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      Retroperitoneal Leiomyosarcoma From the Radiologic Pathology Archives

      1 , 1
      RadioGraphics
      Radiological Society of North America (RSNA)

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          Abstract

          <p class="first" id="d4881591e157">Leiomyosarcoma is a malignant neoplasm that shows smooth muscle differentiation. It is the second most common sarcoma to affect the retroperitoneum. Retroperitoneal leiomyosarcomas may grow to large sizes before detection and may be an incidental finding at imaging. When symptomatic, retroperitoneal leiomyosarcoma may cause compressive symptoms, including pain. Retroperitoneal leiomyosarcoma most commonly manifests as a large soft-tissue mass, with areas of necrosis. The most frequent pattern of growth is an entirely extravascular mass. Less commonly, leiomyosarcoma may demonstrate both extravascular and intravascular components. Rarely, retroperitoneal leiomyosarcomas are completely intravascular, typically arising from the inferior vena cava. Given its variable imaging features, a large variety of neoplastic and nonneoplastic conditions are included in the differential diagnosis of retroperitoneal leiomyosarcoma. In this review, the authors discuss retroperitoneal leiomyosarcoma, with emphasis on the pathologic basis of disease, and illustrate the multimodality imaging appearances of retroperitoneal leiomyosarcoma using cases from the Radiologic Pathology Archives of the American Institute for Radiologic Pathology. The authors review important differential considerations of retroperitoneal leiomyosarcoma, focusing on the extravascular pattern of growth, and emphasize clinical and imaging features that help radiologists differentiate leiomyosarcoma from the most frequent mimics. The information presented in this review will aid radiologists in fulfilling their key roles in the diagnosis, operative planning, and follow-up of patients with retroperitoneal leiomyosarcoma. </p>

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          Most cited references43

          • Record: found
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          Cancer Screening Recommendations for Individuals with Li-Fraumeni Syndrome

          Li-Fraumeni syndrome (LFS) is an autosomal dominantly inherited condition caused by germline mutations of the TP53 tumor suppressor gene encoding p53, a transcription factor triggered as a protective cellular mechanism against different stressors. Loss of p53 function renders affected individuals highly susceptible to a broad range of solid and hematologic cancers. It has recently become evident that children and adults with LFS benefit from intensive surveillance aimed at early tumor detection. In October 2016, the American Association for Cancer Research held a meeting of international LFS experts to evaluate the current knowledge on LFS and propose consensus surveillance recommendations. Herein, we briefly summarize clinical and genetic aspects of this aggressive cancer predisposition syndrome. In addition, the expert panel concludes that there are sufficient existing data to recommend that all patients with LFS be offered cancer surveillance as soon as the clinical or molecular LFS diagnosis is established. Specifically, the panel recommends adoption of a modified version of the "Toronto protocol" that includes a combination of physical exams, blood tests, and imaging. The panel also recommends that further research be promoted to explore the feasibility and effectiveness of these risk-adapted surveillance and cancer prevention strategies while addressing the psychosocial needs of individuals and families with LFS. Clin Cancer Res; 23(11); e38-e45. ©2017 AACRSee all articles in the online-only CCR Pediatric Oncology Series.
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            Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.

            Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma are tumors of the sympathetic nervous system that arise from primitive sympathogonia and are referred to collectively as neuroblastic tumors. They arise wherever sympathetic tissue exists and may be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis. The three tumors differ in their degree of cellular and extracellular maturation; immature tumors tend to be aggressive and occur in younger patients (median age, just under 2 years), whereas mature tumors occur in older children (median age, approximately 7 years) and tend to behave in a benign fashion. The most benign tumor is the ganglioneuroma, which is composed of gangliocytes and mature stroma. Ganglioneuroblastoma is composed of both mature gangliocytes and immature neuroblasts and has intermediate malignant potential. Neuroblastoma is the most immature, undifferentiated, and malignant tumor of the three. Neuroblastoma, however, may have a relatively benign course, even when metastatic. Thus, these neuroblastic tumors vary widely in their biologic behavior. Features such as DNA content, tumor proto-oncogenes, and catecholamine synthesis influence prognosis, and their presence or absence aids in categorizing patients as high, intermediate, or low risk. Treatment consists of surgery and, usually, chemotherapy. Despite recent advances in treatment, including bone marrow transplantation, neuroblastoma remains a relatively lethal tumor, accounting for 10% of pediatric cancers but 15% of cancer deaths in children. Copyright RSNA, 2002
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              Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review.

              Cystic lesions of the retroperitoneum can be classified as either neoplastic or nonneoplastic. Neoplastic lesions include cystic lymphangioma, mucinous cystadenoma, cystic teratoma, cystic mesothelioma, müllerian cyst, epidermoid cyst, tailgut cyst, bronchogenic cyst, cystic change in solid neoplasms, pseudomyxoma retroperitonei, and perianal mucinous carcinoma. Nonneoplastic lesions include pancreatic pseudocyst, nonpancreatic pseudocyst, lymphocele, urinoma, and hematoma. Because the clinical implications of and therapeutic strategies for retroperitoneal cystic masses vary depending on the cause, the ability to noninvasively differentiate between masses is important. Although there is substantial overlap of computed tomographic (CT) findings in various retroperitoneal cysts, some CT features, along with clinical characteristics, may suggest a specific diagnosis. CT may provide important information regarding lesion location, size, and shape; the presence and thickness of a wall; the presence of septa, calcifications, or fat; and involvement of adjacent structures. The most important clinical parameters include patient gender, age, symptoms, and clinical history. Familiarity with the CT and clinical features of various retroperitoneal cystic masses facilitates accurate diagnosis and treatment. Copyright RSNA, 2004
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                Author and article information

                Journal
                RadioGraphics
                RadioGraphics
                Radiological Society of North America (RSNA)
                0271-5333
                1527-1323
                September 2018
                September 2018
                : 38
                : 5
                : 1403-1420
                Affiliations
                [1 ]From the Department of Radiology and Imaging Sciences, National Institutes of Health, Bethesda, Md (J.M.); American Institute for Radiologic Pathology, 1100 Wayne Ave, Suite 1020, Silver Spring, MD 20910 (J.M., D.J.W.); and Community Radiology Division, Johns Hopkins School of Medicine, Washington, DC (D.J.W.).
                Article
                10.1148/rg.2018180006
                6166742
                30207936
                849c367d-ed91-4c5d-8e6e-bfe9c8d144d5
                © 2018
                History

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