Multiple Endocrine Neoplasia type 1 (MEN1) is a hereditary autosomal dominant syndrome clinically defined by the presence of at least two of the following endocrine neoplasias: parathyroid tumors, entero-pancreatic tumors and pituitary tumors. Primary Hyperparathyroidism (HPT) usually due to one or more adenomas of the parathyroid glands is the most frequent manifestation of MEN1 syndrome and often the first endocrinopathy to occur. The consequences of HPT become manifest at a young age with low bone mineral density and urolithiasis. Therefore, monitoring patients who have inherited the disease is mandatory in order to make a tempestive diagnosis and treatment of the HPT. Surgery is the treatment of choice. The surgical objective is the achievement of eucalcemia for as long as possible avoiding a permanent hypoparathyroidism.
The risk of persistence or recurrence of HPT in MEN1 is dramatically higher than in sporadic HPT. Several surgical options have been proposed: the removal only of the enlarged glands, the removal of at least 3 parathyroid glands preserving part of all the fourth gland, or the removal of all the parathyroid glands with autotransplant of pieces of fragments of the less involved parathyroid gland in the muscular or subcutaneous tissue. A concomitant cervical thymectomy is part of the major parathyroidectomies in order to remove any eventually ectopic or supernumerary parathyroid gland. The choice of these different surgical procedures is controversial and should be balanced between the risk of cervical reoperation and the disturbances of hypoparathyroidism.
The aim of this topic is to consider two aspects that have been little explored in the literature: the quality of life before and after surgery, and the long term HPT recurrence after the different types of parathyroidectomy.
Within this Research Topic, we encourage the submission of manuscripts related to the management of primary hyperparathyroidism in Multiple Endocrine Neoplasia type 1. This research topic includes, but is not limited to, the following areas:
- Choice of parathyroidectomy type in MEN1 HPT;
- Localization tests: which and why;
- Pharmacologic therapy of MEN1 HPT;
- Value of intraoperative dosage of PTH in parathyroidectomy for MEN1 HPT;
- Quality of life before and after parathyroidectomy;
- Bone involvement in MEN1 HPT.
Keywords:
Multiple Endocrine Neoplasia type 1, MEN1, Primary Hyperparathyroidism, HPT
Important Note:
All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.
Multiple Endocrine Neoplasia type 1 (MEN1) is a hereditary autosomal dominant syndrome clinically defined by the presence of at least two of the following endocrine neoplasias: parathyroid tumors, entero-pancreatic tumors and pituitary tumors. Primary Hyperparathyroidism (HPT) usually due to one or more adenomas of the parathyroid glands is the most frequent manifestation of MEN1 syndrome and often the first endocrinopathy to occur. The consequences of HPT become manifest at a young age with low bone mineral density and urolithiasis. Therefore, monitoring patients who have inherited the disease is mandatory in order to make a tempestive diagnosis and treatment of the HPT. Surgery is the treatment of choice. The surgical objective is the achievement of eucalcemia for as long as possible avoiding a permanent hypoparathyroidism.
The risk of persistence or recurrence of HPT in MEN1 is dramatically higher than in sporadic HPT. Several surgical options have been proposed: the removal only of the enlarged glands, the removal of at least 3 parathyroid glands preserving part of all the fourth gland, or the removal of all the parathyroid glands with autotransplant of pieces of fragments of the less involved parathyroid gland in the muscular or subcutaneous tissue. A concomitant cervical thymectomy is part of the major parathyroidectomies in order to remove any eventually ectopic or supernumerary parathyroid gland. The choice of these different surgical procedures is controversial and should be balanced between the risk of cervical reoperation and the disturbances of hypoparathyroidism.
The aim of this topic is to consider two aspects that have been little explored in the literature: the quality of life before and after surgery, and the long term HPT recurrence after the different types of parathyroidectomy.
Within this Research Topic, we encourage the submission of manuscripts related to the management of primary hyperparathyroidism in Multiple Endocrine Neoplasia type 1. This research topic includes, but is not limited to, the following areas:
- Choice of parathyroidectomy type in MEN1 HPT;
- Localization tests: which and why;
- Pharmacologic therapy of MEN1 HPT;
- Value of intraoperative dosage of PTH in parathyroidectomy for MEN1 HPT;
- Quality of life before and after parathyroidectomy;
- Bone involvement in MEN1 HPT.
Keywords:
Multiple Endocrine Neoplasia type 1, MEN1, Primary Hyperparathyroidism, HPT
Important Note:
All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.