Tenosynovial giant cell tumor.

Tenosynovial Giant Cell Tumor (TGCT) is a group of typically benign lesions arising from the synovium of joints, bursae and tendon sheaths. Depending on their growth pattern and clinical course, they are divided into localized and diffuse types. It is predominantly caused by a mutation in the stromal cells of the synovial membrane leading to overexpression of the colony stimulating factor 1 that recruits CSF1R-expressing cells of the mononuclear phagocyte lineage into the tumor mass. The lesions contain mainly histiocyte-like and synovial cells accompanied by varying numbers of multinucleated giant cells, mononuclear cells, foam cells, inflammatory cells and hemosiderin deposits. The gold standard for detect- ing and monitoring the disease is MRI, where the characteristic hemosiderin accumulation can be best appreciated, but it is a histological examination that is most conclusive. The main treatment is surgical resection of all pathological tissue, but radio- and chemotherapy are also viable options for certain groups of patients.

Long-term functional outcomes of diffuse pigmented villonodular synovitis of knee: The role of adjuvant radiotherapy.

ABSTRACT: Diffuse pigmented villonodular synovitis (PVNS) of knee is a rare benign disease that has a destructive clinical course. Synovectomy and adjuvant radiotherapy (RT) have been reported as treatment options but literatures reporting functional outcomes were sparse. This study aimed to evaluate the long-term functional outcomes and disease control among treatment modalities through the 22 years of experience.A single-center database was searched for patients who received synovectomy of knee with the pathologic diagnosis of PVNS. General data, treatment modalities, and recurrent status were retrospectively collected from medical records. Functional outcomes were evaluated by Western Ontario and McMaster Universities Osteoarthritis Index through phone interviews by an independent orthopedist.From January 1995 to December 2017, 24 patients with diffuse PVNS of knee were identified, including 19 receiving open synovectomy (OP) and 5 undergoing arthroscopic surgery. Adjuvant RT was performed on 14 patients with a median dose of 35 Gy (range 20-40 Gy). After median follow up of 6 years, recurrences were recorded in 10 cases. The recurrence rate was significantly lower in the OP + RT group than the OP group (8.3% vs 57.1%, P = .038). Among those with preserved knee joints, there was no significant difference in the Western Ontario and McMaster Universities Osteoarthritis Index score and stiffness score between patients in the OP + RT and OP groups.For patients with diffuse PVNS of knee, the addition of moderate-dose adjuvant RT following OP provided excellent local control while maintaining good joint function with limited treatment-related morbidity. Our study emphasized the importance of moderate dose RT in diffuse PVNS of knee joint.

Management of Pigmented Villonodular Synovitis (PVNS): an Orthopedic Surgeon's Perspective.

PURPOSE OF REVIEW: Pigmented villonodular synovitis (PVNS) or tenosynovial giant cell tumor (TGCT) encompasses a wide spectrum of disease and is divided into localized and diffuse variants. Surgical resection remains the principal treatment for nearly all localized type disease and most diffuse type. Recent mechanistic understanding of the disease led to drug discovery that has opened new avenues for patients with recalcitrant disease. In this manuscript, we review the current treatment options for TGCT, presenting outcomes from traditional surgical approaches as well as those from nonsurgical approaches. RECENT FINDINGS: Arthroscopic and/or open surgery remains the mainstay of treatment for TGCT for the vast majority of patients. While radiosynoviorthesis and external beam radiation have been used for recalcitrant disease, recent understanding of the colony stimulating factor 1 receptor (CSF1R) pathway and its paracrine and autocrine role in TGCT has led to the development of targeted inhibitors. Their optimal role and efficacy are unclear due to limited number of high-quality studies and contradictory results; however, recent and ongoing studies suggest there may be a role for their use, especially in diffuse and/or refractory disease. Surgery remains the most common treatment for TGCT, however, there may be an increasing role for adjuvant therapies, including the new targeted agents. Weighing the side effects of these treatments against the symptomatic benefit on a patient-by-patient basis in this benign disease remains critical.

Identification of Patient Needs and Preferences in Pigmented Villonodular Synovitis (PVNS) Using a Qualitative Online Bulletin Board Study.

INTRODUCTION: Pigmented villonodular synovitis (PVNS), also known as giant-cell tumour of the tendon sheath (GCTT), is a rare, benign proliferative tumour affecting the inner lining of synovial joints and tendon sheets. Information on treatment needs of PVNS patients to inform drug development is currently scarce. We conducted an exploratory qualitative study with PVNS patients to generate insights into the objective and emotional aspects related to their medical journey and experiences of living with this disease. METHODS: A 4-day study using an online bulletin board (OBB), an asynchronous, online qualitative research platform, was conducted with patients recruited via physician referral who underwent screening questions to ensure eligibility for the study and willingness to participate. The discussion was moderated, was structured and allowed open answers in response to other participants' posts. RESULTS: Eleven patients (4 from the USA, 4 from the UK and 3 from Canada; 45% female), aged 28-57 years, suffering from PVNS for 2-27 years participated in the study. Key patient insights from the study were: (1) pain was the topmost, spontaneous thought that the participants associated with PVNS, constituting a significant emotional and psychological burden; (2) surgery (arthroscopy) did not completely ameliorate symptoms associated with PVNS, as the relapse rate was high in these patients; (3) PVNS has a substantial negative financial impact on patients, their families and the healthcare system; (4) orthopaedic specialists/surgeons predominantly managed PVNS, as surgery is currently the only therapeutic option. CONCLUSION: PVNS patients expressed an urgent need for a medical drug treatment, which can reduce pain, avoid relapses and provide an alternative to surgery, the current standard of care.

Pigmented villonodular synovitis of the temporomandibular joint: case report and the literature review for postoperative radiotherapy.

Pigmented villonodular synovitis (PVNS) is a benign proliferative disorder of the synovium that usually involves joints, tendon sheaths, and bursae. It presents rarely, however, in the temporomandibular joints (TMJs). This paper reports a 59-year-old female patient with PVNS of the TMJ and its clinico-pathologic features are discussed. The patient was treated with surgery and postoperative radiotherapy (PORT). Follow-up was conducted, and there were no recurrences, metastases, skin changes or joint stiffness noted. The main treatment of PVNS is surgical resection. However, postoperative radiotherapy is important for local control of extensive tumors or positive margins. We conducted a literature review for postoperative radiotherapy case reports related to PVNS of the TMJ.

Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report.

BACKGROUND/AIMS: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. METHODS: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. RESULTS: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. INTERVENTION: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. FOLLOW-UP: At the 16-month follow-up there was no evidence of disease recurrence. CONCLUSION: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.

Diagnostic Imaging and Management of Common Intra-articular and Peri-articular Soft Tissue Tumors and Tumorlike Conditions of the Knee.

Intra-articular (IA) and peri-articular (PA) tumors of the knee are frequently encountered by orthopaedic surgeons. Nonetheless, due to the possibility of great morbidity and potential mortality, it is important to recognize and differentiate between benign and malignant lesions in a timely manner. Therefore, the purpose of this article is to provide a concise, practical, and updated review of commonly encountered IA and PA tumors including intratendinous gout, synovial chondromatosis, schwannoma, pigmented villonodular synovitis, and synovial sarcoma, and a detailed description of differentiating features to include various imaging modalities.

The effects of radiosynoviorthesis in pigmented villonodular synovitis of the knee.

INTRODUCTION: Tenosynovial giant-cell tumor also known as pigmented villonodular synovitis (PVS) is a benign but aggressive synovial proliferative disease most often affecting the knee joint. The mainstay of therapy is surgical resection. Due to a high rate of local recurrence, radiosynoviorthesis (RSO) is used as an adjuvant method in many cases. The aim of this study was to compare local recurrence (LR) rates after surgical synovectomy with and without adjuvant RSO. MATERIALS AND METHODS: From 1996 to 2014, 37 surgical interventions were performed in 32 patients with diffuse pigmented villonodular synovitis of the knee. All patients underwent open synovectomy. Adjuvant radiosynoviorthesis (RSO) was applied in 26 cases, the control group consists of 11 cases without RSO. RESULTS: 9 (24%) lesions recurred within a median of 19 months after surgery. Of those 9 recurrences, 3 (17%) were seen in primary disease, 6 (32%) in already recurring cases (n.s.). In 26 RSO treated patients 6 (23%) recurred, in 11 patients of the control group, 3 (27%) recurred (n.s.). CONCLUSIONS: RSO is effective in PVS as also shown in some smaller reports in the literature. But surgery is still the mainstay of therapy. RSO is not a method of compensating for an insufficient surgical approach, but it may reduce the high rate of LR in patients with large and even recurrent diffuse forms of the disease.

Cystic bone tumors of the foot and ankle.

Bone tumors are relatively rare in the foot and ankle region. Many of them present as cystic lesions on plain films. Due to the relative rarity of these lesions and the complex anatomy of the foot and ankle region, identification of such lesions is often delayed or they get misdiagnosed and mismanaged. This review discusses the most common cystic tumors of the foot and ankle including their radiographic features and principles of management.

Burden of Illness Associated with Tenosynovial Giant Cell Tumors.

PURPOSE: Little is known about the burden of illness in patients with tenosynovial giant cell tumors (TGCT), which are rare, typically benign, lesions of the synovial tissue including giant cell tumor of the tendon sheath (GCT-TS) and pigmented villonodular synovitis (PVNS). The objective of this study was to describe health care resource use and costs for patients with GCT-TS and PVNS, which are rare and typically benign TGCT. METHODS: A retrospective cohort study design was used to analyze administrative claims for adult commercial and Medicare Advantage health plan enrollees with evidence of GCT-TS and PVNS from January 1, 2006 through March 31, 2015. Participants were continuously enrolled for 12 months before (pre-index period) and 12 months after (post-index period) the date of the first tenosynovial giant cell tumor (TGCT) claim (index date). Preindex and postindex measures were compared using the McNemar test and Wilcoxon signed-rank test. Results were stratified by TGCT type. FINDINGS: The study identified 4664 patients with TGCT, 284 with GCT-TS, and 4380 with PVNS. Mean age (GCT-TS group: 50 years; PVNS group: 51 years) and sex distributions (GCT-TS group: 60.2% female; PVNS group: 59.5% female) were similar for each group. Most patients with GCT-TS (78.2%) had at least one postindex surgery, compared with 38.7% of patients with PVNS. Mean total health care costs increased from $8943 in the preindex period to $14,880 in the postindex period (P < 0.001) for GCT-TS and from $13,221 in the preindex period to $17,728 in the postindex period (P < 0.001) for PVNS. Preindex to postindex ambulatory costs increased nearly 120% for patients with GCT-TS ($4340 to $9570, P < 0.001) and 50% for patients with PVNS ($6782 to $10,278, P < 0.001), and physical therapy use increased significantly during the same period (GCT-TS: 18% to 40%, P < 0.001; PVNS: 38% to 60%, P < 0.001). IMPLICATIONS: Costs increased substantially 1 year after the first TGCT claim, with more than half the costs covering ambulatory care. These results suggest a high health care burden once TGCT is identified.

Long-Term Outcome of Hip Arthroplasty in the Setting of Pigmented Villonodular Synovitis.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare, benign, painful proliferation of the synovium previously treated successfully with total hip arthroplasty (THA). Published results come from small series; therefore, the purpose of this study is to investigate the outcomes of THA in the setting of PVNS. METHODS: We identified 25 patients with histologically confirmed, diffuse PVNS who underwent THA between 1971 and 2013. Mean follow-up and age was 10 years and 39 years. Before arthroplasty, 16 patients (64%) had at least 1 surgical procedure (mean, 1; range, 1-3) to treat PVNS. Twenty (80%) patients had "active" disease and underwent synovectomy. No constrained acetabular components were used. RESULTS: The 10-year disease free-survival was 100%. Recurrence occurred in 1 patient at 24 years postoperatively. Nineteen patients (76%) sustained a complication (most commonly component loosening (n = 12 [48%]), and 16 required revision surgery. The 10-year revision-free survival was 66% for conventional polyethylene implants and 100% for highly cross-linked polyethylene devices. Mean Harris Hip Score improved significantly from 48 (range, 23-69) preoperatively to 78 (range, 47-96) postoperatively (P < .001). CONCLUSION: THA in the setting of PVNS improves patient function with a low rate of local recurrence. Complication and revision rates are high in this series likely owing to the young and active patient population and the use of conventional polyethylene. Modern bearings theoretically reduce the risk of revision.

Pigmented villonodular synovitis.

Pigmented villonodular synovitis (PVNS) is a benign disease that rarely undergoes malignant transformation. There are two types of disease: localized (nodular tenosynovitis) and di used (pigmented villonodular synovitis/tenosynovitis) with intra- or extra-articular locations. The second one is limited to synovium of the burse (PVNB) or tendon sheath (PVNTS). The intraarticular lesions are usually located in the knee, hip, ankle and elbow joints. Histologically, PVNS is a tenosynovial giant cell tumor, characterized by proliferation of two types of mononuclear cells - predominantly small, histiocyte-like cells and larger cells with dense cytoplasm, reniform or lobulated nucleus, with accompanying multinucleated giant cells and macrophages overloaded with hemosiderin that give typical image on MRI - currently selected as a gold standard for its diagnosis. The classic X-ray and CT are non-specific but similar to ultrasound should be used to evaluate disease progression and treatment response if radiotherapeutic and pharmacological methods were selected for treatment. An open arthroscopic surgery could also be applied in selected cases.

Localized pigmented villonodular synovitis of posterior compartment of the knee.

Pigmented villonodular synovitis (PVNS) is an uncommon entity involving articular or extra-articular tissues and maybe localized or diffuse in extent. The knee is by far the commonest joint to get involved. Localized PVNS of the knee can occur in any location but its confinement to the posterior compartment is infrequent. We present our experience of managing localized posterior compartment PVNS of the knee. There were 10 patients (7 males and 3 females) with average age of 33 years. These patients had symptoms of pain, locking, or swelling for a mean of 13.9 years before diagnosis. All the patients underwent arthroscopic synovectomy without adjuvant therapy, and PVNS was proven on histopathology. At an average follow-up of 23 months, no patient had recurrence of symptoms. The average International Knee Documentation Committee (IKDC) score at last follow-up was 85.21. Magnetic resonance imaging evaluation at final follow-up did not reveal any residual disease or recurrence in any patient.

Pigmented Villonodular Synovitis - Various Manifestations, Inconsistent Terminology and Treatment. Cases Study.

Pigmented villonodular synovitis (PVNS) as well as tenosynovial giant cell tumor (TGCT) are both diagnosed and described in two forms, namely a localized and diffuse type. They form a diverse group of proliferative lesions in articular synovium and tendon sheaths. The inconsistent terminology impedes communication between patients, orthopedic surgeons, radiologists and pathologists, leading to misunderstandings and delaying treatment. In this paper, we present three cases of PVNS/TGCT (involving the fourth toe extensor tendon sheath, hip joint after resurfacing and flexor hallucis longus tendon sheath) together with suggestions regarding the unification of the terminology as well as diagnosis and treatment.

Extra-articular diffuse-type tenosynovial giant cell tumor with benign histological features resulting in fatal pulmonary metastases.

Diffuse-type tenosynovial giant cell tumor (TS-GCT) is categorized as a locally aggressive but non-metastasizing neoplasm according to the WHO classification. Herein, we report an extremely rare case of a 41-year-old woman who developed multiple metastases from diffuse TS-GCT with benign histological features. The patient complained of a painful buttock mass and imaging studies revealed a soft tissue tumor of the buttock and multiple pulmonary nodules. The buttock tumor was excised and the final diagnosis was extra-articular diffuse-type TS-GCT. By video-assisted thoracic surgery, pulmonary nodules were pathologically identical to the primary tumor. Residual pulmonary nodules progressively grew, and she developed a muscle metastasis and a subcutis metastasis. She died of respiratory dysfunction due to multiple pulmonary metastases 1 year after primary surgery. Very few reports on histologically benign metastases from TS-GCT have been published, and the natural course of this rare condition remains unclarified. This rare case could provide important information for further clinical evaluation of this tumor.

Ultrasound-guided synovial Tru-cut biopsy: indications, technique, and outcome in 111 cases.

OBJECTIVE: To investigate the diagnostic performance of ultrasound-guided synovial biopsy. METHODS: Clinical notes, pathology and microbiology reports, ultrasound and other imaging studies of 100 patients who underwent 111 ultrasound-guided synovial biopsies were reviewed. Biopsies were compared with the final clinical diagnosis established after synovectomy (n = 43) or clinical/imaging follow-up (n = 57) (mean 30 months). RESULTS: Other than a single vasovagal episode, no complication of synovial biopsy was encountered. One hundred and seven (96 %) of the 111 biopsies yielded synovium histologically. Pathology ± microbiology findings for these 107 conclusive biopsies comprised synovial tumour (n = 30, 28 %), synovial infection (n = 18, 17 %), synovial inflammation (n = 45, 42 %), including gouty arthritis (n = 3), and no abnormality (n = 14, 13 %). The accuracy, sensitivity, and specificity of synovial biopsy was 99 %, 97 %, and 100 % for synovial tumour; 100 %, 100 %, and 100 % for native joint infection; and 78 %, 45 %, and 100 % for prosthetic joint infection. False-negative synovial biopsy did not seem to be related to antibiotic therapy. CONCLUSION: Ultrasound-guided Tru-cut synovial biopsy is a safe and reliable technique with a high diagnostic yield for diagnosing synovial tumour and also, most likely, for joint infection. Regarding joint infection, synovial biopsy of native joints seems to have a higher diagnostic yield than that for infected prosthetic joints. KEY POINTS: • Ultrasound-guided Tru-cut synovial biopsy has high accuracy (99 %) for diagnosing synovial tumour. • It has good accuracy, sensitivity, and high specificity for diagnosis of joint infection. • Synovial biopsy of native joints works better than biopsy of prosthetic joints. • A negative synovial biopsy culture from a native joint largely excludes septic arthritis. • Ultrasound-guided Tru-cut synovial biopsy is a safe and well-tolerated procedure.

Pigmented Villonodular Synovitis: A Comprehensive Review and Proposed Treatment Algorithm.

Evidence now exists suggesting a neoplastic origin for pigmented villonodular synovitis, including a thorough description of the translocations involved. Arthroscopic excision for localized pigmented villonodular synovitis allows for both minimal recurrence and morbidity when compared with open excision; however, open excision still plays an important role in treating posterior compartment nodules. The optimal surgical treatment for diffuse pigmented villonodular synovitis is still a matter for debate, with combined anterior arthroscopic and open posterior excision being considered the preferred method. Radiation therapy has re-emerged as an additional treatment method for pigmented villonodular synovitis; however, the potential for serious toxicity makes it a questionable option, especially for such a benign condition. Small molecule and monoclonal antibody targeted therapies are being investigated as novel treatment methods for pigmented villonodular synovitis, with promising results.

A systematic literature review of synovial chondromatosis and pigmented villonodular synovitis of the hip.

OBJECTIVES: Benign synovial diseases of the hip including Synovial Chondromatosis (SC) and Pigmented Villonodular Synovitis (PVNS) are devastating diseases. Initially, patients present with hip pain unrelieved by conservative measures. The diagnosis of PVNS and SC are often delayed, leading to progression of joint damage. The purpose of this review is to present the latest on the diagnosis, management, and prognosis of SC and PVNS of the hip. METHODS: An extensive systematic search of MEDLINE and PUBMED Databases was performed. Data parameters were set from 2005 to present day with set inclusion criteria. Systematic reviews were excluded. RESULTS: 427 abstracts were identified, with 12 articles meeting all inclusion criteria. Eight studies focused on SC, and 5 on PVNS. 233 patients with SC of the hip and 98 patients with PVNS of the hip were identified, a total of 331 patients. DISCUSSION: Benign Synovial disorders of the hip are rare. In patients with chronic hip pain secondary to benign synovial disorders, early diagnosis and surgical intervention demonstrate good outcomes, and patients may benefit due to prevention of morbidity from further joint destruction. There is no clear consensus between higher successes through open versus arthroscopic surgical debridement. In the final phase of benign synovial disorders of the hip, THA of different types based on the patient's age should be considered.

Isolated limb perfusion as a treatment option for rare types of tumours.

BACKGROUND: Isolated limb perfusion (ILP) is an established and effective treatment for advanced melanoma and soft tissue sarcomas of the extremities with a high overall response rate. The aim of this study was to describe our experience of ILP for more rare types of tumours. METHODS: Patients with Merkel cell carcinoma (MCC) (n = 4), squamous cell carcinoma (SCC) (n = 2), B-cell lymphoma (n = 1), desmoid tumours (n = 3), pigmented villonodular synovitis (PVNS) (n = 1) and giant cell tumour (n = 1) were treated with ILP and analysed retrospectively. RESULTS: The four patients with in-transit MCC had three complete responses (CR) and one partial response (PR); the two patients with SCC had one CR and one stable disease (SD); the patients with desmoid tumours had two PR and one SD. A CR was also observed for the patient with a giant cell tumour, but the patient with PVNS had a SD. The patient with cutaneous metastases of B-cell lymphoma showed a CR, however with rapid systemic progression. Local toxicity according to Wieberdink was grade II in 10 patients (83%) and grade III in two patients (17%). CONCLUSIONS: These results show that ILP can be used as a treatment option also for more rare disease entities when other treatments have failed.