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Wide resection for clear cell carcinoma
26%
631/2461
Wide resection for alveolar soft part sarcoma
52%
1271/2461
Radiation therapy alone for large B-cell lymphoma
3%
79/2461
Marginal resection for renal cell carcinoma
6%
147/2461
Marginal resection for well-differentiated liposarcoma
13%
320/2461
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The patient has alveolar soft part sarcoma (ASPS), which after appropriate staging studies, is best treated with wide resection. ASPS is a rare soft tissue sarcoma of unclear etiology representing less than 1% of all soft tissue sarcomas. Similar to other soft tissue sarcomas, it presents as a painless, slow-growing mass. It is a particularly vascular tumor with a poor prognosis and limited treatment options. Although wide resection is the standard of care, local recurrence rates range from 11-50%. There is a high prevalence of metastasis to the lungs, bone, or central nervous system at the time of presentation; lung metastectomy is often part of the treatment protocol. Zarrin-Khameh et al. review the clinical and pathologic features of ASPS. They report ASPS is a rare soft tissue sarcoma that typically occurs in patients aged 15-35. The majority of patients have metastatic disease at the time of diagnosis. It is characterized cytogenetically by a chromosomal translocation of t(X;17)(p11;q25) creating the fusion gene TFE3-ASPSCR1. The prognosis is generally poor with frequent metastatic disease at the time of presentation. Wide surgical resection and metastectomy is the recommended treatment due the tumor's resistance to chemotherapy. Stacy et al. describe some of the characteristic MRI findings of ASPS. The mass is typically well-circumscribed and has high signal on T1 and T2 imaging. The tumor can enhance in a homogenous or heterogeneous fashion, but often has internal areas of flow void due to its vascularity. Figure A demonstrates T2 and T1 axial MRI images of a heterogeneous soft tissue sarcoma of the posterior leg. Figure B demonstrates classic histology for ASPS with nests of cells separated by fibrous stroma in an alveolar pattern. As seen in the slide, the cells can be large and polygonal with cytoplasmic vacuolization. Incorrect answers: Answer 1: Wide resection would be appropriate if this was clear cell carcinoma Answer 3: While radiation therapy may be an appropriate treatment for certian types of lymphoma, the histology represents ASPS Answer 4: The patient's histology is not consistent with renal cell carcinoma Answer 5: The patient's histology is not consistent with liposarcoma
1.6
(10)
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