Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review
Pheochromocytomas and paragangliomas (PPGLs) are rare tumors that secrete catecholamines and arise from the adrenal medulla or extra-adrenal sympathetic ganglia. These tumors secrete adrenaline and noradrenaline, but paragangliomas usually produce only noradrenaline because of the lack of phenyletha...
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| Format: | Article |
| Language: | English |
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The Japan Endocrine Society
2024-01-01
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| Series: | Endocrine Journal |
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| Online Access: | https://www.jstage.jst.go.jp/article/endocrj/71/1/71_EJ23-0271/_html/-char/en |
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| author | Yuriko Sasaki Maki Kanzawa Masaaki Yamamoto Keitaro Kanie Hironori Bando Kei Yoshino Yushi Hirota Katsumi Shigemura Masato Fujisawa Wataru Ogawa Hidenori Fukuoka |
| author_facet | Yuriko Sasaki Maki Kanzawa Masaaki Yamamoto Keitaro Kanie Hironori Bando Kei Yoshino Yushi Hirota Katsumi Shigemura Masato Fujisawa Wataru Ogawa Hidenori Fukuoka |
| author_sort | Yuriko Sasaki |
| collection | DOAJ |
| description | Pheochromocytomas and paragangliomas (PPGLs) are rare tumors that secrete catecholamines and arise from the adrenal medulla or extra-adrenal sympathetic ganglia. These tumors secrete adrenaline and noradrenaline, but paragangliomas usually produce only noradrenaline because of the lack of phenylethanolamine N-methyltransferase (PNMT) expression. Composite paragangliomas, which are complex tumors consisting of multiple types of neuroblastic cells, are extremely rare. We present the case of a 46-year-old woman with an atypical catecholamine profile who was preoperatively diagnosed with pheochromocytoma. However, postoperative pathology revealed that the patient had an extra-adrenal paraganglioma accompanied by a ganglioneuroma, which led to the diagnosis of a composite tumor. Interestingly, PNMT is expressed in both paragangliomas and ganglioneuromas. In addition, we reviewed reported composite paragangliomas and compared their clinical features with those of composite pheochromocytomas. We also discuss various aspects of the etiology of composite paragangliomas and the mechanism by which PNMT is expressed in tumors. |
| format | Article |
| id | doaj-art-6ce2d70e071741ed810ffff72dc969b1 |
| institution | Kabale University |
| issn | 1348-4540 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | The Japan Endocrine Society |
| record_format | Article |
| series | Endocrine Journal |
| spelling | doaj-art-6ce2d70e071741ed810ffff72dc969b12025-01-22T06:31:09ZengThe Japan Endocrine SocietyEndocrine Journal1348-45402024-01-01711657410.1507/endocrj.EJ23-0271endocrjComposite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature reviewYuriko Sasaki0Maki Kanzawa1Masaaki Yamamoto2Keitaro Kanie3Hironori Bando4Kei Yoshino5Yushi Hirota6Katsumi Shigemura7Masato Fujisawa8Wataru Ogawa9Hidenori Fukuoka10Division of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe 650-0017, JapanDepartment of Diagnostic Pathology, Kobe University Hospital, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Kobe University Hospital, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Kobe University Hospital, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Kobe University Hospital, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe 650-0017, JapanDivision of Urology, Department of Organ Therapeutics, Faculty of Medicine, Kobe University Graduate School of Medicine, Kobe 650-0017, JapanDivision of Urology, Department of Organ Therapeutics, Faculty of Medicine, Kobe University Graduate School of Medicine, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe 650-0017, JapanDivision of Diabetes and Endocrinology, Kobe University Hospital, Kobe 650-0017, JapanPheochromocytomas and paragangliomas (PPGLs) are rare tumors that secrete catecholamines and arise from the adrenal medulla or extra-adrenal sympathetic ganglia. These tumors secrete adrenaline and noradrenaline, but paragangliomas usually produce only noradrenaline because of the lack of phenylethanolamine N-methyltransferase (PNMT) expression. Composite paragangliomas, which are complex tumors consisting of multiple types of neuroblastic cells, are extremely rare. We present the case of a 46-year-old woman with an atypical catecholamine profile who was preoperatively diagnosed with pheochromocytoma. However, postoperative pathology revealed that the patient had an extra-adrenal paraganglioma accompanied by a ganglioneuroma, which led to the diagnosis of a composite tumor. Interestingly, PNMT is expressed in both paragangliomas and ganglioneuromas. In addition, we reviewed reported composite paragangliomas and compared their clinical features with those of composite pheochromocytomas. We also discuss various aspects of the etiology of composite paragangliomas and the mechanism by which PNMT is expressed in tumors.https://www.jstage.jst.go.jp/article/endocrj/71/1/71_EJ23-0271/_html/-char/encomposite tumorsphenylethanolamine n-methyltransferasepheochromocytomas and paragangliomas |
| spellingShingle | Yuriko Sasaki Maki Kanzawa Masaaki Yamamoto Keitaro Kanie Hironori Bando Kei Yoshino Yushi Hirota Katsumi Shigemura Masato Fujisawa Wataru Ogawa Hidenori Fukuoka Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review Endocrine Journal composite tumors phenylethanolamine n-methyltransferase pheochromocytomas and paragangliomas |
| title | Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review |
| title_full | Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review |
| title_fullStr | Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review |
| title_full_unstemmed | Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review |
| title_short | Composite paraganglioma-ganglioneuroma with atypical catecholamine profile and phenylethanolamine N-methyltransferase expression: a case report and literature review |
| title_sort | composite paraganglioma ganglioneuroma with atypical catecholamine profile and phenylethanolamine n methyltransferase expression a case report and literature review |
| topic | composite tumors phenylethanolamine n-methyltransferase pheochromocytomas and paragangliomas |
| url | https://www.jstage.jst.go.jp/article/endocrj/71/1/71_EJ23-0271/_html/-char/en |
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