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

BACKGROUND: Pexidartinib (Turalio) is the only systemic therapy approved by the FDA for the treatment of adult patients with symptomatic tenosynovial giant-cell tumor (TGCT) associated with severe morbidity or functional limitations, and not amenable to improvement with surgery. This study assessed patient-reported treatment experiences and symptom improvement among patients receiving pexidartinib. METHODS: A cross-sectional, web-based survey collected data on demographics, disease history, pexidartinib dosing, and symptoms before and after pexidartinib use. RESULTS: Of 288 patients enrolled in the Turalio REMS program in May 2021, 83 completed the survey: mean age was 44.2 years, 62.7% were female, and most common tumor sites were in knee (61%) and ankle (12%). Mean initial dose was 622 mg/day: 29 patients reported reduction from initial dose and 8 had dose reduction after titrating up to a higher dose. At the time of survey completion, median time on pexidartinib was 6.0 months; 22 (26.5%) patients discontinued pexidartinib due to physician suggestion, abnormal laboratory results, side effect, or symptom improvement. Compared with before pexidartinib initiation, most patients reported improvement in overall TGCT symptom (78.3%) and physical function (77.2%) during pexidartinib treatment. Significant improvement was reported during pexidartinib treatment in worst stiffness numeric rating scale (NRS) (3.0 vs. 6.2, P < .05) and worst pain NRS (2.7 vs. 5.7, P < .05). CONCLUSION: Findings from this cross-sectional survey confirmed the benefit of pexidartinib in improving symptoms and functional outcomes among patients with symptomatic TGCTs from the patients' perspective. Future research is warranted to examine the long-term benefit and risk of pexidartinib.

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

BACKGROUND: Diffuse-type tenosynovial giant-cell tumor (D-TGCT), formerly known as pigmented villonodular synovitis, is a rare, locally aggressive, invasive soft tissue tumor that primarily occurs in the knee. Surgical excision is the main treatment option, but there is a high recurrence rate. Arthroscopic surgical techniques are emphasized because they are less traumatic and offer faster postoperative recovery, but detailed reports on arthroscopic techniques and outcomes of D-TGCT in large cohorts are still lacking. QUESTIONS/PURPOSES: (1) What is the recurrence rate of knee D-TGCT after multiportal arthroscopic synovectomy? (2) What are the complications, knee ROM, pain score, and patient-reported outcomes for patients, and do they differ between patients with and without recurrence? (3) What factors are associated with recurrence after arthroscopic treatment in patients with D-TGCT? METHODS: In this single-center, retrospective study conducted between January 2010 and April 2021, we treated 295 patients with knee D-TGCTs. We considered patients undergoing initial surgical treatment with multiportal arthroscopic synovectomy as potentially eligible. Based on that, 27% (81 of 295) of patients were excluded because of recurrence after synovectomy performed at another institution. Of the 214 patients who met the inclusion criteria, 17% (36 of 214) were lost to follow-up, leaving 83% (178 of 214) of patients in the analysis. Twenty-eight percent (50 of 178) of patients were men and 72% (128 of 178) were women, with a median (range) age of 36 years (7 to 69). The median follow-up duration was 80 months (26 to 149). All patients underwent multiportal (anterior and posterior approaches) arthroscopic synovectomy, and all surgical protocols were determined by discussion among four surgeons after preoperative MRI. A combined open posterior incision was used for patients with lesions that invaded or surrounded the blood vessels and nerves or invaded the muscle space extraarticularly. Standard postoperative adjuvant radiotherapy was recommended for all patients with D-TGCT who had extraarticular and posterior compartment invasion; for patients with only anterior compartment invasion, radiotherapy was recommended for severe cases as assessed by the surgeons and radiologists based on preoperative MRI and intraoperative descriptions. Postoperative recurrence at 5 years was calculated using a Kaplan-Meier survivorship estimator. The WOMAC score (0 to 96, with higher scores representing a worse outcome; minimum clinically important difference [MCID] 8.5), the Lysholm knee score (0 to 100, with higher scores being better knee function; MCID 25.4), the VAS for pain (0 to 10, with higher scores representing more pain; MCID 2.46), and knee ROM were used to evaluate functional outcomes. Because we did not have preoperative patient-reported outcomes scores, we present data on the proportion of patients who achieved the patient-acceptable symptom state (PASS) for each of those outcome metrics, which were 14.6 of 96 points on the WOMAC, 52.5 of 100 points on the Lysholm, and 2.32 of 10 points on the VAS. RESULTS: The symptomatic or radiographically documented recurrence at 5 years was 12% (95% confidence interval [CI] 7% to 17%) using the Kaplan-Meier estimator, with a mean recurrence time of 33 ± 19 months. Of these, three were asymptomatic recurrences found during regular MRI reviews, and the remaining 19 underwent repeat surgery. There was one intraoperative complication (vascular injury) with no effect on postoperative limb function and eight patients with postoperative joint stiffness, seven of whom improved with prolonged rehabilitation and one with manipulation under anesthesia. No postradiotherapy complications were found. The proportion of patients who achieved the preestablished PASS was 99% (176 of 178) for the VAS pain score, 97% (173 of 178) for the WOMAC score, and 100% (178 of 178) for the Lysholm score. A lower percentage of patients with recurrence achieved the PASS for WOMAC score than patients without recurrence (86% [19] versus 99% [154], OR 0.08 [95% CI 0.01 to 0.52]; p = 0.01), whereas no difference was found in the percentage of VAS score (95% [21] versus 99% [155], OR 0.14 [95% CI 0.01 to 2.25]; p = 0.23) or Lysholm score (100% [22] versus 100% [156], OR 1 [95% CI 1 to 1]; p = 0.99). Moreover, patients in the recurrence group showed worse knee flexion (median 135° [100° to 135°] versus median 135° [80° to 135°]; difference of medians 0°; p = 0.03), worse WOMAC score (median 3.5 [0 to 19] versus median 1 [0 to 29]; difference of medians 2.5; p = 0.01), and higher VAS pain score (median 1 [0 to 4] versus median 0 [0 to 4]; difference of medians 1; p < 0.01) than those in the nonrecurrence group, although no differences reached the MCID. No factors were associated with D-TGCT recurrence, including the use of postoperative radiotherapy, surgical technique, and invasion extent. CONCLUSION: This single-center, large-cohort retrospective study confirmed that multiportal arthroscopic surgery can be used to treat knee D-TGCTs with a low recurrence rate, few complications, and satisfactory postoperative outcomes. Surgeons should conduct a thorough preoperative evaluation, meticulous arthroscopic synovectomy, and regular postoperative follow-up when treating patients with D-TGCT to reduce postoperative recurrence. Because the available evidence does not appear to fully support the use of postoperative adjuvant radiotherapy in all patients with D-TGCTs and our study design is inadequate to resolve this controversial issue, future studies should look for more appropriate indications for radiotherapy, such as planning based on a more precise classification of lesion invasion. LEVEL OF EVIDENCE: Level III, therapeutic study.

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

Background and Objectives: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive, benign neoplasm arising from the synovium of joints, tendon sheaths, and bursa. There are two main subtypes of TGCT: localized-type TGCT(L-TGCT) and diffuse-type TGCT (D-TGCT). While surgical excision is still considered the gold standard of treatment, the high recurrence rate, especially for D-TGCT, may suggest the need for other treatment modalities. Materials and Methods: This study reviews current literature on the current treatment modalities for refractory-relapsed TGCT disease. Results: The gold standard of treatment modality in TGCT remains surgical excision of the tumor nevertheless, the elevated recurrence rate and refractory disease, particularly in D-TGCT indicates and underscores the necessity for additional treatment alternatives. Conclusions: TGCT is a benign tumor with inflammatory features and a potential destructive and aggressive course that can lead to significant morbidity and functional impairment with a high impact on quality of life. Surgical resection remains the gold standard current treatment and the optimal surgical approach depends on the location and extent of the tumor. Systemic therapies have been recently used for relapsed mainly cases.

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

BACKGROUND: The Tenosynovial giant cell tumor Observational Platform Project (TOPP) registry is an international prospective study that -previously described the impact of diffuse-type tenosynovial giant cell tumour (D-TGCT) on patient-reported outcomes (PROs) from a baseline snapshot. This analysis describes the impact of D-TGCT at 2-year follow-up based on treatment strategies. MATERIAL AND METHODS: TOPP was conducted at 12 sites (EU: 10; US: 2). Captured PRO measurements assessed at baseline, 1-year, and 2-year follow-ups were Brief Pain Inventory (BPI), Pain Interference, BPI Pain Severity, Worst Pain, EQ-5D-5L, Worst Stiffness, and -Patient-Reported Outcomes Measurement Information System. Treatment interventions were no current/planned treatment (Off-Treatment) and systemic treatment/surgery (On-Treatment). RESULTS: A total of 176 patients (mean age: 43.5 years) were included in the full analysis set. For patients without active treatment strategy -(Off-Treatment) at baseline (n = 79), BPI Pain Interference (1.00 vs. 2.86) and BPI Pain Severity scores (1.50 vs. 3.00) were numerically favorable in patients remaining Off-Treatment compared with those who switched to an active treatment strategy at year 1. From 1-year to 2-year -follow-ups, patients who remained Off-Treatment had better BPI Pain Interference (0.57 vs. 2.57) and Worst Pain (2.0 vs. 4.5) scores compared with patients who switched to an alternative treatment strategy. In addition, EQ-5D VAS scores (80.0 vs. 65.0) were higher in patients who remained -Off-Treatment between 1-year and 2-year follow-ups compared with patients who changed treatment strategy. For patients receiving systemic treatment at baseline, numerically favorable scores were seen in patients remaining on systemic therapy at 1-year follow-up: BPI Pain Interference (2.79 vs. 5.93), BPI Pain Severity (3.63 vs. 6.38), Worst Pain (4.5 vs. 7.5), and Worst Stiffness (4.0 vs. 7.5). From 1-year to 2-year follow-up, EQ-5D VAS scores (77.5 vs. 65.0) were higher in patients who changed from systemic treatment to a different treatment strategy. CONCLUSION: These findings highlight the impact D-TGCT has on patient quality of life, and how treatment strategies may be influenced by these outcome measures. (ClinicalTrials.gov number: NCT02948088).

Giant cell tumor of tendon sheath: A report of 216 cases.

BACKGROUND: In this article on giant cell tumor of tendon sheath (GCTTS), we intend to summarize and analyze the clinical and pathological features of GCTTS hoping to improve clinical management and patient treatment. METHODS: The study retrospectively reviewed 216 patients of GCTTS, registered at the Affiliated Hospital of Southwest Medical University from January 2010 to December 2020. These cases were diagnosed by surgical excision. The clinicopathological features and the prognosis were reviewed in the light of the current literature. RESULTS: Of these 216 GCTTS patients, 72 were males (33.3%) and 144 females (66.7%), with a ratio male-to-female of 1:2. The patients' age ranged from 5 to 82, the average being 41.5 years at diagnosis. A total of 96 cases (44.4%) occurred in the hand region, followed by 35 cases (16.2%) in the knee, 32 cases (14.8%) in the foot, 25 cases (11.6%) in the ankle, 12 cases (5.6%) in the wrist, 12 cases (5.6%) in the leg, 2 cases (0.9%) in the head, 1 case (0.5%) in the forearm, and 1 case (0.5%) inside and outside the spinal channel. Histopathology mainly revealed large synovial-like monocytes, small monocytes, and osteoclast-like giant cells. CONCLUSION: Our results confirm that GCTTS predominantly occurs in the hands of young women. Complete surgical resection with long-term follow-up is the preferred management.

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

Tenosynovial giant cell tumor (TGCT) is a rare disease characterized by the proliferation of the synovial membrane of a joint, tendon sheath, or bursa. TGCTs in joints are subdivided into the diffuse or localized type. The localized TGCT most frequently affects the knee and may occur in any knee compartment. The most common localization is the Hoffa's fat pad, followed by the suprapatellar pouch and the posterior capsule. Here, we describe a case of a histopathologically proven TGCT of the knee, found in an unusual localization in the deep infrapatellar bursa, which was diagnosed by magnetic resonance imaging. The tumor was entirely arthroscopically resected. The patient had no further complaints following the operation, and there was no recurrence at the 18-month follow-up. Even though TGCT of the knee is uncommon, it should not be overlooked by orthopedic and trauma surgeons, and excision should be regarded as a reliable treatment option. The form of surgical treatment, either open or arthroscopic, should be determined based on a combination of the surgeon's preference and the best approach to the anatomical location of the disease.

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

Tenosynovial Giant Cell Tumor (TSGCT) or formerly pigmented villonodular synovitis (PVNS) is a rare nonmalignant tumor of the synovia seldom affecting the hip. MRI and surgical resection are the gold standards in its diagnosis and treatment. However, the accuracy of MRI is unknown, and only few reports on its surgical treatment results exist. The goal of the study was to investigate the MRI accuracy, results after surgical treatment, and natural history of untreated MRI-diagnosed hip TSGCT. Twenty-four consecutive patients with suspected TSGCT on hip MRI, between December 2006 and January 2018, were identified from our medical database. Six refused to participate. About 18 patients with a minimal follow-up of 18 months were enrolled. Charts were reviewed for histopathology results, specific treatment and recurrence. At the last follow-up, all patients had a clinical (Harris Hip Score [HHS]) and radiological examination (x-ray and MRI). Out of 18 patients with suspected TSGCT on MRI, with a mean age of 35y (range 17-52), 14 had surgi- cal resection and 4 refused surgery 1 of whom had a CT-guided biopsy. Out of 15 cases with biopsies, in 10 TSGCT was confirmed. Three surgically-treated patients showed recurrence on MRI after 24, 31 and 43 months. Two non-treated patients showed progression after 18 and 116 months. At the last follow-up (65 m; range 18-159), the mean HHS with or without recurrence was 90 and 80pts (ns). Operative vs. non-operative treatment showed HHS of 86 and 90pts (ns). In the conservatively-treated group, HHS with and without progression was 98 and 82pts (ns), respectively. MRI-suspected TSGCT of the hip was confirmed with biopsy in two-thirds of the cases. 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.

[Diagnosis and management of tenosynovial giant cell tumor]. / Diagnostic et prise en charge de la tumeur à cellules géantes ténosynoviale.

Tenosynovial giant cell tumor is a benign condition that originates from synovial cells within joints, tendon sheaths, or bursae and may present either in localized (benign) or diffuse (locally aggressive) forms. Currently, the primary treatment approach is surgical, yielding satisfactory results with low recurrence rates in the localized forms, whereas the diffuse type displays high recurrence rates. In parallel, clinical trials are underway to explore pharmaceutical treatment options for the advanced diffuse type. This article aims at consolidating current knowledge about diagnosis and management of this rare tumor, additionally proposing a brief overview of novel therapeutic approaches.

La tumeur à cellules géantes ténosynoviale, bénigne, prend son origine dans les cellules synoviales des articulations, des gaines tendineuses ou des bourses et se présente dans une forme soit localisée (bénigne), soit diffuse (localement agressive). Le traitement principal est chirurgical, offrant des résultats satisfaisants à long terme, avec un faible risque de récidive dans la forme localisée, alors que le taux de récidives est élevé dans la forme diffuse. Parallèlement, des essais cliniques sont en cours pour explorer des options de traitement systémique pour les formes diffuses sévères. Cet article rappelle les connaissances actuelles pour le diagnostic et la prise en charge de cette tumeur rare. De plus, nous proposons un aperçu succinct des nouvelles approches thérapeutiques.

Treatment updates on tenosynovial giant cell tumor.

PURPOSE OF REVIEW: Diffuse-type tenosynovial giant cell tumor (dt-TGCT) is a benign clonal neoplastic proliferation arising from the synovium. Patients are often symptomatic, require multiple surgical procedures during their lifetime, and have reduced quality of life (QoL). Surgery is the main treatment with relapse rates ranging from 14 to 55%. The treatment strategy for patients with dt-TGCT is evolving. The purpose of this review is to describe current treatment options, and to highlight recent developments in the knowledge of the molecular pathogenesis of dt-TGCT as well as related therapeutic implications. RECENT FINDINGS: TGCT cells overexpress colony-stimulating factor 1 (CSF1), resulting in recruitment of CSF1 receptor (CSF1R)-bearing macrophages that are polyclonal and make up the bulk of the tumor, has led to clinical trials with CSF1R inhibitors. These inhibitors include small molecules such as pexidatinib, imatinib, nilotinib, DCC-3014 (vimseltinib), and the monoclonal antibody RG7155 (emactuzumab). SUMMARY: In conclusion, D-TGCT impairs patients' QoL. The evidence that the pathogenetic loop of D-TGCT can be inhibited has changed the therapeutic armamentarium for this condition. Clinical trials of agents that target CSF1R are currently ongoing. All this new evidence should be taken into consideration within multidisciplinary management.

A prospective real-world study of the diffuse-type tenosynovial giant cell tumor patient journey: A 2-year observational analysis.

BACKGROUND AND OBJECTIVES: Diffuse-tenosynovial giant cell tumor (D-TGCT) is a rare, locally aggressive, typically benign neoplasm affecting mainly large joints, representing a wide clinical spectrum. We provide a picture of the treatment journey of D-TGCT patients as a 2-year observational follow-up. METHODS: The TGCT Observational Platform Project registry was a multinational, multicenter, prospective observational study at tertiary sarcoma centers spanning seven European countries and two US sites. Histologically confirmed D-TGCT patients were categorized as either those who remained on initial treatment strategy (determined at baseline visit) or those who changed treatment strategy with specific changes documented (e.g., systemic treatment to surgery) at the 1-year and/or 2-year follow-up visits. RESULTS: A total of 176 patients were assessed, mean diagnosis age was 38.4 (SD ± 14.6) years; most patients had a knee tumor (120/176, 68.2%). For the 2-year observation period, most patients (75.5%) remained on the baseline treatment strategy throughout, 54/79 patients (68.4%) remained no treatment, 30/45 patients (66.7%) remained systemic treatment, 39/39 patients (100%) remained surgery. Those who changed treatment strategy utilized multimodal treatment options. CONCLUSIONS: This is the first prospectively collected analysis to describe D-TGCT patient treatments over an extended follow-up and demonstrates the need for multidisciplinary teams to determine an optimal treatment strategy.

Surgical management of 144 diffuse-type TGCT patients in a single institution: A 20-year cohort study.

BACKGROUND AND OBJECTIVES: Surgery is the mainstay of treatment for tenosynovial giant cell tumors (TGCTs). However, achieving a cure through surgery alone remains challenging, especially for the diffuse-type (D-TGCT). METHODS: Our goal was to describe the surgical management of patients with D-TGCT related to large joints, treated between 2000 and 2020. We analyzed the effect of (in)complete resections and the presence of postoperative tumor (POT) on magnetic resonance imaging (MRI) on radiological and clinical outcomes. RESULTS: A total of 144 patients underwent open surgery for D-TGCT, of which 58 (40%) had treatment before. The median follow-up was 65 months. One hundred twenty-five patients underwent isolated open surgeries, in which 25 (20%) patients' D-TGCT was intentionally removed incompletely. POT presence on the first postoperative MRI was observed in 64%. Both incomplete resections and POT presence were associated with higher rates of radiological progression (73% vs. 44%; Kaplan-Meier [KM] analysis p = 0.021) and 59% versus 7%; KM analysis p < 0.001), respectively. Furthermore, patients with POT presence clinically worsened more often than patients without having POT (49% vs. 24%; KM analysis p = 0.003). CONCLUSIONS: D-TGCT is often resected incompletely and tumor presence is commonly observed on the first postoperative MRI, resulting in worse radiological and clinical outcomes. Therefore, surgeons should try to remove D-TGCT in toto and consider other multimodal therapeutic strategies.

A Rare Case of Diffuse-type Tenosynovial Giant Cell Tumor in a Teenager With Noonan Syndrome.

Noonan syndrome is a common autosomal dominant disorder associated with an increased risk of malignancy. We report a 16-year-old female with Noonan syndrome (KRAS gene variant, Q22R) and diffuse-type tenosynovial giant cell tumor, a proliferative disorder that has been rarely reported in this population. These tumors may represent a complication of the dysregulated RAS/MAPK signaling pathway that underlies Noonan syndrome. They lack typical clinical features, causing misdiagnosis and delays in management, which could lead to osseous invasion requiring more complicated surgical procedures. Increased awareness of this association will improve the clinical outcomes of patients with Noonan syndrome who develop diffuse-type tenosynovial giant cell tumors.

Localized tenosynovial giant cell tumor: a rare case of snapping hip.

We report on a 40-year-old male with a 9-month-long history of snapping of his right hip caused by a previously undescribed etiology of internal extra-articular snapping hip, namely due to a localized tenosynovial giant cell tumor. Both dynamic ultrasound evaluation and MRI proved to be crucial in the diagnosis of this rare entity. Auto-provocation of the snapping showed an anterior hip mass moving posteriorly to the psoas tendon which elucidated the pain and clicking sensation. Subsequent MRI demonstrated a peripheral low-intensity rim due to hemosiderin deposition around the synovial mass which is indicative for pigmented villonodular tenosynovitis. Treatment consisted of arthroscopic shaver burr resection. Immediately postoperatively, the snapping sensation could not be provoked anymore by the patient. The purpose of reporting on this case report is to emphasize several successive learning points. First, dynamic ultrasound aids in diagnosis and differentiation of the types of snapping hip. Second, specific MRI features are suggestive of tenosynovial giant cell tumor, recognizing these traits may prevent delayed diagnosis and subsequent aggravated clinical course. Third, localized pigmented villonodular tenosynovitis around the hip may present as an internal extra-articular snapping hip and is of consideration in the differential diagnosis of recurrent snapping hip.

Cervical spine tenosynovial giant cell tumor involving the atlantoaxial joint in a pediatric patient with medulloblastoma.

Tenosynovial giant cell tumor (TGCT) is a benign condition that arises from tendon sheaths, synovium, or bursae and is classified according to the site of involvement (intra-articular versus extra-articular) and pattern of growth (localized versus diffuse). The diffuse form tends to present as peri-articular masses and are locally aggressive. It usually presents as a mono-articular process affecting larger joints. Spinal involvement is extremely rare, particularly the cervical spine. In this case report, we present a pediatric case of spinal TGCT involving the C1-C2 joint which was incidentally detected in a 13-year-old girl undergoing surveillance for medulloblastoma recurrence. Although spinal TGCT is a benign condition, it remains a diagnostic challenge, which specific to our case can raise the concern for malignancy or metastasis. We also described a percutaneous biopsy approach using a spring-loaded blunt tip coaxial needle to avoid inadvertent vascular injury. The imaging features of spinal TGCT and biopsy approach for atlantoaxial lesion are discussed together with a comprehensive review of the literature.

[MULTIPLE GIANT CELL TUMOR OF TENDON SHEATH: A CASE REPORT OF THREE LESIONS ON THE SAME FLEXOR TENDON].

INTRODUCTION: We present a case report of a triple location Giant Cell Tumor of tendon sheath appearance on the same flexor tendon sheath of a single digit. There have been scarce descriptions of multiple Giant Cell Tumors of tendon sheath. Multiple tumors may predispose patients to a higher recurrence rate; therefore, recognition and treatment of this rare entity is important.

[Imminent fracture of the tibia due to an osteolytic lesion caused by an intraosseous tenosynovial giant cell tumor : An unusual clinico-histopathological constellation]. / Stabilitätsgefährdende Osteolyse der Tibia durch einen intraossären tenosynovialen Riesenzelltumor : Eine außergewöhnliche klinisch-histopathologische Konstellation.

The tenosynovial giant cell tumor is a rare disease of the joint mucosa, tendon sheaths and bursa. We report on the rare constellation of an intraosseous manifestation of the proximal tibia of a lower leg stump after Burgess amputation as a result of a locally uncontrollable tenosynovial giant cell tumor of the upper ankle. The curettage of the local findings and operative stabilization through an intramedullary composite osteosynthesis led to an early rehabilitation of the exoprosthesis care with regaining patient autonomy.

Tenosynovial giant cell tumor of the pes anserinus bursa with secondary involvement of a reconstructed autologous anterior cruciate ligament - A case report.

Tenosynovial giant cell tumor (TGCT) is defined by the World Health Organization (WHO) as a family of lesions most often arising from the synovium of joints, bursae and tendon sheaths. It is composed of synovial- like mononuclear cells, admixed with multinucleate giant cells, foam cells, siderophages and inflammatory cells (1). It can have various clinical manifestations, and is therefore subdivided in a diffuse and a localized/ nodular subtype. Furthermore, the lesions can have an intra- or extra-articular location. The purpose of this paper is to present the case of a 41-year-old male suffering from multifocal extra- and intra-articular TGCT of the right knee, with involvement of the pes anserinus bursa and an anterior cruciate ligament (ACL) autograft respectively. The ACL reconstruction was performed 11 years prior to the diagnosis of the TGCT, using tendons harvested from the pes anserinus. Our case illustrates the risk of transferring TGCT from an extra- to intra-articular location during ACL reconstruction, when using tendons of a pes anserinus prone to develop this condition. To our knowledge, no similar case was published in the literature so far.

Multiple systemic treatment options in a patient with malignant tenosynovial giant cell tumour.

Tenosynovial giant cell tumour (TGCT) is a group of rare soft tissues neoplasia affecting synovial joints, bursae and tendon sheaths and is classified as localized type or diffuse type. The diffuse type (TGCT-D), also known as 'pigmented villonodular (teno)synovitis' is characterized by local aggressivity, with invasion and destruction of adjacent soft-tissue structures, and high local recurrence rate. Radical surgery remains the standard therapy while adjuvant radiotherapy may help to control local spread. Malignant TGCT is characterized by high rate of local recurrences and distant metastasis. Few cases of malignant TGCT and very few evidences on systemic therapies are described in the literature, so, to date, no systemic treatment is approved for this rare disease. We report the case of a malignant TGCT patient treated with many different systemic therapies, including chemotherapy and tyrosine-kinase inhibitors, and performed a review of the literature on the systemic treatment options of this rare tumour.

Tenosynovial Giant Cell Tumor in an Infant.

In this report, the authors describe a child presenting at 6 months old with a rapidly expanding extracranial left temporal mass concerning for malignancy. The mass was successfully treated at 16 months with radical surgical excision. The patient was found to have a tenosynovial giant cell tumor, diffuse type, completely encased by the temporalis muscle. To our knowledge, this is the first report of a case of diffuse type tenosynovial giant cell tumor in the temporalis muscle, without articular involvement, presenting in an infant.