A unique dedifferentiated tumor of the retroperitoneum

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Case report

A unique dedifferentiated tumor of the retroperitoneum Shahzeer Karmali1, Halligrimur Benediktson2, Walley Temple3 and Oliver F Bathe*3 Address: 1Department of Surgery, University of Calgary, Calgary, AB, Canada, 2Department of Pathology, University of Calgary, Calgary, AB, Canada and 3Departments of Surgery and Oncology, University of Calgary, Calgary, AB, Canada Email: Shahzeer Karmali - [email protected]; Halligrimur Benediktson - [email protected]; Walley Temple - [email protected]; Oliver F Bathe* - [email protected] * Corresponding author

Published: 23 July 2004 World Journal of Surgical Oncology 2004, 2:25

doi:10.1186/1477-7819-2-25

Received: 30 April 2004 Accepted: 23 July 2004

This article is available from: http://www.wjso.com/content/2/1/25 © 2004 Karmali et al; licensee BioMed Central Ltd. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract Background: Dedifferentiated liposarcomas represent heterogeneous tumors with lipomatous and nonlipomatous elements starkly juxtaposed. It is thought that the high grade nonlipomatous elements of the tumor portend a worse prognosis. Case Presentation: A 19.8 kg heterogeneous retroperitoneal tumor was successfully and completely resected. Because of its extent, no additional treatment modalities were practicable. The tumor soon recurred. The recurrent tumor differed from the primary tumor in that it was more homogeneous, consisting mainly of nonlipogenic, calcific tissue. Conclusions: Dedifferentiated liposarcomas are known to have a very high recurrence rate. The biological behavior of dedifferentiated liposarcomas is likely dictated by the most aggressive element of these heterogeneous tumors.

Background Sarcomas arising from the retroperitoneum are rare tumors, accounting for 10–15% of all soft tissue sarcomas [1]. Liposarcoma is the single most common soft tissue sarcoma and accounts for at least 20% of all sarcomas in adults [1]. Classification of liposarcoma into four types, based on morphologic features and cytogenic aberrations, is now widely accepted [2]. These four types are (a) well differentiated; (b) dedifferentiated; (c) myxoid/round cell and (d) pleomorphic. The extent of differentiation, as reflected by histological grade, remains the most important determinant of clinical course and of ultimate prognosis for patients with liposarcoma after resection. The following case illustrates the great morphological and biological heterogeneity of these tumors. A very rapid recurrence was observed, and this recurrence was considerably

less heterogeneous than the primary tumor, consisting mainly of the calcific, nonlipomatous component.

Case presentation A 65-year-old male presented with a three-week history of progressively worsening abdominal distension. He denied any abdominal pain but s