Retroperitoneal Sarcomas: An Update on the Diagnostic Pathology Approach

Overview

Journal Diagnostics (Basel)

Specialty Radiology

Date 2020 Sep 2

PMID 32867125

Citations 16

Authors

Joon Hyuk Choi

Jae Y Ro

Affiliations

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Abstract

Retroperitoneal sarcomas are a heterogenous group of rare tumors arising in the retroperitoneum. Retroperitoneal sarcomas comprise approximately 10% of all soft tissue sarcomas. Though any soft tissue sarcoma histologic types may arise in the retroperitoneal space, liposarcoma (especially well-differentiated and dedifferentiated types) and leiomyosarcoma do so most commonly. Retroperitoneal sarcomas are diagnostically challenging, owing to their diversity and morphological overlap with other tumors arising in the retroperitoneum. An accurate diagnosis is necessary for correct management and prognostication. Herein, we provide an update on the diagnostic approach to retroperitoneal sarcomas and review their key histologic findings and differential diagnoses.

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References

Ordonez N . Desmoplastic small round cell tumor: I: a histopathologic study of 39 cases with emphasis on unusual histological patterns. Am J Surg Pathol. 1998; 22(11):1303-13. DOI: 10.1097/00000478-199811000-00001. View

Nascimento A, Kurtin P, Guillou L, Fletcher C . Dedifferentiated liposarcoma: a report of nine cases with a peculiar neurallike whorling pattern associated with metaplastic bone formation. Am J Surg Pathol. 1998; 22(8):945-55. DOI: 10.1097/00000478-199808000-00004. View

Rubin B, Fletcher C . Myxoid leiomyosarcoma of soft tissue, an underrecognized variant. Am J Surg Pathol. 2000; 24(7):927-36. DOI: 10.1097/00000478-200007000-00004. View

Hornick J, Bosenberg M, Mentzel T, McMenamin M, Oliveira A, Fletcher C . Pleomorphic liposarcoma: clinicopathologic analysis of 57 cases. Am J Surg Pathol. 2004; 28(10):1257-67. DOI: 10.1097/01.pas.0000135524.73447.4a. View

Cash T, McIlvaine E, Krailo M, Lessnick S, Lawlor E, Laack N . Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group. Pediatr Blood Cancer. 2016; 63(10):1771-9. PMC: 4995129. DOI: 10.1002/pbc.26096. View

Folpe A, Kwiatkowski D . Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2009; 41(1):1-15. DOI: 10.1016/j.humpath.2009.05.011. View

Henricks W, Chu Y, Goldblum J, Weiss S . Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. Am J Surg Pathol. 1997; 21(3):271-81. DOI: 10.1097/00000478-199703000-00002. View

Schaefer I, Fletcher C, Hornick J . Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics. Mod Pathol. 2015; 29(1):4-13. DOI: 10.1038/modpathol.2015.134. View

Fisher C, Folpe A, Hashimoto H, Weiss S . Intra-abdominal synovial sarcoma: a clinicopathological study. Histopathology. 2004; 45(3):245-53. DOI: 10.1111/j.1365-2559.2004.01950.x. View

10.

Pan C, Chung M, Ng K, Liu C, Wang J, Chai C . Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol. 2007; 214(3):387-93. DOI: 10.1002/path.2289. View

11.

Creytens D . Malignant solitary fibrous tumour of the kidney with lymph node and liver metastases: beware of STAT6 expression in dedifferentiated liposarcoma with a solitary fibrous tumour-like morphology. Pathology. 2017; 49(6):671. DOI: 10.1016/j.pathol.2017.06.006. View

12.

Wile A, Evans H, Romsdahl M . Leiomyosarcoma of soft tissue: a clinicopathologic study. Cancer. 1981; 48(4):1022-32. DOI: 10.1002/1097-0142(19810815)48:4<1022::aid-cncr2820480429>3.0.co;2-5. View

13.

Shern J, Yohe M, Khan J . Pediatric Rhabdomyosarcoma. Crit Rev Oncog. 2015; 20(3-4):227-43. PMC: 5486973. DOI: 10.1615/critrevoncog.2015013800. View

14.

Widemann B . Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009; 11(4):322-8. PMC: 6689400. DOI: 10.1007/s11912-009-0045-z. View

15.

Mito J, Mitra D, Doyle L . Radiation-Associated Sarcomas: An Update on Clinical, Histologic, and Molecular Features. Surg Pathol Clin. 2019; 12(1):139-148. DOI: 10.1016/j.path.2018.10.010. View

16.

Samaratunga H, Delahunt B, Srigley J, Berney D, Cheng L, Evans A . Granular necrosis: a distinctive form of cell death in malignant tumours. Pathology. 2020; 52(5):507-514. DOI: 10.1016/j.pathol.2020.06.002. View

17.

Argani P, Zhong M, Reuter V, Fallon J, Epstein J, Netto G . TFE3-Fusion Variant Analysis Defines Specific Clinicopathologic Associations Among Xp11 Translocation Cancers. Am J Surg Pathol. 2016; 40(6):723-37. PMC: 4864095. DOI: 10.1097/PAS.0000000000000631. View

18.

Borden E, Baker L, Bell R, Bramwell V, Demetri G, Eisenberg B . Soft tissue sarcomas of adults: state of the translational science. Clin Cancer Res. 2003; 9(6):1941-56. View

19.

Chen E, OConnell F, Fletcher C . Dedifferentiated leiomyosarcoma: clinicopathological analysis of 18 cases. Histopathology. 2011; 59(6):1135-43. DOI: 10.1111/j.1365-2559.2011.04070.x. View

20.

Setsu N, Miyake M, Wakai S, Nakatani F, Kobayashi E, Chuman H . Primary Retroperitoneal Myxoid Liposarcomas. Am J Surg Pathol. 2016; 40(9):1286-90. PMC: 5029446. DOI: 10.1097/PAS.0000000000000657. View