PMID: 3697657Apr 1, 1986Paper

Familial medullary thyroid carcinoma without associated endocrinopathies: a distinct clinical entity

The British Journal of Surgery
J R FarndonS A Wells

Abstract

In an evaluation of 213 patients from 15 kindreds with familial medullary thyroid carcinoma (MTC), we detected 41 subjects from two kindreds (L and O) who had MTC but no extra-thyroidal manifestations (hyperparathyroidism, phaeochromocytomas or mucosal neuromas) of multiple endocrine neoplasia (MEN) type IIa or IIb. In screening 178 members of the L and O kindreds, we found no evidence that any of them had died from MTC. To assess whether the malignancy was relatively indolent in these families, 20 selected subjects from the two kindreds were compared with 33 MEN IIa subjects. Both groups had clinically occult disease which was diagnosed biochemically by documenting elevated plasma calcitonin (CT) levels following stimulation with intravenous calcium and pentagastrin. There were no differences in the peak stimulated plasma CT levels at the time of diagnosis (1055 +/- 236 pg/ml versus 1096 +/- 191 pg/ml) or the incidence of regional lymph node metastases (0/20 versus 1/33) in the two groups. The mean age at diagnosis, however, was significantly higher in patients of the L and O kindreds than in patients with MEN IIa (43.1 +/- 3.4 years versus 21.1 +/- 2.2 years; P less than 0.001) indicating that in the two kindreds the MTC eith...Continue Reading

References

Mar 1, 1979·The Journal of Clinical Endocrinology and Metabolism·S B BaylinM A Beaven
Mar 1, 1975·Cancer·G C ChongL H Woolner
Jun 1, 1973·Annals of Internal Medicine·C E JacksonM A Block
Sep 1, 1967·Annals of Surgery·M A BlockB E Brush
Jan 1, 1959·The Journal of Clinical Endocrinology and Metabolism·J B HAZARDG CRILE
Mar 1, 1962·Clinica Chimica Acta; International Journal of Clinical Chemistry·J J PISANOD ABRAHAM

❮ Previous
Next ❯

Citations

Jan 1, 1996·Journal of Surgical Oncology·Y MizukamiT Ishizaki
Mar 1, 1995·Genes, Chromosomes & Cancer·C EngM J Lorenzo
Mar 1, 1991·Seminars in Surgical Oncology·T C Lairmore, S A Wells
Jan 1, 1997·Pediatric Surgery International·T Kusafuka, P Puri
Jan 31, 2012·Current Oncology Reports·Alessandro AntonelliClodoveo Ferri
Oct 6, 2007·Endocrine Pathology·Andrea Z LaiLois M Mulligan
Apr 1, 1995·Cancer Genetics and Cytogenetics·L D CooleyR F Gagel
Feb 1, 1995·Critical Reviews in Oncology/hematology·Y MizukamiH Nakamura
Jul 11, 2001·Endocrinology and Metabolism Clinics of North America·E Puxeddu, J A Fagin
Nov 1, 1988·Baillière's Clinical Endocrinology and Metabolism·R V Thakker, B A Ponder
Jul 1, 1995·Baillière's Clinical Endocrinology and Metabolism·G J CoteR F Gagel
May 18, 2001·Surgical Oncology·N C Lee, J A Norton
Sep 29, 1994·The New England Journal of Medicine·C J LipsM J Berends
Sep 16, 2005·The New England Journal of Medicine·Michael A SkinnerSamuel A Wells
Feb 15, 1994·Proceedings of the National Academy of Sciences of the United States of America·K M CarlsonH Donis-Keller
Oct 1, 1995·Thyroid : Official Journal of the American Thyroid Association·D J MarshB G Robinson
Jul 21, 1998·Thyroid : Official Journal of the American Thyroid Association·M E GirelliB Busnardo
Dec 14, 2004·Thyroid : Official Journal of the American Thyroid Association·Yun Jae ChungJae Hoon Chung
Jul 21, 2005·Thyroid : Official Journal of the American Thyroid Association·Maria A KouvarakiDouglas B Evans
Feb 24, 2007·Thyroid : Official Journal of the American Thyroid Association·Giovanni PinnaStefano Mariotti
May 28, 2009·Thyroid : Official Journal of the American Thyroid Association·UNKNOWN American Thyroid Association Guidelines Task ForceSamuel A Wells
May 10, 2011·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Jessica Moline, Charis Eng
Jun 1, 1991·Clinical Endocrinology·J P FerrerE Vilardell
May 1, 1993·Clinical Endocrinology·S CasanovaC Houdent
Aug 1, 1996·European Journal of Clinical Investigation·J W Höppener, C J Lips
Sep 16, 2008·Pediatric Dermatology·Janete AlmeidaAdriana Aigotti Brandão
Nov 10, 2000·Journal of Medical Genetics·J R Hansford, L M Mulligan
Jun 9, 2000·Annual Review of Physiology·A O HoffR F Gagel
Feb 9, 2012·International Journal of Molecular Sciences·Lucieli CeolinAna Luiza Maia
Jun 22, 2014·Current Problems in Cancer·Amir H Lebastchi, Glenda G Callender
Dec 1, 1994·American Journal of Human Genetics·K M CarlsonP J Goodfellow
Feb 20, 2007·World Journal of Surgery·Y Nancy YouSamuel A Wells
Jan 12, 2008·World Journal of Surgery·Elizabeth FialkowskiJeffrey Moley
May 16, 2013·Current Problems in Surgery·Judy JinChristopher R McHenry
Sep 14, 2010·Best Practice & Research. Clinical Endocrinology & Metabolism·Nelson WohllkHartmut P H Neumann
Oct 20, 2009·The Surgical Clinics of North America·Göran Akerström, Peter Stålberg

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Polyendocrine Syndromes

This feed focuses on a rare genetic condition called Autoimmune Polyendocrine Syndromes, which are characterized by autoantibodies against multiple endocrine organs. This can lead to Type I Diabetes.