Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding

Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neu...

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Main Authors: Randhir Sagar Yadav, Ashik Pokharel, Deepshikha Gaire, Shumneva Shrestha, Ashbita Pokharel, Sumita Pradhan, Prasan Bir Singh Kansakar
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Endocrinology
Online Access:http://dx.doi.org/10.1155/2021/8842667
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author Randhir Sagar Yadav
Ashik Pokharel
Deepshikha Gaire
Shumneva Shrestha
Ashbita Pokharel
Sumita Pradhan
Prasan Bir Singh Kansakar
author_facet Randhir Sagar Yadav
Ashik Pokharel
Deepshikha Gaire
Shumneva Shrestha
Ashbita Pokharel
Sumita Pradhan
Prasan Bir Singh Kansakar
author_sort Randhir Sagar Yadav
collection DOAJ
description Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neuroendocrine tumor. Malignant insulinoma is extremely rare, while primary hyperparathyroidism is a common occurrence in MEN1. We present a case of MEN1 syndrome with 2.6 cm insulinoma in the pancreatic head and parathyroid adenoma in a 56-year-old female who presented with symptoms suggestive of hypoglycemia like multiple episodes of loss of consciousness for four years. Classical pancreaticoduodenectomy was carried out, and the postoperative period was uneventful. Later, subtotal parathyroidectomy was performed, which showed parathyroid adenoma. Patients presenting with features of hypoglycemia should be vigilantly assessed for the presence of a sinister pathology.
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series Case Reports in Endocrinology
spelling doaj-art-fed34fcd93304f0fbb1c1b5f4a8adfd52025-02-03T01:08:58ZengWileyCase Reports in Endocrinology2090-65012090-651X2021-01-01202110.1155/2021/88426678842667Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare FindingRandhir Sagar Yadav0Ashik Pokharel1Deepshikha Gaire2Shumneva Shrestha3Ashbita Pokharel4Sumita Pradhan5Prasan Bir Singh Kansakar6Maharajgunj Medical Campus, Institute of Medicine, Tribhuvan University, Kathmandu, NepalDepartment of Gastrointestinal and General Surgery, Tribhuvan University Teaching Hospital, Kathmandu, NepalDepartment of Pathology, Tribhuvan University Teaching Hospital, Kathmandu, NepalMaharajgunj Medical Campus, Institute of Medicine, Tribhuvan University, Kathmandu, NepalMaharajgunj Medical Campus, Institute of Medicine, Tribhuvan University, Kathmandu, NepalDepartment of Gastrointestinal and General Surgery, Tribhuvan University Teaching Hospital, Kathmandu, NepalDepartment of Gastrointestinal and General Surgery, Tribhuvan University Teaching Hospital, Kathmandu, NepalMultiple endocrine neoplasia type 1 (MEN1) is a rare syndrome of autosomal dominant inheritance defined by co-occurrence of two or more tumors originating from the parathyroid gland, pancreatic islet cells, and/or anterior pituitary. Insulinoma which has an incidence of 0.4% is a rare pancreatic neuroendocrine tumor. Malignant insulinoma is extremely rare, while primary hyperparathyroidism is a common occurrence in MEN1. We present a case of MEN1 syndrome with 2.6 cm insulinoma in the pancreatic head and parathyroid adenoma in a 56-year-old female who presented with symptoms suggestive of hypoglycemia like multiple episodes of loss of consciousness for four years. Classical pancreaticoduodenectomy was carried out, and the postoperative period was uneventful. Later, subtotal parathyroidectomy was performed, which showed parathyroid adenoma. Patients presenting with features of hypoglycemia should be vigilantly assessed for the presence of a sinister pathology.http://dx.doi.org/10.1155/2021/8842667
spellingShingle Randhir Sagar Yadav
Ashik Pokharel
Deepshikha Gaire
Shumneva Shrestha
Ashbita Pokharel
Sumita Pradhan
Prasan Bir Singh Kansakar
Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding
Case Reports in Endocrinology
title Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding
title_full Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding
title_fullStr Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding
title_full_unstemmed Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding
title_short Multiple Endocrine Neoplasia Type 1 with Concomitant Existence of Malignant Insulinoma: A Rare Finding
title_sort multiple endocrine neoplasia type 1 with concomitant existence of malignant insulinoma a rare finding
url http://dx.doi.org/10.1155/2021/8842667
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