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The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18) chromosomal translocation.
Radiologically
- Odontogenic Myxoma
- Ameloblastoma
- Central Giant Cell Granuloma
- Adenomatoid odontogenic tumor
Histologically
- Orthokeratocyst
- Radicular cyst (particularly if the OKC is very inflamed)
- Unicystic ameloblastoma
Two cell types can be seen microscopically in synovial sarcoma. One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform, and found in sheets. The other is epithelial in appearance. Classical synovial sarcoma has a biphasic appearance with both types present. Synovial sarcoma can also appear to be poorly differentiated or to be monophasic fibrous, consisting only of sheets of spindle cells. Some authorities state that, extremely rarely, there can be a monophasic epithelial form which causes difficulty in differential diagnosis. Depending on the site, there is similarity to biphenotypic sinonasal sarcoma, although the genetic findings are distinctive.
Like other soft tissue sarcomas, there is no universal grading system for reporting histopathology results. In Europe, the Trojani or French system is gaining in popularity while the NCI grading system is more common in the United States. The Trojani system scores the sample, depending on tumour differentiation, mitotic index, and tumour necrosis, between 0 and 6 and then converts this into a grade of between 1 and 3, with 1 representing a less aggressive tumour. The NCI system is also a three-grade one, but takes a number of other factors into account.
The definitive diagnosis is by histologic analysis, i.e. and examination under the microscope.
Under the microscope, OKCs vaguely resemble keratinized squamous epithelium; however, they lack rete ridges and often have an artifactual separation from their basement membrane.
On a CT scan, The radiodensity of a keratocystic odontogenic tumour is about 30 Hounsfield units, which is about the same as ameloblastomas. Yet, ameloblastomas show more bone expansion and seldom show high density areas.
Patients treated with complete surgical excision can expect an excellent long term outcome without any problems. Recurrences may be seen in tumors which are incompletely excised.
While there is a wide age range at clinical presentation (12–85 years), most patients come to clinical attention at 55 years (mean). There is no gender difference.
Complete surgical excision is the treatment of choice, associated with an excellent long term clinical outcome.
Chondromyxoid fibromas can share characteristics with chondroblastomas with regards to histologic and radiographic findings. However they more commonly originate from the metaphysis, lack calcification and have a different histologic organization pattern. Other differential diagnoses for chondroblastoma consist of giant cell tumors, bone cysts, eosinophilic granulomas, clear cell chondrosarcomas, and enchondromas (this list is not exhaustive).
It is important to separate hiberoma from adult rhabdomyoma, a granular cell tumor and a true liposarcoma.
Fibrosarcoma occurs most frequently in the mouth in dogs . The tumor is locally invasive, and often recurs following surgery . Radiation therapy and chemotherapy are also used in treatment. Fibrosarcoma is also a rare bone tumor in dogs.
In cats, fibrosarcoma occurs on the skin. It is also the most common vaccine-associated sarcoma. In 2014, Merial launched Oncept IL-2 in Europe for the management of such feline fibrosarcomas.
Chondroid differentiation is a common feature of chondroblastoma. A typical histological appearance consists of a combination of oval mononuclear and multi-nucleated osteoclast-type giant cells. However this is not a prerequisite for diagnosis, as cells with epithelioid characteristics have been observed in lesions of the skull and facial bones. A "chicken-wire" appearance is characteristic of chondroblastoma cells and is the result of dystrophic calcification that may surround individual cells. Although, calcification may not be present and is not a prerequisite for diagnosis. Mitotic figures can be observed in chondroblastoma tissue but are not considered atypical in nature, and therefore, should not be viewed as a sign of a more serious pathology. There is no correlation between mitotic activity and location of the lesion. Furthermore, the presence of atypical cells is rare and is not associated with malignant chondroblastoma. There are no discernible histological differences observed when comparing the aggressive form of chondroblastoma that can cause recurrence or metastases with its less aggressive, benign, counterpart.
Ancillary testing for fibrosarcoma includes IHC, where vimentin is positive, cytokeratin and S100 are negative, and actin is variable.
Villonodular synovitis is a type of synovial swelling.
Types include:
- Pigmented villonodular synovitis (PVNS)
- Giant cell tumor of the tendon sheath (GCTS)
Though they have very different names, they have the same histology, and stain positive for CD68, HAM56, and vimentin.
They are sometimes discussed together.
There is no specific test for this condition. Diagnosis is based on signs and symptoms, and exclusion of other conditions.
Treatment is frequently by means of removal of the loose bodies and of a partial or full synovectomy (removal of the synovium)
Full synovectomy is a moderately major operation and involves completely exposing the joint and removing the affected tissue. Partial synovectomy is normally done arthroscopically. Synovectomies are normally carried out by shaving the lining of the knee but there are other ways of achieving this by either freezing the synovium or by the use of radiation treatment.
The need for further procedures is greater than 25% although normally the frequency of the required removal of loose bodies is reduced by the previous synovectomy. There have been documented cases of malignant transformation however this is rare.
Whilst the condition can be described as a ‘benign growth’ it seldom affects more than one joint, and does not usually affect surrounding tissue.
Ganglion cysts are diagnosed easily, as they are visible and pliable to touch.
Radiographs in AP and lateral views should be obtained to exclude any more serious underlying pathology. Ultrasonography (US) may be used to increase diagnostic confidence in clinically suspected lesions or to depict cysts, because intratendinous ganglia are readily distinguished from extratendinous ganglia during dynamic ultrasonography, as microscopically, ganglionic cysts are thin-walled cysts containing clear, mucinous fluid.
FDG positron emission tomography (PET) may be useful to detect the condition early. Other imaging studies including MRI, CT scans, and X-rays may reveal inflammation and/or damaged cartilage facilitating diagnosis.
Biopsy of the cartilage tissue (for example, ear) may show tissue inflammation and destruction, and may help with the diagnosis. The Biopsy of cartilage in patients with relapsing polychondritis may demonstrate chondrolysis, chondritis, and perichondritis.
PVNS is radiologically diagnosed by magnetic resonance imaging (MRI). The disorder is difficult to identify and is often not diagnosed for four years or more after presentation due to nonspecific symptoms or a general paucity of symptoms.
Diagnosis is by examination. A Baker's cyst is easier to see from behind with the patient standing with knees fully extended. It is most easily palpated (felt) with the knee partially flexed. Diagnosis is confirmed by ultrasonography, although if needed and there is no suspicion of a popliteal artery aneurysm then aspiration of synovial fluid from the cyst may be undertaken with care. An MRI image can reveal presence of a Baker's cyst.
An infrequent but potentially life-threatening complication, which may need to be excluded by blood tests and ultrasonography, is a deep vein thrombosis (DVT). Quick assessment of the possibility of DVT may be required where a Baker's cyst has compressed vascular structures, causing leg edema, as this sets up conditions for a DVT to develop.
A burst cyst commonly causes calf pain, swelling and redness that may mimic thrombophlebitis.
Patients usually complain of pain in one joint, which persists for months, or even years, does not ease with exercise, steroid injection or heat treatment, shows nothing on X-ray, but shows a definite restriction of movement.
There are 3 defined stages to this disease:
- early: no loose bodies but active synovial disease;
- transitional: active synovial disease, and loose bodies;
- late: loose bodies but no synovial disease;
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 xray fails. In experienced hands, US is also useful for the diagnosis.
In the disease, the thin flexible membrane of the synovium gradually forms blisters which calcify and enlarge. These nodules eventually break free and float around the joint space becoming larger – these add to the discomfort and stiffness of the joint.
The disease is rare and little known and there is currently no known cure. The affected tissue will show up as a semi-solid mass in a MRI scan, final diagnosis is usually confirmed by taking a biopsy.
Synovial chondromatosis occurs twice as commonly in males as females and usually in their forties. However, online communities for synovial chondromatosis patients have yielded a stark contrast, with equal representation from both genders and members diagnosed as young as late teenage/early 20s.
The disease generally affects only one of the larger weight bearing joints (hip, ankle, knee) – although the elbow, and wrist can also be affected. Rarely involves the temporal mandibular joint.
Non-steroidal anti-inflammatory drugs (NSAIDs) can give significant relief of the symptoms. Treatment of lung cancer or other causes of hypertrophic osteoarthropathy results in regression of symptoms for some patients.
Ultrasonography and magnetic resonance imaging of the hands and/or feet have been proposed as useful diagnostic investigations in RS3PE.
Some studies linked RS3PE to HLA-B27 whereas others have not.
Laboratory testing includes white blood cell count, ESR, and CRP. These values are usually elevated in those with septic arthritis; however, these can be elevated by other infections or inflammatory conditions and are, therefore, nonspecific. Procalcitonin may be more useful than CRP.
Blood cultures can be positive in up to half of patients with septic arthritis.
Once PVNS is confirmed by biopsy of the synovium of an affected joint, a synovectomy of the affected area is the most common treatment. Bone lesions caused by the disorder are removed and bone grafting is performed as needed. Because diffuse PVNS has a relatively high rate of recurrence, radiation therapy may be considered as a treatment option. In some cases, a total joint replacement is needed to relieve symptoms when PVNS causes significant joint destruction.