GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1
A male patient with a germline mutation in MEN1 presented at the age of 18 with classical features of gigantism. Previously, he had undergone resection of an insulin-secreting pancreatic neuroendocrine tumour (pNET) at the age of 10 years and had subtotal parathyroidectomy due to primary hyperparath...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Bioscientifica
2021-06-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
| Online Access: | https://edm.bioscientifica.com/view/journals/edm/2021/1/EDM20-0208.xml |
| _version_ | 1828960388096458752 |
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| author | Vinaya Srirangam Nadhamuni Donato Iacovazzo Jane Evanson Anju Sahdev Jacqueline Trouillas Lorraine McAndrew Tom R Kurzawinski David Bryant Khalid Hussain Satya Bhattacharya Márta Korbonits |
| author_facet | Vinaya Srirangam Nadhamuni Donato Iacovazzo Jane Evanson Anju Sahdev Jacqueline Trouillas Lorraine McAndrew Tom R Kurzawinski David Bryant Khalid Hussain Satya Bhattacharya Márta Korbonits |
| author_sort | Vinaya Srirangam Nadhamuni |
| collection | DOAJ |
| description | A male patient with a germline mutation in MEN1 presented at the age of 18 with classical features of gigantism. Previously, he had undergone resection of an insulin-secreting pancreatic neuroendocrine tumour (pNET) at the age of 10 years and had subtotal parathyroidectomy due to primary hyperparathyroidism at the age of 15 years. He was found to have significantly elevated serum IGF-1, GH, GHRH and calcitonin levels. Pituitary MRI showed an overall bulky gland with a 3 mm hypoechoic area. Abdominal MRI showed a 27 mm mass in the head of the pancreas and a 6 mm lesion in the tail. Lanreotide-Autogel 120 mg/month reduced GHRH by 45% and IGF-1 by 20%. Following pancreaticoduodenectomy, four NETs were identified with positive GHRH and calcitonin staining and Ki-67 index of 2% in the largest lesion. The pancreas tail lesion was not removed. Post-operatively, GHRH and calcitonin levels were undetectable, IGF-1 levels normalised and GH suppressed normally on glucose challenge. Post-operative fasting glucose and HbA1c levels have remained normal at the last check-up. While adolescent-onset cases of GHRH-secreting pNETs have been described, to the best of our knowledge, this is the first reported case of ectopic GHRH in a paediatric setting leading to gigantism in a patient with MEN1. Our case highlights the importance of distinguishing between pituitary and ectopic causes of gigantism, especially in the setting of MEN1, where paediatric somatotroph adenomas causing gigantism are extremely rare. |
| first_indexed | 2024-12-14T09:26:26Z |
| format | Article |
| id | doaj.art-7583af6b1091489fb6d38735ee931edf |
| institution | Directory Open Access Journal |
| issn | 2052-0573 2052-0573 |
| language | English |
| last_indexed | 2024-12-14T09:26:26Z |
| publishDate | 2021-06-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj.art-7583af6b1091489fb6d38735ee931edf2022-12-21T23:08:11ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732052-05732021-06-01111810.1530/EDM-20-0208GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1Vinaya Srirangam Nadhamuni0Donato Iacovazzo1Jane Evanson2Anju Sahdev3Jacqueline Trouillas4Lorraine McAndrew5Tom R Kurzawinski6David Bryant7Khalid Hussain8Satya Bhattacharya9Márta Korbonits10Department of Endocrinology, Barts and the London School of Medicine and Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UKDepartment of Endocrinology, Barts and the London School of Medicine and Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UKSt. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UKSt. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UKDepartment of Pathology, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, FranceSt. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UKDivision of Endocrine Surgery, University College Hospital, London, UKSunderland Royal Hospital, South Tyneside and Sunderland NHS Foundation Trust, South Shields, Tyne and Wear, UKDivision of Endocrinology, Sidra Medicine, Doha, Ad Dawhah, QatarSt. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UKDepartment of Endocrinology, Barts and the London School of Medicine and Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UKA male patient with a germline mutation in MEN1 presented at the age of 18 with classical features of gigantism. Previously, he had undergone resection of an insulin-secreting pancreatic neuroendocrine tumour (pNET) at the age of 10 years and had subtotal parathyroidectomy due to primary hyperparathyroidism at the age of 15 years. He was found to have significantly elevated serum IGF-1, GH, GHRH and calcitonin levels. Pituitary MRI showed an overall bulky gland with a 3 mm hypoechoic area. Abdominal MRI showed a 27 mm mass in the head of the pancreas and a 6 mm lesion in the tail. Lanreotide-Autogel 120 mg/month reduced GHRH by 45% and IGF-1 by 20%. Following pancreaticoduodenectomy, four NETs were identified with positive GHRH and calcitonin staining and Ki-67 index of 2% in the largest lesion. The pancreas tail lesion was not removed. Post-operatively, GHRH and calcitonin levels were undetectable, IGF-1 levels normalised and GH suppressed normally on glucose challenge. Post-operative fasting glucose and HbA1c levels have remained normal at the last check-up. While adolescent-onset cases of GHRH-secreting pNETs have been described, to the best of our knowledge, this is the first reported case of ectopic GHRH in a paediatric setting leading to gigantism in a patient with MEN1. Our case highlights the importance of distinguishing between pituitary and ectopic causes of gigantism, especially in the setting of MEN1, where paediatric somatotroph adenomas causing gigantism are extremely rare.https://edm.bioscientifica.com/view/journals/edm/2021/1/EDM20-0208.xml |
| spellingShingle | Vinaya Srirangam Nadhamuni Donato Iacovazzo Jane Evanson Anju Sahdev Jacqueline Trouillas Lorraine McAndrew Tom R Kurzawinski David Bryant Khalid Hussain Satya Bhattacharya Márta Korbonits GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1 Endocrinology, Diabetes & Metabolism Case Reports |
| title | GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1 |
| title_full | GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1 |
| title_fullStr | GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1 |
| title_full_unstemmed | GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1 |
| title_short | GHRH secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with MEN1 |
| title_sort | ghrh secretion from a pancreatic neuroendocrine tumor causing gigantism in a patient with men1 |
| url | https://edm.bioscientifica.com/view/journals/edm/2021/1/EDM20-0208.xml |
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