Medullary Thyroid Cancer (medullary + thyroid_cancer)

Distribution by Scientific Domains


Selected Abstracts


Bilateral Breast Metastases of Medullary Thyroid Cancer

THE BREAST JOURNAL, Issue 2 2004
Makoto Ishitobi MD
No abstract is available for this article. [source]


Pretargeted radioimmunotherapy in rapidly progressing, metastatic, medullary thyroid cancer,

CANCER, Issue S4 2010
Françoise Kraeber-Bodéré MD
Abstract Medullary thyroid cancer (MTC) patients with localized residual disease and/or distant metastases may survive for several years or rapidly progress and die of their disease. Thus, highly reliable prognostic factors are needed for an early distinction between high-risk patients who need to be treated and low-risk patients who warrant a watch-and-wait approach. Calcitonin doubling time is an independent predictor of survival, with a high predictive value in a population of patients who have not normalized their calcitonin, even after repeated surgery. Several imaging methods should be proposed for patients with abnormal residual calcitonin levels persisting after complete surgery: ultrasonography and computed tomography (CT) for neck exploration, and CT for chest, abdomen, and pelvis. Magnetic resonance imaging (MRI) appears to have an advantage over CT for the detection of liver metastases from endocrine tumors. Moreover, MRI appears to be a sensitive imaging technique for detecting the spread of MTC to bone/bone marrow. 2-Fluoro-2-deoxy-D-glucose positron emission tomography/CT could be used for staging patients with progressive MTC, with possible prognostication by standard uptake value quantification. For systemic treatment of patients with rapidly progressing metastatic MTC, chemotherapy is not considered a valid therapeutic option. It is too early to evaluate the potential effectiveness of multikinase inhibitors, although interesting results of phase 2 studies have shown a transient stabilization in 30% to 50% of patients. Pretargeted radioimmunotherapy has been the only innovative treatment modality convincingly showing some survival benefit when compared with a historical untreated control group. Cancer 2010;116(4 suppl):1118,25. © 2010 American Cancer Society. [source]


Neurofibromatosis type 1: should we screen for other genetic syndromes?

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 9 2009
A case report of co-existence with multiple endocrine neoplasia 2A
Abstract Background, NF 1 is a genetic disorder with an autosomal dominant pattern of inheritence. It is associated with neoplastic disorders mainly derived from the neural seath. However, the co-existence of NF1 with the full spectrum of MEN 2A has rarely been reported. The aim of the study was to investigate the presence of secondary neoplasias in a patient with diagnosed NF1, and in particular the presence of hyperparathyroidism and the possible co-existence with another pheochromocytoma-related syndrome. Methods, We report a case of a 70 years old female patient who had NF1. The patient was referred to our center and was diagnosed with an isolated pheochromocytoma of the right adrenal gland for which she underwent right adrenalectomy. We further investigated for the presence of another pheochromocytoma-related syndrome and in particular for the presence of hyperparathyroidism and medullary thyroid cancer. Molecular screening for germline mutations of the genes NF1, RET and VHL has also been performed. Results, The patient was further diagnosed with hyperparathyroidism and medullary thyroid cancer, having the full spectrum of the clinical picture of the MEN2A syndrome. The genetic testing revealed the germline mutation for NF1 but not for the RET proto-oncogene which is generally found in MEN2A cases. Conclusion, To our knowledge this is a rare case of co-existence of two pheochromocytoma-related genetic syndromes, and generates the question of whether all patients with these syndromes should undergo a thorough clinical and laboratory investigation for the possibility of another co-existing pheochromocytoma-related genetic syndrome. [source]


NEW ERA: PROPHYLACTIC SURGERY FOR PATIENTS WITH MULTIPLE ENDOCRINE NEOPLASIA-2A

ANZ JOURNAL OF SURGERY, Issue 7 2006
Jessica E. Gosnell
Background: The surgical management of patients with multiple endocrine neoplasia-2A (MEN-2A) continues to evolve with specific genotype,phenotype correlations allowing for a more tailored approach. In this study, we report the surgical management of one of the largest MEN-2A families with a rearranged during transfection (RET) codon 804 mutation. Method: This is a cohort study comprising all at-risk kindred within a single known MEN-2A family. Prophylactic total thyroidectomy with lymph node dissection was recommended to all mutation carriers aged 5 years and older. Results: There were a total of 48 at-risk individuals in the MEN-2A kindred, with 22 patients undergoing thyroidectomy after appropriate preoperative evaluation. A total of 9 patients had medullary thyroid cancer including 5 with a normal preoperative calcitonin level. A total of 11 patients had C-cell hyperplasia and 7 showed histological evidence of parathyroid disease. Only the index case had a phaeochromocytoma. Conclusion: Genetic testing for germline mutations in the RET proto-oncogene has allowed precise identification of affected RET carriers and provided the opportunity for prophylactic or ,preclinical' surgery to treat and in fact to prevent medullary thyroid cancer. This concept of prophylactic surgery based on a genetic test is likely to be applied more widely as the tools of molecular biology advance. [source]


Mild hypercalcitoninaemia and sporadic thyroid disease,

BRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 5 2010
M. Cherenko
Background: Not operating on patients with mild hypercalcitoninaemia (MHCT) and sporadic thyroid disease carries the risk of omitting curative surgery for medullary thyroid cancer, but systematic surgery would result in unnecessary treatment of benign pathology. This study reviewed the management of MCHT and non-hereditary thyroid disease in one centre. Methods: MCHT was defined as an increase in basal and stimulated calcitonin levels not exceeding 30 and 200 pg/ml respectively. Over 15 years, 125 patients who presented with MCHT and sporadic thyroid disease were followed. Surgery was indicated only if there were local pressure symptoms or suspicious histomorphological changes in solitary nodules. Results: Fifty-five patients underwent total thyroidectomy and 18 unilateral total lobectomy. Histological examination revealed medullary microcarcinoma in six patients (two women and four men). C-cell hyperplasia was found in 54 patients (74 per cent) and 13 (18 per cent) harboured no C-cell pathology. Calcitonin levels stabilized after lobectomy and became undetectable following thyroidectomy. They normalized during follow-up in a third of patients who did not have surgery. Conclusion: Not all patients with MHCT and sporadic thyroid disease require surgery. Copyright © 2010 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd. [source]


Toxicity and efficacy of combined radioimmunotherapy and bevacizumab in a mouse model of medullary thyroid carcinoma,

CANCER, Issue S4 2010
Pierre-Yves Salaun MD
Abstract BACKGROUND: Significant antitumor effects were previously observed with radioimmunotherapy (RIT) using an anti-carcinoembryonic antigen (CEA) monoclonal antibody (F6) labeled with iodine-131 in medullary thyroid cancer (MTC)-bearing nude mice. Nevertheless, no complete response was achieved. Because angiogenesis is critical for tumor growth, bevacizumab is used to treat solid tumor in clinical practice. The present pilot study evaluated toxicity and efficacy of RIT combined with bevacizumab in mice subcutaneously grafted with TT MTC cells. METHODS: Groups of 4-6 nude mice were treated with 5 ,g/g bevacizumab twice weekly during 4 weeks and/or 100 MBq of 131I-F6. For combined therapy, bevacizumab was given at Day 0 followed by 131I-F6 at Day 30. The control group received no treatment. Animal weight, hematological toxicity, tumor volume, and serum calcitonin were monitored for 2 or 4 months. RESULTS: Bevacizumab alone induced no cytopenia and no significant weight loss. A weight loss of 12 ± 1% and 15 ± 2% was observed in mice treated by RIT alone or bevacizumab + RIT, respectively. RIT alone and combined treatment induced leukopenia and anemia. RIT alone and RIT plus bevacizumab induced tumor responses with minimum relative tumor volume of 0.38 ± 0.24 and 0.15 ± 0.07%, respectively, and time to progression of 35 ± 5 and 56 ± 11 days, respectively. CONCLUSIONS: Pretreatment with bevacizumab improved RIT efficacy, with similar toxicity as compared as RIT alone. Cancer 2010;116(4 suppl):1053,8. © 2010 American Cancer Society. [source]


Pretargeted radioimmunotherapy in rapidly progressing, metastatic, medullary thyroid cancer,

CANCER, Issue S4 2010
Françoise Kraeber-Bodéré MD
Abstract Medullary thyroid cancer (MTC) patients with localized residual disease and/or distant metastases may survive for several years or rapidly progress and die of their disease. Thus, highly reliable prognostic factors are needed for an early distinction between high-risk patients who need to be treated and low-risk patients who warrant a watch-and-wait approach. Calcitonin doubling time is an independent predictor of survival, with a high predictive value in a population of patients who have not normalized their calcitonin, even after repeated surgery. Several imaging methods should be proposed for patients with abnormal residual calcitonin levels persisting after complete surgery: ultrasonography and computed tomography (CT) for neck exploration, and CT for chest, abdomen, and pelvis. Magnetic resonance imaging (MRI) appears to have an advantage over CT for the detection of liver metastases from endocrine tumors. Moreover, MRI appears to be a sensitive imaging technique for detecting the spread of MTC to bone/bone marrow. 2-Fluoro-2-deoxy-D-glucose positron emission tomography/CT could be used for staging patients with progressive MTC, with possible prognostication by standard uptake value quantification. For systemic treatment of patients with rapidly progressing metastatic MTC, chemotherapy is not considered a valid therapeutic option. It is too early to evaluate the potential effectiveness of multikinase inhibitors, although interesting results of phase 2 studies have shown a transient stabilization in 30% to 50% of patients. Pretargeted radioimmunotherapy has been the only innovative treatment modality convincingly showing some survival benefit when compared with a historical untreated control group. Cancer 2010;116(4 suppl):1118,25. © 2010 American Cancer Society. [source]


Procalcitonin levels predict clinical course and progression-free survival in patients with medullary thyroid cancer

CANCER, Issue 1 2010
Martin A. Walter MD
Abstract BACKGROUND: Procalcitonin has been well established as an important marker of sepsis and systemic infection. The authors evaluated the diagnostic and predictive value of calcitonin and its prohormone procalcitonin in medullary thyroid cancer. METHODS: The authors systematically explored the ability of calcitonin and procalcitonin to identify medullary thyroid cancer and predict the endpoints local recurrence and distant metastases, as well as the progression-free survival. Patients with C-cell hyperplasia; patients after thyroidectomy for differentiated thyroid cancer, goiter, or Graves disease; and healthy subjects served as controls. The study was performed in accordance with the Reporting Recommendations for Tumor Marker Prognostic Studies of the National Cancer Institute. RESULTS: Sixty-nine medullary thyroid cancer patients and 96 controls were included (median observed interval: 10.9 years [range, 1.4-47.5 years]; 981.8 patient-years). The 1-year, 5-year, 10-year, and 20-year recurrence rates were 9%, 34%, 45%, and 56%, respectively. Calcitonin had a higher diagnostic accuracy for detecting medullary thyroid cancer than procalcitonin (area under the curve [AUC], 0.94; 95% confidence interval [95% CI], 0.90-0.99 vs AUC, 0.89; 95% CI, 0.83-0.95 [P = .038]). The procalcitonin:calcitonin ratio predicted disease progression (AUC, 0.63; 95% CI, 0.51-0.75 [P = .036]) and progression-free survival (hazards ratio, 1.49; 95% CI, 1.09-2.04 [P = .013]). CONCLUSIONS: The results of the current study indicate a superior diagnostic accuracy of calcitonin and an independent predictive value of the procalcitonin:calcitonin ratio. These findings may lead to improved diagnostic and therapeutic strategies for medullary thyroid cancer patients. Cancer 2010. © 2010 American Cancer Society. [source]


RET genotypes in sporadic medullary thyroid cancer: studies in a large Italian series

CLINICAL ENDOCRINOLOGY, Issue 3 2008
Laura Fugazzola
Summary Background, Highly discrepant data about the different distribution of RET germline single nucleotide polymorphisms (SNPs) among patients with sporadic medullary thyroid cancer (sMTC) and controls are available. Design and patients, In the present case-control study, a wide panel of seven RET SNPs has been tested in the largest sMTC series and in a matched control group. Results, None of the investigated polymorphisms show a significantly different distribution in patients with sMTC when compared to controls. Twenty haplotypes and 57 genotypes were generated, and their association with the disease and with the clinical features were statistically evaluated. Interestingly, 14 genotypes were found to be unique to sMTC patients and 25 to controls. Two haplotypes and three genotypes, all including the intronic variants IVS1-126 and IVS14-24, were significantly associated with sMTC patients and with a higher tumour aggression. The functional activity of the only nonsynonymous RET variant (c.2071C > A, G691S) was tested for the first time. Interestingly, Western blot analyses showed that the fraction of Ret9-G691S protein located at the plasma membrane level was overrepresented when compared to Ret9-WT, suggesting facilitated targeting at the cell membrane for this variant. However, no transforming activity was shown in a focus formation assay on cells carrying the Ret9-G691S, against a possible oncogenic role of G691S variant. Conclusions,RET genotypes including two intronic RET variants were associated with the risk of developing sMTC and to more aggressive behaviour. Further studies are warranted to elucidate whether these RET genotypes are in linkage disequilibrium with another susceptibility gene or whether these variants could play a role in the genesis of sMTC per se. [source]