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.

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.

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.

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.

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.

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.

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.

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.

Malignant giant cell tumor of the tendon sheaths and joints (malignant pigmented villonodular synovitis).

Malignant pigmented villonodular synovitis (PVNS) is a rare lesion whose existence may be debatable. We studied eight cases that we consider to be examples of malignant PVNS. The three male and five female patients were aged 12 to 79 years. The knee was involved in three cases; the ankle in two; and the cheek, dorsum of the foot, and thigh in one each. Four patients had swelling for 6 months to 17 years before presentation. Three cases of malignant PVNS were secondary, arising in patients in whom PVNS had been documented previously, and five cases were primary, with histologic features similar to those of the secondary ones. Important histologic features of malignancy were (a) a nodular, solid infiltrative pattern of the lesion; (b) large, plump, round or oval cells with deep eosino philic cytoplasm and indistinct borders; (c) large nuclei with prominent nucleoli; and (d) necrotic areas. Atypical mitoses were occasionally seen. Four patients died with pulmonary metastasis (two also had metastasis to inguinal lymph nodes). Four patients are alive from 3 1/2 to 5 years after the last surgical treatment. The malignant nature of this lesion, the histologic architecture similar to that of PVNS, and the fibrohistiocytic appearance of the cells suggest that malignant PVNS is an entity.

Pigmented villonodular synovitis. Diagnosis and differential diagnosis.

PVNS is a proliferative disorder that can affect joints, tendons and bursae. Its etiology is unknown. The most widely accepted theories attribute this disorder to a chronic inflammatory response or a benign neoplasm of fibrohistiocytic origin. On gross specimens, the synovial changes are characterized by villous and/or nodular hyperplasia. This disorder occurs most frequently in the third or fourth decades of life and has no sex predilection. As a joint problem, it favors the knee and hip. As a tendon abnormality, it favors the digits. Plain film findings include 1) soft tissue swelling, 2) increased density of the synovium secondary to hemosiderin deposition, 3) multiple subchondral cysts, and 4) extrinsic cortical pressure erosions. Cartilage space narrowing, if present, is gradual and occurs later than the other plain film findings. Arthrography can demonstrate the nodular synovial changes and determine if the process is diffuse or focal. MRI can, in some cases, reveal areas of decreased signal within the hyperplastic synovium reflecting the deposition of hemosiderin. The major roentgen differential diagnoses of articular PVNS include uncalcified synovial chondromatosis, tuberculous arthritis, and hemophilic arthropathy.

Microcirculatory changes in posttraumatic pigmented villonodular synovitis.

The synovium from a case of pigmented villonodular synovitis was examined by light and electron microscopy. The hyperplastic villous processes were found to contain, besides many blood vessels, a dense population of phagocytic cells actively engulfing and digesting the erythrocytes; some of their phagosomes ("siderosome") contained iron-laden micelles sometimes arranged in a crystalline lattice. The synovial vessels were remarkable in two respects: first, there was evidence of endothelial breakdown; second, the basement membranes were strikingly multilayered.

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.

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.

Localized pigmented villonodular synovitis as a rare cause of chronic anterolateral ankle pain in an equestrienne.

An unusual case of localized pigmented villonodular synovitis of the ankle as a rare cause of chronic anterolateral ankle pain in a 16-year-old horsewoman is presented. Intra-articular nodular forms of pigmented villonodular synovitis can only be diagnosed arthroscopically, macroscopic and microscopic aspects being typical. We believe that this lesion is more likely a reactive process secondary to repetitive microtrauma rather than a true neoplasm. Our patient presented with pathology in the left ankle, the side by which one mounts and dismounts a horse, forcing, in both activities, ankle dorsiflexion. Moreover, an English saddle was used by our patient, upon which one rides with the ankle maintained in dorsiflexion. At arthroscopy, the soft-tissue mass was seen to be entrapped in the joint between the talus and the tibia at dorsiflexion of the ankle. This had caused a slowly progressive enlargement of the lesion because of fibrosis resulting from reactive inflammation associated with this repetitive microtrauma, thus causing irritation, pain, and synovitis due to impingement.

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.

[Hemopigmented villonodular synovitis]. / La synovite villonodulaire hémopigmentée.

Pigmented Villonodular Synovitis (PVNS) in an infrequent tumoral like disease and there are only a few MRI studies published. Concerning our series of five cases compared with the literature, the readers attention is drawn to the etiologies still under debate and to the respective contribution of the different imaging methods. MRI known for its high sensibility, but also for its low specificity in tumoral pathology, has turned out to be, in the case of PVNS, quite remarkable in both regards. The RMI aspect is quite well correlated to the histological structure of this synovial hyperplasia and to its evolution: highly vascular mass at the beginning and then low cellular density stroma, fibrous, with deposition of hemosiderin. In our five cases, as in almost all those previously reported in the literature, MR imaging shows heterogeneous areas of decreased signal intensity on T2 weighted sequences and on two of our cases after administration of gadolinium. Still the same MR aspects can be found in rheumatoid, hemophilic arthritis, as well as synovial chondromatosis, and therefore the clinical background and findings as well as plain films become essential. MR imaging should be the first examination to be undertaken after plain films, leading in most cases to a precise local preoperative assessment.

[Extra-articular localization of nodular synovitis: a case report]. / Die extraartikuläre lokalisierte noduläre Synovialitis--ein Fallbericht.

INTRODUCTION: The extra-articular nodular synovitis is regarded as a rare case of the common pigmented villonodular synovitis. The following report points out a case with wide extension in a young man's proximal lower leg. CASE-REPORT: A 24-year-old man complained about a non-painful tumor at his left proximal lower leg without any clinical handicap. Some years before he got a fracture of the proximal left tibia caused by an adequate sports trauma. So he had to undergo two operative investigations. The radiographs showed a transparent soft tissue tumor, which displaced the corticalis of the bone for some centimeters. The magnetic resonance imaging did not hint at a malignant process. After operative treatment the diagnosis was confirmed by histology. CONCLUSION: By operative and histological investigation advice could given to a benign tumor. The etiology of the extra-articular nodular synovitis is to be regarded as unknown. In our case a dislocation of synovial cells to extra-articular should be discussed.