Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma

Miguel A. Rodriguez-Bigas, Hans F A Vasen, Henry T. Lynch, Patrice Watson, Torben Myrhøj, Heikki J. Järvinen, Jukka Pekka Mecklin, Finlay Macrae, D. James B St. John, Lucio Bertario, Paulo Fidalgo, Lisa Madlensky, Paul Rozen

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Abstract

BACKGROUND. Small bowel carcinoma is uncommon. However, hereditary nonpolyposis colorectal carcinoma (HNPCC) patients are at increased risk of small bowel carcinoma. The purpose of this study was to characterize small bowel tumors in HNPCC patients. METHODS. A questionnaire was mailed to the members of International Collaborative Group on HNPCC (ICG-HNPCC) requesting clinicopathologic data in their registries on HNPCC patients with small bowel carcinoma. Survival was estimated utilizing the Kaplan-Meier method. RESULTS. Forty-two individuals from 40 HNPCC families developed 42 primary and 7 metachronous small bowel tumors. There were 46 adenocarcinomas and 3 carcinoid tumors. The median age at diagnosis of the index small bowel tumor was 49 years. Mismatch repair gene mutations were present in 15 of 42 patients (36%). There were nine hMLH1 and six hMSH2 mutations. The small bowel was the first site of carcinoma in 24 patients (57%). The median survival for the 42 patients was 47 months (range, 0-447 months). The overall 5- and 10-year survival rates were 44% and 33%, respectively. CONCLUSIONS. Small bowel tumors can be the presenting neoplasms in HNPCC patients. Similar to colorectal carcinoma in HNPCC, small bowel adenocarcinomas in HNPCC patients occur at an earlier age and appear to have a better prognosis than those occurring in the general population.

Original languageEnglish
Pages (from-to)240-244
Number of pages5
JournalCancer
Volume83
Issue number2
DOIs
StatePublished - Jul 15 1998

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Colorectal Neoplasms
Carcinoma
Neoplasms
Adenocarcinoma
Mutation
DNA Mismatch Repair
Survival
Carcinoid Tumor
Registries
Survival Rate
Population
Genes

All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Oncology

Cite this

Rodriguez-Bigas, M. A., Vasen, H. F. A., Lynch, H. T., Watson, P., Myrhøj, T., Järvinen, H. J., ... Rozen, P. (1998). Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma. Cancer, 83(2), 240-244. https://doi.org/10.1002/(SICI)1097-0142(19980715)83:2<240::AID-CNCR6>3.0.CO;2-U

Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma. / Rodriguez-Bigas, Miguel A.; Vasen, Hans F A; Lynch, Henry T.; Watson, Patrice; Myrhøj, Torben; Järvinen, Heikki J.; Mecklin, Jukka Pekka; Macrae, Finlay; St. John, D. James B; Bertario, Lucio; Fidalgo, Paulo; Madlensky, Lisa; Rozen, Paul.

In: Cancer, Vol. 83, No. 2, 15.07.1998, p. 240-244.

Research output: Contribution to journalArticle

Rodriguez-Bigas, MA, Vasen, HFA, Lynch, HT, Watson, P, Myrhøj, T, Järvinen, HJ, Mecklin, JP, Macrae, F, St. John, DJB, Bertario, L, Fidalgo, P, Madlensky, L & Rozen, P 1998, 'Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma', Cancer, vol. 83, no. 2, pp. 240-244. https://doi.org/10.1002/(SICI)1097-0142(19980715)83:2<240::AID-CNCR6>3.0.CO;2-U
Rodriguez-Bigas, Miguel A. ; Vasen, Hans F A ; Lynch, Henry T. ; Watson, Patrice ; Myrhøj, Torben ; Järvinen, Heikki J. ; Mecklin, Jukka Pekka ; Macrae, Finlay ; St. John, D. James B ; Bertario, Lucio ; Fidalgo, Paulo ; Madlensky, Lisa ; Rozen, Paul. / Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma. In: Cancer. 1998 ; Vol. 83, No. 2. pp. 240-244.
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abstract = "BACKGROUND. Small bowel carcinoma is uncommon. However, hereditary nonpolyposis colorectal carcinoma (HNPCC) patients are at increased risk of small bowel carcinoma. The purpose of this study was to characterize small bowel tumors in HNPCC patients. METHODS. A questionnaire was mailed to the members of International Collaborative Group on HNPCC (ICG-HNPCC) requesting clinicopathologic data in their registries on HNPCC patients with small bowel carcinoma. Survival was estimated utilizing the Kaplan-Meier method. RESULTS. Forty-two individuals from 40 HNPCC families developed 42 primary and 7 metachronous small bowel tumors. There were 46 adenocarcinomas and 3 carcinoid tumors. The median age at diagnosis of the index small bowel tumor was 49 years. Mismatch repair gene mutations were present in 15 of 42 patients (36{\%}). There were nine hMLH1 and six hMSH2 mutations. The small bowel was the first site of carcinoma in 24 patients (57{\%}). The median survival for the 42 patients was 47 months (range, 0-447 months). The overall 5- and 10-year survival rates were 44{\%} and 33{\%}, respectively. CONCLUSIONS. Small bowel tumors can be the presenting neoplasms in HNPCC patients. Similar to colorectal carcinoma in HNPCC, small bowel adenocarcinomas in HNPCC patients occur at an earlier age and appear to have a better prognosis than those occurring in the general population.",
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AU - Myrhøj, Torben

AU - Järvinen, Heikki J.

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