UNCOMMON PRESENTATION OF BILATERAL PIGMENTED

VILLONODULAR SYNOVITIS

ABSTRACT ID : IRIA 1149

Pigmented Villonodular Synovitis (PVNS)- Benign proliferative

disorder that affects synovial lined joints, bursae, and tendon sheaths .

PVNS is a slow-growing lesion of uncertain etiology arising from the synovial membrane

Two primary forms –

a) Diffuse form affects the entire synovial lining of a joint, bursa or tendon sheath, involves the large joints.

B)Localized form - occurs around the tendon sheaths of small

joints.

BACKGROUND

The disease may show extraarticular soft tissue involvement, along with a primary intraarticular process.

PVNS involving the shoulder joint is uncommon and bilateral involvement of shoulder is < 1 % cases.

Common presentation in 3rd to 4th decade.

It is usually monoarticular, polyarticular involvement is rare.

Commonly involved joints- knee/ ankle/ hip/ finger joints.

Shoulder joint involvement is extremely rare.

Two types:Diffuse - Affects the entire synovial lining of the joint,

bursa, or tendon, involves the large joints such as the knee and hip joints.

Localized - Most common and involves small joints of the hand/feet.

The extra-articular form of PVNS is very rare and often represents an extension of a primary intra-articular process.

CLINICAL FEATURES:

--Pain of long duration/ Decreased range of motion/ Joint locking/ swelling of joint.

--Extra articular present with soft-tissue mass.

--Slight female predominance seen.

ETIOLOGY:

--Inflammatory process

--Repeated hemorrhage into the joint

--Repetitive mild trauma

--Neoplasia

--Disorder of lipid metabolism

--Genetic rearrangement in both localized and diffuse types of PVNS : chromosome 1p11-13, a site for CSF-1 gene, which most commonly fuses to COL6a3 on chromosome 2q35.

CASE REPORTS A 41 yr old female came with complaints of –

Pain and swelling in both shoulders for past 5 months.

Shoulder joint movement restriction since 5 months.

Past history- No history of trauma.

Not a known case of Tuberculosis/ Hypertension/ Diabetes mellitus.

Previous CT imaging was done but images were not available.

Family history- Not significant

EXAMINATION

GENERAL EXAMINATION: - Afebrile, vitals stable

SYSTEMIC EXAMINATION:

OTHER SYSTEMS- RS/ CVS/ CNS – No abnormality detected.P/A- Soft.

LOCAL EXAMINATION:

-Inspection : Bilateral shoulder joint mild swelling. Shoulder joint movement was restricted. -Palpation: Bilateral warmth with tenderness present. Bilateral shoulder joint soft – firm mobile masses . Measurement- 5 x 4cm in right shoulder. 10 x 8cm in left shoulder.

CLINICAL DIAGNOSIS: ? Tubercular villonodular synovitis.

IMAGING

PLAIN RADIOGRAPH of both the shoulder joints showing homogenous soft tissue opacity infiltrating around the humerus.

ULTRASOUND of both shoulder showing markedly thickened hypo echoic synovium with nodular and villous projections with multiple heterogeneous echogenic masses

Gross distension of the sub acromial  sub deltoid bursa (red arrows) which is filled with fluid and a large extra articular multilobulated heterogeneous signal intensity lesion appearing low to intermediate signal intensity in T1w images (green arrows) with variable signal intensity in T2w images (yellow arrows) within it, superior to supraspinatus.

T1W Cor

T2W Cor

PDW Cor

RTRTRT

T2 FFE sequence showing few blooming artefacts (yellow arrow) signifying hemosiderin. Post contrast, the lesion shows intense contrast enhancement.

RT RTT2 FFE Cor

T1c + Gd Cor

PDW sequence showing few similar signal intensity lesions in the intra-articular region (yellow arrows) and supraspinatus muscle. Few bony lesions also noted (green arrow).

Post contrast sequence showing similar enhancement pattern as the extra-articular component.

RT RT T1c + Gd Cor

PDW Axial

MRI PDW axial sequence showing few subchondral cystic changes in the head of humerus (yellow arrow).

Few lesions showing similar signal intensity and enhancement pattern also seen in left head of humerus.

DIAGNOSISEnhancing proliferative synovium with a lobulated

mass in the subacromial subdeltoid bursa

Erosions in the humeral head with minimal involvement of the shoulder joint and subscapularis muscle

Features suggestive of extraarticular and intra articular form of diffuse type of pigmented villonodular synovitis

DIFFERENTIAL DIAGNOSISPrimary synovial chondromatosis --T2: high signal --loose bodies formation

Lipoma arborescens --T2: high signal; will saturate on fat suppressed sequences

Synovial sarcoma--Mass around, but outside of, joint--Frequently calcify

Degenerative arthritis--Joint space narrowing--Subchondral sclerosis

CONCLUSIONPVNS involvement of the shoulder joint is a rare

location with bilateral joints involved is uncommon.

Findings of both intraarticular and extraarticular involvement is a rare presentation

The radiologic appearances of intraarticular PVNS, particularly its MR imaging findings, are frequently pathognomonic.

MR imaging is the optimal modality to depict and define the intraarticular and, particularly, extraarticular disease extent in order to guide complete surgical excision

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JaffeHL, Lichtenstein L, Sutro CJ. Pigmented villonodular synovitis, bursitis and tenosynovitis. Arch Pathol1941; 31: 731–765.

WeissSW, Goldblum JR. Benign tumors and tumor-like lesions of synovial tissue. In: Enzinger & Weiss’s soft tissue tumors. Philadelphia, Pa: Mosby Elsevier, 2008; 769–788.

KempsonRL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley RK. Synovial tumors. In: Atlas of tumor pathology: tumors of the soft tissues. 3rd series. Washington, DC: Armed Forces Institute of Pathology, 2001;138:387–394.

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