ankle (5%), wrist, and ... Coronal T2-weighted MRI with fat suppression demonstrates the erosive destruction ..... Synovial osteochondromatosis of the wrist.
Synovial Neoplasm Prof. Shereen Refaat Kamel Professor of Rheumatology&Rehabilitation Minia University - Egypt
Contents I- What is synovium? II- GCT of the tendon sheath. III- PVNS IV- Synovial chondromatosis V- Synovial sarcoma VI- Giant Cell Tumor of Bone VII- Metastatic Tumors in Bone
What is synovium? The term synovium refers to the soft tissue lining the spaces of diarthrodial joints, tendon sheaths and bursae. The word "synovium" comes from a Latin word meaning "with egg," because the synovial fluid in joints that have a cavity between the bearing surfaces is like egg white. It includes the continuous surface layer of cells (intima) and the underlying tissue (subintima).
Structure
Synovium is very variable but often has two layers: The inner layer, or intima, consists of a sheet of cells thinner than a piece of paper. The outer layer, or subintima, can be of almost any type: fibrous, fatty or loosely "areolar".
▪The intimal cells are of two types, fibroblasts and macrophages. ▪The fibroblasts manufacture a long chain sugar polymer called hyaluronan, which is what makes the synovial fluid "ropy" like egg-white, together with a molecule called lubricin, which lubricates the joint surfaces. ▪The macrophages are responsible for the removal of undesirable substances from the synovial fluid. ▪Just beneath the intima most synovium has a dense net of small blood vessels which provide nutrients not only for synovium, but also for the avascular cartilage.
Giant cell tumour of tendon sheath (GCT-TS)
Giant cell tumour of tendon sheath (GCT-TS) ▪It is a rare, benign soft tissue tumor . ▪Giant cell tumors of the tendon sheath are the second most common tumors of the hand, with simple ganglion cysts being the most common. ▪ It can occur in any age group but principally affects those between 30 and 50 years of age, with a female predominance. ▪ Most commonly it presents as a soft-tissue swelling in the hand or foot, adjacent to a small joint and arising from a tendon sheath or the synovial lining of a joint or bursa.
▪ It is often painless and slow growing, fixed to deep structures and abutting the bone, which can be eroded by pressure to cause scalloping. ▪ In > 10% of patients it arises from the synovium. ▪Solitary disease is also common in the knee and may present as an incidental finding on MRI or, more commonly, with mechanical symptoms mimicking a meniscal injury or ‘loose body’. ▪ Extra-articular disease presents as a slowgrowing non-painful, often palpable periarticular mass. It is a benign condition, but may recur if incompletely excised.
❖ A 67-year-old female presented with a history of painful nodularities beneath the skin over the dorsal aspect of her distal IP joint. ❖ DD: I. Tophaceous gout. or II. Giant cell tumor of tendon sheath.
Pigmented Villonodular Synovitis
Pigmented Villonodular Synovitis (PVNS) •“Pigmented” - contains hemosiderin. Pigmented means colored. The synovium and its fluid is often a brown color instead of clear. This is because blood, which contains iron, is deposited in the fluid. • “Villonodular” - appearance of gross specimen. Villous means hair-like. In pigmented villonodular synovitis, the tissue that is affected may look frayed or hair-like. The synovial tissue can also appear folded.
• Proliferative disorder of synovium
• PVNS is a rare, usually benign proliferative disorder of the synovial lining of joints that produces localized or diffuse nodular thickening of the synovial membrane.
• Incidence: The estimated prevalence is 1.8 per million. It affects patients in the age-group of 20 to 40 years with no sex predilection. Pigmented villonodular synovitis is rare in children.
Etiology of this disease is unknown, although a neoplastic or inflammatory origin has been suggested. ▪An inflammatory process is considered to play a part in the pathogenesis of PVS, an influence of trauma or repeated bleeding is possible. ▪Proliferation of synovial cells and fibroblasts associated with collagen production is characteristic. Hemosiderin and lipids accumulate in the cells, giant multinuclear cells are present.
• Any synovial joint may be affected, but the knee is the most affected J (80%). • Followed by the hip (15%), ankle (5%), wrist, and shoulder; involvement of the elbow, foot and TMJ rare. • The spine may be affected, notably the sacroiliac joints and posterior vertebral elements.
▪Patients typically complain of pain, swelling, or both of the affected joint. ▪Characteristically there is a long history, with a delay in diagnosis. ▪In the knee and ankle there may be recurrent swelling with spontaneous haemarthrosis.
Is it Mono or Polyarticular? ➢It is usually monoarticular and are unassociated with other disorders.
➢Polyarticular involvement is unusual, but it appears more often in children. ➢PVNS in children has also been associated with a variety of other abnormalities, including vascular lesions, cherubism, multiple lentigines syndrome, extremity lymphedema, Noonan syndrome, and jaw lesions
Case of 30-year old male followed-up since the age of 6 for severe type of rare combination of polyarticular form of PVNS with hereditary malformation consistent with Noonan-like syndrome. ❖Polyarticular form of PVNS affecting children younger than ten years, associated with various congenital malformations, is very rare. ❖Characteristic features of Noonan syndrome are: short stature, hypertelorism, ptosis, epicanthus, low set ears, short neck, pterygium, pectus carinatum (broad deformed chest ), kyphosis, scoliosis, pulmonic stenosis, skin changes (nevi, lentiginosis, depigmentations), osteoporosis, developmental delay and others.
PVNS - Subtypes
▪ Diffuse: Entire synovium. It is the most common form of intra-articular disease. ▪ Local: Discrete region. It is most common and usually extra-articular (PVNB and PVNTS).
▪In intra-articular disease there is no gender predilection, whereas extra-articular disease has a slight female predominance. ▪Although considered to be a benign condition, the diffuse form is more aggressive, with a high recurrence rate after surgery of 25% with intra-articular and 25% to 50% with extra-articular disease.
Arthrocentesis
Brown red or yellow fluid
PVNS – Radiographic Features •Soft tissue swelling – 80% (appear dense because of high iron content (haemosiderin) in the synovium).
•Bony destruction (Erosion, or lytic lesion) •Joint space narrowing - Later in disease -70-75% of hip cases •Subchondral cysts -95% of hip cases •No calcifications & No osteoporosis
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Diffuse PVNS of the knee in a 45-year-old man ▪large suprapatellar effusion and soft-tissue fullness about the knee. ▪erosions with marginal sclerosis
▪patellofemoral degenerative changes.
Diffuse intraarticular PVNS of the knee in an 8-year-old girl with a 5-year history of intermittent knee pain and swelling.
Radiopaque suprapatellar effusion and thickening about the patellar tendon.
Diffuse intraarticular PVNS of the hip in an 18-year-old man with hip pain of 2 years duration. ▪extensive erosion of the femoral neck and acetabulum with sclerotic margins ▪maintained hip joint space.
22-year-old man with a three-year history of progressive pain in his left groin, showing diffuse-type giant cell tumour (Dt-GCT) with erosive destruction of the hip joint. There are lytic lesions with well-defined sclerotic margins on both sides of the joint, consistent with a synovial process. Coronal T2-weighted MRI with fat suppression demonstrates the erosive destruction of the acetabulum, femoral head and neck as a result of extensive synovial proliferation. The areas of low signal intensity within the synovial mass indicate the presence of haemosiderin deposits characteristic of Dt-GCT (white arrows).
(A) Mild anterior joint space narrowing with a trace osteophytic lipping. Findings typical for chronic ankle pain. The only unusual characteristics noted in retrospect were a scalloped margin at the dorsal neck of the talus (small arrow head), an enlarged posterior hiatus (long arrow) and a spherical scalloped margin in the distal anterior tibial plafond (Large arrow head). (B) The ankle mortise was. There was subtle evidence of a large circular lucency in the subchodral aspect of the medial talar shoulder indicated by the arrow.
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Radiology Case Reports, Vol 3, No 2 (2008)
A Rare Manifestation of Pigmented Villonodular Synovitis of the Elbow in a Child
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8-year-old girl with PVNS. Lateral (a) and AP (b) elbow radiographs demonstrate large elbow joint effusion with preserved joint spaces.
There are large, sharply defined lytic lesions of several bones in the carpal and carpometacarpal region. A soft-tissue mass of involved synovial tissue (not apparent on the image) is eroding and replacing the bony structure. The joint spaces are well maintained, and no reactive bone change, such as sclerosis or osteophyte formation, is seen.
In the bones of the hands, localized nodular form, the cysts appear as sharply marginated radiolucent defects in multiple bones.
Localized extraarticular PVNTS involving the thumb in a 26year-old woman with a palpable, slowly enlarging, soft-tissue mass.
MSUS
Ultrasound reveals a homogenous mass measuring 7 x 3 cm in diameter. The tumor is well defined.
PVNS – MRI Features
• Low –intermediate signal intensity on PD, T1 and T2 weighted images - Hemosiderin deposition • Hyperplastic synovium - Lobulated mass
• Bone erosions • Bone density preserved Frassica FJ, Bhimani MA, McCarthy EF, Wenz J. Pigmented Villonodular Synovitis of the Hip and Knee. Am Fam Physician 1999; 60(5): 1404-15.
Dark on MRI images
▪Sagittal gradient echo MRI in a 41-year-old man with persistent pain in the right knee. ▪MRI shows a well-defined softtissue mass dorsal to the posterior cruciate ligament and in close relationship to the knee capsule (white arrow)
Areas of darkness on T1 weighted image
MRI – Dark on T1
•Metal/ foreign body •Cortical Bone •Tendons •Air •Hemosiderin •Fast flowing blood
Sanders TG, Parsons TW 3rd. Radiographic imaging of musculoskeletal neoplasia. Cancer Control 2001; 8(3): 221-31.
Diffuse PVNS of the knee in a 43 y old woman.
Focal PVNS of the knee in a 47 y old woman.
(A) MRI T-1 imaging sagittal section reveals a prominent multi nodular synovial mass predominantly situated in the anterior ankle (arrow head). There is a bifid nodule within the posterior ankle capsule (small arrow). (B) MRI Split Tau Inversion Recovery (STIR) sequence sagittal section reveals the mass within the anterior ankle juxtaposed to the dorsum of the talar neck. The inflammatory process appears most intense within the distal tibia with intra medullary edema less intense throughout the talus. The morphology and the location of the anterior ankle mass are commensurate with scalloping of the dorsal talar neck appreciated on plain radiographs (fat arrow). The fluid within the posterior hiatus (small arrow)
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CT
T1-weighted MRI
T2-weighted MRI 37-year-old man with rapidly growing right-sided periauricular mass. A, Axial CT scan of the skull base shows a large heterogeneous mass in the region of the right TMJ. E, Superselective digital subtraction angiogram of the right internal maxillary artery shows extensive hypervascularity of the tumor F, Photomicrograph of a surgical pathologic specimen shows extensive iron deposition (black stain) within histiocytes (arrows). Note also the presence of multinucleated giant cells (not staining) and a fibrous background
Macroscopically
• Proliferation of synovium
• Brownish villi
• villous and nodular thickening of the synovial membrane. • Diffuse involvement of the synovium results in thin, finger-like excrescences admixed with 0.5- to 2.0-cm rounded nodules that cover the normally smooth synovial membrane.
Microscopically • • • •
Fibrous stroma Hemosiderin deposition Histiocytic infiltrate Multinucleated osteoclast-like giant cells.
Arthroscopy
It is another imaging modality that can be used in the diagnosis of PVNS and may show a richly vascular process,increased articular cavity, joint effusion or synovial villae.
knee arthroscopy performed because of suspected synovitis. Innumerable reddish brown villous synovial projections are noted throughout the visualized joint.
Differential diagnosis
On plain film the differential is wide, and findings are non-specific. 1. joint effusion 2. synovial chondromatosis (non-ossified) 3. lipoma arborescen
On MRI there is little differential in classic examples. 1. scarring / capsulitis 2. siderotic synovitis
Others ➢Rheumatoid Arthritis ➢Systemic lupus erythematosus ➢Osteoarthritis ➢Gouty Arthritis ➢Psoriatic Arthritis ➢Amyloidosis ➢Tuberculous Arthritis ➢Avascular Necrosis (hip only)
Rheumatoid Arthritis: Knees
symmetric narrowing of medial and lateral joint spaces
OA
Chronic tophaceous Gout well-defined sclerotic margin
osteolytic lesion
Psoriatic Arthritis
Radiograph of both hands demonstrates cup-and-pencil deformities of both thumbs and erosion of DIP joint of left middle finger
Amyloid arthropathy soft-tissue swelling erosions
Tuberculous arthritis
periarticular osteopenia
peripheral osseous erosions
gradual diminution of the JS
(Phemister triad)
Avascular necrosis Crescent Sign
subchondral lucency
rim of sclerosis
Other conditions causing recurrent haemarthrosis ▪Hemophilia ▪Hemochromatois ▪Hemosiderosis Demonstrate haemosiderin on histological examination, however, the pigment is largely confined to the synovial cells and macrophages, whereas the distribution in PVNS is more diffuse. Giant cells and histiocytes seen in PVNS are not features of these other conditions.
Hemophilic arthropathy ▪extensive osteoporosis ▪enlargement of the epiphyses ▪mild irregularities of the subchondral bone
▪widened intercondylar notch
Hemochromatosis ➢degenerative arthritis in the second through fifth metacarpophalangeal joints. ➢Joint-space narrowing ➢subchondral cyst formation ➢hypertrophic changes ➢degenerative changes of the first carpometacarpal joint as well as the scaphoid, trapezium, and trapezoid articulation ➢No erosion ➢Chondrocalcinosis of the triangular fibrocartilage of the wrist located just distal to the ulna
Linear calcification
PVNS – Treatment Options Synovectomy Local recurrence (45%)
Radiation therapy Arthroplasty
Proposed treatment and monitoring algorithm for PVNS
Rehabilitation After Surgery ▪ Range-of-motion exercises are important and may be started right away. ▪ Strengthening and conditioning exercises. ▪ When surgery involves the leg, walking with a walker or crutches may be recommended at first.
▪ If knee joint replacement is required, patient hospitalized for three to five days. A continuous passive motion (CPM) machine may be used.
❖The recurrence rate of diffuse intraarticular PVNS following initial treatment ranges from 8% to 56%, although the actual rate may be higher if MR imaging were used as the detection modality. ❖Malignant transformation of PVNS is rare, and it can occur de novo or be associated with recurrent disease. The prevalence of malignant transformation of PVNS was 3% in a study by Bertoni and colleagues.
Which of the following are typical feature of pigmented villonodular synovitis: a) tends to involve multiple joints at the same time b) rarely occurs under the age of forty years of age c) early extensive soft tissue calcification d) Elevated ESR e) low-intensity signal areas on both T1- and T2-weighted sequences
Synovial chondromatosis
Synovial chondromatosis Other names:
Synovial osteochondromatosis Synoviochondrometaplasia Synovial chondrosis Articular chondrosis Synovial chondromata
•It is a condition in which there is proliferation of the synovial lining. The synovial lining proliferation can then calcify and break off, leaving a loose body in the joint capsule. •This disease is commonly seen in the 3rd to 5th decades of life, with a male to female ratio of 2-4:1. •The onset is described as insidious and it occurs over months to years.
•Monoarticular disease is the rule. The most frequent site of involvement is the knee (70%), followed by the hip (20%), shoulder, elbow and the ankle. •It is also encountered in the tendon sheaths and in the periarticular bursa (extra-articular form).
Forms of synovial chondromatosis
There are two forms of synovial chondrometaplasia: primary and secondary. The primary form occurs in an otherwise normal joint and it is thought to be progressive and more likely to recur, and it may lead to severe degenerative arthritis with its long-term presence. Secondary synovial chondromatosis is thought to be caused by the irritation of the synovial tissue of the affected joint. This form is associated with degenerative joint disease, trauma, inflammatory and non-inflammatory arthropathies, avascular necrosis, and osteochondritis dissecans. This form is not likely to recur following its surgical removal.
Pathology There are 3 defined stages to this disease:
early: no loose bodies but metablastic activity transitional: metablastic activity, and loose bodies; late: loose bodies but no metablastic activity; In the early stages of the disease it is often confused with tendinitis and/or arthritis. Once it reaches transitional the loose bodies become apparent with X-ray in greater than 70% of cases, with MRI often showing where x-ray fails.
cartilaginous nodule
synovial proliferation binucleate cells
•Multi-lobulated synovium with multiple white/bluish nodules that are composed of hyaline cartilage attached to the synovium. •These nodules may detach to form loose bodies.
Diagnosis i. Full history ii. Physical examination iii. Radiographic examination. When imaging does not provide the specific diagnostic features, the definitive diagnosis is made histologically on the basis of a synovial tissue biopsy.
Plain film ➢The radiographic features depend on the degree of ossification which has occurred.
➢When calcification is absent (25 - 30% of cases) plain radiographs may be normal or reveal a non-specific findings, e.g. soft-tissue mass surrounding the joint, widening of the joint space, erosions of adjacent bones, or early osteoarthritic changes. ➢When extensive ossification is present then many calcific joint bodies are present, either fully ossified, or demonstrating the ring and arc calcification characteristic of chondroid calcificaiton. They are most often multiple and of uniform size.
1ry SC
Primary synovial osteochondromatosis of the surapatellar pouch of knee. Muhammad Umar Amin, Pervez Salem Qureshi, Abdul Ghaffar, and Mobeen Shafique. J Radiol Case Rep. 2010; 4(8): 7–14. ossified nodules
calcification within synovium
2ry SC
Knee synovial chondromatosis with coexistent osteoarthritis.
Cluster of calcified densities overlying the hip joint consistent with synovial osteochondromatosis. The densities are cartilaginous nodules that have undergone ossification.
Synovial osteochondromatosis in a 24-year-old man with hip pain. Radiograph shows multiple small, dense, punctate calcifications
MSUS
Synovial osteochondromatosis of hip
Female 20 y old, CT scan through the hip area showing multiple calcified loose osteocartilaginous bodies in the hip joint. This patient was treated by an anterior subtotal synovectomy. Multiple loose bodies were removed from the hip joint. Following the procedure, the patient did well.
Female 26 y old, CT scan shows the multiple loose calcifying cartilaginous bodies both in front and posterior to the hip joint.
MRI MRI appearance is variable and depends on the stage of the disease.
The most frequent pattern is one of predominantly unmineralized nodules which demonstrate typical chondroid signal characteristics : T1 : intermediate to low signal T2 : high signal Focal areas of signal void within these nodules represent areas of mineralisation.
Synovial osteochondromatosis of the right knee in a 42year-old patient. (a) Lateral radiograph shows multiple calcified nodules anterior to the proximal tibia. (b) Sagittal proton densityweighted MR image demonstrates that these low-signal-intensity calcified nodules are located in the deep infrapatellar bursa.
The radiograph shows an erosive process of the left femoral head and neck. The magnetic resonance image (coronal view) demonstrates multiple confluent cartilaginous bodies in the synovial recesses with adjacent bone erosions. The intraoperative gross specimen shows severe bone loss in the femoral head and multiple cartilaginous bodies.
Numerous ossified bodies in the hip joint
Synovial osteochondromatosis of the wrist. Anteroposterior radiograph of the wrist shows multiple small, calcified nodules (white arrows) of characteristically uniform size in the radioulnar joint. Note the erosive changes (black arrows) of the distal radius and ulna. Coronal T2-weighted image shows that the mass (*) is hyperintense, note the multiple low-signal-intensity areas (arrowhead), which correspond to areas of calcification seen on the radiograph.
CASE REPORT Korean J Spine 9(3):253-256, 2012 www.e-kjs.org Copyright © 2012 The Korean Spinal Neurosurgery Society. Regrowing Synovial Chondromatosis in a Cervical Facet Joint with Radiculopathy. Jae-Suk Han, Seung Hoon Lee, Eun-Sang Kim, Whan Eoh Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
Preoperative computed tomography (CT) scans (A) showing a small ill-defined intra-articular mass accompanied by bone erosion and focal calcification. A preoperative cervical T2weighted axial (B) and Gadolinium-enhanced T1-weighted axial (C) MRIs, demonstrating partial enhancement and hyperintensity of the mass at the right C6-7 facet joint, respectively.
Case Report
Primary synovial osteochondromatosis of a subdeltoid bursa in 52 y old female.
Arthroscopy
Female 30 y old
Differential diagnosis of primary synovial chondromatosis
secondary osteochondromatosis older age group extensive degenerative change fragments are fewer and often larger pigmented villonodular synovitis (PVNS) more confluent masses diffuse characteristic low density on MRI synovial haemangioma lipoma arborescens characteristic fat signal / density synovial chondrosarcoma soft tissue mass extension beyond the joint presence of metastases siderotic synovitis
Secondary osteochondromatosis
•several osteocartilaginous nodules seen around the knee joint space. •They are relatively few in number, less uniform in size than in primary form, and associated with osteoarthritis of the knee.
synovial haemangioma
A, T1-weighted sagittal MRI scan of synovial hemangioma of the knee demonstrating a heterogeneous mass in the suprapatellar pouch (arrow). B, T2-weighted axial MRI scan demonstrating an intra-articular enhancing lesion in the medial knee (arrow).
lipoma arborescens
Sagittal T1 weighted MRI
Sagittal STIR MRI
synovial chondrosarcoma
well-defined soft-tissue opacity in popliteal region (small white arrows). Note also faintly mineralized loose bodies in posterior capsular recess (black arrow) and suprapatellar pouch (large white arrow).
Sagittal T2-weighted images show well-defined synovial masses and loose bodies. Synovial masses present as predominantly high signal intensity, but areas of intermediate to low signal intensity (arrowheads) are seen. Calcified loose bodies show low signal intensity.
Siderotic synovitis
Sagittal T2-weighted image show well-defined focal lesion of very low signal intensity (arrows) in medial patellofemoral recess.
Note diffuse synovial thickening of intermediate to high signal (arrowheads).
Treatment ▪As radiotherapy and chemotherapy have no effect on synovial chondromatosis, surgical excision is the preferred treatment for it. ▪In asymptomatic patients, the nodules may resorb over time and invasive procedures should be avoided.
▪In localized intra-articular disease, the complete excision of the abnormal synovium seems to provide a cure. ▪In the phase III disease, the removal of the loose bodies alone is sufficient. When synovitis is present, resection of the loose bodies and synovectomy is done.
▪The extra-articular disease treatment aims for the complete excision of the abnormal synovium.
▪The recurrence rates for synovial chondromatosis after surgical treatment have been reported to vary from 7% to 23%. ▪Complications like secondary degenerative osteoarthritis due to chronic mechanical irritation and bone destruction by the loose bodies is the rule. ▪Surgery predisposes the patients to tissue scarring, subsequently compromising the joint function.
True about synovial chondromatosis is: A. Elderly men are affected. B. Bone scan is usually normal. C. Metaplastic cartilage formation occurs throughout the synovium. D. Chondrosarcoma is a common complication.
Synovial sarcoma
Synovial sarcoma is a highly aggressive soft tissue sarcoma predominantly affecting young people, causing death in more than half of the affected children, adolescents, and young adults (15-35y). It is a rare tumor, comprising 5% to 10% of all soft tissue sarcomas. The majority (80–95%) of tumours are reported in the extremities, with two-thirds being located in the lower limbs. Other sites of origin, although rare, include the head and neck, paravertebral region, chest and abdominal wall.
• Extremity lesions typically occur either in a periarticular location or close to a bursa or tendon sheath. The most common location is within 10 cm of the knee. • Most are extra-articular; true intra-articular tumours are rare (10%) and may be difficult to differentiate from other intra-articular lesions such as synovial osteochondromatosis and intra-articular loose bodies.
Synovial sarcoma tumors are uniquely composed of two morphologically distinct cell types: spindle cells and epithelioid cells. The presence of the two cell types in varying proportions lends to the classification of tumors into three histologic subtypes : • Biphasic • monophasic (monophasic fibrous or rare monophasic epithelial) • poorly differentiated.
Biphasic synovial sarcoma
Causes of it are… • Generally unknown • Some studies suggest that genetic alterations are key in formation • What happens in this genetic alteration is a: rearrangement in chromosome material between X & number 18 ║ which changes position and function of genes, causing fusion of genes; a.k.a “fusion transcript” ║ SYT-SSX2
And the symptoms are… • • • •
Deep-seated swelling pain Limping or difficulty using legs, arms, hands or feet The symptoms may resemble other conditions like arthritis and bursitis
Diagnosed by…Plain films • Plain films can reveal I. well-defined or lobulated soft tissue mass II.up to one-third of cases demonstrating punctate calcification, often in the periphery of the lesion III.Tumour growth is usually slow and this can result in superficial pressure erosions, periosteal reaction and osteoporosis. Frank osseous invasion can also occur
A 28-year-old female with synovial sarcoma of the right antecubital fossa. Lateral radiograph shows ‘‘punctuate’’ curvilinear areas of calcification, around a circular appearing mass.
A 48-year-old male with synovial sarcoma of the lower limb. peripheral ‘‘punctuate’’ curvilinear calcification within a soft-tissue mass.
A 42-year-old male with synovial sarcoma of the foot. Destructive calcified soft-tissue mass between the third and fourth metatarsals. The fine ‘‘sand-like’’ calcification is suggestive of synovial sarcoma.
Ultrasound • Ultrasound can be used to localize the position and size of superficial or extremity lesions. It may reveal a focal nodular lesion or cystic components, but is nonspecific in suggesting a diagnosis.
CT • CT is helpful in identifying subtle soft-tissue calcifications • and local bony changes (Figure 7b), particularly in • regions of complex anatomy. Axial CT shows gross bony destruction of the 4th metatarsal, with a 6 cm invasive soft-tissue mass displacing the adjacent metatarsals.
MRI • MRI is considered the modality of choice for the detection and staging of soft-tissue tumours. • Lesions are usually multilocular with internal septa, and the majority are well defined (91%) • Over 40% of lesions demonstrate high signal on both T1 and T2 weighted images, consistent with haemorrhage.
This 35yr old patient presented with swelling on the back of his knee for 6 months. The swelling is not particularly tender and was initially discovered incidentally.
Treatments • Surgery • Radiation Therapy • Chemotherapy • Angiogenesis inhibitors
Surgery • Limb-sparing: when the tumor CAN be removed, all of the tissue involved with the tumor are taken out, while unaffected tendons, nerves, vessels are left in the body • Amputation: when the tumor CANNOT be removed because it involves the nerves and blood vessels
Angiogenesis inhibitors • Substances that may be able to prevent the growth of tumors by blocking formation of new blood vessels that feed the tumors
Giant Cell Tumor of Bone
There are several facts regarding Giant Cell Tumors: Usually discovered between 20 and 40 (75%). M=F. ? more common in Chinese 50-65% occur around the knee. Radius, humerus also common. Spine (esp. sacrum), ilia, less commonly involved. In long tubular bones, the diagnosis of GCT is made by finding: an eccentric, lytic lesion extending to the subchondral region in a patient with closed epiphyses with minimal or absent periosteal reaction and almost always absent sclerotic rim Often recurs after curretage/resection. Up to 15% can be malignant. Mets to lung occur even with "benign" tumors. DDX: Aneurysmal Bone Cyst Brown tumor of HPTH Fibrous Dysplasia Eosinophilic granuloma & Giant Cell reparative granuloma
An association with Paget's disease is frequently quoted
calvarial thickening
* areas of lysis
areas of sclerosis (‘cotton-wool spots)
Plain x-ray • • • • •
Eccentric lytic lesion Epiphyseal Cortical thinning Expansile No sclerotic margin
Left and Center, Giant cell tumor in the upper tibia. Right, the MRI appearance of the tumor.
21 years old female with wrist pain. Note sub-articular location and expansile nature.
Mimics on Radiography of Giant Cell Tumor of Bone
ABC Expansile osteolytic lesion with a thin wall, containing bloodfilled cystic cavities.
Brown tumors of hyperparathyroidism
subperiosteal resorption along radial aspect
radiolucent lesion
pathologic fracture
Multiple Myeloma
multiple "punched-out" holes
Treatment • Controversial • Traditionally: – Intralesional curettage / resection & bone graft – Recurrence 35-42%
• En Bloc resection – Recurrence ~10% – Multiple complications
• Adjuvant
Metastatic Tumors in Bone Metastatic tumors are cancers that started in another location and spread to the bones. More than 90% of all these metastatic lesions in bone are caused by a small number of primary tumors, including breast, lung, kidney, prostate, and thyroid.
Typical x-ray appearance of osteoblastic bone metastases. This plain pelvic x-ray film of a patient with prostate cancer shows multiple osteoblastic metastases to the pelvis and lumbar (L4) and sacral (S1) vertebral bodies.
Typical x-ray appearance of osteolytic bone metastases. This plain pelvic x-ray film of a 75-year-old patient with breast carcinoma shows multiple osteolytic bone lesions.
Bronchus” metastases. (A) AP and (B) lateral views of the lumbar spine reveal multiple sclerotic metastases from lung cancer. The most impressive lesion involves the L5 vertebral body (white arrows). The AP view also demonstrates sclerotic metastases in the pelvis (black arrows).
