Primary tumours of the synovium
A REPORT OF FOUR CASES OF MALIGNANT TUMOUR
A. K. Bhadra,
R. Pollock,
R. P. Tirabosco,
J. A. M. Skinner,
S. R. Cannon,
T. W. R. Briggs,
A. M. Flanagan
From the Royal
National
Orthopaedic
Hospital, Stanmore,
England

A. K. Bhadra, MBBS,
MRCSEd, Orthopaedic
Registrar

R. Pollock, FRCS, FRCS(Orth),
Consultant Orthopaedic
Surgeon

R. P. Tirabosco, MD,
Histopathologist

J. A. M. Skinner, FRCS,
FRCS(Orth), Consultant
Orthopaedic Surgeon

S. R. Cannon, MA,
MCh(Orth), FRCS, Consultant
Orthopaedic Surgeon

T. W. R. Briggs, MCh,
FRCS(Orth), Consultant
Orthopaedic Surgeon

A. M. Flanagan, PhD,
FRCPath, Professor of
Histopathology
Royal National Orthopaedic
Hospital, Brockley Hill,
Stanmore, Middlesex HA7 4LP,
UK.
Correspondence should be sent
to Mr A. K. Bhadra; e-mail:
arupbhadra@yahoo.com
2007 British Editorial Society
of Bone and Joint Surgery
doi:10.1302/0301-620X.89B11.
18963 $2.00
J Bone Joint Surg [Br]
2007;89-B:1504-8.
Received 27 November 2006;
Accepted after revision 11 June
2007
1504
Four patients who developed malignant synovial tumours are described; one with
chondromatosis developed a synovial chondrosarcoma and three with pigmented
villonodular synovitis developed malignant change. The relevant literature is discussed.
Primary synovial pathology is almost always
benign. The most commonly observed change
is inflammation, but conditions such as syn-
ovial chondromatosis and pigmented villo-
nodular synovitis are sometimes seen. Primary
malignant synovial pathology is extremely
rare. Over the last ten years in our specialist
musculoskeletal tumour unit we have treated
only four patients with primary malignant syn-
ovial tumours. We present their cases and
review the literature.
Synovial chondrosarcoma
Case 1. An 81-year-old man presented with a
six-month history of swelling and stiffness of
his right knee. Clinical examination revealed a
tender nodule in the popliteal fossa and a range
of flexion of 60 to 90. Radiographs and sub-
sequent MRI showed popcorn calcification
consistent with synovial chondromatosis
(Fig. 1). A core biopsy of the lesion revealed an
atypical chondroid neoplasm consistent with
synovial chondromatosis. He underwent open
debulking of the tumour through a posterior
approach. A piecemeal excision was per-
formed in order to preserve the popliteal neu-
rovascular structures.
The specimen was examined by histo-
pathology and was reported as atypical chon-
droid neoplasm. Initially he made a good post-
operative recovery and the fixed-flexion defor-
mity resolved, but within three months his swell-
ing and stiffness had returned. Further
radiographs and MRI confirmed local recur-
rence. Given the severity of his symptoms and
the volume of the recurrent tumour, an above-
knee amputation was performed. Histological
analysis of the amputated limb confirmed the
presence of a synovial chondrosarcoma (Fig. 2).
CT of the chest revealed no pulmonary
metastases. He was not given any adjuvant
treatment and is alive and well six months after
amputation.
Malignant pigmented villonodular synovitis
Case 2. A 65-year-old man presented with pain
and swelling of his left knee. Clinical examina-
tion revealed a firm mass 3 cm 4 cm in size in
front of the patellar tendon. He had a full
range of movement. A core biopsy confirmed
the diagnosis of pigmented villonodular syno-
vitis with atypical features of focal necrosis
and myxoid change. Local excision was per-
formed, but this was intralesional since the
tumour was adherent to the patellar tendon.
Within three months he developed a local
recurrence. He underwent a further excision
with sacrifice of the patellar tendon because of
recurrence and heavy involvement of the patel-
lar tendon, and reconstruction with a gastro-
cnemius flap and split-skin graft. Histological
analysis revealed a malignant spindle-cell
tumour within pigmented villonodular syno-
vitis with clear margins. Post-operatively, he
was given radiotherapy (60 Gy). Two years
Fig. 1a Fig. 1b
a) Lateral radiograph showing scattered calcification in
synovial chondromatosis (white arrow), and b) T2-
weighted sagittal MR scan of the knee showing hetero-
genous malignant synovial chondromatosis (white
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 PRIMARY TUMOURS OF THE SYNOVIUM
Fig. 2a
Photomicrographs of a) synovial chondrosarcoma permeating bone and b) infiltrating branches of a major nerve (haematoxylin and eosin 100).
later he presented again with swelling around the left knee
and pain in the left hip associated with loss of weight. Clin-
ical examination showed obvious recurrent tumour in the
knee and further MR imaging revealed metastases in his
pelvis and lungs. He had local radiotherapy to the pelvis
and systemic treatment with Doxorubicin. He died two
years and ten months after the initial diagnosis.
Case 3. A 72-year-old woman presented with a four-year
history of pain and intermittent swelling of the left knee.
She had undergone arthroscopic debridement on four sep-
arate occasions with little benefit. Clinical examination
revealed an effusion and a reduced range of movement.
Plain radiographs showed early tricompartmental osteo-
arthritis and a well-defined lytic lateral femoral lesion. MRI
showed diffuse, low-signal synovial thickening with ero-
sion of the lateral femoral condyle extending into the
patellofemoral joint. A core biopsy confirmed the diagnosis
of pigmented villonodular synovitis. In view of the size of
the bony lesion and its intra-articular extension she under-
went an en bloc excision of the distal femur and endopros-
thetic replacement. The histology of the resected femur
confirmed the diagnosis of pigmented villonodular synovi-
tis and showed its margins to be clear of tumour. She made
a good recovery, regained a full range of movement and was
able to walk unaided. Eight months later she presented
again with pain and swelling of the thigh. MRI showed a
lobular recurrent tumour surrounding the distal femur and
extending into the muscles of the proximal thigh. CT of the
chest showed no metastases to the lungs. An open biopsy
confirmed malignant change in pigmented villonodular
synovitis (Fig. 3). She underwent a hip disarticulation. His-
tological analysis of the amputated limb confirmed malig-
nant pigmented villonodular synovitis with clear margins.
Six months later she is alive and well.
VOL. 89-B, No. 11, NOVEMBER 2007
1505
Fig. 2b
Case 4. A 53-year-old woman presented with a swelling on
the ulnar aspect of the right ring finger in its proximal part.
It had been present for approximately five years. It had
become gradually larger but caused no pain. On examina-
tion she had a normal range of movement in the fourth
metacarpophalangeal and interphalangeal joints and
neurological examination was normal. A clinical diagnosis
of a ganglion was made and she underwent local excision.
The histological diagnosis was thought to be a low-grade
(Trojani grade I, low grade soft-tissue tumour) spindle-cell
sarcoma with myofibroblastic differentiation and a posi-
tive margin. Two months later she presented again with
further local swelling. Clinical examination revealed a
nodule on the radial side of the flexor tendons overlying
the proximal phalanx. MRI confirmed a local recurrence.
She underwent a wide local excision although was given
the option of disarticulation of the ring finger at the meta-
carpophalangeal joint. Histological examination showed
fibrous connective tissue including synovium containing a
multinodular fibroblastic neoplasm. There was a fascicu-
late growth pattern of slightly atypical spindle cells. The
lesion was considered to represent low-grade sarcomatous
transformation of pigmented villonodular synovitis. The
patient remains well eight months after her last operation.
Discussion
In our unit over the last ten years we have treated 94 cases
of synovial chondromatosis and 184 of pigmented villo-
nodular synovitis. We have observed one case (1.06%) of
malignant synovial chondromatosis and three (1.63%) of
malignant pigmented villonodular synovitis. These four
cases represent 1.43% of our case load which confirms
that primary malignant synovial pathology is extremely
rare.
1506 A. K. BHADRA, R. POLLOCK, R. P. TIRABOSCO, J. A. M. SKINNER, S. R. CANNON, T. W. R. BRIGGS, A. M. FLANAGAN

Fig. 3a Fig. 3b

Photomicrographs showing a) conventional pigmented villonodular synovitis with a mixed population of mononuclear cells admixed with multi-
nucleated giant cells, foamy histiocytes and haemosiderin pigment, and b) malignant pigmented villonodular synovitis characterised by diffuse,
compact sheets and fascicles of uniform and clearly atypical round-to-spindle cells (haematoxylin and eosin 100).

Synovial chondromatosis is a benign neoplasm of syn-
ovial tissue. It most commonly affects the knee, but can
affect any synovial joint. It is characterised by the presence
of multiple cartilaginous bodies arising from the internal
surface of the joint or from other structures lined by syn-
ovium.1 Its treatment is surgical, usually arthroscopic,
removal of loose bodies, synovectomy or, more rarely, exci-
sion followed by joint replacement.2 The clinical and radio-
logical features of synovial chondromatosis and synovial
chondrosarcoma, whether primary or secondary, are the
same. It is therefore difficult to distinguish between the two
entities. Recurrence is not uncommon even after synovec-
tomy. Histologically, synovial chondromatosis is character-
ised by cartilaginous metaplasia of the intimal layer of the
synovial membrane in a solid matrix without myxoid
change. In synovial chondrosarcoma the stroma shows
myxoid change and the chondrocytes show marked cyto-
logical atypia.1,3,4 The most important feature favouring
synovial chondrosarcoma is the permeation of bone.
Although malignant change in synovial chondromatosis is
well documented in the literature (Table I),5-15 it is
extremely rare.
In our case of synovial chondrosarcoma the tumour
recurred after excision and required above-knee amputa-
tion. We believe that it was originally benign, but had
undergone malignant transformation in some areas before
the first operation. It is possible that the biopsy was
unrepresentative of the areas which had undergone malig-
nant transformation and therefore did not show this.
Pigmented villonodular synovitis commonly affects large
joints such as the knee and hip but also any area of synovium.
There are reports of cases involving the temporomandibular
joint, the zygapophyseal joint and the calcaneonavicular, mid-
tarsal and interphalangeal joints.16-18 The presentation is
usually insidious and patients usually present months or
years after the onset of symptoms with slowly progressive
disease.
Histologically, pigmented villonodular synovitis is a
benign condition of the synovial membrane and is pre-
sumed to be of histiocytic origin.19 Its cause is unknown.
Jaffe, Lichtenstein and Sutro20 originally described its main
histopathological features and proposed an inflammatory
pathogenesis. Others19,21 believe that it is a neoplasm and
this view is supported by chromosomal studies.11
Malignant pigmented villonodular synovitis is
extremely rare. A review of the literature (Table I) shows
that there are only a few reported cases.22-28 The largest
series of eight cases was reported by Bertoni et al22 in
1997. It may occur as a primary malignancy or may arise
in an area of pigmented villonodular synovitis. It can be
difficult to differentiate from benign aggressive pig-
mented villonodular synovitis. The pathological criteria
currently used to diagnose malignant pigmented villon-
odular synovitis are a sarcoma arising within it or at the
site of a previously diagnosed synovitis, or a synovial-
based sarcoma with cytological features similar to sar-
coma arising in a well-documented pigmented villonod-
ular synovitis.
Histologically, malignant pigmented villonodular syn-
ovitis is characterised by a uniform proliferation of atyp-
ical round-to-spindle shaped cells arranged in sheets and
fascicles (Fig. 3b).
Multinucleate cells and foamy histiocytes are not typ-
ical features of malignant pigmented villonodular syno-
vitis. Diffuse areas of ischaemic-like necrosis are
generally present.

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 PRIMARY TUMOURS OF THE SYNOVIUM 1507

Table I. Review of the literature on synovial chondrosarcoma secondary to synovial chondromatosis and pigmented villonodular synovitis

Age of patient
Author/s (yrs) Gender Site Treatment Follow-up
Synovial chondrosarcoma
Kenan et al3 74 M Knee Synovectomy No metastases
Arthrotomy of knee
4
Bertoni et al 30 to 66 M4:F2 Hip (2) Hemipelvectomy Lung metastases (2)
Knee (3) Above-knee amputation No metastases (4)
Elbow (1) Shoulder disarticulation

Brannon and Tracey5 34 M Hip Synovectomy Lung metastases

Hemipelvectomy

Dunn et al6 67 M Knee Hip disarticulation Lung metastases

13
Nixon et al 33 M Knee Above-knee amputation Lung metastases
7
Goldman and Lichtenstein 58 M Knee (all) Synovectomy (all) Not available
37 Above-knee amputation (1)
41

Hamilton et al8 51 M Knee Hip disarticulation No metastases

Kaiser et al9 47 M Ankle Synovectomy No metastases

Below-knee amputation

King et al10 43 M Knee Synovectomy Lung metastases

Above-knee amputation

Manivel et al11 50 M Knee Synovectomy Not available

12
Mullins et al 43 F Knee Synovectomy Lung metastases
Hip disarticulation

Perry et al14 51 F Knee Synovectomy Lung metastases

Above-knee amputation

Hallam et al15 45 M Knee Synovectomy No metastases

Total knee replacement

Pigmented villonodular synovitis

Enzinger and Weiss19 Foot Wide local excision No follow-up
Lung metastases after several
years

Bertoni et al22 12 to 79 M3:F5 Knee (3) Wide local excision (3) Two alive in 13 years
Ankle (2) Intralesional excision (5) Lung metastases five
Foot (1) years
Cheek (1) Dead 5.5 years
Thigh (1) Lung metastases (3)
and
Dead 2 to 4 years
Above-knee amputa-
tion (2), alive 13 to 22
years

Castens and Howell23 48 F Dorsum to right foot Wide local excision, below- Alive 13 years
knee amputation, re-excision Multiple local
stump recurrence
No lung metastases

Ushijima et al24 59 M Right knee Synovectomy 14 years alive

Three local recurrence No lung metastases
Below-knee amputation
Stump and groin recurrence
Hemipelvectomy

Nielsen and Kiaer25 67 M Left knee Incision biopsy (pathological Radiotherapy

fracture of tibial plateau) Dead eight months
Above-knee amputation, after above-knee
multiple bony metastases amputation

Abdul-Karim et al26 Right knee Synovectomy

Local recurrence 11 years later

Shinjo et al27 72 F Right hip Open biopsy Lung metastases

Chemotherapy Dead four months
Wide local excision later
Local recurrence (10 months)
Hemipelvectomy

Schajowicz28 Knee Wide local excision Two years dead

Two recurrence Lung metastases
Above-knee amputation

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1508 A. K. BHADRA, R. POLLOCK, R. P. TIRABOSCO, J. A. M. SKINNER, S. R. CANNON, T. W. R. BRIGGS, A. M. FLANAGAN
Most of the reported malignant cases19,22-29 had multiple
local recurrences or wide bony metastases particularly
those which had first been treated by local excision. More
than half of these patients needed an amputation or more
extensive surgery at a later date and also had pulmonary
metastases. Local recurrence or metastatic disease may
occur years after the initial presentation. It is therefore
important that patients with synovial malignancy are fol-
lowed up for a prolonged period. Amputation should be
considered at the outset as a life-saving procedure rather
than an attempt to salvage the limb.
Most cases of synovial disease are inflammatory or
benign in nature. Our recent experience suggests that
malignancy should be considered in patients with atypical
symptoms. If there is any doubt about the diagnosis pre-
operatively we recommend MRI and a core biopsy. If syn-
ovial malignancy involving a joint is confirmed then either
an extra-articular resection or amputation is required. It is
essential to achieve local control of these tumours since
they are resistant to both radiotherapy and chemotherapy.
No benefits in any form have been received or will be received from a commer-
cial party related directly or indirectly to the subject of this article.
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