Skip to main content
Top
Published in: World Journal of Surgical Oncology 1/2018

Open Access 01-12-2018 | Research

Clinicopathological characteristics and prognosis of primary appendiceal stromal tumors

Authors: Bao Zhang, Guo Liang Zheng, Hai Tao Zhu, Yan Zhao, Zhi Chao Zheng

Published in: World Journal of Surgical Oncology | Issue 1/2018

Login to get access

Abstract

Background

Gastrointestinal stromal tumors (GISTs) account for less than 1% of all gastrointestinal tumors. The biological behaviors of GISTs vary from benign to malignant. GISTs are common in the stomach (55.6%) and small intestine (31.8%), but rarely in the rectum, colon (6%), and other sites (5.5%). Currently, the majority of published reports of primary appendiceal stromal tumors (PASTs) are case reports or case series.

Methods

The PASTs described in this study were identified from a literature review (23 cases) and our center (one case). The relationship between PAST gross types and clinicopathological factors was analyzed and summarized. At the same time, the study also analyzed the related risk factors and survival of PASTs and GISTs.

Results

Twenty-four cases of PASTs were compared with 254 cases of GISTs from our center. The results showed that there was a significant difference between the two groups in tumor size (P < 0.001), histological type (P = 0.013), CD34 expression (P < 0.001), and DOG-1 expression (P < 0.001). Disease-free survival (DFS) analysis of 11 cases of PASTs and 227 cases of GISTs found that a comparison of 3-year and 5-year DFS was not statistically significant (P = 0.894 and P = 0.846, respectively). In the DFS multivariate analysis, tumor mucosal ulceration, tumor size, and NIH risk classification were independent prognostic factors in 3-year and 5-year DFS.

Conclusion

In this study, there was no significance in the survival of patients with appendix and gastric stromal tumors, which we hypothesized to be associated with the low sample size and incomplete follow-up records. Based on this, we conclude that the prognosis of primary appendiceal stromal tumors may be better than gastric tumors, but this needs to be confirmed in further prospective studies.
Literature
1.
go back to reference Sircar K, Hewlett BR, Huizinga JD, Chorneyko K, Berezin I, Riddell RH. Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors. Am J Surg Pathol. 1999;23(4):377.CrossRef Sircar K, Hewlett BR, Huizinga JD, Chorneyko K, Berezin I, Riddell RH. Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors. Am J Surg Pathol. 1999;23(4):377.CrossRef
2.
go back to reference Lim KT, Tan KY. Current research and treatment for gastrointestinal stromal tumors. World J Gastroenterol. 2017;23(27):4856–66.CrossRef Lim KT, Tan KY. Current research and treatment for gastrointestinal stromal tumors. World J Gastroenterol. 2017;23(27):4856–66.CrossRef
3.
go back to reference Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998;152:1259–69.PubMedPubMedCentral Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998;152:1259–69.PubMedPubMedCentral
4.
go back to reference Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol. 1983;7(6):507–19.CrossRef Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol. 1983;7(6):507–19.CrossRef
5.
go back to reference Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. Journal of Clinical Oncology. Proc Am Soc Clin Oncol. 2004;22(18):3813.CrossRef Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. Journal of Clinical Oncology. Proc Am Soc Clin Oncol. 2004;22(18):3813.CrossRef
6.
go back to reference Søreide K, Sandvik OM, Søreide JA, Giljaca V, Jureckova A, Bulusu VR. Global epidemiology of gastrointestinal stromal tumours (GIST): a systematic review of population-based cohort studies. Cancer Epidemiol. 2016;40:39–46.CrossRef Søreide K, Sandvik OM, Søreide JA, Giljaca V, Jureckova A, Bulusu VR. Global epidemiology of gastrointestinal stromal tumours (GIST): a systematic review of population-based cohort studies. Cancer Epidemiol. 2016;40:39–46.CrossRef
7.
go back to reference Misdraji J, Graemecook FM. Miscellaneous conditions of the appendix. Semin Diagn Pathol. 2004;21(2):151–63.CrossRef Misdraji J, Graemecook FM. Miscellaneous conditions of the appendix. Semin Diagn Pathol. 2004;21(2):151–63.CrossRef
8.
go back to reference Vassos N, Agaimy A, Günther K, Hohenberger W, Schneider-Stock R, Croner RS. A novel complex KIT mutation in a gastrointestinal stromal tumor of the vermiform appendix. Hum Pathol. 2013;44(4):651–5.CrossRef Vassos N, Agaimy A, Günther K, Hohenberger W, Schneider-Stock R, Croner RS. A novel complex KIT mutation in a gastrointestinal stromal tumor of the vermiform appendix. Hum Pathol. 2013;44(4):651–5.CrossRef
9.
go back to reference Guo ALWZQ, Wang W. A case of low grade appendix malignant stromal tumor. Chin J Pathol. 2001;03:29. Guo ALWZQ, Wang W. A case of low grade appendix malignant stromal tumor. Chin J Pathol. 2001;03:29.
10.
go back to reference Miettinen M, Sobin LH. Gastrointestinal stromal tumors in the appendix: a clinicopathologic and immunohistochemical study of four cases. Am J Surg Pathol. 2001;25(11):1433–7.CrossRef Miettinen M, Sobin LH. Gastrointestinal stromal tumors in the appendix: a clinicopathologic and immunohistochemical study of four cases. Am J Surg Pathol. 2001;25(11):1433–7.CrossRef
11.
go back to reference He JFLSL. A case of malignant tumor of appendix. Chin J Pathol. 2002;02:31. He JFLSL. A case of malignant tumor of appendix. Chin J Pathol. 2002;02:31.
12.
go back to reference Wu YQWY. A case of misdiagnosis of malignant stromal tumors in the ileocecal region. Chin J Misdiagnistics. 2004;04:639. Wu YQWY. A case of misdiagnosis of malignant stromal tumors in the ileocecal region. Chin J Misdiagnistics. 2004;04:639.
13.
go back to reference Yap WM, Tan HW, Goh SG, Chuah KL. Appendiceal gastrointestinal stromal tumor. Am J Surg Pathol. 2005;29(11):1545–7.CrossRef Yap WM, Tan HW, Goh SG, Chuah KL. Appendiceal gastrointestinal stromal tumor. Am J Surg Pathol. 2005;29(11):1545–7.CrossRef
14.
go back to reference Guo HNLXM, Huang JYWJX. One case of giant low grade gastrointestinal stromal tumor of appendix. J Diag PAthol. 2006;1:48. Guo HNLXM, Huang JYWJX. One case of giant low grade gastrointestinal stromal tumor of appendix. J Diag PAthol. 2006;1:48.
15.
go back to reference Kyu-Jong K, Park S, Park S, Hyun B, Kwon C. Gastrointestinal stromal tumor of appendix incidentally diagnosed by appendiceal hemorrhage. World J Gastroenterol. 2007;13(23):3265–7.CrossRef Kyu-Jong K, Park S, Park S, Hyun B, Kwon C. Gastrointestinal stromal tumor of appendix incidentally diagnosed by appendiceal hemorrhage. World J Gastroenterol. 2007;13(23):3265–7.CrossRef
16.
go back to reference Agaimy A, Pelz AF, Wieacker P, Roessner A, Wünsch PH, Schneider-Stock R. Gastrointestinal stromal tumors of the vermiform appendix: clinicopathologic, immunohistochemical, and molecular study of 2 cases with literature review. Hum Pathol. 2008;39(8):1252–7.CrossRef Agaimy A, Pelz AF, Wieacker P, Roessner A, Wünsch PH, Schneider-Stock R. Gastrointestinal stromal tumors of the vermiform appendix: clinicopathologic, immunohistochemical, and molecular study of 2 cases with literature review. Hum Pathol. 2008;39(8):1252–7.CrossRef
17.
go back to reference Agaimy A, Wünsch PH, Dirnhofer S, Bihl MP, Terracciano LM, Tornillo L. Microscopic gastrointestinal stromal tumors in esophageal and intestinal surgical resection specimens: a clinicopathologic, immunohistochemical, and molecular study of 19 lesions. Am J Surg Pathol. 2008;32(6):867–73.CrossRef Agaimy A, Wünsch PH, Dirnhofer S, Bihl MP, Terracciano LM, Tornillo L. Microscopic gastrointestinal stromal tumors in esophageal and intestinal surgical resection specimens: a clinicopathologic, immunohistochemical, and molecular study of 19 lesions. Am J Surg Pathol. 2008;32(6):867–73.CrossRef
18.
go back to reference Elazary R, Schlager A, Khalaileh A, Appelbaum L, Bala M, Abu-Gazala M, Khatib A, Neuman T, Rivkind AI, Almogy G. Malignant appendiceal GIST: case report and review of the literature. J Gastrointest Cancer. 2010;41(1):9–12.CrossRef Elazary R, Schlager A, Khalaileh A, Appelbaum L, Bala M, Abu-Gazala M, Khatib A, Neuman T, Rivkind AI, Almogy G. Malignant appendiceal GIST: case report and review of the literature. J Gastrointest Cancer. 2010;41(1):9–12.CrossRef
19.
go back to reference Yang F. A case of Appendiceal stromal tumor. Chin Health Care Nutrition. 2012;22(14):2647. Yang F. A case of Appendiceal stromal tumor. Chin Health Care Nutrition. 2012;22(14):2647.
20.
go back to reference Tran S, Dingeldein M, Mengshol SC, Kay S, Chin AC. Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs. Pediatr Surg Int. 2014;30(4):457–66.CrossRef Tran S, Dingeldein M, Mengshol SC, Kay S, Chin AC. Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs. Pediatr Surg Int. 2014;30(4):457–66.CrossRef
21.
go back to reference Back J, Jeanty J, Landas S. Gastrointestinal stromal tumor of the appendix: case report and review of the literature. Human Pathology Case Reports. 2015;2(4):94–8.CrossRef Back J, Jeanty J, Landas S. Gastrointestinal stromal tumor of the appendix: case report and review of the literature. Human Pathology Case Reports. 2015;2(4):94–8.CrossRef
22.
go back to reference Luo WXZHH, Cui XHLDN, et al. Misdiagnosis of stromal tumors of gynecological tumors in 2 cases and literature review. Chin J Clin Obstet Gynecol. 2015;16(01):75–6. Luo WXZHH, Cui XHLDN, et al. Misdiagnosis of stromal tumors of gynecological tumors in 2 cases and literature review. Chin J Clin Obstet Gynecol. 2015;16(01):75–6.
23.
go back to reference Pan YLXY. A case of low grade appendix malignant stromal tumor. Chin J Postgrad Med (z1). 2015:193–4. Pan YLXY. A case of low grade appendix malignant stromal tumor. Chin J Postgrad Med (z1). 2015:193–4.
24.
go back to reference Zhu CY, Zhu YM. Gastrointestinal stromal tumor of the vermiform appendix: a case report and literature review. World Chin J Digestol. 2015;23(1):176.CrossRef Zhu CY, Zhu YM. Gastrointestinal stromal tumor of the vermiform appendix: a case report and literature review. World Chin J Digestol. 2015;23(1):176.CrossRef
25.
go back to reference Rahimi K, Gologan A, Haliotis T, Lamoureux E, Chetty R. Gastrointestinal stromal tumor with autonomic nerve differentiation and coexistent mantle cell lymphoma involving the appendix. Int J Clin Exp Pathol. 2009;2(6):608–13. Rahimi K, Gologan A, Haliotis T, Lamoureux E, Chetty R. Gastrointestinal stromal tumor with autonomic nerve differentiation and coexistent mantle cell lymphoma involving the appendix. Int J Clin Exp Pathol. 2009;2(6):608–13.
26.
go back to reference Chung JC, Song OP. Gastrointestinal stromal tumor of the appendix. Turk J Gastroenterol. 2012;23:303–4.CrossRef Chung JC, Song OP. Gastrointestinal stromal tumor of the appendix. Turk J Gastroenterol. 2012;23:303–4.CrossRef
27.
go back to reference Bouassida M, Chtourou MF, Chalbi E, Chebbi F, Hamzaoui L, Sassi S, et al. Appendiceal GIST: report of an exceptional case and review of the literature. Pan Afr Med J. 2013;15:85.PubMedPubMedCentral Bouassida M, Chtourou MF, Chalbi E, Chebbi F, Hamzaoui L, Sassi S, et al. Appendiceal GIST: report of an exceptional case and review of the literature. Pan Afr Med J. 2013;15:85.PubMedPubMedCentral
28.
go back to reference Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O'Leary TJ, Remotti H, Rubin BP. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002;33(5):459–65.CrossRef Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O'Leary TJ, Remotti H, Rubin BP. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002;33(5):459–65.CrossRef
29.
go back to reference Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008;39:1411–9.CrossRef Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor. Hum Pathol. 2008;39:1411–9.CrossRef
30.
go back to reference Liu Z, Tian Y, Xu G, Liu S, Guo M, Lian X, et al. Pancreatic gastrointestinal stromal tumor: clinicopathologic features and prognosis. J Clin Gastroenterol. 2017;51:850–6.PubMed Liu Z, Tian Y, Xu G, Liu S, Guo M, Lian X, et al. Pancreatic gastrointestinal stromal tumor: clinicopathologic features and prognosis. J Clin Gastroenterol. 2017;51:850–6.PubMed
31.
go back to reference Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol. 2006;23(2):70–83.CrossRef Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol. 2006;23(2):70–83.CrossRef
32.
go back to reference Demetri GD, Von MM, Antonescu CR, Dematteo RP, Ganjoo KN, Maki RG, Pisters PW, Raut CP, Riedel RF, Schuetze S. NCCN task force report: update on the management of patients with gastrointestinal stromal tumors. J Natl Compr Canc Netw Jnccn 8 Suppl. 2010;2(4):S1. Demetri GD, Von MM, Antonescu CR, Dematteo RP, Ganjoo KN, Maki RG, Pisters PW, Raut CP, Riedel RF, Schuetze S. NCCN task force report: update on the management of patients with gastrointestinal stromal tumors. J Natl Compr Canc Netw Jnccn 8 Suppl. 2010;2(4):S1.
33.
go back to reference Lopes LF, West RB, Bacchi LM, Van dRM, Bacchi CE. DOG1 for the diagnosis of gastrointestinal stromal tumor (GIST): comparison between 2 different antibodies. Appl Immunohistochem Mol Morphol. 2010;18(4):333–7.CrossRef Lopes LF, West RB, Bacchi LM, Van dRM, Bacchi CE. DOG1 for the diagnosis of gastrointestinal stromal tumor (GIST): comparison between 2 different antibodies. Appl Immunohistochem Mol Morphol. 2010;18(4):333–7.CrossRef
34.
go back to reference Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med. 2006;130:1466–78.PubMed Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Arch Pathol Lab Med. 2006;130:1466–78.PubMed
35.
go back to reference Rubin BP, Cooper K, Fletcher CD, Folpe AL, Gannon FH, Hunt JL, et al. Protocol for the examination of specimens from patients with tumors of soft tissue. Arch Pathol Lab Med. 2010;134:e31–9.PubMed Rubin BP, Cooper K, Fletcher CD, Folpe AL, Gannon FH, Hunt JL, et al. Protocol for the examination of specimens from patients with tumors of soft tissue. Arch Pathol Lab Med. 2010;134:e31–9.PubMed
36.
go back to reference 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;279(5350):577.CrossRef 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;279(5350):577.CrossRef
37.
go back to reference Sarlomo-Rikala M, Kovatich AJ, Barusevicius A, Miettinen M. CD117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD34. Mod Pathol. 1998;11:728–34.PubMed Sarlomo-Rikala M, Kovatich AJ, Barusevicius A, Miettinen M. CD117: a sensitive marker for gastrointestinal stromal tumors that is more specific than CD34. Mod Pathol. 1998;11:728–34.PubMed
38.
go back to reference Miettinen M, Wang ZF, Lasota J. DOG1 antibody in the differential diagnosis of gastrointestinal stromal tumors: a study of 1840 cases. Am J Surg Pathol. 2009;33(9):1401–8.CrossRef Miettinen M, Wang ZF, Lasota J. DOG1 antibody in the differential diagnosis of gastrointestinal stromal tumors: a study of 1840 cases. Am J Surg Pathol. 2009;33(9):1401–8.CrossRef
39.
go back to reference Li K, Cheng H, Li Z, Pang Y, Jia X, Xie F, et al. Genetic progression in gastrointestinal stromal tumors: mechanisms and molecular interventions. Oncotarget. 2017;8:60589–604.PubMedPubMedCentral Li K, Cheng H, Li Z, Pang Y, Jia X, Xie F, et al. Genetic progression in gastrointestinal stromal tumors: mechanisms and molecular interventions. Oncotarget. 2017;8:60589–604.PubMedPubMedCentral
40.
go back to reference Valsangkar N, Sehdev A, Misra S, Zimmers TA, O'Neil BH, Koniaris LG. Current management of gastrointestinal stromal tumors: surgery, current biomarkers, mutations, and therapy. Surgery. 2015;158(5):1149–64.CrossRef Valsangkar N, Sehdev A, Misra S, Zimmers TA, O'Neil BH, Koniaris LG. Current management of gastrointestinal stromal tumors: surgery, current biomarkers, mutations, and therapy. Surgery. 2015;158(5):1149–64.CrossRef
41.
go back to reference Jian Y, Jian W, Zhang S, Yingqiang Y, Xiaobo L. Chinese consensus guidelines for diagnosis and management of gastrointestinal stromal tumor. Chin J Cancer Res. 2017;29(4):281–93.CrossRef Jian Y, Jian W, Zhang S, Yingqiang Y, Xiaobo L. Chinese consensus guidelines for diagnosis and management of gastrointestinal stromal tumor. Chin J Cancer Res. 2017;29(4):281–93.CrossRef
42.
go back to reference Ford SJ, Gronchi A. Indications for surgery in advanced/metastatic GIST. Eur J Cancer. 2016;63:154–67.CrossRef Ford SJ, Gronchi A. Indications for surgery in advanced/metastatic GIST. Eur J Cancer. 2016;63:154–67.CrossRef
43.
go back to reference Huang CM, Chen QF, Lin JX, Lin M, Zheng CH, Li P, Xie JW, Wang JB, Lu J, Chen QY. Can laparoscopic surgery be applied in gastric gastrointestinal stromal tumors located in unfavorable sites?: a study based on the NCCN guidelines. Medicine (Abingdon). 2017;96(14):e6535. Huang CM, Chen QF, Lin JX, Lin M, Zheng CH, Li P, Xie JW, Wang JB, Lu J, Chen QY. Can laparoscopic surgery be applied in gastric gastrointestinal stromal tumors located in unfavorable sites?: a study based on the NCCN guidelines. Medicine (Abingdon). 2017;96(14):e6535.
44.
go back to reference Dematteo RP, Gold JS, Saran L, Gönen M, Liau KH, Maki RG, Singer S, Besmer P, Brennan MF, Antonescu CR. Tumor mitotic rate, size, and location independently predict recurrence after resection of primary gastrointestinal stromal tumor (GIST). Cancer. 2008;112(3):608.CrossRef Dematteo RP, Gold JS, Saran L, Gönen M, Liau KH, Maki RG, Singer S, Besmer P, Brennan MF, Antonescu CR. Tumor mitotic rate, size, and location independently predict recurrence after resection of primary gastrointestinal stromal tumor (GIST). Cancer. 2008;112(3):608.CrossRef
45.
go back to reference Rutkowski P, Nowecki ZI, Michej W, Debiecrychter M, Woźniak A, Limon J, Siedlecki J, Grzesiakowska U, Kakol M, Osuch C. Risk criteria and prognostic factors for predicting recurrences after resection of primary gastrointestinal stromal tumor. Ann Surg Oncol. 2007;14(7):2018–27.CrossRef Rutkowski P, Nowecki ZI, Michej W, Debiecrychter M, Woźniak A, Limon J, Siedlecki J, Grzesiakowska U, Kakol M, Osuch C. Risk criteria and prognostic factors for predicting recurrences after resection of primary gastrointestinal stromal tumor. Ann Surg Oncol. 2007;14(7):2018–27.CrossRef
Metadata
Title
Clinicopathological characteristics and prognosis of primary appendiceal stromal tumors
Authors
Bao Zhang
Guo Liang Zheng
Hai Tao Zhu
Yan Zhao
Zhi Chao Zheng
Publication date
01-12-2018
Publisher
BioMed Central
Published in
World Journal of Surgical Oncology / Issue 1/2018
Electronic ISSN: 1477-7819
DOI
https://doi.org/10.1186/s12957-018-1524-1

Other articles of this Issue 1/2018

World Journal of Surgical Oncology 1/2018 Go to the issue