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...
Saved in:
Main Authors: | , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Wiley
2021-01-01
|
Series: | Case Reports in Endocrinology |
Online Access: | http://dx.doi.org/10.1155/2021/8842667 |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
_version_ | 1832565278798512128 |
---|---|
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. |
format | Article |
id | doaj-art-fed34fcd93304f0fbb1c1b5f4a8adfd5 |
institution | Kabale University |
issn | 2090-6501 2090-651X |
language | English |
publishDate | 2021-01-01 |
publisher | Wiley |
record_format | Article |
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 |
work_keys_str_mv | AT randhirsagaryadav multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding AT ashikpokharel multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding AT deepshikhagaire multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding AT shumnevashrestha multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding AT ashbitapokharel multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding AT sumitapradhan multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding AT prasanbirsinghkansakar multipleendocrineneoplasiatype1withconcomitantexistenceofmalignantinsulinomaararefinding |