Abstract
Purpose. This review summarizes the more prevalent soft tissue tumours arising in the retroperitoneum and highlights some recent fundamental and diagnostic developments relevant to mesenchymal tumours.
Discussion. The retroperitoneum is an underestimated site for benign and malignant neoplastic disease, and represents the second most common site of origin of primary malignant soft tissue tumours (sarcomas) after the deep tissues of the lower extremity. In contrast to the predominance of benign soft tissue lesions over malignant sarcomas elsewhere, retroperitoneal mesenchymal lesions are far more likely to be malignant. The differential diagnosis is primarily with the more common lymphoproliferative and parenchymatous epithelial lesions arising in this area, and with metastatic disease from known or unknown primary sites elsewhere.The most prevalent mesenchymal tumours at this site are of a lipomatous, myogenic or neural nature.Their generally late clinical presentation and poorly accessible location provides numerous clinical challenges; optimal radiological imaging and a properly performed biopsy are essential cogs in the management route. Histopathological diagnosis may be complicated, but has been aided by developments in the fields of immunohistochemistry and tumour (cyto)genetics. Despite significant advances in oncological management protocols, the prognosis remains generally less favourable than for similar tumours at more accessible sites.
Full Text
The Full Text of this article is available as a PDF (149.8 KB).
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Abe S., Imamura T., Park P., Nakano H., Okita H., Hata J., Tateishi A. Small round-cell type of malignant peripheral nerve sheath tumor. Mod Pathol. 1998 Aug;11(8):747–753. [PubMed] [Google Scholar]
- Bane B. L., Evans H. L., Ro J. Y., Carrasco C. H., Grignon D. J., Benjamin R. S., Ayala A. G. Extraskeletal osteosarcoma. A clinicopathologic review of 26 cases. Cancer. 1990 Jun 15;65(12):2762–2770. doi: 10.1002/1097-0142(19900615)65:12<2762::aid-cncr2820651226>3.0.co;2-k. [DOI] [PubMed] [Google Scholar]
- Barr F. G., Galili N., Holick J., Biegel J. A., Rovera G., Emanuel B. S. Rearrangement of the PAX3 paired box gene in the paediatric solid tumour alveolar rhabdomyosarcoma. Nat Genet. 1993 Feb;3(2):113–117. doi: 10.1038/ng0293-113. [DOI] [PubMed] [Google Scholar]
- Beckwith J. B., Kiviat N. B., Bonadio J. F. Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms' tumor. Pediatr Pathol. 1990;10(1-2):1–36. doi: 10.3109/15513819009067094. [DOI] [PubMed] [Google Scholar]
- Brooks J. S., Freeman M., Enterline H. T. Malignant "Triton" tumors. Natural history and immunohistochemistry of nine new cases with literature review. Cancer. 1985 Jun 1;55(11):2543–2549. doi: 10.1002/1097-0142(19850601)55:11<2543::aid-cncr2820551105>3.0.co;2-4. [DOI] [PubMed] [Google Scholar]
- Casadei G. P., Scheithauer B. W., Hirose T., Manfrini M., Van Houton C., Wood M. B. Cellular schwannoma. A clinicopathologic, DNA flow cytometric, and proliferation marker study of 70 patients. Cancer. 1995 Mar 1;75(5):1109–1119. doi: 10.1002/1097-0142(19950301)75:5<1109::aid-cncr2820750510>3.0.co;2-m. [DOI] [PubMed] [Google Scholar]
- Chan J. K. Mesenchymal tumors of the gastrointestinal tract: a paradise for acronyms (STUMP, GIST, GANT, and now GIPACT), implication of c-kit in genesis, and yet another of the many emerging roles of the interstitial cell of Cajal in the pathogenesis of gastrointestinal diseases? Adv Anat Pathol. 1999 Jan;6(1):19–40. doi: 10.1097/00125480-199901000-00003. [DOI] [PubMed] [Google Scholar]
- Coindre J. M., Trojani M., Contesso G., David M., Rouesse J., Bui N. B., Bodaert A., De Mascarel I., De Mascarel A., Goussot J. F. Reproducibility of a histopathologic grading system for adult soft tissue sarcoma. Cancer. 1986 Jul 15;58(2):306–309. doi: 10.1002/1097-0142(19860715)58:2<306::aid-cncr2820580216>3.0.co;2-7. [DOI] [PubMed] [Google Scholar]
- Costa J., Wesley R. A., Glatstein E., Rosenberg S. A. The grading of soft tissue sarcomas. Results of a clinicohistopathologic correlation in a series of 163 cases. Cancer. 1984 Feb 1;53(3):530–541. doi: 10.1002/1097-0142(19840201)53:3<530::aid-cncr2820530327>3.0.co;2-d. [DOI] [PubMed] [Google Scholar]
- Crist W. M., Kun L. E. Common solid tumors of childhood. N Engl J Med. 1991 Feb 14;324(7):461–471. doi: 10.1056/NEJM199102143240706. [DOI] [PubMed] [Google Scholar]
- Davis R. J., D'Cruz C. M., Lovell M. A., Biegel J. A., Barr F. G. Fusion of PAX7 to FKHR by the variant t(1;13)(p36;q14) translocation in alveolar rhabdomyosarcoma. Cancer Res. 1994 Jun 1;54(11):2869–2872. [PubMed] [Google Scholar]
- Ducatman B. S., Scheithauer B. W., Piepgras D. G., Reiman H. M., Ilstrup D. M. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986 May 15;57(10):2006–2021. doi: 10.1002/1097-0142(19860515)57:10<2006::aid-cncr2820571022>3.0.co;2-6. [DOI] [PubMed] [Google Scholar]
- Elgar F., Goldblum J. R. Well-differentiated liposarcoma of the retroperitoneum: a clinicopathologic analysis of 20 cases, with particular attention to the extent of low-grade dedifferentiation. Mod Pathol. 1997 Feb;10(2):113–120. [PubMed] [Google Scholar]
- Enneking W. F. Musculoskeletal tumor staging: 1988 update. Cancer Treat Res. 1989;44:39–49. doi: 10.1007/978-1-4613-1757-9_3. [DOI] [PubMed] [Google Scholar]
- Enneking W. F., Spanier S. S., Goodman M. A. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res. 1980 Nov-Dec;(153):106–120. [PubMed] [Google Scholar]
- Erlandson R. A., Klimstra D. S., Woodruff J. M. Subclassification of gastrointestinal stromal tumors based on evaluation by electron microscopy and immunohistochemistry. Ultrastruct Pathol. 1996 Jul-Aug;20(4):373–393. doi: 10.3109/01913129609016340. [DOI] [PubMed] [Google Scholar]
- Fletcher C. D., Akerman M., Dal Cin P., de Wever I., Mandahl N., Mertens F., Mitelman F., Rosai J., Rydholm A., Sciot R. Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group. Am J Pathol. 1996 Feb;148(2):623–630. [PMC free article] [PubMed] [Google Scholar]
- Fletcher C. D. Pleomorphic malignant fibrous histiocytoma: fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma. Am J Surg Pathol. 1992 Mar;16(3):213–228. [PubMed] [Google Scholar]
- Gerald W. L., Rosai J., Ladanyi M. Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor. Proc Natl Acad Sci U S A. 1995 Feb 14;92(4):1028–1032. doi: 10.1073/pnas.92.4.1028. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Graadt van Roggen J. F., Bovée J. V., Morreau J., Hogendoorn P. C. Diagnostic and prognostic implications of the unfolding molecular biology of bone and soft tissue tumours. J Clin Pathol. 1999 Jul;52(7):481–489. doi: 10.1136/jcp.52.7.481. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Graadt van Roggen J. F., Welvaart K., de Roos A., Offerhaus G. J., Hogendoorn P. C. Adenocarcinoma arising within a tailgut cyst: clinicopathological description and follow up of an unusual case. J Clin Pathol. 1999 Apr;52(4):310–312. doi: 10.1136/jcp.52.4.310. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Gururangan S., Bowman L. C., Parham D. M., Wilimas J. A., Rao B., Pratt C. B., Douglass E. C. Primary extracranial rhabdoid tumors. Clinicopathologic features and response to ifosfamide. Cancer. 1993 Apr 15;71(8):2653–2659. doi: 10.1002/1097-0142(19930415)71:8<2653::aid-cncr2820710834>3.0.co;2-#. [DOI] [PubMed] [Google Scholar]
- Haggitt R. C., Reid B. J. Hereditary gastrointestinal polyposis syndromes. Am J Surg Pathol. 1986 Dec;10(12):871–887. doi: 10.1097/00000478-198612000-00006. [DOI] [PubMed] [Google Scholar]
- Hashimoto H., Enjoji M. Liposarcoma. A clinicopathologic subtyping of 52 cases. Acta Pathol Jpn. 1982 Nov;32(6):933–948. [PubMed] [Google Scholar]
- Henricks W. H., Chu Y. C., Goldblum J. R., Weiss S. W. Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol. 1997 Mar;21(3):271–281. doi: 10.1097/00000478-199703000-00002. [DOI] [PubMed] [Google Scholar]
- Hirota S., Isozaki K., Moriyama Y., Hashimoto K., Nishida T., Ishiguro S., Kawano K., Hanada M., Kurata A., Takeda M. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998 Jan 23;279(5350):577–580. doi: 10.1126/science.279.5350.577. [DOI] [PubMed] [Google Scholar]
- Huang C. J. Rhabdomyosarcoma involving the genitourinary organs, retroperitoneum, and pelvis. J Pediatr Surg. 1986 Feb;21(2):101–107. doi: 10.1016/s0022-3468(86)80058-6. [DOI] [PubMed] [Google Scholar]
- Jones J. H., Ross E. J., Matz L. R., Edwards D., Davies D. R. Retroperitoneal fibrosis. Am J Med. 1970 Feb;48(2):203–208. doi: 10.1016/0002-9343(70)90116-6. [DOI] [PubMed] [Google Scholar]
- Kelly K. M., Womer R. B., Sorensen P. H., Xiong Q. B., Barr F. G. Common and variant gene fusions predict distinct clinical phenotypes in rhabdomyosarcoma. J Clin Oncol. 1997 May;15(5):1831–1836. doi: 10.1200/JCO.1997.15.5.1831. [DOI] [PubMed] [Google Scholar]
- Kilpatrick S. E., Doyon J., Choong P. F., Sim F. H., Nascimento A. G. The clinicopathologic spectrum of myxoid and round cell liposarcoma. A study of 95 cases. Cancer. 1996 Apr 15;77(8):1450–1458. doi: 10.1002/(SICI)1097-0142(19960415)77:8<1450::AID-CNCR5>3.0.CO;2-G. [DOI] [PubMed] [Google Scholar]
- Kodet R., Newton W. A., Jr, Hamoudi A. B., Asmar L., Jacobs D. L., Maurer H. M. Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study. Am J Surg Pathol. 1993 May;17(5):443–453. doi: 10.1097/00000478-199305000-00002. [DOI] [PubMed] [Google Scholar]
- Kransdorf M. J. Benign soft-tissue tumors in a large referral population: distribution of specific diagnoses by age, sex, and location. AJR Am J Roentgenol. 1995 Feb;164(2):395–402. doi: 10.2214/ajr.164.2.7839977. [DOI] [PubMed] [Google Scholar]
- Kubben F. J., Kroon F. P., Hogendoorn P. C., Shaw P. C., Veenendaal R. A., de Roos A., Lamers C. B. Absence of Epstein-Barr virus (EBV) in a gastrointestinal stromal cell tumour (GIST) in an adult human immunodeficiency virus-seropositive patient with past EBV infection. Eur J Gastroenterol Hepatol. 1997 Jul;9(7):721–724. doi: 10.1097/00042737-199707000-00014. [DOI] [PubMed] [Google Scholar]
- Lack E. E., Cubilla A. L., Woodruff J. M., Lieberman P. H. Extra-adrenal paragangliomas of the retroperitoneum: A clinicopathologic study of 12 tumors. Am J Surg Pathol. 1980 Apr;4(2):109–120. doi: 10.1097/00000478-198004000-00002. [DOI] [PubMed] [Google Scholar]
- Lack E. E., Travis W. D., Welch K. J. Retroperitoneal germ cell tumors in childhood. A clinical and pathologic study of 11 cases. Cancer. 1985 Aug 1;56(3):602–608. doi: 10.1002/1097-0142(19850801)56:3<602::aid-cncr2820560329>3.0.co;2-d. [DOI] [PubMed] [Google Scholar]
- Lodding P., Kindblom L. G., Angervall L., Stenman G. Cellular schwannoma. A clinicopathologic study of 29 cases. Virchows Arch A Pathol Anat Histopathol. 1990;416(3):237–248. doi: 10.1007/BF01678983. [DOI] [PubMed] [Google Scholar]
- Lucas D. R., Nascimento A. G., Sanjay B. K., Rock M. G. Well-differentiated liposarcoma. The Mayo Clinic experience with 58 cases. Am J Clin Pathol. 1994 Nov;102(5):677–683. doi: 10.1093/ajcp/102.5.677. [DOI] [PubMed] [Google Scholar]
- Lucas J. D., O'Doherty M. J., Cronin B. F., Marsden P. K., Lodge M. A., McKee P. H., Smith M. A. Prospective evaluation of soft tissue masses and sarcomas using fluorodeoxyglucose positron emission tomography. Br J Surg. 1999 Apr;86(4):550–556. doi: 10.1046/j.1365-2168.1999.01090.x. [DOI] [PubMed] [Google Scholar]
- Lucas J. D., O'Doherty M. J., Wong J. C., Bingham J. B., McKee P. H., Fletcher C. D., Smith M. A. Evaluation of fluorodeoxyglucose positron emission tomography in the management of soft-tissue sarcomas. J Bone Joint Surg Br. 1998 May;80(3):441–447. doi: 10.1302/0301-620x.80b3.8232. [DOI] [PubMed] [Google Scholar]
- Lüttges J., Mentzel T., Hübner G., Klöppel G. Solitary fibrous tumour of the pancreas: a new member of the small group of mesenchymal pancreatic tumours. Virchows Arch. 1999 Jul;435(1):37–42. doi: 10.1007/s004280050392. [DOI] [PubMed] [Google Scholar]
- Meis J. M., Enzinger F. M. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor. Am J Surg Pathol. 1991 Dec;15(12):1146–1156. doi: 10.1097/00000478-199112000-00005. [DOI] [PubMed] [Google Scholar]
- Meis J. M., Enzinger F. M. Myolipoma of soft tissue. Am J Surg Pathol. 1991 Feb;15(2):121–125. doi: 10.1097/00000478-199102000-00003. [DOI] [PubMed] [Google Scholar]
- Mentzel T., Fletcher C. D. Dedifferentiated myxoid liposarcoma: a clinicopathological study suggesting a closer relationship between myxoid and well-differentiated liposarcoma. Histopathology. 1997 May;30(5):457–463. doi: 10.1046/j.1365-2559.1997.5510791.x. [DOI] [PubMed] [Google Scholar]
- Mentzel T., Fletcher C. D. Lipomatous tumours of soft tissues: an update. Virchows Arch. 1995;427(4):353–363. doi: 10.1007/BF00199383. [DOI] [PubMed] [Google Scholar]
- Moran C. A., Albores-Saavedra J., Wenig B. M., Mena H. Pigmented extraadrenal paragangliomas. A clinicopathologic and immunohistochemical study of five cases. Cancer. 1997 Jan 15;79(2):398–402. doi: 10.1002/(sici)1097-0142(19970115)79:2<398::aid-cncr24>3.0.co;2-v. [DOI] [PubMed] [Google Scholar]
- Nappi O., Ritter J. H., Pettinato G., Wick M. R. Hemangiopericytoma: histopathological pattern or clinicopathologic entity? Semin Diagn Pathol. 1995 Aug;12(3):221–232. [PubMed] [Google Scholar]
- Newman P. L., Fletcher C. D. Malignant mesenchymoma. Clinicopathologic analysis of a series with evidence of low-grade behaviour. Am J Surg Pathol. 1991 Jul;15(7):607–614. doi: 10.1097/00000478-199107000-00001. [DOI] [PubMed] [Google Scholar]
- Osborne B. M., Butler J. J., Bloustein P., Sumner G. Idiopathic retroperitoneal fibrosis (sclerosing retroperitonitis). Hum Pathol. 1987 Jul;18(7):735–739. doi: 10.1016/s0046-8177(87)80245-9. [DOI] [PubMed] [Google Scholar]
- Parsons M. A., Harris S. C., Longstaff A. J., Grainger R. G. Xanthogranulomatous pyelonephritis: a pathological, clinical and aetiological analysis of 87 cases. Diagn Histopathol. 1983 Jul-Dec;6(3-4):203–219. [PubMed] [Google Scholar]
- Rajani B., Smith T. A., Reith J. D., Goldblum J. R. Retroperitoneal leiomyosarcomas unassociated with the gastrointestinal tract: a clinicopathologic analysis of 17 cases. Mod Pathol. 1999 Jan;12(1):21–28. [PubMed] [Google Scholar]
- Regan J. F., Juler G. L., Schmutzer K. J. Retroperitoneal neurilemoma. Am J Surg. 1977 Jul;134(1):140–145. doi: 10.1016/0002-9610(77)90297-5. [DOI] [PubMed] [Google Scholar]
- Shimada H., Newton W. A., Jr, Soule E. H., Qualman S. J., Aoyama C., Maurer H. M. Pathologic features of extraosseous Ewing's sarcoma: a report from the Intergroup Rhabdomyosarcoma Study. Hum Pathol. 1988 Apr;19(4):442–453. doi: 10.1016/s0046-8177(88)80495-7. [DOI] [PubMed] [Google Scholar]
- Shmookler B. M. Retroperitoneal synovial sarcoma. A report of four cases. Am J Clin Pathol. 1982 Jun;77(6):686–691. doi: 10.1093/ajcp/77.6.686. [DOI] [PubMed] [Google Scholar]
- Smith T. A., Easley K. A., Goldblum J. R. Myxoid/round cell liposarcoma of the extremities. A clinicopathologic study of 29 cases with particular attention to extent of round cell liposarcoma. Am J Surg Pathol. 1996 Feb;20(2):171–180. doi: 10.1097/00000478-199602000-00005. [DOI] [PubMed] [Google Scholar]
- Sreekantaiah C., Davis J. R., Sandberg A. A. Chromosomal abnormalities in leiomyosarcomas. Am J Pathol. 1993 Jan;142(1):293–305. [PMC free article] [PubMed] [Google Scholar]
- Tomera K. M., Farrow G. M., Lieber M. M. Sarcomatoid renal carcinoma. J Urol. 1983 Oct;130(4):657–659. doi: 10.1016/s0022-5347(17)51388-9. [DOI] [PubMed] [Google Scholar]
- Tsang W. Y., Chan J. K. Kaposi-like infantile hemangioendothelioma. A distinctive vascular neoplasm of the retroperitoneum. Am J Surg Pathol. 1991 Oct;15(10):982–989. doi: 10.1097/00000478-199110000-00009. [DOI] [PubMed] [Google Scholar]
- Vallat-Decouvelaere A. V., Dry S. M., Fletcher C. D. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol. 1998 Dec;22(12):1501–1511. doi: 10.1097/00000478-199812000-00007. [DOI] [PubMed] [Google Scholar]
- Verstraete K. L., Van der Woude H. J., Hogendoorn P. C., De-Deene Y., Kunnen M., Bloem J. L. Dynamic contrast-enhanced MR imaging of musculoskeletal tumors: basic principles and clinical applications. J Magn Reson Imaging. 1996 Mar-Apr;6(2):311–321. doi: 10.1002/jmri.1880060210. [DOI] [PubMed] [Google Scholar]
- Weiss S. W. Lipomatous tumors. Monogr Pathol. 1996;38:207–239. [PubMed] [Google Scholar]
- Weiss S. W. Proliferative fibroblastic lesions. From hyperplasia to neoplasia. Am J Surg Pathol. 1986;10 (Suppl 1):14–25. [PubMed] [Google Scholar]
- Weiss S. W., Rao V. K. Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites. A follow-up study of 92 cases with analysis of the incidence of "dedifferentiation". Am J Surg Pathol. 1992 Nov;16(11):1051–1058. doi: 10.1097/00000478-199211000-00003. [DOI] [PubMed] [Google Scholar]
- Weiss S. W., Rao V. K. Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites. A follow-up study of 92 cases with analysis of the incidence of "dedifferentiation". Am J Surg Pathol. 1992 Nov;16(11):1051–1058. doi: 10.1097/00000478-199211000-00003. [DOI] [PubMed] [Google Scholar]
- White W., Shiu M. H., Rosenblum M. K., Erlandson R. A., Woodruff J. M. Cellular schwannoma. A clinicopathologic study of 57 patients and 58 tumors. Cancer. 1990 Sep 15;66(6):1266–1275. doi: 10.1002/1097-0142(19900915)66:6<1266::aid-cncr2820660628>3.0.co;2-e. [DOI] [PubMed] [Google Scholar]
- Woodruff J. M. The pathology and treatment of peripheral nerve tumors and tumor-like conditions. CA Cancer J Clin. 1993 Sep-Oct;43(5):290–308. doi: 10.3322/canjclin.43.5.290. [DOI] [PubMed] [Google Scholar]
