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.

Pigmented villonodular synovitis diagnosed during revision total knee arthroplasty for flexion instability and patellar fracture.

Occurring in either a localized or diffuse form, pigmented villonodular synovitis (PVNS) is a disease of unknown etiology that typically presents with insidious onset of pain, swelling, stiffness, or mechanical symptoms as a result of synovial tissue proliferation. PVNS preferentially affects large joints, most commonly the knee. Currently there is no known association with PVNS and total knee arthroplasty (TKA), and to date, there are only a few cases reported in the orthopedic literature in which PVNS was diagnosed after primary TKA. To our knowledge, this is the first case of diffuse PVNS that was discovered at the time of revision TKA for flexion instability and patellar fracture. In this patient, with no known history of PVNS, the diagnosis of diffuse PVNS was made at the time of surgery. She underwent revision TKA, partial patellectomy, and extensive synovectomy. Level of evidence: V, Case Report.

Multi-lineage differentiation and angiogenesis potentials of pigmented villonodular synovitis derived mesenchymal stem cells--pathological implication.

Pigmented villonodular synovitis (PVNS) is a benign tissue proliferation characterized by its hyper-vascularity within the lesion. The true etiology and cell source of this disease entity still remain unclear. Mesenchymal stem cells (MSCs) exist in various tissues of human body. However, it has not been clarified whether MSCs could be isolated from tissue of PVNS. Here, we isolated MSCs from PVNS (PVNS-SCs), and by comparing to the MSCs from normal synovium (Syn-SCs) of the same individual, we investigated whether PVNS-SCs differed in the capacity for multi-differentiation and inducing angiogenesis. We first demonstrated that PVNS-SCs existed in the lesion of PVNS of three individuals. Moreover, we showed PVNS-SCs had better osteogenic differentiation potential than Syn-SCs, whereas Syn-SCs had better capacity for adipogenic and chondrogenic differentiation. By genome-wide analysis of gene expression profile using a complementary DNA microarray and comparing to Syn-SCs, we identified in PVNS-SCs a distinct gene expression profile characterized by up-regulation of genes involved in angiogenesis. In vitro and in vivo studies further confirmed that PVNS-SCs had better capacities for promoting angiogenesis. In summary, the identification of PVNS-SCs in PVNS tissue and their distinct angiogenic potential may help elucidate the underlying etiology of this disease.

Pediatric giant cell tumor of the tendon sheath of the craniocervical junction involving the occipital condyle.

INTRODUCTION: Giant cell tumor of the tendon sheath (GCTTS), also called pigmented villonodular synovitis, is a common lesion of the synovial membrane of the hand joint, but it uncommonly involves the axial skeleton, especially in pediatric populations. Furthermore, GCTTS originating from the occipital condyle has not been reported previously. CASE REPORT: A 15-year-old girl presented with a palpable neck mass for 1 year, and imaging studies revealed a less demarcated and heterogeneously enhanced mass in the suboccipital region. The tumor was originating from the occipital condyle that eroded the skull and atlas, and it was completely resected via a far lateral transcondylar approach followed by transarticular screw fixation. After the resection, we performed occipitocervical fusion to prevent spinal instability. The patient made an uneventful recovery after surgery. Recurrence has not been observed after 5 years of follow-up. DISCUSSION: We report this rare case and briefly review the general features and unusual locations of GCTTS with recommendations for treatment modalities.

A severe systemic presentation of pigmented villonodular synovitis in a child with underlying Chediak-Higashi syndrome.

Pigmented villonodular synovitis (PVNS), a condition of synovial hyperproliferation that mostly affects large joints, is rare in children and conventionally lacks systemic symptoms. This report describes a complex paediatric patient who underwent bone marrow transplantation to control the accelerated phase of the Chediak-Higashi syndrome. Diffuse PVNS developed in one knee 2.75 years later. Progression of PVNS was accompanied by the development of severe systemic symptoms, which resolved rapidly following subtotal surgical debridement. The patient remains well with minimal elevation of inflammatory marker levels 10.5 years later. As PVNS and Chediak-Higashi syndrome are both very rare diseases we propose a potential unifying hypothesis for this combination.

Pigmented villonodular synovitis: a retrospective study of seventy five cases (eighty one joints).

PURPOSE: Pigmented villonodular synovitis (PVNS) is a relatively rare, benign proliferation lesion of the synovium of large joints, but there is not much information available about the disease's aetiology, clinical history, differential diagnosis, treatment, and long-term effects. We conducted a study to analyse these aspects of PVNS. METHODS: We reviewed all clinical data for 75 patients with PVNS (81 joints) who were treated either by synovectomy alone or synovectomy plus arthroplasty. RESULTS: In all cases, the diagnosis of PVNS was confirmed by pathological examination. The ratio of males to females was 27:48, and the average age of patients was 46 years (range, 15-80 years). Lesions were located in the knee, hip, or ankle, and pain and swelling were the main symptoms. Of 75 patients, 42 had a history of trauma to the involved joint. Forty-one patients (43 joints) underwent synovectomy alone, and 34 patients (38 joints) underwent synovectomy and arthroplasty together. Of the 75 patients, 61 had full follow-up data. Twelve patients had recurrent legions detected by pathological examinations; four patients had more than two recurrences. Moreover, five patients developed PVNS after arthroplasty. CONCLUSIONS: PVNS occurs most often in middle-aged women and most frequently involves the knee, followed by the hip and ankle. The disease's etiology is varied and unclear. Surgical excision alone or with arthroplasty is an effective treatment, but there is a high rate of recurrence.

Severe destructive arthritis of the carpometacarpal joint: a diagnosis of exclusion case report.

We present a case of severe destruction of the thumb carpometacarpal joint (CMCJ) and surrounding structures on a background of osteoarthritis and Seronegative Rheumatoid arthritis. Imaging studies suggested a soft tissue lesion consistent with Pigmented Villonodular Synovitis (PVNS), Synovial Osteochondromatosis or Giant Cell Tumour (GCT). Due to the possibility of malignant transformation and deteriorating symptoms the mass was excised. Histological analysis of the lesion revealed severe degenerative disease with no evidence of malignancy or infection. This represents an atypical presentation of thumb carpometacarpal joint arthritis, which should be diagnosed once more sinister pathology has been excluded.

An unusual case of pigmented villonodular synovitis 14 years after total hip arthroplasty.

Pigmented villonodular synovitis (PVNS) is a rare benign proliferation lesion of the synovium of the joint, bursa, and the tendon sheath. We report here a case of PVNS in a 78-year-old woman 14 years after she underwent total arthroplasty of her right hip. Diffuse PVNS was detected in her right hip during surgery to replace her prosthesis, which had loosened. Macroscopically, the surface of the resected tissue was black and composed of papillae and nodules. Histologically, the tissue consisted of proliferative synoviocytes with black pigment in the cytoplasm. Beneath the synoviocytes were foamy cells. Pathologic analysis confirmed the diagnosis of PVNS with black pigment and the presence of hemosiderin. This indicates that implantation of the prosthesis might have caused the lesion or might have caused its proliferation.

Pigmented villonodular synovitis of the knee: diagnosis and treatment.

Pigmented villonodular synovitis (PVNS) of the knee is a benign but locally aggressive disease of synovial proliferation that occurs in localized nodular and diffuse villous growth patterns. Although inflammatory and neoplastic causes have been hypothesized, etiology remains unknown. Presenting as unilateral knee pain and swelling, PVNS mimics other knee ailments. Radiographs are often unremarkable, whereas magnetic resonance imaging may show characteristic intra-articular masses with signal dropout on T2-weighted sequences. Pigmented villonodular synovitis is surgically treated with open or arthroscopic total or partial synovectomy. High recurrence rates are associated with all treatments of diffuse PVNS. Complications of open synovectomy include arthrofibrosis and wound breakdown. Total arthroscopic synovectomy is technically demanding but can be advantageous. Transcondylar notch views, accessory posterior portals, and the posterior transseptal portal maximize arthroscopic access to the posterior knee. Intra-articular radioisotope injection and external beam radiation may be beneficial adjuvant therapy for extensive diffuse and recurrent PVNS of the knee.

An unusual case of pigmented villonodular synovitis after total knee arthroplasty.

We describe a case of focal pigmented villonodular synovitis in the knee presenting 12 months after total knee arthroplasty. The abnormal synovial proliferation was noted at arthroscopy, and histological analysis of the resected tissue confirmed the diagnosis.

Primary tumours of the synovium. A report of four cases of malignant tumour.

Four patients who developed malignant synovial tumours are described; one with chondromatosis developed a synovial chondrosarcoma and three with pigmented villonodular synovitis developed malignant change. The relevant literature is discussed.

[Progression of bone and articular destruction and QOL].

The joint dysfunction caused by bone and articular destruction is the most important pathology in rheumatoid arthritis (RA) patients. The progression of bone and articular destruction starts within five years from disease onset, however depending each case inflammation. Quality of life (QOL) of RA patients needs early stage treatment to prevent joint destruction. We recognized villonodular synovial proliferation is significantly correlated with early stage RA. Therefore to decrease those synovium leads to prevent joint destruction. Biological therapy itself can not inhibit villonodular synovial proliferation in effect attenuation cases. Arthroscopical synovectomy is effective to remove those synovium and restore the effect of biological therapy. It is needed that detail analysis of improvement of joint destruction by biological therapy near future to lead the improvement QOL of RA patients.

[Pigmented villonodular synovitis]. / Seltene Schleimhauterkrankung zerstört die Gelenke. Frühzeitige Diagnose erspart Gelenkersatz.

Pigmented villonodular synovitis (PVNS) is a rare, strongly proliferative disease of the lining of thejoint, synovial bursa and tendon (synovial) sheath. If left untreated, it leads to severe destruction of the joint resulting in an early need for endoprosthetic replacement. The clinical signs are unspecific. Using the diagnostic gold standard MRI, the complete extent of PVNS can usually be determined non-invasively. Once histological confirmation has been obtained, radical tumor resection, synovectomy, possibly curettage, and postoperative irradiation must be applied.

Increased expression of humanin peptide in diffuse-type pigmented villonodular synovitis: implication of its mitochondrial abnormality.

OBJECTIVES: To define the pathogenesis of pigmented villonodular synovitis (PVNS), by searching for highly expressed genes in primary synovial cells from patients with PVNS. METHODS: A combination of subtraction cloning and Southern colony hybridisation was used to detect highly expressed genes in PVNS in comparison with rheumatoid synovial cells. Northern hybridisation was performed to confirm the differential expression of the humanin gene in PVNS. Expression of the humanin peptide was analysed by western blotting and immunohistochemistry. Electron microscopic immunohistochemistry was performed to investigate the distribution of this peptide within the cell. RESULTS: 68 highly expressed genes were identified in PVNS. Humanin genes were strongly expressed in diffuse-type PVNS, but were barely detected in nodular-type PVNS, rheumatoid arthritis, or osteoarthritis. Humanin peptide was identified in synovium from diffuse-type PVNS, and most of the positive cells were distributed in the deep layer of the synovial tissue. Double staining with anti-humanin and anti-heat shock protein 60 showed that humanin was expressed mainly in mitochondria. Electron microscopy disclosed immunolocalisation of this peptide, predominantly around dense iron deposits within the siderosome. CONCLUSIONS: Increased expression of the humanin peptide in mitochondria and siderosomes is characteristic of synovial cells from diffuse-type PVNS. Humanin is an anti-apoptotic peptide which is encoded in the mitochondrial genome. Present findings suggest that mitochondrial dysfunction may be the principal factor in pathogenesis of diffuse-type PVNS and that humanin peptide may play a part in the neoplastic process in this form of PVNS.

Subcutaneous pigmented villonodular synovitis caused by portal contamination during knee arthroscopy and open synovectomy.

The etiology of pigmented villonodular synovitis (PVNS) is not clear. Researchers have suggested that localized nodular synovitis is an inflammatory process, but more recent studies tend to describe the lesion as benign synovial neoplasm with the potential of local recurrence. Although the theoretical risk of secondarily seeding the remainder of the knee is evident, this is the first report of a subcutaneous PVNS caused by portal contamination during knee arthroscopy and open synovectomy. It supports a neoplastic origin of this lesion.

Pigmented villonodular synovitis of the ankle occurring in a patient on anticoagulation therapy.

A case report is presented of a 45-year-old woman with an 18-month history of pain and swelling in her right ankle. There was no history of trauma. Routine investigations failed to elicit a diagnosis. The patient had been on warfarin anticoagulation therapy for 12 years. The onset of symptoms coincided with a period of poor control of her anticoagulation therapy and her international normalized ratio was recorded at 5 or above on three occasions. A diagnosis of pigmented villonodular synovitis (PVNS) was made on arthroscopic examination of her ankle; this was confirmed histologically. The etiology of PVNS remains controversial. Hemarthrosis has been suggested as an etiological factor. Although there are reports of PVNS in patients with hemophilia, there are no reports of PVNS occurring in patients on anticoagulation therapy. This case report supports a possible role for hemarthrosis in the etiology of PVNS.

Pigmented villonodular synovitis following arthroscopic laser surgery of the knee.

We report a case of a nodular variant of pigmented villonodular synovitis of the knee that developed after an arthroscopic lateral release of the synovium by laser for an unrelated condition. This case, the first reported case of pigmented villonodular synovitis following laser treatment, lends weight to the etiological association with the pathological processes of the response to trauma.