Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors
MEN1-associated pancreatic neuroendocrine tumors (pNETs) may potentially express distinct hormones, but the mechanism has not been elucidated. Transcription factors such as MafA and Pdx1 have been identified to lead to beta cell differentiation, while Arx and Brn4 to alpha cell differentiation in de...
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Format: | Article |
Language: | English |
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Bioscientifica
2017-09-01
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Series: | Endocrinology, Diabetes & Metabolism Case Reports |
Online Access: | https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-17-0088 |
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author | Yasutaka Takeda Yukihiro Fujita Kentaro Sakai Tomoe Abe Tomonobu Nakamura Tsuyoshi Yanagimachi Hidemitsu Sakagami Jun Honjo Atsuko Abiko Yuichi Makino Masakazu Haneda |
author_facet | Yasutaka Takeda Yukihiro Fujita Kentaro Sakai Tomoe Abe Tomonobu Nakamura Tsuyoshi Yanagimachi Hidemitsu Sakagami Jun Honjo Atsuko Abiko Yuichi Makino Masakazu Haneda |
author_sort | Yasutaka Takeda |
collection | DOAJ |
description | MEN1-associated pancreatic neuroendocrine tumors (pNETs) may potentially express distinct hormones, but the mechanism has not been elucidated. Transcription factors such as MafA and Pdx1 have been identified to lead to beta cell differentiation, while Arx and Brn4 to alpha cell differentiation in developing pancreas. We hypothesized those transcription factors are important to produce specific hormones in pNETs, similarly to developing pancreas, and examined the expression of transcription factors in a case of MEN1 who showed immunohistological coexistence of several hormone-producing pNETs including insulinoma. A 70-year-old woman was found to manifest hypoglycemia with non-suppressed insulinemia and hypercalcemia with elevated PTH level. She was diagnosed as MEN1 based on the manifestation of primary hyperparathyroidism, pituitary adenoma and insulinoma, with genetic variation of MEN1 gene. She had pylorus-preserving pancreaticoduodenectomy because CT scan and SACI test indicated that insulinoma was localized in the head of the pancreas. Histopathological finding was MEN1-associated NET, G1. Interestingly, immunohistological examination of the resected pancreas revealed that two insulinomas, a glucagon-positive NET and a multiple hormone-positive NET coexisted. Hence, we examined the expression of transcription factors immunohistochemically to elucidate the role of the transcription factors in MEN1-associated hormone-producing pNETs. We observed homogeneous expressions of MafA and Pdx1 in insulinomas and Arx in glucagon-positive NET, respectively. Moreover, multiple hormone-positive NETs expressed several transcription factors heterogeneously. Collectively, our results suggested that transcription factors could play important roles in the production of specific hormones in
MEN1-associated pNETs, similar to islet differentiation. |
first_indexed | 2024-12-12T09:03:17Z |
format | Article |
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institution | Directory Open Access Journal |
issn | 2052-0573 2052-0573 |
language | English |
last_indexed | 2024-12-12T09:03:17Z |
publishDate | 2017-09-01 |
publisher | Bioscientifica |
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series | Endocrinology, Diabetes & Metabolism Case Reports |
spelling | doaj.art-e78f6851309f4bbabb7579ce9024c9b82022-12-22T00:29:45ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732052-05732017-09-01111610.1530/EDM-17-0088Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumorsYasutaka Takeda0Yukihiro Fujita1Kentaro Sakai2Tomoe Abe3Tomonobu Nakamura4Tsuyoshi Yanagimachi5Hidemitsu Sakagami6Jun Honjo7Atsuko Abiko8Yuichi Makino9Masakazu Haneda10Division of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanDivision of Metabolism and Biosystemic Science, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, JapanMEN1-associated pancreatic neuroendocrine tumors (pNETs) may potentially express distinct hormones, but the mechanism has not been elucidated. Transcription factors such as MafA and Pdx1 have been identified to lead to beta cell differentiation, while Arx and Brn4 to alpha cell differentiation in developing pancreas. We hypothesized those transcription factors are important to produce specific hormones in pNETs, similarly to developing pancreas, and examined the expression of transcription factors in a case of MEN1 who showed immunohistological coexistence of several hormone-producing pNETs including insulinoma. A 70-year-old woman was found to manifest hypoglycemia with non-suppressed insulinemia and hypercalcemia with elevated PTH level. She was diagnosed as MEN1 based on the manifestation of primary hyperparathyroidism, pituitary adenoma and insulinoma, with genetic variation of MEN1 gene. She had pylorus-preserving pancreaticoduodenectomy because CT scan and SACI test indicated that insulinoma was localized in the head of the pancreas. Histopathological finding was MEN1-associated NET, G1. Interestingly, immunohistological examination of the resected pancreas revealed that two insulinomas, a glucagon-positive NET and a multiple hormone-positive NET coexisted. Hence, we examined the expression of transcription factors immunohistochemically to elucidate the role of the transcription factors in MEN1-associated hormone-producing pNETs. We observed homogeneous expressions of MafA and Pdx1 in insulinomas and Arx in glucagon-positive NET, respectively. Moreover, multiple hormone-positive NETs expressed several transcription factors heterogeneously. Collectively, our results suggested that transcription factors could play important roles in the production of specific hormones in MEN1-associated pNETs, similar to islet differentiation.https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-17-0088 |
spellingShingle | Yasutaka Takeda Yukihiro Fujita Kentaro Sakai Tomoe Abe Tomonobu Nakamura Tsuyoshi Yanagimachi Hidemitsu Sakagami Jun Honjo Atsuko Abiko Yuichi Makino Masakazu Haneda Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors Endocrinology, Diabetes & Metabolism Case Reports |
title | Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors |
title_full | Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors |
title_fullStr | Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors |
title_full_unstemmed | Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors |
title_short | Expression of transcription factors in MEN1-associated pancreatic neuroendocrine tumors |
title_sort | expression of transcription factors in men1 associated pancreatic neuroendocrine tumors |
url | https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-17-0088 |
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