Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 1—Common Sarcomas: From the Radiologic Pathology Archives

Published Online:https://doi.org/10.1148/rg.2017160157

The classification of soft-tissue sarcomas and the clinical, pathologic, and imaging features of the most common primary soft-tissue sarcomas of the abdomen and pelvis (liposarcoma, leiomyosarcoma, and gastrointestinal stromal tumor) are reviewed, with emphasis on the radiologic features that aid in differential diagnosis and preoperative planning.

Soft-tissue sarcomas are a diverse group of rare mesenchymal malignancies that can arise at any location in the body and affect all age groups. These sarcomas are most common in the extremities, trunk wall, retroperitoneum, and head and neck. In the adult population, soft-tissue sarcomas arising in the abdomen and pelvis are often large masses at the time of diagnosis because they are usually clinically silent or cause vague or mild symptoms until they invade or compress vital organs. In contrast, soft-tissue sarcomas arising from the abdominal wall come to clinical attention earlier in the course of disease because they cause a palpable mass, abdominal wall deformity, or pain that is more clinically apparent. The imaging features of abdominal and pelvic sarcomas and abdominal wall sarcomas can be nonspecific and overlap with more common pathologic conditions, making diagnosis difficult or, in some cases, delaying diagnosis. Liposarcoma (well-differentiated and dedifferentiated liposarcomas), leiomyosarcoma, and gastrointestinal stromal tumor (GIST) are the most common intra-abdominal primary sarcomas. Any soft-tissue sarcoma can arise in the abdominal wall. Knowledge of the classification and pathologic features of soft-tissue sarcomas, the anatomic locations where they occur, and their cross-sectional imaging features helps the radiologist establish the diagnosis or differential diagnosis so that patients with soft-tissue sarcomas can receive optimal treatment and management. In part 1 of this article, the most common soft-tissue sarcomas (liposarcoma, leiomyosarcoma, and GIST) are reviewed, with a discussion on anatomic locations, classification, clinical considerations, and differential diagnosis. Part 2 will focus on the remainder of the soft-tissue sarcomas occurring in the abdomen and pelvis.

References

  • 1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon, France: IARC, 2013; 10–11.
  • 2. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2016. CA Cancer J Clin 2016;66(1):7–30.
  • 3. Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin 2004;54(2):94–109.
  • 4. Baheti AD, O’Malley RB, Kim S, et al. Soft-tissue sarcomas: an update for radiologists based on the revised 2013 World Health Organization classification. AJR Am J Roentgenol 2016;206(5):924–932.
  • 5. Gerota D. Beitrage zur kenntnis des befestigungsapparates der niere. Arch Anat Entwicklungsgesch 1895;19:265–286.
  • 6. Zuckerkandl E. Beitrage zur anatomie des menschlichen korpers. I. Ueber den fixationsapparat der nieren. Med Jahr 1883;13(2):59–67.
  • 7. Raptopoulos V, Kleinman PK, Marks S Jr, Snyder M, Silverman PM. Renal fascial pathway: posterior extension of pancreatic effusions within the anterior pararenal space. Radiology 1986;158(2):367–374.
  • 8. Mindell HJ, Mastromatteo JF, Dickey KW, et al. Anatomic communications between the three retroperitoneal spaces: determination by CT-guided injections of contrast material in cadavers. AJR Am J Roentgenol 1995;164(5):1173–1178.
  • 9. Raptopoulos V, Touliopoulos P, Lei QF, Vrachliotis TG, Marks SC Jr. Medial border of the perirenal space: CT and anatomic correlation. Radiology 1997;205(3):777–784.
  • 10. Raptopoulos V, Lei QF, Touliopoulos P, Vrachliotis TG, Marks SC Jr. Why perirenal disease does not extend into the pelvis: the importance of closure of the cone of the renal fasciae. AJR Am J Roentgenol 1995;164(5):1179–1184.
  • 11. Coindre JM. Grading of soft tissue sarcomas: review and update. Arch Pathol Lab Med 2006;130(10):1448–1453.
  • 12. Edge SB, Byrd DB, Compton CC, Fritz AG, Greene FL, Trotti A, eds. AJCC cancer staging manual. 7th ed. New York, NY: Springer, 2010.
  • 13. Pisters PW. Clinical evaluation and treatment of soft tissue tumors. In: Goldblum JR, Folpe AL, Weiss SW, eds. Enzinger and Weiss’s soft tissue tumors. 6th ed. Philadelphia, Pa: Saunders/Elsevier, 2013; 11–24.
  • 14. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 1980;153:106–120.
  • 15. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma: 1980. Clin Orthop Relat Res 2003;415:4–18.
  • 16. Goldblum JR, Folpe AL, Weiss SW. Liposarcoma. In: Goldblum JR, Folpe AL, Weiss SW, eds. Enzinger and Weiss’s soft tissue tumors. 6th ed. Philadelphia, Pa: Saunders/Elsevier, 2013; 484–523.
  • 17. Enzinger FM, Winslow DJ. Liposarcoma: a study of 103 cases. Virchows Arch Pathol Anat Physiol Klin Med 1962;335:367–388.
  • 18. Dei Tos AP, Pedeutour F. Atypical lipomatous tumor. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon, France: IARC, 2013; 33–36.
  • 19. Weiss SW. Lipomatous tumors. Monogr Pathol 1996;38:207–239.
  • 20. Lucas DR, Nascimento AG, Sanjay BK, Rock MG. Well-differentiated liposarcoma: the Mayo Clinic experience with 58 cases. Am J Clin Pathol 1994;102(5):677–683.
  • 21. Dei Tos AP. Liposarcoma: new entities and evolving concepts. Ann Diagn Pathol 2000;4(4):252–266.
  • 22. Henricks WH, Chu YC, Goldblum JR, Weiss SW. Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol 1997;21(3):271–281.
  • 23. Huang HY, Brennan MF, Singer S, Antonescu CR. Distant metastasis in retroperitoneal dedifferentiated liposarcoma is rare and rapidly fatal: a clinicopathological study with emphasis on the low-grade myxofibrosarcoma-like pattern as an early sign of dedifferentiation. Mod Pathol 2005;18(7):976–984.
  • 24. Fiore M, Grosso F, Lo Vullo S, et al. Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution. Cancer 2007;109(12):2522–2531.
  • 25. Kim ES, Jang SH, Park HC, Jung EH, Moon GB. Dedifferentiated liposarcoma of the retroperitoneum. Cancer Res Treat 2010;42(1):57–60.
  • 26. Kilpatrick SE, Doyon J, Choong PF, Sim FH, Nascimento AG. The clinicopathologic spectrum of myxoid and round cell liposarcoma: a study of 95 cases. Cancer 1996;77(8):1450–1458.
  • 27. Azumi N, Curtis J, Kempson RL, Hendrickson MR. Atypical and malignant neoplasms showing lipomatous differentiation: a study of 111 cases. Am J Surg Pathol 1987;11(3):161–183.
  • 28. Gebhard S, Coindre JM, Michels JJ, et al. Pleomorphic liposarcoma: clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases—a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol 2002;26(5):601–616.
  • 29. Song T, Shen J, Liang BL, Mai WW, Li Y, Guo HC. Retroperitoneal liposarcoma: MR characteristics and pathological correlative analysis. Abdom Imaging 2007;32(5):668–674.
  • 30. Murphey MD, Arcara LK, Fanburg-Smith J. Imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation. RadioGraphics 2005;25(5):1371–1395.
  • 31. Kim T, Murakami T, Oi H, et al. CT and MR imaging of abdominal liposarcoma. AJR Am J Roentgenol 1996;166(4):829–833.
  • 32. Bestic JM, Kransdorf MJ, White LM, et al. Sclerosing variant of well-differentiated liposarcoma: relative prevalence and spectrum of CT and MRI features. AJR Am J Roentgenol 2013;201(1):154–161.
  • 33. Tateishi U, Hasegawa T, Beppu Y, Satake M, Moriyama N. Primary dedifferentiated liposarcoma of the retroperitoneum: prognostic significance of computed tomography and magnetic resonance imaging features. J Comput Assist Tomogr 2003;27(5):799–804.
  • 34. Tallini G, Erlandson RA, Brennan MF, Woodruff JM. Divergent myosarcomatous differentiation in retroperitoneal liposarcoma. Am J Surg Pathol 1993;17(6):546–556.
  • 35. Kim EY, Kim SJ, Choi D, et al. Recurrence of retroperitoneal liposarcoma: imaging findings and growth rates at follow-up CT. AJR Am J Roentgenol 2008;191(6):1841–1846.
  • 36. Ghadimi MP, Al-Zaid T, Madewell J, et al. Diagnosis, management, and outcome of patients with dedifferentiated liposarcoma systemic metastasis. Ann Surg Oncol 2011;18(13):3762–3770.
  • 37. Lazar A, Evans HL, Shipley J. Leiomyosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon, France: IARC, 2013; 111–113.
  • 38. Hartman DS, Hayes WS, Choyke PL, Tibbetts GP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation. RadioGraphics 1992;12(6):1203–1220.
  • 39. Rajani B, Smith TA, Reith JD, Goldblum JR. Retroperitoneal leiomyosarcomas unassociated with the gastrointestinal tract: a clinicopathologic analysis of 17 cases. Mod Pathol 1999;12(1):21–28.
  • 40. Kempson RL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley RK. Atlas of tumor pathology: tumors of the soft tissues. Washington, DC: Armed Forces Institute of Pathology, 2001.
  • 41. Lane RH, Stephens DH, Reiman HM. Primary retroperitoneal neoplasms: CT findings in 90 cases with clinical and pathologic correlation. AJR Am J Roentgenol 1989;152(1):83–89.
  • 42. Cooley CL, Jagannathan JP, Kurra V, et al. Imaging features and metastatic pattern of non-IVC retroperitoneal leiomyosarcomas: are they different from IVC leiomyosarcomas? J Comput Assist Tomogr 2014;38(5):687–692.
  • 43. O’Sullivan PJ, Harris AC, Munk PL. Radiological imaging features of non-uterine leiomyosarcoma. Br J Radiol 2008;81(961):73–81.
  • 44. Bharti JN, Dey B, Desai P, Gupta R, Khurana N, Gandhi G. Primary leiomyosarcoma of peritoneal cavity. Rare Tumors 2014;6(1):5165. doi:10.4081/rt.2014.5165. Published online March 26, 2014.
  • 45. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol 1998;152(5):1259–1269.
  • 46. Ricci R. Syndromic gastrointestinal stromal tumors. Hered Cancer Clin Pract 2016;14:15. doi:10.1186/s13053-016-0055-4. Published online July 19, 2016.
  • 47. Stratakis CA, Carney JA. The triad of paragangliomas, gastric stromal tumours and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal sarcomas (Carney-Stratakis syndrome): molecular genetics and clinical implications. J Intern Med 2009;266(1):43–52.
  • 48. Miettinen M, Wang ZF, Sarlomo-Rikala M, Osuch C, Rutkowski P, Lasota J. Succinate dehydrogenase-deficient GISTs: a clinicopathologic, immunohistochemical, and molecular genetic study of 66 gastric GISTs with predilection to young age. Am J Surg Pathol 2011;35(11):1712–1721.
  • 49. Miettinen M, Lasota J. Gastrointestinal stromal tumors: definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438(1):1–12.
  • 50. Levy AD, Remotti HE, Thompson WM, Sobin LH, Miettinen M. Gastrointestinal stromal tumors: radiologic features with pathologic correlation. RadioGraphics 2003;23(2):283–304.
  • 51. Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol 1999;30(10):1213–1220.
  • 52. Miettinen M, Lasota J. Histopathology of gastrointestinal stromal tumor. J Surg Oncol 2011;104(8):865–873.
  • 53. Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med 2006;130(10): 1466–1478.
  • 54. Miettinen M, Makhlouf H, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up. Am J Surg Pathol 2006;30(4):477–489.
  • 55. Miettinen M, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up. Am J Surg Pathol 2005;29(1):52–68.
  • 56. Burkill GJ, Badran M, Al-Muderis O, et al. Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread. Radiology 2003;226(2):527–532.
  • 57. Rana R, Nikolaidis P, Miller F. Calcified gastrointestinal stromal tumor of the sigmoid colon mimicking inspissated barium on CT. AJR Am J Roentgenol 2006;187(3):W322–W323.
  • 58. Kim HC, Lee JM, Kim SH, et al. Primary gastrointestinal stromal tumors in the omentum and mesentery: CT findings and pathologic correlations. AJR Am J Roentgenol 2004;182(6):1463–1467
  • 59. Tirumani SH, Tirumani H, Jagannathan JP, et al. MDCT features of succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumours. Br J Radiol 2014;87(1043):20140476. http://dx.doi.org/10.1259/bjr.20140476. Published online October 13, 2014.
  • 60. Israel GM, Bosniak MA, Slywotzky CM, Rosen RJ. CT differentiation of large exophytic renal angiomyolipomas and perirenal liposarcomas. AJR Am J Roentgenol 2002;179(3):769–773.
  • 61. Craig WD, Fanburg-Smith JC, Henry LR, Guerrero R, Barton JH. Fat-containing lesions of the retroperitoneum: radiologic-pathologic correlation. RadioGraphics 2009;29(1):261–290.
  • 62. Kamaya A, Federle MP, Desser TS. Imaging manifestations of abdominal fat necrosis and its mimics. RadioGraphics 2011;31(7):2021–2034.
  • 63. Aufforth RD, Baker JJ, Kim HJ. Soft tissue sarcoma. In: Chu QD, Gibbs JF, Zibari GB, eds. Surgical oncology: a practical and comprehensive approach. New York, NY: Springer, 2015; 605–626.

Article History

Received: June 14 2016
Revision requested: Aug 8 2016
Revision received: Sept 28 2016
Accepted: Oct 11 2016
Published online: Mar 13 2017
Published in print: Mar 2017