When is a GIST not a GIST? A case report of synchronous metastatic gastrointestinal stromal tumor and fibromatosis
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Case report
When is a GIST not a GIST? A case report of synchronous metastatic gastrointestinal stromal tumor and fibromatosis Chee Khoon Lee*1, Alison Hadley2, Keshani Desilva3, Gareth Smith4 and David Goldstein1 Address: 1Department of Medical Oncology, Prince of Wales Hospital, NSW, Australia, 2Department of Medical Oncology, St George Hospital, NSW, Australia, 3Department of Anatomical Pathology, Pacific Laboratory Medicine Services, NSW, Australia and 4Department of Surgery, Royal North Shore Hospital, NSW, Australia Email: Chee Khoon Lee* - [email protected]; Alison Hadley - [email protected]; Keshani Desilva - [email protected]; Gareth Smith - [email protected]; David Goldstein - [email protected] * Corresponding author
Published: 21 January 2009 World Journal of Surgical Oncology 2009, 7:8
doi:10.1186/1477-7819-7-8
Received: 17 October 2008 Accepted: 21 January 2009
This article is available from: http://www.wjso.com/content/7/1/8 © 2009 Lee 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: A number of non-malignant diseases that share similar morphological features as gastrointestinal stromal tumor (GIST) have been reported. Co-existence of GIST with these other diseases is rarely recognized or reported. Case presentation: We report a case of a 62 year-old man with long-term stable control of metastatic GIST with systemic therapy, presented with an apparent intra-abdominal progression but not supported by imaging with positron emission tomography. Subsequent resection of the intra-abdominal tumor identified a non-malignant fibroid. Conclusion: Differentiating localized progression of GIST from other diseases has important prognostic and therapeutic implications. The potential for co-existence of non-malignant soft tissue neoplasm should always be considered.
Background The finding of gain-of-function mutation of KIT has revolutionized the treatment of advanced gastrointestinal stromal tumor (GIST). This has subsequently led to development of effective systemic therapy utilizing tyrosine kinase inhibitors (TKI). Imatinib is the prototype TKI that was initially reported to achieve a partial response rate of 53.7% and stable disease rate of 27.9%[1]. With the increasing use of TKI in the treatment of advanced GIST, the pattern of disease evolutions are changing which will ultimately impact on the approach to management.
A number of soft tissue neoplasm share many similarities in the morphological and immunophenotypic profiles with GIST. Aggressive fibromatosis (AF) and keloid type fibromatosis scar tissues are distinct soft tissue tumors. AF is a fibroproliferative disease with a propensity for intraabdominal presentation[2]; it may be locally aggressive but gen
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