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Desar I. Systemic treatment for adults with synovial sarcoma. Options Oncol. Trassard M. Cancer Discov. The morphologic diagnosis of sarcoma relies on the evaluation as well as the integration of four main features: the shape of the neoplastic cells; the pattern of growth; the quality of the background; the architecture of the vascular network.
Immunohistochemical characterization plays a key role in the diagnostic workup of soft tissue sarcomas. Skip to main content. By using our site, you agree to our collection of information through the use of cookies. To learn more, view our Privacy Policy. Log In Sign Up. Download Free PDF. Sana Arooj. Download PDF. A short summary of this paper. These mostly arise in the diaphyses of long bones but can also occur in various extra skeletal tissues and carry poor prognoses.
Multimo- dality treatment of surgery and chemoradiation is needed to improve survival. J Med Sci ; 2 James Ewing in She then developed generalized tumor. She was treated for hypertension and her cases involving the diaphyses of long bones. But the weight gain was attributed to her pregnancy. She then flat bones, chest wall and extra skeletal soft tissues started feeling headache, decreased vision, nausea or occasionally, the solid organs are also involved.
The extra skeletal EWS has been reported in various She kept switching from one to the other hospital locations of the body, including scalp, central nervous and was delivered through a cesarean section in Paki- system CNS , nose, lips, arms, hands, retroperitoneum, stan. Her symptoms were a little bit alleviated but con- paravertebral tissues, kidney, small intestine, pelvis, tinued. Her post operative course remained uneventful. While, limited cases of primary adrenal EWS have been report- during the work up for hypertension, she was found ed in the literature.
Ziauddin Afridi Corresponding Author her body, predominantly lower limbs. An ultrasound Assistant Professor US abdomen, during the routine work up showed a Department of Surgery, Khyber Teaching Hospital, complex mass in the left supra-renal location.
While the contrast enhanced CT Email: ziafridi hotmail. USS may play a role in guidance for core-biopsy sampling of superficial masses. However, if a malignancy is suspected, then magnetic resonance imaging MRI and computed tomography CT scans are required prior to biopsy sampling, allowing for assessment and staging to be completed using images which have not yet been altered by intervention. Although X-rays are not particularly useful for the assessment of STS, they can provide valuable information.
Calcification of a soft-tissue mass may indicate an extraosseous bone-forming sarcoma or synovial sarcoma. Myositis ossificans, a calcified hematoma and hemangiomas with phleboliths, while common, is associated with distinct presenting histories. Its high level of soft-tissue contrast and anatomical detail mean that even small soft tissue can be detected with a great accuracy.
This patient successfully underwent wide resection and plastic reconstruction with a latissimus dorsi flap. Signs on MRI which can be indicative not diagnostic of a malignancy are: Deep-seated large mass, heterogeneous signal, surrounding edema, associated destruction of bone.
MRI plays an essential role in the local staging of tumors such that no surgery is to be performed nor biopsy taken until an MRI is carried out. Biopsy planning with the use of cross-sectional imaging, such as MRI, ensures that other compartments are not contaminated and that the interpretation of images is not compromised by post biopsy edema or hemorrhage. MRI is extremely valuable in locating neurovascular structures and defining specific muscular compartments being affected, as well as showing the spread of individual muscles by the tumor.
These factors play an essential role in determining the tumor's resectability and the anticipated quality of surgical margins. CT is readily available and can be used in cases where MRI is contraindicated or where CT may better delineate areas of periosteal bone formation, micro-calcification, and cortical destruction. Positron emission tomography-CT PET-CT is a nuclear medicine investigation which combines PET's ability to detect the distribution of metabolic or biochemical activity with CT's precise anatomic imaging, thereby defining the location of STS primary tumors and metastasis.
Previously, identifying disease during the initial staging of sarcomas has been done by plain chest X-ray or chest CT and bone scintigraphy. Evidence has shown that PET added to conventional imaging improved preoperative staging, 13 and recently, PET-CT scans have been shown to have a higher sensitivity, specificity, accuracy, negative predictive value, and positive predictive value than PET or CT alone and is therefore recommended as a part of routine diagnostic imaging in STS.
Tissue biopsy of STS is an essential part of both diagnosis and management, with inadequate or inaccurate biopsies usually leading to poorer outcomes. Such poor outcomes include higher mortality rates and reduced opportunity for limb salvage. Biopsy techniques include core-needle biopsy with CT guidance [ Figure 2 ] and open biopsy, with fine-needle biopsy not being sufficient as it does not provide information regarding the structure of the tumor, which is essential for accurate tumor diagnosis.
Biopsy specimens undergo a range of investigations, including immunohistochemistry, flow cytometry, and cytogenetics. Poorly planned biopsies have been shown to result in greater adverse outcomes than those which are appropriately planned, this includes the need for more amputations due to biopsy-related local contamination and subsequent spread of disease.
If malignancy is suspected, then an MRI is required. Beware the diagnosis of a chronic hematoma. MRI: Adequate scans will include the whole anatomical compartment, the involved site, and its adjacent joints. Essential for local staging of disease. CT: Essential in systemic staging where its role is to exclude pulmonary metastatic disease. Used for guidance in core-biopsy sampling. Tissue biopsy: Gold standard for confirming sarcoma diagnosis. Recommended to be performed by a specialist referral center.
Management of STS outside of specialist sarcoma referral centers results in significantly worse clinical outcomes. Adverse outcomes include increased morbidity from subsequent, potentially more complex surgeries and increased mortality. Evaluation prior to a planned oncological excision involves complete anatomic MRI of the affected part, CT of the chest, and tissue biopsy. A general practitioner GP will see many hundreds of benign tumors in their lifetime of clinical practice, but can be expected to only see one or two patients with STS.
Earlier diagnosis would result in improved survival and allow for less-damaging surgery being performed. Surgeons inappropriately excising STS include general surgeons, plastic surgeons, and nononcologically trained orthopedic surgeons, at times excising masses without definite knowledge what is contained inside [ Figure 3a ].
Inappropriate excisions. This patient had undergone an unplanned excision of a small mass by a general surgeon, which turned out to be a sarcoma.
Subsequently, the sarcoma locally recurred, rapidly grew, and began to fungate. Local recurrence resulted in the patient requiring above-elbow amputation. Knowledge that reexcision referrals are coming from these clinicians can allow for targeted education regarding safe and appropriate management. Educational interventions have been directed at these groups of surgeons, as well as GPs, however this problem has persisted.
Numerous studies have demonstrated that, as a downstream effect of inappropriate excision, 7. A recent study by Potter et al. The larger a STS is at referral, the worse the chance of cure. The intervention involved a mail package with a golf ball 4. Golf ball sized 4. Consider any lump bigger than this to be a sarcoma until proven otherwise.
Excision of a STS should be performed by an orthopedic surgeon with specialist sarcoma training. Surgery remains the primary treatment modality for STS. Decisions about the optimal surgical procedure for the primary tumor are based on the tumor location, tumor size, involvement of adjacent anatomical structures, patient preference, and response to neoadjuvant therapies.
Such decisions are often made with the consultation of an MDT. The primary aim of curative surgery is to excise the whole tumor with tumor-negative margins. Local staging of disease is essential for surgical planning. The tumor size, capsule, consistency, site, shape, edge, and adjacent structures are vital pieces of information for planning the surgical margins and reconstructions after assessing response to neoadjuvant therapies.
STS have a predilection to grow in a centrifugal manner, which pushes the surrounding tissue aside as they grow. During this process, a pseudocapsule of compressed tissue and inflammation develops around the tumor, which often contains micro-satellites of tumor tissue. Axial MRI showing surgical margins of a right-sided thigh undifferentiated pleomorphic sarcoma. Red circle innermost represents an intralesional margin.
Yellow circle 2 nd from center represents a marginal excision. Green circle outermost line represents a wide excision. This form includes excision of a STS with microscopic disease remaining and potentially macroscopic disease.
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