The role of imaging techniques in the diagnosis of

Pigmented villonodular synovitis

Poster No.: C-2153

Congress: ECR 2015
Type: Educational Exhibit
Authors: C. B. Lazu; Targu-Mures/RO
Keywords: Neoplasia, Hemorrhage, Education and training, Imaging
sequences, MR, CT, Conventional radiography, Musculoskeletal
joint
DOI: 10.1594/ecr2015/C-2153

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Learning objectives

To identify the pathologic basis of radiologic features of pigmented villonodular synovitis

(PVNS).

To describe the radiologic manifestations of PVNS - the diffuse intraarticular form.

Background

Pigmented villonodular synovitis (PVNS) is a rare, predominantly benign neoplastic lesion

of uncertain etiology that affects the synovium that lines joints, bursae and tendon
sheaths.

PVNS is characterized, grossly, by friable villous, nodular, or villonodular synovial

proliferations. These proliferations project into the joint and may bleed, resulting in
hemosiderin pigmentation and bloody or xanthochromic synovial fluid.

Monoarticular involvement is the most common and occurs in two forms: localized
and diffuse. The localized form is characterized by focal involvement of the synovium,
with either nodular or pedunculated masses; the diffuse form affects virtually the entire
synovium.

The diffuse form typically involves the large joints: the knee joint in 80 % of cases. But
PVNS can also affect the hip, ankle, elbow, shoulder and spine.

PVNS occurs predominantly in second to fifth decades. In intra-articular disease there is

no gender predilection, whereas extra-articular disease has a slight female predominance
(female-to-male predominance ratio 1,5-2:1).

The diagnostic is suggested clinically by joint swelling, pain and occasionally joint
dysfunction on a young adult.

Diagnosis of PVNS can be clinically difficult and radiographic findings are relatively non-
specific.

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Magnetic resonance imaging (MRI) is a highly diagnostic modality in 95% of cases that
demonstrates extent and reveals characteristic but not pathognomonic aspects of the
disease.

Even if it is a benign condition, PVNS may result in significant morbidity if left untreated.
Pain, loss of function, and eventual joint destruction may result. The primary treatment
option is surgical resection via synovectomy. Radiation therapy may be used as the
primary treatment for diffuse intraarticular PVNS, but it is best used to augment surgery
following the incomplete resection of disease. Arthroplasty and arthrodesis are also
treatment options. Amputation is used in rare cases, typically only those with multiple
recurrences.

Malignant transformation of PVNS is rare, and it can occur de novo or be associated with
recurrent disease (usually multiple episodes). These lesions are high-grade aggressive
sarcomatous tumors with evidence of synovial origin and typically poor outcome

Malignant PVNS is rare and difficult to distinguish, both pathologically and radiologically,
from multiple local recurrences of benign disease unless there is metastatic involvement
of the lungs or lymph nodes.

Images for this section:

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Fig. 4: PVNS is characterized, grossly, by friable villous, nodular, or villonodular synovial
proliferations. These proliferations project into the joint. The knee joint is affected in 80
% of cases, but PVNS can also affect the hip, ankle, elbow, shoulder and spine.

Fig. 5: PVNS is characterized, grossly, by friable villous, nodular, or villonodular synovial

proliferations. These proliferations project into the joint and may bleed, resulting in
hemosiderin pigmentation and bloody or xanthochromic synovial fluid.

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Findings and procedure details

Findings on plain radiographs are not specific. The mineralization of the bones around
the lesion is normal until late stages. As in gout, integrity of the articular surfaces and
preservation of the width of the joint space are maintained until relatively late in the
disease.

In late stages, secondary degenerative changes may occur in the affected joint, with
concentric joint space narrowing, subchondral cyst, and osteophyte formation.

Computed tomography (CT) findings are also nonspecific. CT scanning is hobbled by its
inability to completely show the extent of disease and other pathology around or within
the joint.

Because of improved tissue contrast, CT scanning is valuable in delineating bone cysts

and erosions.

The hypertrophic synovium appears as a soft tissue mass, which on account

of haemosiderin, may appear slightly hyperdense compared to adjacent muscle.
Affected synovium is hypervascular and generally enhances following administration of
radiographic contrast. Joint effusions commonly co-exist.

Radiographic contrast may be injected into the joint following joint aspiration. With
contrast filling of the joint, findings demonstrate multiple irregular nodular-filling defects
of variable sizes, which produce the typical cobblestone appearance of the synovium
seen on arthrography. The fluid that is aspirated before injection of contrast material for
arthrography typically demonstrates bloody effusion (69%-75% of cases), yellow fluid
(22%-25%), or brownish fluid (9%).

In the evaluation of soft-tissue and synovial lesions MRI has become the imaging
modality of choice. PVNS demonstrates variable appearance on MRI, depending on the
composition of the lesion and its relative proportions of hemosiderin, lipid, fibrous tissue,
cyst formation, and cellular elements and on the imaging parameters.

Characteristic on MRI scans are mass-like synovial proliferations, nodular with lobulated
margins, in a joint with effusion. Those lesions may be extensive in the diffuse form or
limited to a well-defined single nodule in the localized form with low signal intensity on T1-,
T2-, and proton density-weighted sequences. Low signal intensity is due to hemosiderin
deposits and is accentuated on T2-weighted sequences, most notably on gradient echo

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sequence that shows "blooming" phenomenon of hemosiderin-laden nodules. On T2-
weighted sequences some areas of high signal may be present likely due to joint fluid
or inflamed synovium. STIR MR images have been reported to reveal predominantly
high signal intensity in PVNS. PVNS lesions characteristically show prominent contrast
enhancement of synovium with the administration of gadolinium.

Another MRI finding is scalloping or truncation of prefemoral fat pad.

The differential diagnosis of heterogeneous joint effusion with intra articular low signal
are PVNS, chronic effusion containing hemorrhage and hemosiderin from entities such
as hemophilia, trauma, intraarticular hemangiomatosis/hemangioma and inflammatory
conditions with chronic effusions such as rheumatoid arthritis.

Images for this section:

Fig. 2: Pigmented villonodular synovitis of the knee. Plain radiographic findings of the
knee apparently are normal except for the curvilinear calcification seen peripherally to
the medial femoral condyle.

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Fig. 1: Intraarticular PVNS of the knee in a 22-year-old man. Oblic PD frFSE HiRes,
Sagittal PD frFSE HiRes, Sag T1 FSE and Oblic PD frFSE HiRes images reveal the
diffuse intraarticular low signal intensity mass around lateral femoral condyle in right knee.

Fig. 3: PVNS of the ankle joint and giant cell tumor of the FHL tendon sheath Sagittal T1
C+ fat sat image and Coronal T2W image showing hypointense masses around tarsal
bones and tendon sheats.

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Fig. 6: A well defined nodular lesion is seen within knee joint appearing heterogenously
hypointense on T1W and T2W images. It is seen to indent Hoffa's fat pad, and seen close
to tibial attachment of ACL.

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Conclusion

PVNS of the knee presents a difficult differential diagnosis.

Only MRI is an effective, noninvasive technique to define and characterize the disease
by being able to identify the presence of haemosiderin precipitates within the nodules
characterizing the PVNS lesion. MRI findins are diagnostic in 95% of cases.

A combination of plain films and MRI should be used in preoperative evaluation of a

patient with PVNS. This combination provides an accurate diagnosis and maps out the
extent of disease for the surgeon prior to treatment.

Personal information

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