Retroperitoneal Leiomyosarcoma

  • Fuzhen Chen
  • Wengang Li


Leiomyosarcoma is a soft tissue sarcoma arising from the uterus, gastrointestinal tract, and soft tissue smooth muscle cells, accounting for 5–10% of all soft tissue sarcomas. It is the third most common soft tissue sarcomas following liposarcoma and malignant fibrous histiocytoma. Nowadays, leiomyosarcoma is generally classified into three categories: (a) leiomyosarcoma in the retroperitoneum or peritoneal cavity, which is the most common type, of which 1/2 to 2/3 occurs in the retroperitoneum; (b) skin and subcutaneous leiomyosarcoma, with the best prognosis among all the three; and (c) prototype vascular smooth muscle sarcoma, which is the rarest type. Primary retroperitoneal leiomyosarcoma, accounting for 11% of all malignant retroperitoneal tumors, can occur at any age, is mostly seen in people aged 40–70 years, and is more prevalent in women than in men (ratio of 2:1). Most of the tumors grow invasively and are difficult to treat, with dismal prognosis and the lowest survival rate among all soft tissue sarcomas.


  1. De Beuckeleer LHL, Vanhoenacker FM. Fibrohistiocytic tumors. In: Kransdorf MJ, Murphy MD, editors. Imaging of soft tissue tumors. 3rd ed. Philadelphia: WB Saunders; 1997. p. 221–6.Google Scholar
  2. Felix EL, Word DK, Gupta TKD. Tumors of the retroperitoneum. Curr Probl Cancer. 1981;6(1):1–47.CrossRefGoogle Scholar
  3. Gustafson P, Willén H, Baldetorp B, et al. Soft tissue leiomyosarcoma: a population-based epidemiologic and prognostic study of 48 patients, including cellular DNA center. Cancer. 1992;70(1):114–9.CrossRefGoogle Scholar
  4. Hartman DS, Hayes WS, Choyke PL, et al. Leiomyosarcoma of the retroperitoneum and inferior vena cava; radiologic-pathologic correlation. Radiographics. 1992;12(8):1203–20.CrossRefGoogle Scholar
  5. Mankin HJ, Casas-Ganem J, Kim JI, et al. Leiomyosarcoma of somatic soft tissues. Clin Orthop Relat Res. 2004;421:225–31.CrossRefGoogle Scholar
  6. Paal E, Miettinen M. Retroperitoneal leiomyomas a clinicopathologic and immunohistochemical study of 56 cases with a comparison to retroperitoneal leiomyosarcomas. Am J Surg Pathol. 2001;25(11):1355–63.CrossRefGoogle Scholar
  7. Shvarts O, Han KP, Lain JS. Belldegrun AS. Primary leiomyosarcoma of the inferior vena cava presenting as a renal mass. Rev Urol. 2004;6(1):39–42.PubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2018

Authors and Affiliations

  1. 1.Chenggong Hospital Affiliated to Medical College of Xiamen UniversityXiamenChina

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