphanet journal of rare diseases· 2026· PMID 41612360mais citado
  2. TGCT of the shoulder - a case series and literature review.
    Archives of orthopaedic and trauma surgery· 2026· PMID 41801507mais citado
  3. Tenosynovial Giant Cell Tumor With Bone Erosion of the Tibial Midshaft: A Case Report.
    JBJS case connector· 2026· PMID 41785371mais citado
  4. Soft tissue and juxtaarticular tumors of the knee.
    Skeletal radiology· 2026· PMID 41706083mais citado
  5. Bilateral Localized-Tenosynovial Giant Cell Tumor of the Knee: Case Report and Review.
    Journal of clinical medicine· 2026· PMID 41682697mais citado
  6. Functional Outcomes and Recurrence After Arthroscopy-Based Treatment of Pigmented Villonodular Synovitis of the Knee: A 20-Year Single-Center Series.
    Cancers (Basel)· 2026· PMID 41976400recente
  7. Tenosynovial Giant Cell Tumor masked as de Quervain Tenosynovitis.
    Acta Orthop Belg· 2025· PMID 41928761recente
  8. Pexidartinib: Current advances in the symptomatic treatment of tenosynovial giant cell tumors.
    Cancer Metastasis Rev· 2026· PMID 41922828recente
  9. [Vimseltinib in non-resecable tenosynovial giant cell tumor].
    Bull Cancer· 2026· PMID 41791925recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:66627(Orphanet)
  2. MONDO:0024686(MONDO)
  3. GARD:7396(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q3768153(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Tumor tenossinovial de células gigantes
Compêndio · Raras BR

Tumor tenossinovial de células gigantes

ORPHA:66627 · MONDO:0024686
Prevalência
1-5 / 10 000
Herança
Not applicable
CID-10
M12.2 · Sinovite vilonodular (pigmentada)
Ensaios
10 ativos
Início
Adolescent, Adult, Childhood
Prevalência
20.0 (Europe)
MedGen
UMLS
C0039106
EuropePMC
Wikidata
Wikipedia
Papers 10a
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