Pigmented villonodular synovitis of the shoulder

Clinical Cases 07.04.2015
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 32 years, male
Authors: Ereño Ealo M.J., Casado Verdugo O.
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AI Report

Clinical History

A 32-year-old male patient presented with a 1-year history of spontaneous shoulder pain. Physical examination revealed shoulder stiffness.

Imaging Findings

Plain radiograph demonstrated intraosseous cystic lesions and articular erosions in the humerus head (Fig. 1a).
CT shows better the well-defined lytic areas with sclerotic rim together with chronic degenerative changes such as decrease of the joint space and sclerosis (Fig. 1b). No calcifications were seen.
Coronal FSE PD-WI with and without fat saturation (Fig. 2a, b) and axial FSE T2-WI (Fig. 3a, b) showed nodular synovial intraarticular masses of marked low signal intensity (Fig. 4a, b), extending to the long biceps tendon sheath and axillary recess. Cystic osseous erosions and degenerative changes in the glenohumeral joint were also present.

Discussion

Intraarticular giant cell tumour (GCT), also known as pigmented villonodular synovitis (PVNS), is an uncommon benign proliferative process affecting synovial membranes (and can thus also include bursae and tendons).

Depending on the site of origin, it can be divided into iintraarticular or extraarticular and depending on the pattern of growth localized or diffuse. It typically affects patients of 20-50 years of age, with equal frequency in men and women.

The cause of PVNS is not known. Microscopically synovial hyperplasia is accompanied by organizing surface fibrin deposits, stromal fibrosis, chronic inflammation, and generally accumulations of macrophages that often contain cytoplasmic haemosiderin pigment.

Diffuse GCT is usually a monoarticular process of large joints with the knee being the most common (66-80%), followed by the hip, ankle, shoulder [3, 4] and elbow, in descending frequency. Localized intra-articular type of GCT or PVNS occurs almost exclusively within the knee [1, 7].
Our case shows unusual presentation in the shoulder with large bone erosions [4].

Most cases come with joint swelling, pain and occasionally joint dysfunction.
Hypertrophic synovium may appear villous, nodular, or villonodular with prominent haemosiderin deposition. Most patients have a haemorrhagic, dark brown synovial fluid.
Biopsy is diagnostic [5].

Plain radiographs may be normal, show periarticular soft tissue swelling and local osseous changes such as extrinsic bone erosions, osteopaenia, joint space narrowing, intraarticular osteochondral bodies and degenerative disease (Fig. 1a) [1].

CT can show joint effusion, erosions and bone cysts and intraarticular soft tissue mass of synovial tissue, sometimes slightly hyperdense compared to muscle (Fig. 1b).

MRI is the best imaging technique for the diagnosis of GCT. Synovial thickening in MRI characteristically shows intermediate to low signal intensity on T1-WI and markedly low on T2-WI MR-images, due to T2-relaxation time shortening caused by haemosiderin deposits together with collagen bundles. This effect is accentuated on gradient-echo images, the so-called “blooming” effect, caused by increased magnetic susceptibility artefact (nearly pathognomonic of pigmented villonodular synovitis) [5]. Other MRI findings include bone erosion, subchondral cysts, bone marrow oedema and cartilaginous defects (Fig. 2, 3). Marked enhancement of the synovium is common after gadolinium injection.

Treatment usually consists of surgery (synovectomy). Both arthroscopic and open surgery can be used. However, there is a high rate of recurrence, especially on diffuse forms. Adjuvant treatment is often employed, especially in recurrent cases, external beam radiotherapy or new treatments with tyrosine kinase inhibitors [6]. In cases of extensive joint destruction total joint replacement is the best procedure.

Differential Diagnosis List

Diffuse type of GCT of the shoulder
Haemophilic arthropathy
Haemorrhagic synovitis
Rheumatoid arthritis
Synovial osteochondromatosis
Gout

Final Diagnosis

Diffuse type of GCT of the shoulder

Liscense

Figures

Radiografía y TC

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Radiografía y TC
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Radiografía y TC

RM de hombro, plano oblicuo coronal

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RM de hombro, plano oblicuo coronal
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RM de hombro, plano oblicuo coronal

RM de hombro, plano axial

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RM de hombro, plano axial
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RM de hombro, plano axial

Artroscopia

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Artroscopia
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Artroscopia