Localized pigmented villonodular synovitis in the knee associated with locking symptoms

Pigmented villonodular synovitis (PVNS) occurs in the knee more frequently than other joints. Most cases involve diffuse PVNS while the localized type is relatively rare. This report describes a patient who had PVNS and a lateral meniscus injury which induced locking symptoms in the knee. A closed reduction of the meniscus tear was performed under local anesthesia. However, complete extension of the knee was not achieved and the locking symptoms persisted. MRI examination showed a neoplastic lesion measuring approximately 2 x 2 cm in the intercondylar space together with a lateral meniscus tear. The lesion was resected using arthroscopy. Histology of the resected lesion demonstrated localized PVNS. There has not been any recurrence of locking symptoms or PVNS two years after surgery. The findings in this case suggest that localized PVNS may contribute to locking symptoms in a patient with meniscal pathology.     

Outcome of 17 pigmented villonodular synovitis (PVNS) of the knee at 6 years mean follow-up

Between January 1950 and December 2000, 16 patients were identified from Scottish Bone Tumour Registry with 17 histologically proven pigmented villonodular synovitis (PVNS) of the knee. The mean follow-up was 6 years (range, 1-14 years). A knee swelling of chronic duration with dull ache was the common presenting symptom. The mean duration of symptoms prior to presentation was 25 months (1-108 months), however it was much less (mean, 7 months) in four patients with a history of trauma. The mean age was 33 years (range, 16-58 years) with a slight male predominance. The lesion was predominantly anterior in nine patients, posterior in four, anterolateral in two, and medial and lateral in one each. Three patients (four knees) had localised disease and 13 diffuse. Anteroposterior and lateral radiographs of the knee revealed normal findings in 11 patients, features of gonarthrosis in four and a large suprapatellar loose body in one patient (both knees). Open (incisional-eight, excisional-eight) biopsy was carried out in all and all were histologically confirmed as PVNS. Removal of a localised synovial mass or loose body with surrounding partial synovectomy (four) was carried out for the localised variety, whilst open partial (three) or total (radical) synovectomy (10) was performed in all cases of diffuse PVNS. Three of seventeen knees had a recurrence, noted at 4, 6 and 8 years postoperatively (0% - localised, 23% - diffuse variety). A total (radical) synovectomy should be considered in diffuse PVNS in order to obtain optimal outcome.     

Fine needle aspiration of pigmented villonodular synovitis of the temporomandibular joint

Temporomandibular joint (TMJ) problems are usually the result of functional disorders. Tumors of the TMJ are rarer entities, the diagnosis of which may be delayed due to a clinical impression of a functional ailment. We report a case of a patient with a recurrent TMJ area lesion diagnosed cytologically as pigmented villonodular synovitis (PVNS), an entity that very rarely involves the TMJ and is usually diagnosed by histology. The differential diagnosis of PVNS in the context of its location in and around the TMJ is also discussed.     

Recurrent and non-recurrent pigmented villonodular synovitis 1

Pigmented villonodular synovitis (PVNS) is a benign intra-articular lesion. Patients are at risk for local recurrence. Factors that predict recurrence are not established. Two groups of patients were retrieved from our files. One consisted of 25 patients who had one or more recurrences within 5 years after primary surgery. The second group contained 18 historical controls free of recurrence for at least 5 years after primary surgery. Patient medical records and surgical notes were reviewed. We compared proliferative activity and DNA ploidy using digital image analysis, and other clinicopathologic features between the 2 groups of patients. The location of PVNS was significantly different between the two groups (p=0.03). In the recurrence group, there were 7 (28%) cases with disease in the knee. However, none of the controls had disease in the knee. Among recurrent cases, tumors in the knee were, on average, larger than tumors in the small joints. The size of all recurrent tumors was not significantly different than non-recurrent tumors (median of 1.8 cm versus 1.3 cm, respectively; p=0.06). There were no significant differences in age, sex, completeness of surgical removal, MIB-I index, DNA ploidy, or the percent of tumor nuclei in the diploid, S-phase, tetraploid, or hypertetraploid DNA histogram categories between the two groups. Our results indicate that recurrent PVNS tumors were more likely to be located in the knee, which may be related to larger tumor size. Patient age, sex, completeness of surgical removal, DNA ploidy, and MIB-I proliferation were not significantly different between recurrent and non-recurrent lesions.     

Osteoclast formation and function in pigmented villonodular synovitis

Pigmented villonodular synovitis (PVNS) is a synovial tumour-like lesion that frequently causes osteolysis. PVNS contains numerous macrophages and osteoclast-like giant cells. In this study, we have analysed the cytochemical and functional characteristics of mononuclear and multinucleated cells in PVNS and determined the cellular and humoral mechanisms underlying giant cell formation and resorption in PVNS. Giant cells and CD14(+) and CD14(-) mononuclear cell populations were isolated from PVNS synovial tissue and cultured alone or in the presence and absence of the osteoclastogenic factors, RANKL and M-CSF. Osteoclast formation and activity was assessed by expression of TRAP and evidence of lacunar resorption. Giant cells in PVNS expressed an osteoclast-phenotype (CD51(+) , TRAP(+) , CD14(-) , HLA-DR(-) ) and were formed only in cultures of mononuclear cells that expressed the macrophage marker CD14. Osteoclast formation required RANKL and occurred in both the presence and absence of exogenous M-CSF. CD14(-) cells in PVNS expressed RANKL. Lacunar resorption by PVNS-derived giant cells was abolished by the addition of the bisphosphonate, zoledronate. Our findings indicate that osteoclasts form by a RANKL-dependent mechanism from CD14(+) mononuclear phagocytes in PVNS. Osteoclast formation occurred even in the absence of exogenous M-CSF, a finding which is in keeping with over-expression of M-CSF playing a pathogenic role in this condition. Anti-osteoclast resorptive treatment may be useful to control osteolysis in PVNS.     

November 2000: 13 year old girl with back pain and leg weakness

A 13 year-old girl presented with back pain and recurrent falls of one year, with more recent loss of ambulation and bladder control. Examination showed spasticity and a sensory level bilaterally at T8. CT and MRI scans showed an epidural soft tissue mass with spinal cord compression and destruction of the pedicle, transverse process and other portions of a mid-thoracic vertebral body. Histologic examination of the gross total resection showed a pigmented villonodular synovitis (PVNS). PVNS is most common in the knee and only 26 cases have been reported in the spine. Although vertebral bodies are rarely involved, it is important to include PVNS in the differential diagnosis of spinal lesions because of its tendency to recur locally if not totally resected.     

Atypical and bilateral pigmented villonodular synovitis of wrist in an adolescent patient: case report and literature review

Background: Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disorder of the synovium. It rarely occurs in adolescents, particularly in immature patients with bilateral manifestation. Case presentation: We present a case of atypical and bilateral PVNS of wrist in a 14-year-old boy. Initially, the patient presented with left wrist pain and swelling without the history of trauma. Physical examination revealed an obvious lesion in the dorsal part of left wrist. Radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) showed multiple abnormal signal shadows and arthroedema in the left wrist. Arthroscopy operation was performed, and histologic examination suggested the diagnosis of PVNS. Only 10 months later, the patient presented with the similar symptoms and signs in the right wrist. But MIR and histologic examination were atypical. In this article, we also review and summarize 26 studies on 30 adolescent patients with PVNS. Conclusions: This study provides an example of atypical and bilateral PVNS in adolescents.     

Giant cells in pigmented villo nodular synovitis express an osteoclast phenotype.

AIM: To determine the cytochemical and functional phenotype of multinucleated giant cells in pigmented villo nodular synovitis (PVNS). METHODS: Giant cells isolated from a patient with PVNS of the knee were assessed for a number of markers used to distinguish osteoclasts from macrophages/ macrophage polykaryons: evidence of tartrate resistant acid phosphatase (TRAP) activity; expression of CD11b, CD14, CD51, and calcitonin receptors; and the ability of the giant cells to carry out lacunar resorption. RESULTS: Isolated giant cells expressed an osteoclast antigenic phenotype (positive for CD51, negative for CD11b and CD14) and were TRAP and calcitonin receptor positive. They also showed functional evidence of osteoclast differentiation, producing numerous lacunar bone resorption pits on bone slices in short term culture. CONCLUSIONS: The giant cells in this case of PVNS express all the phenotypical features of osteoclasts including the ability to carry out lacunar resorption. This may account for the bone destruction associated with this aggressive synovial lesion.     

Pigmented villonodular synovitis of the knee: A retrospective analysis of 214 cases at a UK tertiary referral centre

Aims

Pigmented villonodular synovitis (PVNS) is a rare, locally aggressive and potentially recurrent synovial disease. We present the largest single-centre experience of knee PVNS. Our aim was to evaluate our tertiary hospital's experience in the management of knee PVNS.

Primary synovial epithelioid sarcoma of the knee: distinctly unusual location leading to its confusion with pigmented villonodular synovitis

Epithelioid sarcoma (ES) is a rare malignant soft tissue tumor occurring in the distal extremities of young adults and is characterized histologically by nodules of epithelioid cells showing central necrosis. Intra‐articular ES is extremely rare; only four cases have been reported, but their radiologic and histologic documentation of intra‐articular origin have been imprecise. We report the first radiologically and histologically well‐documented case of primary synovial ES. A 59‐year‐old woman presented with pain followed by swelling of her right knee for 6 months. MRI revealed an entirely intra‐articular nodular synovial mass in the lateral part of the right knee joint in a background of diffusely thickened synovium. Synovectomy was performed under the clinical impression of pigmented villonodular synovitis (PVNS), a diagnosis erroneously confirmed by the reporting pathologist. The tumor rapidly recurred 3 months afterward and the diagnosis of primary synovial ES was made. Despite above‐knee amputation, the tumor continued to spread proximally to the retroperitoneum. She developed multiple lung metastases and died 20 months after initial presentation. The nodular aggregates of tumor cells with central necrosis resulted in diffuse polypoid synovial thickening mimicking tuberculous synovitis and PVNS. The tumor cells showed positive staining for EMA, CK19, CD34, and complete loss of INI1 staining, establishing the diagnosis of primary synovial ES. The ES spread from the synovium to and along the joint capsule, and then extra‐articularly into the soft tissue surrounding the knee joint, with lymphovascular permeation. Such pattern of spread calls for radical surgical excision as the treatment of choice.     

Pigmented villonodular synovitis secondary to laceration of the perforating branch of the peroneal artery

A case of peroneal artery injury subsequently developed into a lesion resembling an extra-articular tenosynovial giant cell tumor, which is a type of pigmented villonodular synovitis (PVNS). This case supports the hypothesis that accident trauma, such as a vascular injury, can be the etiology of PVNS.     

Trisomy 5 and trisomy 7 are nonrandom aberrations in pigmented villonodular synovitis: confirmation of trisomy 7 in uncultured cells.

Pigmented villonodular synovitis (PVNS) is a proliferative lesion of disputed genesis. Recently, we reported trisomy 7 in short-term cultures of 1 PVNS. In the present report, we describe another specimen of PVNS in which 9 of 26 (35 percent) metaphase cells demonstrated trisomy 7 when analyzed after 3-15 days of tissue culture. In situ hybridization analysis, with a biotinylated probe to chromosome 7 alpha-satellite DNA, revealed trisomy 7 in 53 of 200 uncultured cells from this PVNS sample. Our findings indicate that trisomy 7 is a nonrandom aberration that arises in vivo in PVNS.     

Two-stage TKA for tuberculosis septic arthritis of the knee masquerading as pigmented villonodular synovitis: A case report

A 25-year-old female presented with left knee pain following arthroscopic synovectomy for presumed pigmented villonodular synovitis (PVNS). Radiographs and magnetic resonance imaging demonstrated tricompartmental arthritic changes. She underwent a two-stage procedure first involving antibiotic spacer implantation, followed 1 week later by spacer removal and definitive total knee arthroplasty (TKA) once initial intraoperative culture results were negative. Subsequent cultures confirmed tuberculosis septic arthritis. Repeat evaluation 1 year postoperatively showed no complications and patient satisfaction with left knee function. This is a unique case report in the United States describing 1-year outcomes following staged TKA for tuberculosis septic arthritis masquerading as PVNS.     

Two incision synovectomy and radiation treatment for diffuse pigmented villonodular synovitis of the knee with extra-articular component

Diffuse pigmented villonodular synovitis (PVNS) of the knee is a rare, locally aggressive disease. Since 1995 the senior author has been treating patients with diffuse intra- and extra-articular PVNS of the knee with a bimodality treatment protocol, consisting of anterior and posterior open synovectomies in conjunction with external-beam radiation. The purpose of this study was to describe the surgical technique, evaluate our protocol's efficacy in limiting local recurrences, and assess its functional implications and associated morbidities. The medical records of nine consecutive patients were analyzed. Functional results were measured using the Knee Society score. The mean follow-up was 67 months (range 37-103 months). Eight patients were disease free and one patient had local recurrence, which was extra-articular, localized and non-progressive. The mean knee rating and functional rating had significantly improved from 65.2 and 70 preoperatively to 93.7 and 96.6 postoperatively respectively. Maximal knee flexion ranged from 90 degrees to 130 degrees, and only 3 patients developed flexion contractures of 5 degrees. In conclusion our bimodality protocol of anterior and posterior open synovectomies, in conjunction with external-beam radiation, limited the recurrence of diffuse intra- and extra-articular PVNS of the knee. All patients showed good-to-excellent functional outcomes and were able to return to their previous level of activity.     

Lipoma arborescens of the knee

Lipoma arborescens (diffuse articular lipomatosis) is a rare, benign intra-articular lesion of unknown aetiology. It is characterised by villous proliferation of the synovium and diffuse replacement of the subsynovial tissue by mature fat cells. It forms part of the differential diagnosis for a slowly progressive chronically swollen knee. We present a very rare case of bilateral involvement of the knee and discuss the symptoms, diagnosis and treatment of this condition.     

Malignant giant cell tumor of synovium (malignant pigmented villonodular synovitis)

CONTEXT: Pigmented villonodular synovitis (PVNS) is a well-recognized entity that has the potential for extensive local destruction, even though it rarely metastasizes. Rare reports of malignant forms are recorded in the literature. We observed 2 patients in whom examples of PVNS followed an aggressive course with multiple recurrences, metastasis, or degeneration to an appearance resembling malignant fibrous histiocytoma. OBJECTIVE: We studied the occurrence and persistence of aneuploidy for chromosomes 5 and 7 in 2 patients with clinically aggressive PVNS. DESIGN: Fluorescence in situ hybridization was performed for the detection of chromosomes 5 and 7 in the primary lesions, recurrences, and metastases in 2 examples of PVNS. RESULTS: Fluorescence in situ hybridization demonstrated small but significant numbers of cells with trisomies for chromosomes 7 and/or 5 in both the primary and recurrent lesions of both patients. CONCLUSIONS: The presence of consistent chromosomal trisomies (5 and 7) in both patients' examples of PVNS suggests a neoplastic nature for this lesion. The persistence of these trisomies in the primary lesions, recurrences, and metastases supports a molecular link between the primaries, recurrences, and metastases despite changes in morphologic features. The presence of persistent trisomies in the recurrent and metastatic lesions supports the concept of malignant PVNS.     

Intraarticular nodular fasciitis causing limitation of knee extension: A case report

We report a patient with intraarticular nodular fasciitis who developed mechanical symptoms in the knee, limiting knee extension. A 24 year-old man presented with mechanical restriction of extension and pain at maximum extension. MRI revealed an intraarticular mass within the knee joint. The mass was excised arthroscopically. Histological examination of the excised tissue led to a diagnosis of nodular fasciitis. Pain and limitation of motion of the knee disappeared from one day after surgery. No recurrence was found after 1 year. Intraarticular nodular fasciitis is rare, and although this lesion within the knee has been reported previously, it has not been associated with mechanical symptoms. This case indicates that intra-articular fasciitis should be considered in the differential diagnosis of a patient presenting with mechanical limitation of knee extension and a mass lesion inside the knee joint.     

Two cases of synovial haemangioma of the knee joint: Gd-enhanced image features on MRI and arthroscopic excision

Synovial haemangioma of the knee joint is a relatively rare benign condition with around 200 reported cases. We have recently encountered two cases of synovial haemangioma of the knee joint which preoperative MRI had assessed as highly suspect and which arthroscopic resection and subsequent histological examinations confirmed as synovial hemangiomas. Published studies have identified the following as characteristic MRI features of synovial haemangioma: homogenous low intensity to iso-intensity on T1 sequence; and heterogeneous high intensity with low-intensity septa or spots within the lesion on T2 sequence. However, several other intra-knee disorders mimic these characteristics. In our two cases, we found that gadolinium (Gd)-enhanced images, which have been relatively rarely discussed in the literature, were useful for making the diagnosis and for determining the extent of this condition. These images also were very helpful during arthroscopic excision of the lesion. Nonetheless, even after Gd enhancement, differentiating between malignant conditions such as synovial sarcoma and haemangioma solely from MRI findings is still difficult.     

Case of cutaneous Pseudallescheria boydii infection caused by trauma]

Pseudallescheria boydii is a ubiquitous filamentous fungus. We report a case of cutaneous P. boydii infection of the left knee in a 79-year-old Japanese man who was receiving oral predonisolone (25 mg/day) for radiation pneumonitis after radiation therapy on left breast cancer. He presented with a 2-week-history of a lesion on the left knee. A biopsy specimen from the skin lesion revealed granulomatous inflammation with hyphae. Culture of the pus from the skin specimen confirmed the diagnosis of cutaneous P. boydii infection. rDNA ITS sequence was analyzed to confirm the mycological diagnosis. The patient was treated orally with 200 mg/day of itraconazole. The lesion was gradually cured and left a hypertrophic scar. Cutaneous injury may be responsible for an incidence of localized infection. Such rare fungus infection in immunocompromised patients who have a persistent traumatic skin ulcer needs to be ruled out. An opportunistic infection in immunocompromised patients can be life-threatening and prompt treatment based on accurate diagnosis is important.     

Somatoform pain disorder in the general population

BACKGROUND: Chronic pain disorder is assumed to represent a frequent and disabling condition. However, data on the prevalence of somatoform pain symptoms and somatoform pain disorder in the community are limited to date. METHODS: German versions of the Composite International Diagnostic Interview were administered to a representative national sample of 4,075 people. Somatoform pain disorder was diagnosed by standardized diagnostic algorithm based on the DSM-III-R criteria (absence of adequate physical findings). One subgroup was identified as also meeting the DSM-IV criterion B for 'significant distress or psychosocial impairment due to the somatoform pain'. RESULTS: A lifetime prevalence rate of somatoform pain disorder according to DSM-III-R of 33.7% and a 6-month rate of 17.3% was found. When applying the DSM-IV B criterion, the prevalence rate dropped to 12.3 and 5.4%, respectively. In both groups more than 95% of the probands had contacted their doctor because of the pain. In 25% of the probands the pain was positively assigned to psychological factors. A female:male ratio of 2:1 was found. CONCLUSIONS: Somatoform pain disorder (DSM-III-R) is a frequent condition. However, only about one third of these subjects is severely distressed or impaired by the pain. A clear operationalized concept of the DSM-IV criterion C 'psychological factors are judged to have an important role in the onset, severity, exacerbation or maintenance of the pain' should be provided in the further development of the diagnosis 'pain disorder' in order to make this diagnosis suitable for general population surveys.