Pigmented Villonodular Synovitis of Shoulder

The New Journal of Medicine 2010;27: 122-125
Case report
Pigmented Villonodular Synovitis of Shoulder;
Radiologic Assessment with MRI Findings
Hasan AYDIN 1, Nilay Aydn OKTAY 1, Hüsamettin SARGIN 2, Baki HEKİMOĞLU
1
1
Dşkap Yldrm Beyazit Research Hospital Radiology Department, ANKARA
2
Egerad MR Imaging Center, ANKARA-TURKEY
ÖZET
Omuzda pigmente villonodüler sinovit; MR görüntüleme bulgulari ile radyolojik analiz
Pigmente villonodüler sinovit sinovyumun birikim bozukluğu olup, eklem-tendon ve bursalar snrlayan dokularda
hemosiderin birikimi ile karakterizedir. PVNS sonrasnda
diffüz ve lokalize formlar olarak snflandrlmştr, en çok
etkilenen eklemler diz-kalça ve parmaklardr. Omuz
tutulumu oldukça nadirdir. Bu çalşmada, 45 yaşnda
erkek hastann sağ omzunda görülen diffüz villonodüler
sinovit olgusu sunulmuştur. Tan patognomonik MR
görüntüleme bulgular ve lezyonlarn histopatolojik
analizi ile konmuştur. Buradaki eklem tutulumu monoartiküler tiptedir.
ABSTRACT
Pigmented villonoduler synovitis is a proliferative
disorder of the synovium and deposition of hemosiderin
into the lining tissues of joints, tendons and bursae.
PVNS is further classified into diffuse and localized
forms, the most affected joints are the knees, hips and
fingers. Shoulder involvement is extremely rare. In this
paper, we present diffuse villonoduler synovitis of the
right shoulder of a 45 year old male. The diagnosis is
made by pathognomonic MRI findings and histopathological analysis of the lesions. The joint involvement in
this case is of monoarticular type.
Key Words: Pigmented villonodular synovitis-ShoulderMRI findings
Anahtar Kelimeler: Pigmente villonodüler sinovit, omuzMR görüntüleme bulgular
INTRODUCTION
PVNS is a benign tissue proliferation which
presents as a borderline case between a reactive
and a neoplastic process and emanates from the
tendosynovial layers, joint capsule or the synovial
bursa1. Two forms of PVNS can be differentiated
macroscopically. The diffuse form, which involves
the entire synovia is of a joint and often occurs in
large joints; the localized nodular form with
isolated circumscribed or pedunculated tissue
masses in the synovia most commonly seen at the
flexor tendon sheaths of the hand1-3.
Histologically, hyperplastic synovial villi with many
foam cells and hemosiderin-storing macrophages
are seen, as well as multinuclear giant cells1,4.
Primarily, PVNS appears in the synovial structures
of the knee and fingers in 80%of cases5, it
occasionally involves the hip and ankle joints but
very
rarely
involves
the
shoulder
and
temporomandibuler joints1,3,6. Polyarticular PVNS
appears in less than 1% of all cases5. The rate of
PVNS is approximately 1.8 patients per million
population2,7,8. Diffuse PVNS in the shoulder is
extremely rare and less than 10 cases reported so
far1,9. The total number of diffuse and nodular
forms of PVNS is approximately 251,3,4. The
disease is characteristically monoarticular and
usually slowly progressive, most of the patients
present with progressive painful swelling and
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limited motion of the affected shoulder1,3-5. Joint
stiffness is common in cases of long duration1-6.
We present here diffuse monoarticular PVNS of
the shoulder of a middle aged male with MRI and
histopathological analysis.
CASE REPORT
A 45 year old male without trauma history
developed right shoulder pain and limitation of
movements over 6 months, the pain got worser at
the last 2 months. He also noted progressive
weakness of her right shoulder over a period of 9
months. He was taking aspirin, cataflam and
myadren for relief of this condition but
unfortunately the pain was progressed especially
at the last 2 months. Physical examination
revealed marked discomfort in flexion, internal
and external rotation of the right shoulder. The
abduction is 70%. The acromio-clavicular joint
was mildly tender to palpation. Complete blood
count, serum electrolytes and urine analysis was
within normal limits. Plain radiography of the right
shoulder showed no abnormalities. Magnetic
resonance imaging of the shoulder showed a large
glenohumeral joint effusion especially at the
superior side with increased fluid loculation in the
subdeltoid and subcoracoid bursae (Figure 1,2).
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H. Aydın
et al.
seen in the anterior and inferior part of subdeltoid
bursa, superior part of joint capsule,lesser
tuberosity and local subscapularis insertion area,
inferomedial portion of the supraspinatus muscle,
supraglenoid fossa and near subcoracoid bursa
(Figure 4-5). There are also subchondral
degenerative microcysts in humeral head. These
shoulder changes in the MRI section is thought to
be due to the diffuse extensive pigmented
villonodular synovitis.
Figure 1. Humero-glenoidal effusion
Figure 4. Pigmented villonodular synovitis in glenohumeral joint-subcoracoid bursa-supraglenoid fossa and
etc.
Figure 2. Effusion located at subcoracoid and subdeltoid
bursae
In the T2W images there is a homogeneous
increased signal at the articular side of greater
tuberosity and apparent hemoragic effusion
medially-superolaterally due to 2-2.5 cm rotator
cuff tear, especially supra and infraspinatus
tendon tears (Figure 3).
Figure 5. Pigmented villonodular lesions in the lesser
tuberosity-subscapularis insertion-inferomedial part of
supraspinatus tendon-subdeltoid bursa and inferior part
of humero-glenoidal articular joint
Figure 3. Rotator cuff tears
There is no marked tendon retraction of rotator
cuff. In both T1-T2W images, there are nodular
spheric conglomerated heterogeneous grape
shaped
cummulative
lesions
with
marked
decreased signal intensities and with internal loose
bodies-fluid, surrounded by effusion externally.
The major involved site at the right shoulder is the
inferior part of glenohumeral joint and the
infraglenoid fossa. However, the abnormal
hypointense nodular lesion accumulations are also
Total shoulder arthroplasty and total synovectomy
is offered to the patient both for diagnosis and the
treatment but he refused to get operated. A
serosangineous effusion and synovium is taken
from the inferior gleno-humeral joint afterwards.
Microscopic and histological analysis of the
material revealed hyperplastic synovial tissue and
enlarged synovial villi loaded with foamy cells,
numerous
densely
distributed
mononuclear
fibroblasts hemosiderin laden phagocytes, diffuse
elongated areas with siderophages, histiocytes
and multinucleated giant cells (Figure 6-7).
MRI findings and the histological analysis together
is consistent with the diffuse form of pigmented
villonoduler shoulder synovitis. Due to the
operation refusal, the patient underwent medical
treatment, analgesics-myorelaxants were prescribed.
Hydrotherapy and physical therapy will be started
after the medical therapy.
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H. Aydın et al.
Figure 6. Numerous hemosiderin containing phagocytes-histiocytes and multinuclear giant cells
Figure 7. Hyperplastyic synovial tissue and enlarged villi
DISCUSSION
PVNS is a primarily monoarticular, locally benign
proliferative synovial lesion affecting joints,
bursae and the tendon sheaths3,4,10. In the past,
these lesions have been identified as benign
synovioma, xanthoma, giant cell tumor of the
tendon sheaths, hemorrhagic villous synovitis and
proliferative synovitis10,11. PVNS was first defined
by Jaffe et al in 1941 who described a series of
patients with proliferative lesions arising from the
synovium of various joints12. The knee is the most
common affected site, shoulder and temporomandibular joints involvement are very rare1,3,4,6.
Two separate forms of PVNS is presented up to
now, the diffuse form and the localized nodular
form1-4. The diffuse form is characterized by
general involvement of entire synovia, was
generally associated with pain and muscle tears;
the localized nodular form is an isolated
proliferative solid tumoral lesions of the synovia,
generally presented as a painful enlarging mass
with decreased motion, acute effusion painfull
subluxation and impingement1-4,10. The cause of
PVNS is not obvious, but the possibility of an
inflammatory or traumatic origin has been
raised5,10,11 but in this case there is no traumatic
and enfectious history. Men and women are
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The New Journal of Medicine 2010;27: 122-125
affected equally and it is more common during the
4th and 5th decades of life4,7,10.
The radiographic appearance of the diffuse form of
PVNS is usually normal; localized nodular form
varies from a normal osseous anatomy with soft
tissue mass or small cystic erosions of the
subchondral bone in the early stages of disease,
to juxta-articular cystic lucency of the glenoid and
humeral head in the late stages3,4,10. In the MRI
sections, the diffuse form of PVNS is usually
presented with joint effusion-fluid in the shoulder
bursae, dark hemosiderin pigmented synovium
accumulation in T1-T2W sequences especially in
the glenohumeral joints, rotator cuff tear can also
be seen in some cases. The localized nodular form
is generally presented with a soft tissue mass
involving the shoulder capsule and rotator cuff
muscle tendons in the MR imaging1,10,13. In the
differential
diagnosis:
rheumatoid
arthritis,
tuberculous arthritis, osteoarthritis, hypertrofic
synovitis, hemophilic arthritis can appear similar
to diffuse form of PVNS, especially when the bone
changes are added to synovial abnormalities and
presence of intra-articular hemorrhage. Synovial
chondromatozis, synovial sarcoma, benign or
malignant synovioma, tumoral lesions of the
muscles and bone in the involved area can mimic
the nodular form of PVNS especially when these
lesions don’t contain much more irregular
calcifications1,3-5. The presence of PVNS is
confirmed with histological examinations. In the
histological sections, the presence of hyperplastic
synovial cells, long villi under the proliferative
synovia-histiocytic and foam cells, multinucleated
phagocytic giant cells with hemosiderin deposits,
surface fibrin deposits and stromal fibrosis, a
loose hyalinized collagenous background tissues
are the issues that strongly support the diagnosis
of PVNS1,3,4,10.
Dorwart et al. in reported two cases: He diagnosed a
soft tissue mass in the X-Ray of a left shoulder of
a middle aged male, found filling of subacromial
and subdeltoid bursae of right shoulder of an old
lady in the single-contrast arthrogram consistent
with rotator cuff tear.Both cases underwent
shoulder arthroplasty and final histopathological
diagnosis is consistent with PVNS of diffuse and
nodular forms5.
Mulier et al. in reported a diffuse PVNS with rotator
cuff tear, the patient is an old male with
subacromial calcifications and acromio-clavicular
osteoarthritis. He believed that rotator cuff tear
was the result of a classic impingement
syndrome13.
Tong et al. in presented a case of diffuse PVNS
with rotator cuff tear and cystic erosions at
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H. Aydın
et al.
greater tuberosity, patient is a male at fifties, he
suspected that rotator cuff tear in their case was
caused directly by the locally invasive villonodular
synovial lesions as seen in our case9.
Sawmiller et al. presented an extra-articular
diffuse PVNS, he found complete rotator cuff tear,
large glenohumeral joint effusion with fluid in the
subacromial and subdeltoid bursae, a large
acromial spur in the right shoulder MRI of a 57
year old female. Intra-operatively an abnormal
bursal lesion anterior to the supraspinatus
insertion is noted and there is no contact of the
lesion with synovial lining10. The histopathological
studies are consistent with the PVNS of the extraarticular origin. In his case, he believed that the
rotator cuff tear was most likely secondary to a
classic impingement syndrome due to a large spur
at the anteroinferior tip of the acromial process,
not by direct invasion of the lesions. In our case
we have similar findings but there is no acromial
spur and we believe that rotator cuff tear is due to
invasion of the villonodular synovitis.
Molina et al, in presented another diffuse PVNS in
the left shoulder of or 77 year old female. The
patient had profuse glenohumeral joint effusion
and also had rheumatoid arthritis. Arthroscopy+
arthrocentesis are applied and the histopathological
results are consistent with the diagnosis of PVNS4.
For the localized nodular form of PVNS: Sher et al.
in, Sotje et al. in reported two cases, painful soft
tissue masses and lucent defects in the humeral
heads. After the local excision and synovectomy,
nodular PVNS is diagnosed14,15.
Cheng et al in presented a nodular PVNS after an
anterior capsulolabral reconstruction. The patient
was a 20 year old male, had a large mass in the
inferior portion of the left joint capsule in plain
radiographs. Local excision of the lesion is applied
and results are consistent with nodular form of
PVNS3.
Konrath et al. in reported another case, it is an
extra-articular nodular mass lesion found in the
subacromial bursa,local excision and histopathological studies suggested the diagnosis16.
Muller et al. in presented a case of a 16 year old
male with a left proximal humerus lesion studied
by MRI, the lesion appeared to simulate a
malignant soft tissue tumor. After the local
extirpation of the mass, final diagnosis is nodular
PVNS1.
Upon review of the literature, our case with
abundant hyperplastic hemosiderin pigmented
synovial lesions localized nearly in the entire right
shoulder, inferior glenohumeral joint, infraglenoid
fossa, near the insertion of rotator cuff tendons, at
the region of subcoracoid bursa and etc. is the
first report of diffuse PVNS with profuse shoulder
involvement. As seen in the literature, in our case
there are also joint effusions, fluid in subdeltoid
and subcoracoid bursae and tear in the supra and
infraspinatus tendons of rotator cuff. We believe
that the rotator cuff tear in our case is due to the
direct invasion of villonodular synovitis.
Finally, managing of PVNS based on a review of
literature is, surgical marginal resection in localized
nodular form and broad or radical synovectomy in
diffuse form have been suggested. Most authors
agree that recurrence of both PVNS forms after
surgery is uncommon1,3,4,10.
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Correspondence:
Hasan AYDIN M.D.
Dşkap Yldrm Bayezit Hospital, Department of Radiology, Ankara
e-mail:[email protected]
Arrival date
: 13.10.2009
Acceptance date
: 12.01.2010
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