Materiały źródłowe

• #1 Synovial sarcoma: the misdiagnosed sarcoma in: EFORT Open Reviews Volume 9 Issue 3 (2024)

  https://eor.bioscientifica.com/view/journals/eor/9/3/EOR-23-0193.xml

  Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. […] The management of synovial sarcomas is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. […] The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. […] Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases.

• #1 Synovial Sarcoma – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/synovial-sarcoma

  Synovial sarcoma is a cancer that can come from different types of soft tissue, such as muscle or ligaments. […] One third of patients with synovial sarcoma will be diagnosed under the age of 30. […] Synovial sarcoma accounts for 5% to 10% of soft-tissue tumors. […] The symptoms caused by synovial sarcoma depend on where the tumor forms. Synovial sarcoma is often first noticed as a painless lump. […] If you have symptoms of synovial sarcoma, your doctor will use scans such as X-ray, ultrasound, CT scan, and MRI to take pictures of the tumor. […] Your doctor will perform a biopsy, taking a small sample of the tumor with a needle. A pathologist will study cells from the sample under the microscope to see what kind of tumor it is. […] Doctors will look for this change in chromosomes to confirm that it is synovial sarcoma.

• #1 9 synovial sarcoma questions, answered | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/synovial-sarcoma.h00-159068712.html

  Most synovial sarcoma patients discover their tumor from a lump, and for some, related pain. Its important to see a doctor if you find a lump or have persistent, unprovoked pain in a soft tissue, such as the muscle of an extremity or from within your abdomen. Synovial sarcoma may also occur in the lungs, so see your doctor if you notice shortness of breath. […] Synovial sarcoma can be found in the soft tissues by an MRI, or in the lungs with an X-ray or CT scan. However, an official diagnosis can only be made by a pathologist after obtaining tissue samples through biopsy or surgery. […] Make sure you get your regular scans and follow-ups with your doctor, even if you’ve had no evidence of disease for many years.

• #1 Synovial Sarcoma | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/s/synovial-sarcoma

  Synovial sarcoma is a soft tissue sarcoma that is usually found in the legs or arms but can occur anywhere in the body. […] The first step in diagnosing synovial sarcoma is for the doctor to take a detailed medical history of the patient and family. Next, the patient has a physical exam and other types of tests, which may include: X-rays, Magnetic resonance imaging (MRI), Computed tomography (CT), Bone scans, Positron emission tomography (PET), a test that measures important body functions and also can show whether the cancer has spread, Blood tests, A needle biopsy or surgical procedure. A specialist examines the tissue under a microscope to confirm the diagnosis of synovial sarcoma. […] Prompt diagnosis and treatment can significantly improve the outcome for patients with synovial sarcoma. It is very important for patients to receive specialized care at a hospital such as Cincinnati Childrens. Our sarcoma team has experience in diagnosing and treating this rare type of cancer.

• #1 Diagnosis of soft tissue sarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/diagnosis

  Diagnosis is the process of finding out the cause of a health problem. Diagnosing soft tissue sarcoma can begin with a visit to a doctor, often a specialist. Your doctor will ask you about any symptoms you have and may do a physical exam. The doctor may order tests to check for soft tissue sarcoma or other health problems. But sometimes soft tissue sarcomas are found by chance (incidentally) while checking for a different health problem. […] The following tests are usually used to rule out or diagnose soft tissue sarcoma. Many of the same tests used to diagnose cancer are used to find out how far the cancer has spread (the extent of the cancer). Your doctor may also order other tests to check your general health and to help plan your treatment. […] A physical exam allows your doctor to look for any signs of soft tissue sarcoma. During a physical exam, your doctor may: examine any lumps on your body, including checking their size and shape and how they feel; check how certain parts of your body are moving, such as your arms and legs, and if there is any weakness; examine the lungs; feel the abdomen for an enlarged liver or abnormalities.

• #1 Synovial sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/synovial-sarcoma/diagnosis-treatment/drc-20577401

  Synovial sarcoma is usually slow growing, so it can be years before a diagnosis is made. Sometimes, synovial sarcoma is diagnosed in error as a joint problem, such as arthritis or bursitis. […] Tests and procedures used to diagnose synovial sarcoma include: […] Imaging tests take pictures of the body. They can show where a synovial sarcoma is, how large it is and if it has spread to other areas of the body. Tests for synovial sarcoma might include MRI scans, X-rays and CT scans. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample. […] The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan.

• #1

  https://www.orthobullets.com/pathology/8052/synovial-sarcoma

  Synovial Sarcoma is a malignant, soft tissue sarcoma caused by a t(X;18) chromosomal translocation mutation ( SS18:SSX fusion protein) most commonly found near joints but rarely within the joint. […] Diagnosis is made with a biopsy showing a classic mono/biphasic appearance with atypical spindle cells and epithelial cells. Immunostaining is positive for epithelial membrane antigen and vimentin. […] MRI is the gold standard for diagnostic imaging. […] Biopsy is performed using core-needle versus incisional biopsy. […] FNA is not gold-standard as it does not provide information on tumor structure. […] Classical synovial sarcoma shows a mono/biphasic appearance with two typical cell types. […] Synovial sarcoma stains positive for vimentin and epithelial membrane antigen.

• #1 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  The MRI presentations of synovial sarcomas encompass a spectrum, spanning from small, uniform nodules to extensive, heterogeneous masses. […] Most synovial sarcomas manifest as prominently heterogeneous, multilobulated soft-tissue masses, displaying SI similar to or slightly higher than that of muscle on T1-weighted images and predominantly bright SI on T2-weighted images. […] The presence of peritumoral enhancement, without causing any discernible mass effects on the neighboring tissues, is regarded as a direct sign of tumor margin infiltration. […] A more favorable prognosis, on the other hand, is linked to extensive calcification and tumor size below 5 cm. […] The treatment for synovial sarcomas typically comprises a combination of surgery, radiotherapy, and chemotherapy. […] A biopsy is essential for an accurate diagnosis in patients with synovial sarcoma to differentiate synovial sarcoma from other soft-tissue sarcoma subtypes and define the tumor grade.

• #1 Distinguishing synovial sarcoma from benign and malignant mimics: MR Imaging indicators | Applied Radiology

  https://www.appliedradiology.com/Articles/distinguishing-synovial-sarcoma-from-benign-and-malignant-mimics-mr-imaging-indicators

  Synovial sarcomas (SS) are malignant soft tissue tumors thought to account for 5-10% of soft tissue sarcomas. Synovial sarcomas are rare, with an estimated incidence of 2.75 in 100,000 people. However, they are the second-most prevalent soft-tissue tumors after rhabdomyosarcoma in children, adolescents, and young adults. Since synovial sarcomas are so rare, many clinicians and radiologists are not familiar with their presentation and imaging appearance. For these tumors to be biopsied, it is essential that interpreting radiologists or orthopedic oncologists not deem them benign. Incorrectly assuming that an SS is a benign lesion will lead to delays in diagnosis and possibly prognosis. Conversely, mistaking a benign lesion for an SS may result in increased patient anxiety and costs due to unnecessary procedures and imaging tests. The aim of this article is to describe the key magnetic resonance imaging (MRI) features of SS, as well as to describe those of benign and malignant tumors that are often confused with SS.

• #1 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  Synovial sarcomas are the fourth most prevalent soft-tissue sarcoma, primarily affecting the extremities (in 80–95% of cases) of adolescents and young adults aged 15 to 40. […] The imaging characteristics of synovial sarcomas encompass a wide spectrum, ranging from non-aggressive features to more aggressive appearances. […] Understanding and identifying these diverse presentations, along with their associated prognostic implications, might be helpful in patient evaluations and in achieving optimal management. […] While the radiological features of these tumors are not definitely indicative, the presence of calcification in a soft-tissue mass (occurring in 30% of cases), adjacent to a joint, strongly suggests the diagnosis. […] Cross-sectional imaging characteristics play a crucial role in diagnosing synovial sarcomas.

• #1 Diagnosis of soft tissue sarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/diagnosis

  A complete blood count (CBC) measures the number and quality of white blood cells, red blood cells and platelets. A CBC is done to check your general health, often before a biopsy. […] Blood clotting (coagulation) tests check if your blood is clotting properly. Prothrombin time (PT), partial thromboplastin time (PTT) and international normalized ratio (INR) are common blood clotting tests. They are usually checked before a biopsy is done. […] An MRI is commonly used to examine a lump that doctors think may be a soft tissue sarcoma. It is often the first imaging test done for lumps in the arms or legs. An MRI can check the size of a tumour, what tissues and areas are nearby and if it has spread to other parts of the body. […] A CT scan is commonly used to examine a lump in a specific area, including checking its size and finding out what tissues and areas are nearby. It is often used to check the abdomen, back of the abdomen (retroperitoneum) and chest. If soft tissue sarcoma is diagnosed, a CT scan can be used to check if the cancer has spread to other parts of the body.

• #1 Tests for Soft Tissue Sarcomas | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/detection-diagnosis-staging/how-diagnosed.html

  If you have signs or symptoms that suggest you might have a soft tissue sarcoma, the doctor will likely need to do tests to find out if you have cancer. […] If a soft tissue sarcoma is suspected based on exams and imaging tests, a biopsy is needed to know for sure that it’s a sarcoma and not another type of cancer or a benign (not cancer) disease. In a biopsy, the doctor takes out a small piece of the tumor. This tissue is looked at under a microscope and other lab tests may be done as well. […] Several types of biopsies are used to diagnose sarcomas. Doctors experienced with these tumors will choose one, based on the size and location of the tumor. Most prefer to use a fine needle aspiration or a core needle biopsy as the first step. Proper biopsy technique is a very important part of successfully treating soft tissue sarcomas. An improper biopsy can lead to tumor spread and problems removing the tumor later on.

• #1 Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-022-05752-4

  The biopsy technique of choice in soft tissue sarcoma (STS) diagnosis is controversial. […] We examined the diagnostic accuracy of percutaneous core needle biopsy (CNB) and compared it to open incisional biopsy. […] Furthermore, we investigated the role of molecular pathology in sarcoma diagnostics. […] The pair matched analysis showed that the correct entity was determined in 96% of incisional and 97.6% of core needle biopsies. […] CNB leads to faster diagnosis while reaching the same histological accuracy and is less burdensome for patients. […] A 2009 meta-analysis on 35 studies has found that incisional biopsy has had the highest diagnostic accuracy, followed by CNB, which has been more accurate than fine-needle aspiration biopsy. […] Newer studies, including a comprehensive 2020 meta-analysis, have reported comparable accuracy of percutaneous CNB and incisional biopsy, and an increasing number of centers have cited CNB as the method of the first choice in diagnosing soft tissue tumors.

• #1 Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-022-05752-4

  CNB has thus found recognition as the method of choice in the 2018 guidelines of the National Comprehensive Cancer Network (NCCN) for soft tissue tumor biopsy. […] This study aims to report our institutional experience and investigate the diagnostic accuracy, sensitivity, specificity, safety, and time to diagnosis of CNB in comparison to open incisional biopsy. […] Analysis of all patients regarding the accuracy of biopsy techniques revealed a 96% accuracy for diagnosing malignancy via CNB and 100% for incisional biopsy. […] The correct entity was diagnosed in nearly all cases, and the correct grading was diagnosed in 90% of incisional biopsies and 94% of CNBs. […] During the last decade, reports have been accumulating on the reliability of CNB for the primary diagnosis of soft tissue tumors.

• #1 Synovial Sarcoma | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/types/synovial-sarcoma

  Synovial sarcoma is a rare cancer that is more commonly diagnosed in people under 40. It is a form of soft tissue sarcoma. […] Doctors use imaging to detect synovial sarcoma. These tests typically include MRIs or CTs. The scans can show the size of the tumor, where it started, whether any nearby organs or tissues are involved, and whether the tumor has spread to other areas of the body. […] Biopsy is usually the next step. A biopsy is the only test that can make a definite diagnosis of synovial sarcoma. It involves taking a sample of the tumor. That sample is checked under a microscope for cancer cells by a pathologist (a doctor who is specially trained to diagnose disease). […] It is very important to receive a diagnosis from a team of doctors that is highly experienced in the diagnosis and treatment of soft tissue sarcoma. There are more than 80 subtypes of soft tissue sarcoma, so it can be a difficult disease to diagnose. Getting the correct evaluation and diagnosis is the first step to being cured.

• #1 Synovial Sarcoma: A Clinical Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8161765/

  Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 510% of all STS. […] Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. […] A biopsy and pathologic assessment are required to differentiate synovial sarcoma from other STS subtypes and define the tumor grade. […] The two most commonly utilized staging systems are the American Joint Committee on Cancer (AJCC) system and the Enneking staging system. […] Both fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR) testing have been validated in the diagnosis of this translocation. […] Synovial sarcoma is characterized by a pathognomonic translocation t(X:18) which is present in 95% of cases.

• #1

  https://tumorsurgery.org/tumor-education/soft-tissue-tumors/soft-tissue-tumor-types/synovial-sarcoma.aspx

  Synovial Sarcoma appears heterogeneous and demonstrate areas of nodular enhancement. […] Synovial Sarcoma is composed of two different cell types: Spindle cell (small, uniform, and ovoid cells with pale nuclei and the cytoplasm is sparse) and Epitheloid cell (ovoid nuclei and abundant cytoplasm). […] Wide surgical excision is the mainstay of treatment for Synovial Sarcoma. […] Patients with tumors that present with more than 20% of poorly differentiated patterns have the worse prognosis.

• #1 Final Diagnosis — Case 292

  https://path.upmc.edu/cases/case292/dx.html

  FINAL DIAGNOSIS: SYNOVIAL SARCOMA […] Synovial sarcoma is a soft tissue tumor occurring in the biphasic or monophasic type and consisting of epithelial and/or spindle cell components. […] The reported incidence of synovial sarcomas ranges from 5.6% to 10% among all soft tissue sarcomas. […] According to the AFIP, synovial sarcoma ranks as the fourth most common sarcoma after malignant fibrous histiocytoma, liposarcoma and rhabdosarcoma. […] Although it can occur at any age, the peak incidence is ages 10 to 35. […] The majority of synovial sarcomas occur in the extremities with a predilection for the lower extremities. […] Less frequently, it can occur in the head and neck, abdominal wall, pleura, lung and mediastinum and has been found in almost all anatomical sites. […] The lesions often grow close to joints, particularly the knee, however the intra-articular tissues are only rarely involved.

• #1

  https://link.springer.com/article/10.1245/s10434-017-5855-x

  Synovial sarcoma is a translocation-associated soft-tissue malignancy that frequently affects adolescents and young adults. […] In this review, we present recent discoveries in the pathogenesis, diagnosis, and treatment of synovial sarcoma. […] TLE1 as a diagnostic immunohistochemical marker in synovial sarcoma. […] The clinical implication of SS18-SSX fusion gene in synovial sarcoma. […] Detection of the synovial sarcoma translocation t(X;18) (SYT;SSX) in paraffin-embedded tissues using reverse transcriptase-polymerase chain reaction: a reliable and powerful diagnostic tool for pathologists. […] TLE1 as a diagnostic immunohistochemical marker for synovial sarcoma emerging from gene expression profiling studies. […] Specificity of TLE1 expression in unclassified high-grade sarcomas for the diagnosis of synovial sarcoma.

• #1 Synovial Cell Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK587366/

  Synovial sarcoma is defined by the translocation between chromosome X and 18, which leads to the expression of SS18:SSX fusion proteins. […] The presence of t(X;18)(p11.2;q11.2) is a pathognomonic feature of synovial sarcoma. […] Several molecular techniques are used to determine this translocation. […] The evaluation of a patient with SS includes a biopsy of the mass and radiographic tests. […] To establish the diagnosis, multidisciplinary and interprofessional planning must be pursued to obtain a pretreatment biopsy. […] The treatment of patients with synovial sarcoma is based on the stage of presentation. […] The therapy involves using cytotoxic chemotherapy, tyrosine kinase inhibitors, or enrolling the patient in a clinical trial. […] The evaluation of a patient with SS includes a biopsy of the mass and radiographic tests.

• #1 Synovial Sarcoma Workup: Laboratory Studies, Plain Radiography, Computed Tomography

  https://emedicine.medscape.com/article/1257131-workup

  Cytogenetic analysis aids the physician in detecting the specific chromosomal translocation between chromosome 18 and chromosome Xnamely, t(X;18)(p11;q11). This translocation produces the SYT-SSX1, SYT-SSX2, or SYT-SSX4 fusion gene. Identification of this anomaly is obtained by reverse transcriptasepolymerase chain reaction (RT-PCR) in tumor tissue (and possibly in blood), with a sensitivity of 96% and specificity of 100%. Fusion transcripts can be detected with molecular diagnostic techniques in biopsy samples. […] Fluorescence in-situ hybridization (FISH) is less expensive than RT-PCR. It has been suggested as a good method of first choice for synovial sarcoma (also referred to as synovial cell sarcoma). This method has a lower sensitivity than RT-PCR does, but the results of the two methods are concordant in 76% of cases. […] Aside from molecular diagnostic tests, no laboratory studies are specific for this diagnosis. […] Staging requires local imaging with MRI. The most likely site of distant spread, the lungs, can be observed by means of CT. Synovial sarcoma is a high-grade lesion.

• #1 Synovial Sarcoma: A Clinical Review

  https://www.mdpi.com/1718-7729/28/3/177

  Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5–10% of all STS. […] Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. […] A biopsy and pathologic assessment are required to differentiate synovial sarcoma from other STS subtypes and define the tumor grade. […] Staging investigations are imperative and allow for a better understanding of disease prognosis and risk of recurrence or metastases. […] The two most commonly utilized staging systems are the American Joint Committee on Cancer (AJCC) system and the Enneking staging system. […] Both fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR) testing have been validated in the diagnosis of this translocation.

• #1 Molecular diagnosis of synovial sarcoma: A comparative study with histologic correlation in Ramathibodi Hospital | Asian Archives of Pathology

  https://www.asianarchpath.com/view/33

  Synovial sarcoma (SS) is a relatively common sarcoma of soft tissue arising in extremities of young adult, which is often diagnosed by histology and immunohistochemistry (IHC). […] In this study, we evaluated the application of molecular test namely SS18/SSX fusion transcripts as an additional diagnostic tool for synovial sarcoma. […] We found that the detection of SS18/SSX fusion transcripts by RT-PCR is a valuable method to confirm the diagnosis of SS, especially in those difficult cases arising in uncommon sites or presenting with unusual histology and unconventional immunohistochemical profiles. […] Therefore, molecular analysis for the detection of SS18/ SSX fusion transcripts has been shown to be a very useful tool for making the diagnosis of SS in difficult cases. […] Our molecular test for SS also facilitated the diagnosis of other 5 cases (cases No. 18-22) with inconclusive histologic diagnosis and IHC results by excluding SS from its histologic mimickers such as MPNST, mesenchymal chondrosarcoma and solitary fibrous tumor.

• #1 Soft Tissue Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/21732-soft-tissue-sarcoma

  Healthcare providers will ask you about your symptoms, specifically unusual lumps or bumps on your body. Theyll do a physical examination. They may do several tests, including: […] Your provider obtains tissue that a medical pathologist will examine under a microscope. The pathologist may do several tests to identify the sarcoma type, stage and grade. Knowing tumor grade is how providers determine if a tumor is likely to grow slowly, grow fast or spread (metastasize). […] Yes, they do. Providers use cancer staging systems to develop treatment plans and prognoses (what you can expect if you have a soft tissue sarcoma). Providers consider the following factors in staging soft tissue sarcomas: […] Soft tissue sarcomas have four stages (Stage I to Stage IV) that combine information about tumor size, location and activity, and tumor grade. There are four grades for soft tissue sarcoma (GX to G3). In general, the lower the tumors grade, the better the prognosis. […] Cleveland Clinic offers advanced diagnosis and personalized treatment for malignant soft tissue tumors like sarcomas.

• #1 Final Diagnosis — Case 292

  https://path.upmc.edu/cases/case292/dx.html

  Cytogenetically, 90% of synovial sarcomas are associated with a chromosomal translocation, t(X;18)(p11.2;q11.2), occurring in both biphasic and monophasic forms. […] The translocation results in two fusion genes: SYT-SSX1 and SYT-SSX2. […] The 5-year survival rate is 50% and up to 82% in heavily calcified tumors. […] The 10-year survival rate is 20-30%. […] Better prognosis is related to the following factors: small tumor size (5cm are more likely to metastasize. […] Treatment consists of local excision with wide margins, followed by high dose radiation therapy. […] Adjuvant chemotherapy may also play a role.

• #1 Synovial Sarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1257131-overview

  Synovial sarcoma (also referred to as synovial cell sarcoma) is one of the most common soft-tissue tumors in adolescents and young patients, with approximately one third of cases occurring in the first two decades of life. Mean age of patients at diagnosis is approximately 30 years. […] Adjuvant chemotherapy and neoadjuvant chemotherapy have been proposed for patients with metastatic soft-tissue sarcomas, but chemotherapy in the treatment of synovial sarcoma has been controversial. Surgical excision is still the cornerstone of treatment. […] There is, however, a relative contraindication for treating these patients in primary centers; early referral to tertiary centers for definitive treatment is preferred. Radiation therapy (RT) is usually required in addition to surgery. […] Survival analysis is correlated with the location of the tumor in three anatomic regions: Trunk – Head, neck, thorax, abdomen, and pelvis; Distal extremity – Hands, feet, and ankles; Proximal extremity – Arms, forearms, thighs, and legs.

• #1 Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer

  https://www.explorationpub.com/Journals/emd/Article/100734

  Soft tissue sarcoma (STS) is a rare malignancy with a high incidence. Early diagnosis can reduce the rate of amputations and increase survival, however, this is typically delayed. […] A timely and precise diagnosis is essential because half of people with STS progress toward quietly aggressive illness. […] The purpose of this review is to raise awareness of STSs so that early recognition, accurate work-up, overview of conventional treatment plans, and appropriate referral to a tumor center can be achieved, avoiding whoop situations, and improving patient outcomes. […] The clinical symptoms observed in patients with STS are nonspecific, with the most common finding being a painless, gradually enlarging mass with site-dependent symptoms of increased pressure, such as paraesthesia and distal edema.

• #1 Soft Tissue Sarcoma Diagnosis | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/diagnosis

  Getting an accurate diagnosis is crucial to getting the best care for soft tissue sarcoma. By making absolutely sure that we have diagnosed the correct sarcoma type, we can better determine the most effective treatment approach for you. […] Diagnosing soft tissue sarcoma is not straightforward. When people come to us for a second opinion, our pathologists change their diagnosis approximately 15 percent of the time. […] While these tests are very helpful, the gold standard for diagnosing soft tissue sarcoma is a biopsy. In this procedure, we take a tiny sample of the tumor so that a pathologist (a doctor who is specially trained to diagnose disease) can look at it under a microscope. […] We use genetic and molecular sequencing on the tumors of nearly all our patients with soft tissue sarcoma. By making absolutely sure that we have diagnosed the correct sarcoma type, we can better determine the most effective treatment approach.

• #1 Synovial sarcoma | Applied Radiology

  https://appliedradiology.com/articles/synovial-sarcoma

  Synovial sarcoma […] A biopsy followed by histopathological analysis confirmed the diagnosis suggested by the MRI. […] Synovial sarcoma is graded using the widely accepted grading scheme for all sarcomas, the FNCLCC (French Federation Nationale desCentres de Lutte Contre le Cancer) scheme, which uses a combined score from 3 separate parameters, including degree of differentiation, mitotic activity, and necrosis. […] Though plain radiographs and CT may aid in the diagnosis, MRI is the imaging modality of choice for evaluating soft-tissue sarcomas. […] A large tumor size ( 5 cm) has been consistently proven to be associated with development of distant metastasis and decreased disease-specific survival. […] Many studies have shown neoadjuvant chemotherapy (doxorubicin, ifosfamide, and mensa) to improve disease-specific survival, particularly in patients with extremity tumors of a large size ( 5 cm).

• #1 Synovial sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/synovial-sarcoma?lang=us

  Synovial sarcomas are relatively common intermediate-to-high grade malignant soft tissue tumors, often with an initial indolent course, affecting young patients and most commonly involving the soft tissue surrounding the knees. […] For establishing a correct diagnosis, an IHC study for keratin and EMA is very helpful. Nearly 90% of monophasic variants are at least focally positive for keratin or EMA. […] SS18-SSX fusion antibody is 95% sensitive and specific for synovial sarcoma. […] A soft tissue mass near but not in a joint in a young patient (15-40 years old), particularly if dystrophic calcification is present, is very suggestive. […] Overall 5-year survival is between 36-76%. Both local recurrence (30-50%) and distant metastases are frequent (40-70%), most commonly to the lungs as cannonball metastases (~80%), bones (~15%), regional lymph nodes (~10%), followed by chest wall/abdomen (~7.5%).

• #2 Synovial sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/synovial-sarcoma/diagnosis-treatment/drc-20577401

  Synovial sarcoma is usually slow growing, so it can be years before a diagnosis is made. Sometimes, synovial sarcoma is diagnosed in error as a joint problem, such as arthritis or bursitis. […] Tests and procedures used to diagnose synovial sarcoma include: […] Imaging tests take pictures of the body. They can show where a synovial sarcoma is, how large it is and if it has spread to other areas of the body. Tests for synovial sarcoma might include MRI scans, X-rays and CT scans. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample. […] The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan.

• #2 Synovial Sarcoma: A Clinical Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8161765/

  Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 510% of all STS. […] Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. […] A biopsy and pathologic assessment are required to differentiate synovial sarcoma from other STS subtypes and define the tumor grade. […] The two most commonly utilized staging systems are the American Joint Committee on Cancer (AJCC) system and the Enneking staging system. […] Both fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR) testing have been validated in the diagnosis of this translocation. […] Synovial sarcoma is characterized by a pathognomonic translocation t(X:18) which is present in 95% of cases.

• #2

  https://tumorsurgery.org/tumor-education/soft-tissue-tumors/soft-tissue-tumor-types/synovial-sarcoma.aspx

  Synovial sarcoma is a common soft tissue malignancy accounting for 5-10% of soft tissue sarcomas. Patients with synovial cell sarcoma are often between the ages of 15 and 35 years old; generally younger than patients with other types of soft tissue sarcomas. Synovial sarcoma has a fusion gene, SYT-SSX, which is the result of a chromosomal translocation unique for this tumor, t(x;18)(p11;q11), which is present in up to 90% of cases. […] Symptoms may be present from days to as long as 20 years before initial diagnosis. […] Most synovial sarcomas occur in the extremities (80%). Predilection for lower limbs 60-70%. […] Intra-articular origin is found in less than 10% of the cases. […] This type of mineralization usually presents as calcifications with an irregular contour often in a peripheral distribution.

• #2 Synovial Sarcoma | Cancer Care and Oncology | Bon Secours

  https://www.bonsecours.com/health-care-services/cancer-care-oncology/conditions/synovial-sarcoma

  Doctors use imaging tests, biopsy and physical exams to diagnose synovial sarcoma. […] Your oncologist may use one or more of the following tests to diagnose this condition: […] Physical exam your doctor will perform a complete physical exam, including asking questions about your health history, symptoms and related risk factors. […] Biopsy in this test, your doctor removes a biopsy (small tissue sample) from the area of the suspected cancer. This sample is sent to the laboratory, where a specialist closely checks the biopsy for cancer. […] Computerized tomography (CT) scan this type of imaging test provides a 3D image of the inside of the body that your doctor can use to determine the size and location of the suspected cancer. Your doctor can also use the CT scan to help determine if the cancer has spread beyond the initial site.

• #2 Synovial Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/22012-synovial-sarcoma

  A healthcare provider will do a physical examination and ask you about your symptoms and health history. If they think you might have synovial cell sarcoma, theyll refer you to an oncologist for further testing. […] An oncologist will examine you and use imaging tests to see the size and location of the tumor. […] Imaging tests might include: X-rays, Magnetic resonance imaging (MRI), Ultrasound, Computed tomography (CT) scan. […] Your provider will likely have the tumor biopsied. During this procedure, theyll take a small tissue sample from the lump under your skin. Once they have the sample, theyll send it to a pathologist, who will look at the cells under a microscope.

• #2 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  Nevertheless, the existence of non-aggressive features, such as gradual growth (with an average time to diagnosis of 2–4 years) and small size (initially measuring < 5 cm) with well-defined margins, can lead to an initial misclassification as a benign lesion. [...] Recently, the prognostic importance of CT and MRI characteristics for synovial sarcomas was elucidated. [...] Gaining insight into and identifying the diverse range of presentations of synovial sarcomas, which correlate with the prognosis, might be helpful in achieving the optimal patient management. [...] The objective of this review article is to outline the diverse range of imaging features observed in extremity synovial sarcomas, spanning from non-aggressive to aggressive appearance. [...] MRI stands as the favored imaging technique for delineating the nature of soft tissue sarcomas and establishing the tumor’s extent prior to surgery.

• #2

  https://www.polradiol.com/Primary-synovial-sarcoma-on-MRI-a-case-series-and-review-r-nof-the-literature,167778,0,2.html

  Purpose: Simple MRI features are mandatory to facilitate the diagnostics of synovial sarcomas, especially for radiologists outside multidisciplinary sarcoma centres. In this case-series and review, we investigate the main appearance of synovial sarcoma on MRI. […] Knowing the main appearance of synovial sarcoma can help facilitate the diagnostics of primary synovial sarcomas. […] Therefore, precise diagnostics is essential. Magnetic resonance imaging (MRI) is the imaging modality of choice for detecting synovial sarcomas because it best characterizes intratumoural architecture, perilesional oedema, and involvement of other structures. […] A novel and simple approach is to classify soft-tissue sarcomas (STS) by characterizing their configurations on MRI. […] Our case series revealed that most primary synovial sarcomas are mainly multilobulated, heterogeneous, infiltrative, and T2 hyperintense masses with marked contrast enhancement and triple signs in many cases. Only a few of our cases showed an ovoid/nodular or a fascicular configuration. Ovoid/nodular synovial sarcomas are homogeneous masses with marked contrast enhancement and without triple signs. […] Knowing about the main appearance of synovial sarcomas on MRI allows radiologists and other clinicians to evaluate the presence or absence of a possible synovial sarcoma on MRI.

• #2 Soft Tissue Sarcoma Diagnosis and Detection

  https://www.cancercenter.com/cancer-types/soft-tissue-sarcoma/diagnosis-and-detection

  A thorough and accurate cancer diagnosis is the first step in developing a soft tissue sarcoma treatment plan. During the diagnosis process, the care team performs a complete array of diagnostic tests and thoroughly reviews the patient’s medical records and health history. The care team will also likely conduct a physical exam. This information helps the care team formulate treatment recommendations tailored to each individual patient. […] The most common procedures used for diagnosing soft tissue sarcoma include those listed below. […] CT scans are useful when making the initial soft tissue sarcoma diagnosis, and to see if the cancer has spread to other areas of the body. CT scans also may be used to guide a biopsy needle. […] MRIs may help outline a tumor in the soft tissues, and may also help determine if cancer cells have spread.

• #2 Synovial Sarcoma Treatment | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/childhood-cancer/solid-tumors/synovial-sarcoma.html

  Several tests are used to diagnose synovial sarcoma. These include: […] A health history, physical exam, and blood tests to learn about symptoms, general health, past illnesses, and risk factors […] MRI or CT scan […] Biopsy […] Full-body imaging using bone scans or PET scans to look for cancer that has spread to bone and to other areas of the body.

• #2 Soft Tissue Sarcoma Diagnosis and Detection

  https://www.cancercenter.com/cancer-types/soft-tissue-sarcoma/diagnosis-and-detection

  PET scans are very sensitive but don’t show much detail, they often are performed in combination with a CT scan (called PET/CT). […] Ultrasound scans may be used to provide information about a tumor or surrounding tissues and organs, and to precisely locate the position of a tumor in order to guide a needle biopsy. […] The lungs are a common location for soft tissue sarcomas to metastasize (spread). Therefore, X-rays of the chest may be taken to see if the cancer has spread to the lungs. […] Laparoscopic ultrasound and laparoscopic biopsies may also be performed by the surgeon to diagnose and stage soft tissue sarcoma. For tumors located within the abdomen, a laparoscopic procedure is sometimes used to diagnose or stage the extent of disease.

• #2 Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-022-05752-4

  CNB has thus found recognition as the method of choice in the 2018 guidelines of the National Comprehensive Cancer Network (NCCN) for soft tissue tumor biopsy. […] This study aims to report our institutional experience and investigate the diagnostic accuracy, sensitivity, specificity, safety, and time to diagnosis of CNB in comparison to open incisional biopsy. […] Analysis of all patients regarding the accuracy of biopsy techniques revealed a 96% accuracy for diagnosing malignancy via CNB and 100% for incisional biopsy. […] The correct entity was diagnosed in nearly all cases, and the correct grading was diagnosed in 90% of incisional biopsies and 94% of CNBs. […] During the last decade, reports have been accumulating on the reliability of CNB for the primary diagnosis of soft tissue tumors.

• #2 Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-022-05752-4

  Thus, in many centers, including ours, CNB is the primary method for biopsy soft tissue tumors. […] The ability to diagnose malignancy by CNB is widely accepted. […] The matched-pair analysis showed a 100% sensitivity for malignancy and a 95% correct diagnosis of the entity in both biopsy techniques. […] The diagnostic accuracy increased during the study period. […] The most commonly investigated gene products were MDM2 and CDK4, the mutations of which are markers for liposarcoma. […] In our series, there were no cases where a change in therapy was affected by molecular pathology findings. […] In light of our data and the pertinent literature, we conclude that CNB is reliable and safe as a method of the first choice for soft tissue sarcoma biopsy.

• #2 Soft tissue sarcoma: Recognizing a rare disease | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/89/2/73

  The recognition of a malignant soft tissue mass can be challenging, given the rarity of soft tissue sarcoma and the extensive overlap between benign and malignant presentations. […] Patients suspected of having a soft tissue sarcoma require prompt referral to a sarcoma center for assessment and treatment. […] A smaller tumor at diagnosis ( 5 cm) is associated with better prognosis. […] The UK guidelines suggest that a lump that is larger than 5 cm, exhibits growth, is deep in the body, and is painful should be considered malignant until proven otherwise. […] A high degree of suspicion for STS based on the biopsy results should trigger prompt referral to a sarcoma center for triple assessment of clinical history, imaging, and biopsy, all of which should be done on the same day. […] The standard diagnostic approach must also include biopsy, in most cases multiple percutaneous core needle specimens obtained under ultrasonographic guidance. […] If ultrasonography raises suspicion of STS or is inconclusive, the patient must be referred to a sarcoma center. […] Early referral is important to improve the outcomes.

• #2

  https://winshipcancer.emory.edu/cancer-types-and-treatments/sarcoma/diagnosis.php

  An accurate sarcoma diagnosis is critical to achieving the best outcomes. And because there are more than 150 different types of sarcoma each one biologically unique the correct diagnosis can be challenging for pathologists who lack expertise in the field of sarcoma. […] The first step in making a sarcoma diagnosis is a thorough physical examination, followed by imaging. At Winship, our sarcoma experts use a variety of imaging techniques to pinpoint the exact location, size and stage of the tumor. […] A critical component in accurately diagnosing a sarcoma is having an expert sarcoma pathologist review the biopsy tissue. Winship has world-renowned sarcoma pathologists who analyze sarcoma biopsies on a daily basis.

• #2 Synovial Sarcoma: A Clinical Review

  https://www.mdpi.com/1718-7729/28/3/177

  Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5–10% of all STS. […] Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. […] A biopsy and pathologic assessment are required to differentiate synovial sarcoma from other STS subtypes and define the tumor grade. […] Staging investigations are imperative and allow for a better understanding of disease prognosis and risk of recurrence or metastases. […] The two most commonly utilized staging systems are the American Joint Committee on Cancer (AJCC) system and the Enneking staging system. […] Both fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR) testing have been validated in the diagnosis of this translocation.

• #2

  https://www.ouhsc.edu/pathologyJTY/OUMC/OUMC-COM/Com20/Com2006-1-Diss.htm

  The positive immunoreactivity of TLE-1 is supportive of synovial sarcoma. […] Although the histopathology and immunohistochemical profile are classic for a biphasic synovial sarcoma, it lacks the classic translocation of SS18 which is seen in most synovial sarcomas. […] Synovial sarcomas are common around large joints especially the knee. […] The key to correct diagnosis is, therefore, a high index of suspicion. Given the known success of chemotherapy on synovial sarcoma, correct diagnosis has clinical importance. […] Immunohistochemistry for TLE-1 and FISH or PCR for the detection of SS18 are two powerful tools to confirm the diagnosis. […] The diagnosis of biphasic synovial sarcomas arising from classic locations are usually not a diagnostic challenge. […] When classic synovial sarcoma areas are identified in a poorly differentiated synovial sarcoma, the diagnosis is less challenging but it is not uncommon for the poorly differentiated component to make up over 90% of the tumor.

• #2

  https://link.springer.com/article/10.1245/s10434-017-5855-x

  Immunohistochemical staining for TLE1 distinguishes synovial sarcoma from histologic mimics. […] The SYT-SSX fusion protein and histological epithelial differentiation in synovial sarcoma: relationship with extracellular matrix remodeling. […] Strong association of SYT-SSX fusion type and morphologic epithelial differentiation in synovial sarcoma. […] Recurrent and novel SS18-SSX fusion transcripts in synovial sarcoma: description of three new cases. […] Clinical Neuropathology practice guide 6-2013: morphology and an appropriate immunohistochemical screening panel aid in the identification of synovial sarcoma by neuropathologists. […] Reduced expression of SMARCB1/INI1 protein in synovial sarcoma. […] Synovial sarcoma: an immunohistochemical and ultrastructural study. […] A synovial sarcoma-specific preoperative nomogram supports a survival benefit to ifosfamide-based chemotherapy and improves risk stratification for patients.

• #2 Synovial sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Synovial_sarcoma

  The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18) chromosomal translocation. […] The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18). […] Immunohistochemistry (IHC): SS18-fusion specific antibody and SSX-CT antibody are highly sensitive and specific for synovial sarcoma and when used together may obviate the need for molecular testing in most cases. […] Most, and perhaps all, cases of synovial sarcoma are associated with a reciprocal translocation t(x;18)(p11.2;q11.2).

• #2 FISH, Synovial Sarcoma, SYT, 18q11.2 Rearrangement | Test Detail | Quest Diagnostics

  https://testdirectory.questdiagnostics.com/test/test-detail/19773/fish-synovial-sarcoma-syt-18q112-rearrangement?p=r&cc=MASTER

  FISH, Synovial Sarcoma, SYT, 18q11.2 Rearrangement […] FISH, Synovial Sarcoma, SYT, 18q11.2 Rearrangement – This fluorescence in situ hybridization (FISH) assay detects the chromosome rearrangements that involve the SS18 (SYT) gene on chromosome 18q11.2. The results of this test may aid in the diagnosis of synovial sarcoma. Synovial sarcoma is a soft tissue sarcoma that mainly occurs in the extremities. A histologic and immunohistochemical diagnosis can be difficult because synovial sarcoma often resembles other spindle-cell sarcomas. The majority of the patients with synovial sarcoma have SS18 gene rearrangements, which result in the t(X;18)(p11;q11) with SS18/SSX1 […] This test was developed and its analytical performance characteristics have been determined by Quest Diagnostics. It has not been cleared or approved by FDA. This assay has been validated pursuant to the CLIA regulations and is used for clinical purposes.

• #2 Molecular diagnosis of synovial sarcoma: A comparative study with histologic correlation in Ramathibodi Hospital | Asian Archives of Pathology

  https://www.asianarchpath.com/view/33

  In summary, molecular test for SS may not be routinely required for the diagnosis of SS cases arising in usual locations or having typical histology and IHC profiles. On the other hand, it is proved to be very helpful for the diagnosis of rare SS cases arising in unusual locations, having unusual histology and unconventional IHC profiles, which can be problematic in distinguishing them from other histologic mimickers such as MPNST, Ewing sarcoma, solitary fibrous tumor, undifferentiated pleomorphic sarcoma etc.

• #2 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  The MRI presentations of synovial sarcomas encompass a spectrum, spanning from small, uniform nodules to extensive, heterogeneous masses. […] Most synovial sarcomas manifest as prominently heterogeneous, multilobulated soft-tissue masses, displaying SI similar to or slightly higher than that of muscle on T1-weighted images and predominantly bright SI on T2-weighted images. […] The presence of peritumoral enhancement, without causing any discernible mass effects on the neighboring tissues, is regarded as a direct sign of tumor margin infiltration. […] A more favorable prognosis, on the other hand, is linked to extensive calcification and tumor size below 5 cm. […] The treatment for synovial sarcomas typically comprises a combination of surgery, radiotherapy, and chemotherapy. […] A biopsy is essential for an accurate diagnosis in patients with synovial sarcoma to differentiate synovial sarcoma from other soft-tissue sarcoma subtypes and define the tumor grade.

• #2 Synovial Sarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1257131-overview

  Distal-extremity tumors have a better prognosis than proximal-extremity or truncal tumors do. […] Synovial sarcoma has survival rates of 50-60% at 5 years and 40-50% at 10 years. […] A slight improvement in survival rate has been reported with the use of chemotherapy as adjuvant therapy. […] Recurrence has been reported up to 69 months after treatment and suggests a worse prognosis with low survival rates. […] Distant metastases at presentation suggests a bad prognosis (2-year survival rate, 25%). […] The addition of chemotherapy to postoperative RT was not found to improve survival or disease control. […] RT improved survival, but the benefit of chemotherapy was unclear. […] In another study (N = 130) using data from the SEER database, Zhu et al found that in children with synovial sarcoma, factors associated with poorer overall and cancer-specific survival were chemotherapy (possibly because of the selection bias in chemotherapy, whereby patients receiving chemotherapy tend also to have concurrent metastases and a poor prognosis) and worse SEER stage.

• #2 Synovial Sarcoma | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/s/synovial-sarcoma

  Synovial sarcoma is a soft tissue sarcoma that is usually found in the legs or arms but can occur anywhere in the body. […] The first step in diagnosing synovial sarcoma is for the doctor to take a detailed medical history of the patient and family. Next, the patient has a physical exam and other types of tests, which may include: X-rays, Magnetic resonance imaging (MRI), Computed tomography (CT), Bone scans, Positron emission tomography (PET), a test that measures important body functions and also can show whether the cancer has spread, Blood tests, A needle biopsy or surgical procedure. A specialist examines the tissue under a microscope to confirm the diagnosis of synovial sarcoma. […] Prompt diagnosis and treatment can significantly improve the outcome for patients with synovial sarcoma. It is very important for patients to receive specialized care at a hospital such as Cincinnati Childrens. Our sarcoma team has experience in diagnosing and treating this rare type of cancer.

• #2 Synovial sarcoma

  https://www.mymlc.com/health-information/diseases-and-conditions/s/synovial-sarcoma/?section=Overview

  Synovial sarcoma is usually slow-growing, so it can be years before a definitive diagnosis is made. In some cases, synovial sarcoma initially is diagnosed incorrectly as arthritis or bursitis. […] Imaging methods that may be helpful include: […] A sample of the tumor can be removed with a needle and then studied under a microscope to help determine the best treatment. Synovial sarcoma can be mistaken for other types of sarcomas, so a correct diagnosis depends on experienced pathologists.

• #2 Synovial sarcoma: the misdiagnosed sarcoma in: EFORT Open Reviews Volume 9 Issue 3 (2024)

  https://eor.bioscientifica.com/view/journals/eor/9/3/EOR-23-0193.xml

  SS is considered to be a high-grade STS with a tendency to be locally aggressive and metastatic; therefore, early diagnosis and treatment is crucial for the patients prognosis. However, the high rate of delay and misdiagnosis of SS in current clinical diagnosis has negative consequences for patients. […] Therefore, we recommend that orthopedic physicians perform biopsies and testing for the SS18 gene rearrangements in all patients in whom a SS cannot be excluded, especially those located in the extremities and joints, to minimize misdiagnosis.

• #2 Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer

  https://www.explorationpub.com/Journals/emd/Article/100734

  The best individual indicator of increased malignancy risk is an increase in tumor size. An accurate differential diagnosis can be obtained after a comprehensive history and physical examination, including examination of the site, size, shape, contour, consistency, tenderness and tethering. […] Appropriate imaging and histological analysis will further assist in confirming this diagnosis. […] Timely referral to a specialized center is essential, allowing for early treatment that can improve the survival rate and lower the amputation rate. […] It is essential to diagnose STS early as it is silently aggressive in nature, negligence and unawareness will result in the disease progression from acute to chronic. […] Studies have found that there is a linear relationship between an increasing lesion size with a poorer prognosis, especially for lesions greater than 5 cm.

• #2 Synovial Cell Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK587366/

  The diagnosis follows histopathology, and clinical imaging helps determine the tumor’s stage. […] A pathologist trained in diagnosing STS is needed to establish histology and accurately grade cancer. […] Patients diagnosed with synovial sarcoma should receive treatment at centers that regularly experience a high volume of such patients. […] The principles of treating a patient diagnosed with synovial sarcoma require close coordination of interprofessional team members between the surgical oncology, radiation oncology, and medical oncology teams.

• #2 Sarcoma Diagnosis | Sarcoma Oncology Center

  https://sarcomaoncology.com/services/sarcoma-diagnosis/

  At Sarcoma Oncology Center, we understand the importance of early detection and diagnosis. Our team can answer your questions and address any anxieties you may have about your condition. […] During your diagnostic consultation, our oncologists discuss your medical history, including any symptoms, risk factors, and overall health condition with you. A physical examination is performed to identify potential signs of sarcoma. […] While imaging tests may identify the possible existence of sarcoma, a biopsy test is typically the most definitive step for diagnosis and identifying the specific type of sarcoma you may have. […] This procedure is crucial for confirming a sarcoma diagnosis and determining the type and grade (aggressiveness) of the cancer. A biopsy allows a pathologist to definitively confirm the presence of sarcoma and differentiate it from other conditions. […] Following the biopsy, tissue samples may undergo additional laboratory testing to identify specific genetic markers or protein expressions unique to your sarcoma. Understanding these characteristics helps tailor your treatment plan, offering a more personalized approach.

• #2 Synovial sarcoma | Applied Radiology

  https://appliedradiology.com/articles/synovial-sarcoma

  MRI is extremely helpful in preoperative planning of tumor excision. […] In conclusion, synovial sarcoma is an aggressive tumor and one of the most common soft-tissue neoplasms, which occur in the first 3 decades of life. It has a propensity to involve juxta-articular regions. A large tumor size at the time of diagnosis indicates poor prognosis. MRI is the imaging modality of choice in the preoperative evaluation of these lesions. Neoadjuvant chemotherapy has been found to improve disease-specific survival in some patient groups; particularly those with large-sized extremity tumors. Surgery is the mainstay of treatment.

• #2 9 synovial sarcoma questions, answered | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/synovial-sarcoma.h00-159068712.html

  Most synovial sarcoma patients discover their tumor from a lump, and for some, related pain. Its important to see a doctor if you find a lump or have persistent, unprovoked pain in a soft tissue, such as the muscle of an extremity or from within your abdomen. Synovial sarcoma may also occur in the lungs, so see your doctor if you notice shortness of breath. […] Synovial sarcoma can be found in the soft tissues by an MRI, or in the lungs with an X-ray or CT scan. However, an official diagnosis can only be made by a pathologist after obtaining tissue samples through biopsy or surgery. […] Make sure you get your regular scans and follow-ups with your doctor, even if you’ve had no evidence of disease for many years.

• #2 Synovial Cell Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK587366/

  Synovial sarcoma is defined by the translocation between chromosome X and 18, which leads to the expression of SS18:SSX fusion proteins. […] The presence of t(X;18)(p11.2;q11.2) is a pathognomonic feature of synovial sarcoma. […] Several molecular techniques are used to determine this translocation. […] The evaluation of a patient with SS includes a biopsy of the mass and radiographic tests. […] To establish the diagnosis, multidisciplinary and interprofessional planning must be pursued to obtain a pretreatment biopsy. […] The treatment of patients with synovial sarcoma is based on the stage of presentation. […] The therapy involves using cytotoxic chemotherapy, tyrosine kinase inhibitors, or enrolling the patient in a clinical trial. […] The evaluation of a patient with SS includes a biopsy of the mass and radiographic tests.

• #2 Distinguishing synovial sarcoma from benign and malignant mimics: MR Imaging indicators | Applied Radiology

  https://www.appliedradiology.com/Articles/distinguishing-synovial-sarcoma-from-benign-and-malignant-mimics-mr-imaging-indicators

  Confirmation of the presence of a mass by radiography and MRI is usually performed prior to biopsy. Definitive SS diagnosis is based on biopsy. The biopsied tissue is often tested for the t(X;18)(p11;q11) translocation using fluorescence in situ hybridization (FISH), for which about 90% of SS are positive. […] Accurate diagnosis of synovial sarcoma can impact prognosis and overall survival rates by maximizing the chances of appropriate and aggressive treatment. Although synovial sarcomas are most prevalent in children and young adults, and tend to occur in the extremities and can occur at any age and in any location in the body. We recommend all imaging studies be reviewed by a radiologist with experience in evaluating musculoskeletal tumors. A lesion should be considered a potential synovial sarcoma if it has any suspicious features. The authors recommend biopsy of all suspicious lesions and use of FISH for confirmation of the diagnosis of synovial sarcoma.

• #3 Synovial sarcoma: the misdiagnosed sarcoma in: EFORT Open Reviews Volume 9 Issue 3 (2024)

  https://eor.bioscientifica.com/view/journals/eor/9/3/EOR-23-0193.xml

  SS is considered to be a high-grade STS with a tendency to be locally aggressive and metastatic; therefore, early diagnosis and treatment is crucial for the patients prognosis. However, the high rate of delay and misdiagnosis of SS in current clinical diagnosis has negative consequences for patients. […] Therefore, we recommend that orthopedic physicians perform biopsies and testing for the SS18 gene rearrangements in all patients in whom a SS cannot be excluded, especially those located in the extremities and joints, to minimize misdiagnosis.

• #3 Synovial Sarcoma: A Clinical Review

  https://www.mdpi.com/1718-7729/28/3/177

  Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5–10% of all STS. […] Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. […] A biopsy and pathologic assessment are required to differentiate synovial sarcoma from other STS subtypes and define the tumor grade. […] Staging investigations are imperative and allow for a better understanding of disease prognosis and risk of recurrence or metastases. […] The two most commonly utilized staging systems are the American Joint Committee on Cancer (AJCC) system and the Enneking staging system. […] Both fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR) testing have been validated in the diagnosis of this translocation.

• #3 Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis

  https://www.mdpi.com/2072-6694/15/19/4860

  Synovial sarcomas are the fourth most prevalent soft-tissue sarcoma, primarily affecting the extremities (in 80–95% of cases) of adolescents and young adults aged 15 to 40. […] The imaging characteristics of synovial sarcomas encompass a wide spectrum, ranging from non-aggressive features to more aggressive appearances. […] Understanding and identifying these diverse presentations, along with their associated prognostic implications, might be helpful in patient evaluations and in achieving optimal management. […] While the radiological features of these tumors are not definitely indicative, the presence of calcification in a soft-tissue mass (occurring in 30% of cases), adjacent to a joint, strongly suggests the diagnosis. […] Cross-sectional imaging characteristics play a crucial role in diagnosing synovial sarcomas.

• #3 Synovial sarcoma: Magnetic resonance and computed tomography imaging features and differential diagnostic considerations

  https://www.spandidos-publications.com/10.3892/ol.2014.2774

  In general, the size of the lesions tends to be large, with 85% of tumors being 5 cm in size. […] In the current study, all 24 lesions were deeply located within tissues. […] Furthermore, MRI is one of the most common imaging examinations for soft-tissue tumors and is considered the modality of choice for the detection and staging of soft-tissue tumors. […] The combination of the two approaches can improve the accuracy of the pre-operative diagnosis. However, the final diagnosis relies on pathological investigation.

• #3 Synovial Sarcoma | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/types/synovial-sarcoma

  Synovial sarcoma is a rare cancer that is more commonly diagnosed in people under 40. It is a form of soft tissue sarcoma. […] Doctors use imaging to detect synovial sarcoma. These tests typically include MRIs or CTs. The scans can show the size of the tumor, where it started, whether any nearby organs or tissues are involved, and whether the tumor has spread to other areas of the body. […] Biopsy is usually the next step. A biopsy is the only test that can make a definite diagnosis of synovial sarcoma. It involves taking a sample of the tumor. That sample is checked under a microscope for cancer cells by a pathologist (a doctor who is specially trained to diagnose disease). […] It is very important to receive a diagnosis from a team of doctors that is highly experienced in the diagnosis and treatment of soft tissue sarcoma. There are more than 80 subtypes of soft tissue sarcoma, so it can be a difficult disease to diagnose. Getting the correct evaluation and diagnosis is the first step to being cured.

• #3 Diagnosing Soft Tissue Sarcoma | NYU Langone Health

  https://nyulangone.org/conditions/soft-tissue-sarcoma/diagnosis

  This form of X-ray uses a computer to create cross-sectional, three-dimensional pictures, offering doctors various views of a tumor. CT scans can help your doctor determine if cancer has spread to other organs, such as the lungs. […] Your doctor may use a PET/CT scan, which can generate images of the entire body, if there is a possibility that the cancer has spread. […] A biopsy involves the removal of tissue from the body for extensive molecular and genetic laboratory tests and examination under a microscope for signs of cancer. These tests can help your doctor determine the type of soft tissue sarcoma that’s present and the therapies that may be most successful. […] Doctors may use a core needle biopsy to get a sample of a soft tissue sarcoma. […] Sometimes, diagnosing a soft tissue sarcoma using tissue from a needle biopsy can be challenging. If so, your doctor may perform a surgical biopsy in an operating room. This involves removing a large portion of tumor tissue through a surgical incision.

• #3 Metastatic biomarkers in synovial sarcoma | Biomarker Research | Full Text

  https://biomarkerres.biomedcentral.com/articles/10.1186/s40364-017-0083-x

  Synovial sarcoma (SS) is an aggressive soft tissue sarcoma (STS) that typically occurs in the extremities near a joint. […] Chromosomal translocation with fusion of the SYT and SSX genes has been described and is currently used as a diagnostic marker, although the full impact of the fusion is unknown. […] Over 95% of SS can be characterized by expression of the SYT-SSX gene and it is used as a routine diagnostic marker for this type of cancer. […] Development of a diagnostic blood test to detect SS and assess metastatic risk would be a valuable tool for the management of this disease. […] Detection of a circulating SYT-SSX fusion gene product could be another important method for assessing metastatic risk in patients with SS. […] A number of studies have investigated the association between SYT-SSX gene fusion type and metastatic risk.

• #3 Molecular Diagnostics in Sarcoma… | College of American Pathologists

  https://www.cap.org/member-resources/articles/molecular-diagnostics-in-sarcoma-pathology

  Increasingly, pathologists employ molecular testing as an aid in sarcoma diagnosis. […] Molecular testing (i.e., testing DNA and/or RNA) has become increasingly common in diagnostic testing pathways for sarcomas and is often required for clinical trial protocols. […] The authors conclude that, for all sarcomas with recurrent genetic aberrations (e.g., synovial sarcoma, alveolar rhabdomyosarcoma), molecular testing should be performed. […] Current World Health Organization (WHO) and National Comprehensive Cancer Center (NCCN) guidelines acknowledge the utility of molecular testing in sarcoma diagnosis. […] Current NCCN Soft Tissue Sarcoma guidelines confirm the diagnostic utility of molecular genetic testing and recommend that such testing should be carried out by a pathologist with expertise in sarcoma diagnosis and molecular diagnostic techniques. […] While morphology and IHC remain the bedrock for sarcoma diagnosis, molecular methods have shown potential for improving and/or refining diagnosis of these rare tumors. Additionally, molecular genetic testing is finding greater acceptance in diagnostic guidelines for soft tissue and bone tumors.

• #3 Synovial Sarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1257131-overview

  In cohort 1, independent favorable prognostic factors for overall survival were young age, surgery of the primary tumor, and a single metastatic site; in cohort 2, independent favorable prognostic factors were surgery within an expert FSG center, absence of perioperative chemotherapy, the lungs as a single metastatic site, time to first metastasis greater than 12 months, local therapy, and ifosfamide in the first metastatic line.

• #3 Soft Tissue Sarcoma Diagnosis | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/diagnosis

  Getting an accurate diagnosis is crucial to getting the best care for soft tissue sarcoma. By making absolutely sure that we have diagnosed the correct sarcoma type, we can better determine the most effective treatment approach for you. […] Diagnosing soft tissue sarcoma is not straightforward. When people come to us for a second opinion, our pathologists change their diagnosis approximately 15 percent of the time. […] While these tests are very helpful, the gold standard for diagnosing soft tissue sarcoma is a biopsy. In this procedure, we take a tiny sample of the tumor so that a pathologist (a doctor who is specially trained to diagnose disease) can look at it under a microscope. […] We use genetic and molecular sequencing on the tumors of nearly all our patients with soft tissue sarcoma. By making absolutely sure that we have diagnosed the correct sarcoma type, we can better determine the most effective treatment approach.

• #3 Synovial Sarcoma: A Clinical Review

  https://www.mdpi.com/1718-7729/28/3/177

  The mainstay of treatment for synovial sarcoma remains surgical excision with negative margins with the addition of radiotherapy and/or chemotherapy based on patient and tumour characteristics. […] Neoadjuvant or adjuvant radiation therapy is recommended for larger tumors (>5 cm), or in any case where a close margin may be required to preserve a major neurovascular structure or bone. […] Unlike the majority of STS, synovial sarcoma appears to be more chemosensitive, although there is still controversy surrounding which subgroups of patients benefit from systemic therapy.

• #3 Synovial Sarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/synovial-sarcoma/

  A biopsy — removal of a small sample of the tumor with a needle — can help narrow down the diagnosis. The tissue sample will be examined under a microscope by a pathologist to determine the type of tumor. […] Because synovial sarcoma is an aggressive type of cancer and doesn’t always have initial symptoms, it means it is more likely to have spread to other parts of the body. […] Treatment involves surgically removing the tumor. Radiation therapy may be used before surgery to shrink the tumor or after surgery to attack any remaining cells. If synovial sarcoma cannot be completely removed surgically or has spread, chemotherapy likely will be needed to attack the cancer. […] Synovial sarcoma has the best chance of being cured when it’s treated by pediatric cancer specialists. Young adults may benefit from treatment at a children’s hospital instead of an adult-service hospital because of the care team’s expertise in childhood diseases.

• #3 Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer

  https://www.explorationpub.com/Journals/emd/Article/100734

  Imaging procedures alone do not permit the exact classification of a tumor as benign or malignant, therefore diagnosis is dependent on the histopathological investigation. […] Following confirmation of STS by biopsy a course of action can be determined depending on the histopathological grade and staging. […] A whoops procedure is the process where a mass that was considered benign is resected in an unplanned manner without final diagnosis, preoperative imaging, or planning and is unexpectedly found to be a sarcoma when pathology results come back. […] The consequences of a whoops procedure harm the overall prognosis of the disease. […] To improve this, a soft tissue mass presenting with red flag symptoms should be considered malignant until proven otherwise, and all necessary steps in management should be taken when there is clinical suspicion of STS.

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