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©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Mar 15, 2017; 9(3): 135-141
Published online Mar 15, 2017. doi: 10.4251/wjgo.v9.i3.135
Heavily calcified gastrointestinal stromal tumors: Pathophysiology and implications of a rare clinicopathologic entity
Massimiliano Salati, Giulia Orsi, Luca Reggiani Bonetti, Fabrizio Di Benedetto, Giuseppe Longo, Stefano Cascinu
Massimiliano Salati, Giulia Orsi, Stefano Cascinu, Department of Medical and Surgical Sciences for Children and Adults, Division of Medical Oncology, University Hospital of Modena and Reggio Emilia, 41124 Modena, Italy
Luca Reggiani Bonetti, Department of Diagnostic Medicine and Public Health, Division of Pathology, University Hospital of Modena and Reggio Emilia, 41124 Modena, Italy
Fabrizio Di Benedetto, Hepato-Pancreato-Biliary Surgery and Liver Transplantation Unit, University Hospital of Modena and Reggio Emilia, 41124 Modena, Italy
Giuseppe Longo, Azienda Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy
Author contributions: Reggiani Bonetti L reported the pathological findings; Di Benedetto F provided the surgical care; Salati M, Orsi G, Longo G and Cascinu S performed the research, analyzed the literature data and wrote the paper; all authors read and approved the final manuscript.
Institutional review board statement: This case report was exempt from the Institutional Review Board standards of the University Hospital of Modena and Reggio Emilia.
Informed consent statement: The patient involved in this study gave written informed consent authorizing use and disclosure of protected health information.
Conflict-of-interest statement: None of the authors have any conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Massimiliano Salati, MD, Department of Medical and Surgical Sciences for Children and Adults, Division of Medical Oncology, University Hospital of Modena and Reggio Emilia, via del Pozzo, 41124 Modena, Italy. maxsalati@live.it
Telephone: +39-33-94624778 Fax: +39-05-84222647
Received: September 5, 2016
Peer-review started: September 7, 2016
First decision: September 29, 2016
Revised: October 19, 2016
Accepted: December 13, 2016
Article in press: December 15, 2016
Published online: March 15, 2017
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract, and are characterized by a broad spectrum of clinical, histological and molecular features at presentation. Although focal and scattered calcifications are not uncommon within the primary tumor mass, heavy calcification within a GIST is rarely described in the literature and the clinical-biological meaning of this feature remains unclear. Cases with such an atypical presentation are challenging and may be associated with diagnostic pitfalls. Herein, we report a gastric GIST with the unusual presentation of prominent calcifications that was identified incidentally on imaging during a post-trauma diagnostic work-up. The patient underwent laparoscopic surgery with a radical resection of the mass, which was subsequently characterized by histological analysis as spindle-shaped tumor cells, positive for CD117/c-KIT, CD34 and DOG1, and with calcified areas. Given the intermediate risk of recurrence, no adjuvant therapy was recommended and the patient underwent regular follow-up for 22 mo, with no evidence of relapse. Our case can be considered of interest because of the rarity of clinical presentation and the uniquely large size of the GIST at diagnosis (longest diameter exceeding 9 cm). In closing, we discuss the pathophysiology and clinical implications of calcifications in GISTs by reviewing the most up-to-date relevant literature.
Core tip: Gastrointestinal stromal tumors (GISTs) are heterogeneous neoplasms that may present with a wide range of clinicopathologic characteristics upon diagnosis; among these, massive calcification is an infrequently occurring feature of GIST presentation. By reporting on such a rare clinical case and reviewing the available literature, this manuscript discusses major diagnostic challenges as well as the pathophysiologic and clinical implications related to a heavily calcified mass diagnosed as GIST.
