PIGMENTED VILLONODULAR SYNOVITIS,SYNOVIAL CHONDROMATOSIS & LOOSE BODIES

Dr.Darshan.C.K JSS Medical College

PVNS

Synonyms:(PVNS, xanthoma, xanthogranuloma, fibroxanthoma, giant cell tumor of tendon sheath, benign synovioma, villous arthritis.)

It is a proliferative condition of the synovium characterized by monoarticular involvement (Knee) & the presence of inflammation and hemosiderin deposition in the synovium.

History PVNS was first described in 1852 by Flandry F, Hughston

JC. Originally thought to be a neoplastic process because of its

growth pattern, capacity to erode surrounding bone and joint tissue, and high recurrence rate after resection.

In 1941, Jaffe et al presented the pathologic entity as a synovitis, thereby shifting the focus from a neoplastic process to an inflammatory one.

Recent observations of cytogenetic abnormalities demonstrate that its pathogenesis remains unresolved.

Etiology

1.Unknown

2.Trauma

3. local metabolic activity

4.Neoplastic process.

5.Inflammatory process

Etiology

A) Trauma:

Some author suggest that it occurs as a result of trauma and subsequent recurrent local hemorrhage to the affected joint.

Hemophiliac synovial pathology differs from PVNS, in that it lacks lipid laden histiocytes and giant cells, which are considered classic indications of PVNS

The theory of repeated trauma and hemarthrosis as an explanation for PVNS has fallen out of favor.

EtiologyB) Abnormal local metabolic activity:

suggested as an inciting event for the inflammation seen in PvNS, but it has been an

inconsistent finding.

C) Neoplastic The presence of trisomy 7 and clonal DNA rearrangements & presence of

DNA aneuploidy. Pointers to neoplastic aetiology -invasive character of diffuse pigmented villonodular synovitis -aneuploidy by DNA analysis and chromosomal rearrangements

Etiology

Many chromosomal abnormalities have been detected. -Dysregulation of apoptosis with high cytoplasmic levels of B cell lymphoma protein 2(Bcl2) - mitochondrial dysfunction with high levels of humanin genes further support a neoplastic origin. Humanin genes, anti-apoptotic peptides encoded in the mitochondrial genome that act

against Bax (bcl-2-associated X protein), are strongly expressed in diffuse pigmented villonodular synovitis, but barely detected in localized pigmented villonodular synovitis.

Etiology

There also are more CD57 (natural killer) cells in diffuse pigmented villonodular synovitis contrasting with more CD3 and CD20 cells in localized pigmented villonodular synovitis.

Macrophages, more common in diffuse pigmented villonodular synovitis, have osteoclastic differentiation and proinflammatory cytokines including tumor necrosis factor a (TNF-a)

D) Inflammatory Analysis of cell populations in PVNS strongly support for its being a chronic

inflammatory process .Findings were based on the presence of a cell marker for inflammation within a heterogeneous population of mononuclear cells.

Clinical Presentation

IncidenceThe estimated annual incidence of pigmented villonodular synovitis is 1.8/million

A monoarticular process that often involves the large joints. Site :The knee is the most commonly involved joint, but others include the hip,

ankle, shoulder, and elbow. Age often appears in the 3rd decade of life. Sex: no sex based predilection. slowly progressive and tend to be episodic. Early mild pain is insidious

Clinical Presentation

Later limitation of motion, contracture, and joint locking can occur. Joint effusion is common and aspiration may yield brownish fluid due to

chronic hemorrhage or, less often, fresh blood. When joint involvement is diffuse, there may be warmth and tenderness. PVNS most often is intra-articular and monoarticular. involvement of a bursa is termed pigmented villonodular bursitis.

Symptoms are non-specific

Occurs in two forms: Localized (LPVNS) Diffuse (DPVNS) LPVNS and DPVNS, the two entities exist along a continuum of one

disease process. Histologically, LPVNS and DPVNS are similar, however, they differ in

their Gross apprance ,Clinical presentation, Prognosis, Response to treatment.

LPVNS most often affects the flexor tendons of the fingers Localized pigmented villonodular synovitis at this site is termed a

giant-cell tumor of the tendon sheath is second only to the ganglion cyst in incidence. frequently involves the toes and less often the knee and ankle Less common than DPVNS characterized by a pedunculated, lobular lesion localized to one area

of the synovium.

LPVNS

LPVNS lesions occur more common in the anterior compartment. Most cases lesions arise at the meniscocapsular junction. The synovium in the region of the anterior horn of the medial meniscus

is the mc site of involvement. Pt often present with suspicious meniscal pathology. Additionally, involvement of the infrapatellar fat pad, suprapatellar

pouch, intercondylar notch, ant horn of the lateral meniscus, medial and lateral recesses of the knee have been reported.

LPVNS has a favorable prognosis.

DPVNS

More common form often presents with global joint involvement most of all synovium of the joint is invovled Swelling and pain are more pronounced than in LPVNS DPVNS tends to have a more rapidly destructive course poorer prognosis. can present with extra-articular extension, either at the time of primary

diagnosis or as recurrent disease. can involve any synovial joint but is most common in large joints:

knee, hip, ankle, shoulder, and elbow in decreasing order of frequency.

DPVNS

Spinal involvement is rare. Extra-articular DPVNS can encroach on major neurovascular

structures, making surgical excision more challenging and complete excision difficult.

Despite treatment, the recurrence rate for DPVNS is reportedly as high as 46%.

The end result can be the need for total joint arthroplasty

DIFFERENTIALS

• Tuberculosis,• Synovial osteochondromatosis,• Hemophilic arthropathy,• Synovial hemangioma,• Secondary osteoarthritis and Amyloid arthropathy• early osteoarthritis, rheumatoid arthritis, a meniscal tear

Pathology

The gross appearance of the localized form ranges from a mottled red-brown to yellow-brown

single, large, lobulated mass originating from the synovium of the knee.

The diffuse type thickens the synovium resulting in tangled folds, fronds, and nodules with progressive encroachment on the joint space, bony erosion, and extra-articular extension.

Microscopically-villous hypertrophy of synovium Definitive diagnosis requires histological confirmation of biopsy

material

 Photograph shows the typical appearance of diffuse intraarticular disease, which appears brown (*) owing to hemosiderin deposition and has villonodular protrusions (arrowheads). 

Histology

proliferating oval mononucleated synovial cells appearing as a mixture of fibroblasts and histiocytes, irregularly distributed multinucleated giant cells with phenotypical features of osteoclasts,

islands of hemosiderin-loaded macrophages and foam cells intra- and extracellular deposition of hemosiderin an inflammatory infiltrate containing large numbers of T8 lymphocytes 

Histology differential diagnosis nonossifying fibroma benign fibrous histiocytoma. 

Histology

 Photomicrograph shows the villonodular fronds with overlying synovial tissue (solid arrowheads).

Brownish areas represent extensive hemosiderin deposition.

Inset photomicrograph demonstrates golden brown globular cytoplasmic intracellular hemosiderin (open arrowheads). 

Histology

Photomicrograph of localized extraarticular PVNS shows abrupt transition between the nodular tumor (*) and fibrous pseudocapsule (between arrowheads).

Inset photomicrograph shows both multinucleated giant cells (straight arrow) and reniform mononuclear histiocytoid cells (curved arrows).

Diagnosis

Not always obvious clinically. Various imaging modalities and lab investigations are often necessary to

exclude other conditions and narrow the diagnosis Radiograph Synovial fluid examination MRI Thallium scan

Diagnosis

A. Radiograph With early disease, radiographs may be normal or show only joint

effusion Soft tissue may be distinctive when hemosiderin deposition results in

high density, but calcification & periosteal reaction is rare With progression marginal erosions and cysts become visible well-defined erosions with a sclerotic margin and tend to be prominent

in joints with tight capsules such as the hip, elbow, and wrist. Long-standing disease also can result in corticated bone deformities Lack of joint space narrowing and osteophytes differentiate

pigmented villonodular synovitis from degenerative joint disease

Diagnosis

Radiological DD synovial osteochondromatosis when calcifications are absent tuberculous arthritis, hemophilia synovial hemangiomas

Diagnosis

B) Ultrasonography proliferative synovitis, loculated joint effusions complex heterogeneous

echogenic masses, and markedly thickened synovium Doppler shows increased blood flow in these vascular lesions.

c) Computed Tomography erosions and cysts of bones Soft-tissue density is high when there is hemosiderin deposition. High attenuation characteristics

CT arthrogram showing femoral head erosions

Diagnosis

D) MRI Modality of choice for diagnosing Highly sensitive and specific. Low signal intensity ("dark on dark“)on T1 & T2 images due

to haemorrhage & haemosidderin deposition Helpful in determining the extent of disease involvement

and distinguishing DPVNS from LPVNS. Typical MRI findings for LPVNS include a periarticular or

synovial nodular mass with varying degrees of bone erosion

Diagnosis

In DPVNS, there is a poorly localized mass or diffuse synovial thickening with varying degrees of periarticular erosions.

E) Thallium Scan

Increased TI-201 uptake in PVNS. TI-201 uptake may prove to be a useful diagnostic modality,

particularly in patients in whom there is no clear distinction between recurrent disease and early osteoarthritis..

Localized intraarticular PVNS of the knee in a 40-year-old woman with knee pain. (a)Lateral radiograph shows an ill-defined area of soft-tissue opacity that replaces the normal Hoffa fat pad (arrows). (b, c) Sagittal proton-density–weighted  (b) T1-weighted fat-suppressed postcontrast (c) images reveal the localized intermediate-signal-intensity soft-tissue mass (*), which has prominent diffuse enhancement. 

 Sagittal proton-density–weighted fat-suppressed (d) and gradient-echo  (e) images show overall increased signal intensity of the intraarticular soft-tissue mass (*). The gradient-echo image (e) also shows focal hypointense areas (arrowheads), findings that represent the blooming artifact from hemosiderin.  (f) Photograph of the resected specimen shows the brownish appearance caused by hemosiderin.

 

Diffuse PVNS of the knee in a 45-year-old man; multiple recurrences of PVNS ultimately led to amputation. (a) Lateral radiograph shows a large suprapatellar effusion and soft-tissue fullness about the knee (*). There are extrinsic erosions with marginal sclerosis (arrowheads) on both sides of the knee joint and patellofemoral degenerative changes. (b) Photograph of the sagittally sectioned gross specimen demonstrates the large mass throughout the knee joint (*). The prominent brownish areas (curved arrows) result from more prominent hemosiderin deposition. Extrinsic erosions (straight arrows) of the femur (F) and tibia (T) are also seen  

(c,d) Sagittal T1-weighted  (c) and gradient-echo (d) MR images reveal a large amount of low-signal-intensity tissue (*) with posterior extension (curved arrows) that replaces the entire knee joint. Extrinsic erosions of the femur and tibia with low-signal-intensity margins are also seen (straight arrows)..

Diagnosis

F) Synovial fluid examination Joint aspiration gives fluid that ranges in colour from yellow or straw,

with deep xanthochromia from previous haemorrhage, to brown-stained or frankly bloody.

Reports of synovial fluid analysis are sparse but findings point to inflammation and which include

slight elevation of protein, reduced glucose, low to moderate leucocyte count.

Management

The goal is to eradicate all abnormal synovial tissue, thus removing the source of pain and reducing the risk of joint destruction and recurrence.

A combination of surgical and nonsurgical approaches may be necessary Radiation Therapy Results are often mixed in PVNS. Delivered by two methods external beam radiation, intra-articular radiation synovectomy.

ManagementExternal beam radiation external beam radiation therapy can be highly useful in managing

refractory cases of PVNS or in those with extensive extra articular involvement.

No significant advantage has been reported to using adjuvant external beam radiation compared with surgical synovectomy alone.

Serious potential complications associated with external beam radiation, include

Skin reactions Poor wound healing Joint stiffness sarcomatous transformation

Management

Intra-articular radiation synovectomy it as an adjuvant treatment modality in PVNS. that intra-articular radiation therapy may be useful in the management of

PVNS, particularly in patients with documented postoperative residual disease that is still intra-articular.

Caution with the use of intra-articular radiation therapy is Radionecrosis of the soft tissues,Mild febrile and painful reactions.

Development of postradiation sarcoma and increased risk of other malignancies as a result of total body radiation exposure

SURGICAL Management

Arthroscopic Treatment of PVNS Arthroscopy has been associated with Better functional results and Lower rates of

postoperative stiffness than open techniques. Arthroscopic synovectomy in the knee offers excellent visualization of Anterior

compartment as well as the medial and lateral recesses. Posterior compartment synovectomy can be performed,Localized PVNS Arthroscopic partial synovectomy is preferred surgical option for LPVNS. Limited or partial synovectomy necessitates débridement of the PVNS mass along with a

rim of surrounding healthy synovium Anterior compartment lesions are addressed via standard AL and AM portals. Posterior compartment lesions require PM or PL accessory portals to ensure adequate

visualization and access to the entire lesion. Recurrence is rare after limited local treatment of LPvNS lesions

Arthroscopic Treatment of PVNSDiffuse PVNS In DPVNS the posterior compartment is typically involved and requires a

surgeon who is comfortable with the placement of accessory posterior arthroscopic portals

Patients with large popliteal masses or extra-articular involvement generally are not candidates for an exclusively arthroscopic approach.

Thorough, complete synovectomy is the treatment of choice for DPVNS. In addition to the risk of recurrence, arthroscopic excision carries with it a

theoretical risk of Joint seeding and Portal contamination Extensive joint involvement and Extraarticular spread may result after failed arthroscopic management Subcutaneous contamination of an arthroscopic portal from DPVNS (rare)

Open Surgical Management

Open arthrotomy and complete synovectomy is the standard surgical treatment for DPVNS.

In the knee, this is performed through a combined anterior and posterior approach.

Preferred anterior approach is through a midline incision and antromedial parapatellar arthrotomy.

The incisions must be extensive enough to allow flexion of the knee and lateral inversion of the patella.

This approach permits adequate exposure to the anterior aspect of the knee to perform the synovectomy.

Open Surgical Management

Open Surgical Management

Summary Treatment of each patient must be based on the Type of PVNS (ie, local or diffuse), Presence or absence of extra-articular disease, Level of experience of the surgeon. MRI should be performed on any person in whom the diagnosis of PVNS is being

considered. The presence of extra-articular involvement suggests a more aggressive form of

DPVNS, which should be managed with an aggressive open surgical approach rather than with arthroscopic surgery

LPVNS responds well to arthroscopic resection

Summary Moderately extensive intra-articular DPVNS can be managed with total

synovectomy with results equivalent to those of open synovectomy. Unless a surgeon has a very good understanding of the use of accessory

portals and arthroscopic technique total synovectomy, it is very difficult to treat DPVNS with arthroscopy alone.

For the less experienced arthroscopist, open anterior and posterior synovectomy offers better results and lower recurrence rates.

Total joint arthroplasty for persistent recurrent disease or in cases demonstrating end-stage arthritis is a viable option

External beam radiation is no longer used due to its complications

Summary

Infliximab (anti-TNF-a) in patients with recurrent pigmented villonodular synovitis has been reported to control the reactive synovitis without affecting the size of the neoplasm.

Long-term follow-up is essential since recurrence can occur many years after initial treatment

After recurrence, treatment consists of repeat synovectomy usually with intra-articular yttrium-90 or dysprosium-165.

These radionuclides are used to destroy small amounts of residual abnormal tissue and avoid the disadvantages of a Complete synovectomy

SYNOVIAL CHONDROMATOSIS

Synovial chondromatosis is a benign condition characterized by development of cartilaginous or osteocartilaginous bodies within synovial membranes of joints, bursae or tendon sheaths as a result of metaplasia of synovial fibroblasts

Also is termed synovial osteochondromatosis, synovial chondrometaplasia, articular ecchondrosis, and synovial chondrosis

SYNOVIAL CHONDROMATOSIS

Synovial chondromatosis has been divided into primary and Secondary forms.

Primary synovial chondromatosis, represent chondroid metaplasia in the subserous layer on inner surface of capsule of a joint with resultant formation of multiple intraarticular chondral bodies.

An identical process can also involve the synovium that extends along tendons and bursae and is frequently referred to as tenosynovial or bursal chondromatosis, respectively

Secondary synovial chondromatosis is associated with joint abnormalities, such as mechanical or arthritic conditions, that cause intraarticular chondral bodies

Pathology

Gross pathology- thickened synovial lining with multiple shiny blue-white glistening cartilage nodules- cobble stone appearance

synovium is moist, folded and hyperaemic Loose bodies noted-average 1-2 cm in diameter The cartilage nodules are attached by a synovial pedicle-

provides nutritional support, allows hypertrophy and endochondral ossification

After separation the synovial fluid provides nutrition but the osseous portion dies

Histology

Metaplastic synovial fibroblasts produce nodules of hyaline cartilage

Nodules undergo endochondral ossification with woven and lamellar bone

The hyaline cartilage in primary synovial chondromatosis is often hypercellular with atypical histologic features, including multinucleation, nuclear crowding, nuclear enlargement and hyperchromasia, and mild myxoid changes

Dystrophic mineralization can also occur centrally within the lobules.

Metastasis

No metastasis is noted No sarcomatous change Malignant degeneration to chondrosarcoma or de

novo intra articular chondro sarcoma can occur- incidence less than 1%

Clinical features

Male to female ratio- 1 : 1.4

Middle age adults affected

Not hereditary

10 percent have bilateral involvement

CLINICAL PRESENTATION

Low grade chronic pain Limitation of movement Limp & locking of joint Localised tenderness & swelling due to effusion degenerative disease Is ultimately a common sequel

X-ray Initial stages- effusion & juxta articular osteoporosis multiple intraarticular chondral bodies with "ring-and-arc chondroid

mineralization and extrinsic erosion of bone on both sides of the joint With hypertrophy, opacities become larger with peripheral linear densities

MRI MRI shows chondromas usually helps establish the diagnosis, and the images

demonstrate the true extent of the disease. High signal intensity on t2 weighted images due to hyperplasia of synovium

Synovial chondomatosis- shoulder joint

Synovial chondomatosis- hip joint

Management

The treatment of choice for primary synovial chondromatosis, either intraarticular or extraarticular (bursal or tenosynovial), is surgical resection

The overall recurrence rate for intraarticular disease in larger series appears to range from 3% to 23%, and recurrence may be related to incomplete resection in many cases

controversy exists regarding the extent of surgical treatment required whether removal of chondral bodies alone is sufficient or whether associated synovectomy is also necessary.

Management Milgram advocated synovectomy to treat active phase 1

disease (intrasynovial disease without chondral bodies). synovectomy with removal of chondral fragments for phase

2 disease (transitional form with intrasynovial disease and chondral bodies)

removal of the multiple chondral bodies alone and no synovectomy for phase 3 disease (late inactive disease with chondral bodies but no synovial abnormality)

Prognosis

Good prognosis

Synovial chondromatosis and synovialosteochondromatosis -remain uncommon, benign and self limited conditions

LOOSE BODIES Loose bodies are fragments of cartilage or bone that freely float inside

the knee joint space.  They can be the result of an injury or from generalized wear and tear

over time.  Depending on the severity of the condition, there can be one or many

loose bodies inside the joint.  stable (they don’t move about inside the joint) unstable (they float through the inside of the joint) which can cause pain

or loss of motion The loose bodies can vary in size from a few millimeters (such as the

size of a small pill) to a few centimeters (the size of a quarter). 

Symptoms

The feeling of something moving in knee. Intermittent joint locking clicking Knee pain swelling

LOOSE BODIES

CLASSIFICATION OF LOOSE BODIES

A. FIBROUS LOOSE BODIES Traumatic i e. organization from haemorrhage into villus pathological-in association with tuberculosis, syphilis and osteoarthritis

B. FIBRINOUS LOOSE BODIES Traumatic-after haemorrhage pathological in association with tuberculosis and chronic synovitis.

C. CARTILAGINOUS LOOSEBODIES. Traumatic separation of whole or part of an intraarticular fibro-cartilage

eg.meniscus

LOOSE BODIES

D. OSTEO CARTTLAGINOUSLOOSE BODIES Pathological osteochondritis dissecans, detachment of osteophytes in osteoarthritis, separation of sequestrum in acute osteomyelitis. synovial chondromata

Traumatic- fracture of the tibial spine.

E. MISCELLANEOUS LOOSE BODIESintroduced foreign body lipoma secondary carcinoma.

X-ray: A loose body is typically diagnosed with an x-ray. In most cases, the traumatic

loose body has a bone chip with it or a big chunk of cartilage easily seen on an x-

ray.

MRI: The best way to see the location and character of loose body is with an MRI.

When the loose body is just cartilage or in the case of synovial chondromatosis, a

MRI is the best non radiation diagnostic tool to asses the whole knee joint. It is a

useful preoperative tool to see size, number and location of the loose bodies.

CT scan: This is rarely used but can give details about the loose body when fracture

or bone fragments are expected.

LOOSE BODIES

Diagnosis of presence of loose body inside the knee joint is made by history of recurrent locking at different angles.

There will be associated findings according to the cause of loose bodies.

The most difficult part is to differentiate injury to the meniscus with the

presence of loose bodies inside the joint. This difference is easier when the loose body is radio-opaque ie. bony

or osteo-cartilaginous or radioopaque foreign bodies. This requires only X-ray for diagnosis.

LOOSE BODIES

Two conditions should be kept in mind which are often misdiagnosed as bony loose bodies in the knee joint

Fabella -a sesamoid bone in the lateral head of the gastroenemius

Pellegrini-Stieda's disease in which calcification occurs in the tibial collateral ligament after partial avulsion from the medial condyle of the femur

Treatment Non-surgical management - physical therapy and anti-inflammatory drugs can

be used to help with the symptoms and to keep the joint flexible.

Arthroscopy: The gold standard today is to remove possibly all loose bodies by arthroscopy, popularly known as “clean out “arthroscopies.

It is minimally invasive and allows surgeon to thoroughly examine the knee for loose body and allows to asses any damage to cartilage and treat the condition, hence it is best Diagnostic and therapeutic modality available

  Open arthrotomy: This is less common due to the availability of arthroscopy but

it can be done when the loose bodies are very large and numerous or there is associated complete synovial involvement

REFERENCES

CAMPBELL'S OPERATIVE ORTHOPAEDICS TUREK TEXT BOOK OF ORTHOPAEDICS. BONE TUMORS BY JOSEPH M. MIRRA. ATLAS OF ORTHOPAEDIC PATHOLOGY BY WOLD AND UNNI THE RADIOLOGY OF SKELETAL DISORDERS DUKE RADIOLOGY-CASE REVIEW

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