Endocrine Surgical Aspects of Multiple Endocrine Neoplasia Syndromes in Children

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Background: All patients diagnosed with medullary thyroid carcinoma (MTC) should undergo RET mutation analysis to exclude familial disease – multiple endocrine neoplasia (MEN)-2A and -2B and familial medullary thyroid carcinoma (FMTC). In young patients at risk of genetically determined MTC, the key to a good outcome is an appropriate first operation, and this will depend upon the codon mutation, patient age, calcitonin level and disease extent at presentation. When MTC has already developed, a therapeutic intervention is required. Conclusions: The thyroid, pituitary, adrenal, parathyroid and pancreatic components of MEN-1 and -2 require close collaboration of a specialist and experienced multidisciplinary team.

Related collections

Most cited references 10

Record: found
Abstract: found
Article: not found

Early malignant progression of hereditary medullary thyroid cancer.

Andreas Machens, Josef Hoegel, … (2003)

An age-related progression from C-cell hyperplasia to medullary thyroid carcinoma is associated with various germ-line mutations in the rearranged during transfection (RET) proto-oncogene that could be used to identify the optimal time for prophylactic surgery. In this European multicenter study conducted from July 1993 to February 2001, we enrolled patients who had a RET point mutation in the germ line, were 20 years of age or younger, were asymptomatic, and had undergone total thyroidectomy after confirmation of the RET mutation. Exclusion criteria were medullary thyroid carcinomas of more than 10 mm in greatest dimension and distant metastasis. Altogether, 207 patients from 145 families were identified. There was a significant age-related progression from C-cell hyperplasia to medullary thyroid carcinoma and, ultimately, nodal metastasis in patients whose RET mutations were grouped according to the extracellular- and intracellular-domain codons affected and in those with the codon 634 genotype. No lymph-node metastases were noted in patients younger than 14 years of age. The age-related penetrance was unaffected by the type of amino acid substitution encoded by the various codon 634 mutations. The codon-specific differences in the age at presentation of cancer and the familial rates of concomitant adrenal and parathyroid involvement suggest that the risk of progression was based on the transforming potential of the individual RET mutation. These data provide initial guidelines for the timing of prophylactic thyroidectomy in asymptomatic carriers of RET gene mutations. Copyright 2003 Massachusetts Medical Society

0 comments Cited 62 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Rationale for central and bilateral lymph node dissection in sporadic and hereditary medullary thyroid cancer.

Sophie Leboulleux, N Bellon, Eric Baudin … (2003)

A retrospective study was performed on 101 consecutive medullary thyroid cancer (MTC) patients who underwent at Institut Gustave-Roussy (IGR) total thyroidectomy with central and bilateral lymph node dissection. At histology, lymph node metastases were found in 55% of patients. In sporadic MTC, lymph node metastases were observed in the central compartment in 50% of patients, in the ipsilateral jugulocarotid chain in 57%, and in the contralateral jugulocarotid chain in 28%. In hereditary MTC, lymph node metastases were identified in the central compartment in 45% of patients, in the ipsilateral jugulocarotid chain in 36%, and in the contralateral jugulocarotid chain in 19%. Contralateral lymph nodes were found in 37% of metastatic patients with an unilateral tumoral involvement of the thyroid gland. A strong association was observed between tumor size and lymph node involvement for both hereditary and sporadic MTC (P < 0.02). Permanent hypoparathyroidism occurred in 4% of patients and laryngeal nerve palsy in 5%. An undetectable calcitonin level was obtained after surgery in 61% of patients, in 95% of patients without lymph node metastases, and in 32% of patients with lymph node metastases. Among patients with lymph node involvement, undetectable calcitonin level was obtained in 57% of patients with less than or with 10 lymph node metastases and in 4% of patients with more than 10 (P < 0.01). 1) lymph node metastases occur early in the course of MTC; 2) the pattern of lymph node metastatic distribution in neck areas varied between patients and was not related to the thyroid tumor size; 3) contralateral lymph node metastases were observed even in patients with small thyroid tumor; 4) total thyroidectomy with central and complete bilateral neck dissection should be performed routinely in all patients with sporadic and hereditary MTC, even in those with small thyroid tumors-a contralateral neck dissection may be avoided only in sporadic MTC patients with unilateral involvement of the thyroid gland in the absence of central and ipsilateral neck involvement; and 5) the number of lymph node metastases was predictive of biological cure after surgery.

0 comments Cited 50 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Imaging and localization of islet-cell tumours of the pancreas on CT and MRI.

Tara Noone, Jason Hosey, Zeynep Firat … (2005)

Islet-cell tumours are neuroendocrine tumours that arise from the endocrine pancreas. They may be associated with a variety of syndromes and are subclassified into functioning and non-functioning tumours. They range from benign to malignant. They demonstrate characteristic features when imaged with both computed tomography (CT) and magnetic resonance imaging (MRI). Sensitivity and specificity, as well as detection of extrapancreatic extension, are generally superior with MRI. However, CT is currently still more readily available to patients. Multiphase, post-contrast series are commended for the evaluation of islet-cell tumours with either modality.

0 comments Cited 27 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Journal

Journal ID (publisher-id): HRE

Journal ID (nlm-ta): Horm Res Paediatr

Journal ID (doi): 10.1159/issn.1663-2818

Title: Hormone Research in Paediatrics

Publisher: S. Karger AG

ISBN: 978-3-8055-8475-3

ISBN: 978-3-8055-8476-0

ISSN (Print): 1663-2818

ISSN (Electronic): 1663-2826

Publication date Subscription-year: 2007

Publication date (Print): December 2007

Publication date (Electronic): 10 December 2007

Volume: 68

Issue: Suppl 5

Pages: 105-106

Affiliations

Consultant Endocrine Surgeon, Royal Hallamshire Hospital, Sheffield, UK

Article

Publisher ID: 110590 Other ID: Horm Res 2007;68:105–106

DOI: 10.1159/000110590

PubMed ID: 18174722

SO-VID: a41bc49c-46b5-485c-a0f5-b343c94ae415

License:

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Endocrine Surgical Aspects of Multiple Endocrine Neoplasia Syndromes in Children

Read this article at

Abstract

Related collections

Karger: Endocrinology

Most cited references 10

Early malignant progression of hereditary medullary thyroid cancer.

Rationale for central and bilateral lymph node dissection in sporadic and hereditary medullary thyroid cancer.

Imaging and localization of islet-cell tumours of the pancreas on CT and MRI.

Author and article information

Journal

Affiliations

Article

History

Page count

Categories

Comments

Comment on this article

Similar content 76

Most referenced authors 149