Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma
Abstract Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. In this study, we present...
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| Format: | Article |
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Nature Portfolio
2022-05-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-022-12882-2 |
| _version_ | 1818553380612603904 |
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| author | Wei Li Xue Yang Yuan Deng Yina Jiang Guiping Xu Enxiao Li Yinying Wu Juan Ren Zhenhua Ma Shunbin Dong Liang Han Qingyong Ma Zheng Wu Zheng Wang |
| author_facet | Wei Li Xue Yang Yuan Deng Yina Jiang Guiping Xu Enxiao Li Yinying Wu Juan Ren Zhenhua Ma Shunbin Dong Liang Han Qingyong Ma Zheng Wu Zheng Wang |
| author_sort | Wei Li |
| collection | DOAJ |
| description | Abstract Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi’an Jiaotong University. The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23–51). The average time from onset of symptoms to diagnosis of glucagonoma was 14 months (range 2–36 months). All the patients visited dermatology first for necrolytic migratory erythema (NME) 7/7 (100%), and other presenting symptoms included diabetes mellitus (DM) 4/7 (57%), stomatitis 2/7 (28%), weight loss 4/7 (57%), anemia 4/7 (57%), diarrhea 1/7 (14%), and DVT1/7 (14%). Plasma glucagon levels were increased in all patients (range 216.92–3155 pg/mL) and declined after surgery. Imaging studies revealed that four of seven patients had liver metastasis. Six of seven patients received surgical resection, and all of them received somatostatin analog therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Our data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma. Once diagnosed, surgery and somatostatin analogs are effective for symptom relief and tumor control. |
| first_indexed | 2024-12-12T09:25:05Z |
| format | Article |
| id | doaj.art-b5530acc9dcf4f0fbd994d3ce6140556 |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-12-12T09:25:05Z |
| publishDate | 2022-05-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-b5530acc9dcf4f0fbd994d3ce61405562022-12-22T00:29:03ZengNature PortfolioScientific Reports2045-23222022-05-011211710.1038/s41598-022-12882-2Necrolytic migratory erythema is an important visual cutaneous clue of glucagonomaWei Li0Xue Yang1Yuan Deng2Yina Jiang3Guiping Xu4Enxiao Li5Yinying Wu6Juan Ren7Zhenhua Ma8Shunbin Dong9Liang Han10Qingyong Ma11Zheng Wu12Zheng Wang13Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Pathology, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Pathology, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Radiology, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Medical Oncology, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Medical Oncology, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Radiotherapy Oncology, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityDepartment of Hepatobiliary Surgery, The First Affiliated Hospital of Xi’an Jiaotong UniversityAbstract Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi’an Jiaotong University. The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23–51). The average time from onset of symptoms to diagnosis of glucagonoma was 14 months (range 2–36 months). All the patients visited dermatology first for necrolytic migratory erythema (NME) 7/7 (100%), and other presenting symptoms included diabetes mellitus (DM) 4/7 (57%), stomatitis 2/7 (28%), weight loss 4/7 (57%), anemia 4/7 (57%), diarrhea 1/7 (14%), and DVT1/7 (14%). Plasma glucagon levels were increased in all patients (range 216.92–3155 pg/mL) and declined after surgery. Imaging studies revealed that four of seven patients had liver metastasis. Six of seven patients received surgical resection, and all of them received somatostatin analog therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Our data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma. Once diagnosed, surgery and somatostatin analogs are effective for symptom relief and tumor control.https://doi.org/10.1038/s41598-022-12882-2 |
| spellingShingle | Wei Li Xue Yang Yuan Deng Yina Jiang Guiping Xu Enxiao Li Yinying Wu Juan Ren Zhenhua Ma Shunbin Dong Liang Han Qingyong Ma Zheng Wu Zheng Wang Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma Scientific Reports |
| title | Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma |
| title_full | Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma |
| title_fullStr | Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma |
| title_full_unstemmed | Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma |
| title_short | Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma |
| title_sort | necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma |
| url | https://doi.org/10.1038/s41598-022-12882-2 |
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