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Published in: BMC Cancer 1/2017

Open Access 01-12-2017 | Case report

Synchronous occurrence of hereditary gastric adenocarcinoma, gastrointestinal stromal tumor, and esophageal small cell and squamous carcinoma in situ: an extremely rare case report

Authors: Huijie Fan, Pei Lu, Li Xu, Yanru Qin, Jing Li

Published in: BMC Cancer | Issue 1/2017

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Abstract

Background

Hereditary diffuse gastric carcinoma (HDGC) accounts for 1–3% of all gastric carcinomas. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the gastrointestinal (GI) tract but they comprise fewer than 1% of all GI malignancies. Small-cell carcinoma (SmCC) is a rare histological type of esophageal carcinoma, accounting for 0.4% to 2.8% of all esophageal tumors. Co-occurrence of SmCC with esophageal tumors caused by squamous carcinoma is also very uncommon. Although multiple primary malignancies are no longer rare in clinical practice, the simultaneous appearance of HDGC, GIST, esophageal small cell and squamous carcinoma in situ is extremely rare and very few cases have been reported.

Case presentation

We present a case of a 53 year-old woman with synchronous occurrence of four malignancies including HDGC, GIST, esophageal small cell- and local squamous carcinoma in situ. A total gastrectomy with D2 lymph node dissection and postoperative adjuvant chemotherapy with oxaliplatin and paclitaxel liposome were performed. After a 1-year follow-up, this patient was still in good condition with no evidence of recurrence.

Conclusion

This is the unique case that describes the co-existence of the aforementioned four types of neoplasm. This case demonstrates that a diagnosis of gastric cancer does not preclude the presence of other malignancies and every case should be thoroughly analyzed to avoid missing other problems, which may worsen the prognosis.
Literature
1.
go back to reference Pierko J, Lukaszewicz J, Sawicka-Pierko A, Hady HR, Dadan J. Synchronous gastric and rectal cancer in a 50 year-old man - case report. Pol Przegl Chir. 2012;84(11):582–4.PubMed Pierko J, Lukaszewicz J, Sawicka-Pierko A, Hady HR, Dadan J. Synchronous gastric and rectal cancer in a 50 year-old man - case report. Pol Przegl Chir. 2012;84(11):582–4.PubMed
2.
go back to reference Kim JH, Rha SY, Kim C, Kim GM, Yoon SH, Kim KH, Kim MJ, Ahn JB, Chung HC, Roh JK, et al. Clinicopathologic features of metachronous or synchronous gastric cancer patients with three or more primary sites. Cancer Res Treat. 2010;42(4):217–24.CrossRefPubMedPubMedCentral Kim JH, Rha SY, Kim C, Kim GM, Yoon SH, Kim KH, Kim MJ, Ahn JB, Chung HC, Roh JK, et al. Clinicopathologic features of metachronous or synchronous gastric cancer patients with three or more primary sites. Cancer Res Treat. 2010;42(4):217–24.CrossRefPubMedPubMedCentral
3.
go back to reference Hansford S, Kaurah P, Li-Chang H, Woo M, Senz J, Pinheiro H, Schrader KA, Schaeffer DF, Shumansky K, Zogopoulos G, et al. Hereditary diffuse gastric cancer syndrome: CDH1 mutations and beyond. JAMA Oncol. 2015;1(1):23–32.CrossRefPubMed Hansford S, Kaurah P, Li-Chang H, Woo M, Senz J, Pinheiro H, Schrader KA, Schaeffer DF, Shumansky K, Zogopoulos G, et al. Hereditary diffuse gastric cancer syndrome: CDH1 mutations and beyond. JAMA Oncol. 2015;1(1):23–32.CrossRefPubMed
4.
5.
go back to reference Chen W, Zheng R, Baade PD, Zhang S, Zeng H, Bray F, Jemal A, XQ Y, He J. Cancer statistics in China, 2015. CA Cancer J Clin. 2016;66(2):115–32.CrossRefPubMed Chen W, Zheng R, Baade PD, Zhang S, Zeng H, Bray F, Jemal A, XQ Y, He J. Cancer statistics in China, 2015. CA Cancer J Clin. 2016;66(2):115–32.CrossRefPubMed
6.
go back to reference Hamilton LE, Jones K, Church N, Medlicott S. Synchronous appendiceal and intramucosal gastric signet ring cell carcinomas in an individual with CDH1-associated hereditary diffuse gastric carcinoma: a case report of a novel association and review of the literature. BMC Gastroenterol. 2013;13:114.CrossRefPubMedPubMedCentral Hamilton LE, Jones K, Church N, Medlicott S. Synchronous appendiceal and intramucosal gastric signet ring cell carcinomas in an individual with CDH1-associated hereditary diffuse gastric carcinoma: a case report of a novel association and review of the literature. BMC Gastroenterol. 2013;13:114.CrossRefPubMedPubMedCentral
7.
go back to reference Fitzgerald RC, Hardwick R, Huntsman D, Carneiro F, Guilford P, Blair V, Chung DC, Norton J, Ragunath K, Van Krieken JH, et al. Hereditary diffuse gastric cancer: updated consensus guidelines for clinical management and directions for future research. J Med Genet. 2010;47(7):436–44.CrossRefPubMedPubMedCentral Fitzgerald RC, Hardwick R, Huntsman D, Carneiro F, Guilford P, Blair V, Chung DC, Norton J, Ragunath K, Van Krieken JH, et al. Hereditary diffuse gastric cancer: updated consensus guidelines for clinical management and directions for future research. J Med Genet. 2010;47(7):436–44.CrossRefPubMedPubMedCentral
8.
go back to reference Yuan J, Li Y, Tian T, Li N, Zhu Y, Zou J, Gao J, Shen L. Risk prediction for early-onset gastric carcinoma: a case-control study of polygenic gastric cancer in Han Chinese with hereditary background. Oncotarget. 2016;7(23):33608–15.CrossRefPubMedPubMedCentral Yuan J, Li Y, Tian T, Li N, Zhu Y, Zou J, Gao J, Shen L. Risk prediction for early-onset gastric carcinoma: a case-control study of polygenic gastric cancer in Han Chinese with hereditary background. Oncotarget. 2016;7(23):33608–15.CrossRefPubMedPubMedCentral
9.
go back to reference Study Group of Millennium Genome Project for C, Sakamoto H, Yoshimura K, Saeki N, Katai H, Shimoda T, Matsuno Y, Saito D, Sugimura H, Tanioka F et al: Genetic variation in PSCA is associated with susceptibility to diffuse-type gastric cancer. Nat Genet 2008, 40(6):730-740. Study Group of Millennium Genome Project for C, Sakamoto H, Yoshimura K, Saeki N, Katai H, Shimoda T, Matsuno Y, Saito D, Sugimura H, Tanioka F et al: Genetic variation in PSCA is associated with susceptibility to diffuse-type gastric cancer. Nat Genet 2008, 40(6):730-740.
10.
go back to reference Eshel R, Zanin A, Kapon D, Sagi-Assif O, Brakenhoff R, van Dongen G, Witz IP. Human Ly-6 antigen E48 (Ly-6D) regulates important interaction parameters between endothelial cells and head-and-neck squamous carcinoma cells. Int J Cancer. 2002;98(6):803–10.CrossRefPubMed Eshel R, Zanin A, Kapon D, Sagi-Assif O, Brakenhoff R, van Dongen G, Witz IP. Human Ly-6 antigen E48 (Ly-6D) regulates important interaction parameters between endothelial cells and head-and-neck squamous carcinoma cells. Int J Cancer. 2002;98(6):803–10.CrossRefPubMed
11.
go back to reference Saeki N, Ono H, Yanagihara K, Aoyagi K, Sasaki H, Sakamoto H, Yoshida T. rs2294008T, a risk allele for gastric and gallbladder cancers, suppresses the PSCA promoter by recruiting the transcription factor YY1. Genes Cells. 2015;20(5):382–91.CrossRefPubMed Saeki N, Ono H, Yanagihara K, Aoyagi K, Sasaki H, Sakamoto H, Yoshida T. rs2294008T, a risk allele for gastric and gallbladder cancers, suppresses the PSCA promoter by recruiting the transcription factor YY1. Genes Cells. 2015;20(5):382–91.CrossRefPubMed
12.
go back to reference Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol. 1983;7(6):507–19.CrossRefPubMed Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol. 1983;7(6):507–19.CrossRefPubMed
13.
go back to reference Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol. 1999;30(10):1213–20.CrossRefPubMed Miettinen M, Sarlomo-Rikala M, Lasota J. Gastrointestinal stromal tumors: recent advances in understanding of their biology. Hum Pathol. 1999;30(10):1213–20.CrossRefPubMed
14.
go back to reference van der Zwan SM, DeMatteo RP. Gastrointestinal stromal tumor: 5 years later. Cancer. 2005;104(9):1781–8.CrossRefPubMed van der Zwan SM, DeMatteo RP. Gastrointestinal stromal tumor: 5 years later. Cancer. 2005;104(9):1781–8.CrossRefPubMed
15.
go back to reference Liu YJ, Yang Z, Hao LS, Xia L, Jia QB, XT W. Synchronous incidental gastrointestinal stromal and epithelial malignant tumors. World J Gastroenterol. 2009;15(16):2027–31.CrossRefPubMedPubMedCentral Liu YJ, Yang Z, Hao LS, Xia L, Jia QB, XT W. Synchronous incidental gastrointestinal stromal and epithelial malignant tumors. World J Gastroenterol. 2009;15(16):2027–31.CrossRefPubMedPubMedCentral
16.
go back to reference Ding J, Sun P, Cai XY, Fei SH, Wu J, Qi YK, Liu ZB, Yuan L, He YJ, Song H, et al. Synchronous poorly-differentiated neuroendocrine carcinoma and gastrointestinal stromal tumor of the stomach: a case report with immunohistochemical and molecular genetic analyses of KIT and PDGFRA. Int J Clin Exp Pathol. 2014;7(12):9076–80.PubMedPubMedCentral Ding J, Sun P, Cai XY, Fei SH, Wu J, Qi YK, Liu ZB, Yuan L, He YJ, Song H, et al. Synchronous poorly-differentiated neuroendocrine carcinoma and gastrointestinal stromal tumor of the stomach: a case report with immunohistochemical and molecular genetic analyses of KIT and PDGFRA. Int J Clin Exp Pathol. 2014;7(12):9076–80.PubMedPubMedCentral
17.
go back to reference Nemes C, Rogojan L, Surdea-Blaga T, Seicean A, Dumitrascu DL, Ciuce C. Gastrointestinal stromal tumor (GIST) associated with synchronous colon adenocarcinoma - a case report. J Gastrointestin Liver Dis. 2012;21(1):101–3.PubMed Nemes C, Rogojan L, Surdea-Blaga T, Seicean A, Dumitrascu DL, Ciuce C. Gastrointestinal stromal tumor (GIST) associated with synchronous colon adenocarcinoma - a case report. J Gastrointestin Liver Dis. 2012;21(1):101–3.PubMed
18.
go back to reference Brenner B, Tang LH, Shia J, Klimstra DS, Kelsen DP. Small cell carcinomas of the gastrointestinal tract: clinicopathological features and treatment approach. Semin Oncol. 2007;34(1):43–50.CrossRefPubMed Brenner B, Tang LH, Shia J, Klimstra DS, Kelsen DP. Small cell carcinomas of the gastrointestinal tract: clinicopathological features and treatment approach. Semin Oncol. 2007;34(1):43–50.CrossRefPubMed
19.
go back to reference Jemal A, Bray F, Center MM, Ferlay J, Ward E, Forman D. Global cancer statistics. CA Cancer J Clin. 2011;61(2):69–90.CrossRefPubMed Jemal A, Bray F, Center MM, Ferlay J, Ward E, Forman D. Global cancer statistics. CA Cancer J Clin. 2011;61(2):69–90.CrossRefPubMed
20.
go back to reference Casas F, Ferrer F, Farrus B, Casals J, Biete A. Primary small cell carcinoma of the esophagus: a review of the literature with emphasis on therapy and prognosis. Cancer. 1997;80(8):1366–72.CrossRefPubMed Casas F, Ferrer F, Farrus B, Casals J, Biete A. Primary small cell carcinoma of the esophagus: a review of the literature with emphasis on therapy and prognosis. Cancer. 1997;80(8):1366–72.CrossRefPubMed
21.
go back to reference Law SY, Fok M, Lam KY, Loke SL, Ma LT, Wong J. Small cell carcinoma of the esophagus. Cancer. 1994;73(12):2894–9.CrossRefPubMed Law SY, Fok M, Lam KY, Loke SL, Ma LT, Wong J. Small cell carcinoma of the esophagus. Cancer. 1994;73(12):2894–9.CrossRefPubMed
22.
go back to reference Huncharek M, Muscat J. Small cell carcinoma of the esophagus. The Massachusetts General Hospital experience, 1978 to 1993. Chest. 1995;107(1):179–81.CrossRefPubMed Huncharek M, Muscat J. Small cell carcinoma of the esophagus. The Massachusetts General Hospital experience, 1978 to 1993. Chest. 1995;107(1):179–81.CrossRefPubMed
23.
go back to reference Yau KK, Siu WT, Wong DC, Chau CH, Li AC, Law BK, Li MK. Non-operative management of small cell carcinoma of esophagus. Dis Esophagus. 2007;20(6):487–90.CrossRefPubMed Yau KK, Siu WT, Wong DC, Chau CH, Li AC, Law BK, Li MK. Non-operative management of small cell carcinoma of esophagus. Dis Esophagus. 2007;20(6):487–90.CrossRefPubMed
24.
go back to reference Lv J, Liang J, Wang J, Wang L, He J, Xiao Z, Yin W. Primary small cell carcinoma of the esophagus. J Thorac Oncol. 2008;3(12):1460–5.CrossRefPubMed Lv J, Liang J, Wang J, Wang L, He J, Xiao Z, Yin W. Primary small cell carcinoma of the esophagus. J Thorac Oncol. 2008;3(12):1460–5.CrossRefPubMed
25.
go back to reference Doherty MA, McIntyre M, Arnott SJ. Oat cell carcinoma of esophagus: a report of six British patients with a review of the literature. Int J Radiat Oncol Biol Phys. 1984;10(1):147–52.CrossRefPubMed Doherty MA, McIntyre M, Arnott SJ. Oat cell carcinoma of esophagus: a report of six British patients with a review of the literature. Int J Radiat Oncol Biol Phys. 1984;10(1):147–52.CrossRefPubMed
26.
go back to reference Lee SS, Ha HK, Kim AY, Kim TK, Kim PN, Yu E, Lee MG, Myung SJ, Jung HY, Kim JH, et al. Primary extrapulmonary small cell carcinoma involving the stomach or duodenum or both: findings on CT and barium studies. AJR Am J Roentgenol. 2003;180(5):1325–9.CrossRefPubMed Lee SS, Ha HK, Kim AY, Kim TK, Kim PN, Yu E, Lee MG, Myung SJ, Jung HY, Kim JH, et al. Primary extrapulmonary small cell carcinoma involving the stomach or duodenum or both: findings on CT and barium studies. AJR Am J Roentgenol. 2003;180(5):1325–9.CrossRefPubMed
Metadata
Title
Synchronous occurrence of hereditary gastric adenocarcinoma, gastrointestinal stromal tumor, and esophageal small cell and squamous carcinoma in situ: an extremely rare case report
Authors
Huijie Fan
Pei Lu
Li Xu
Yanru Qin
Jing Li
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Cancer / Issue 1/2017
Electronic ISSN: 1471-2407
DOI
https://doi.org/10.1186/s12885-017-3736-0

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