scholarly journals Medullary thyroid carcinoma: recent advances in identification, treatment, and prognosis

2021 ◽  
Vol 12 ◽  
pp. 204201882110496
Author(s):  
Marisa A. Bartz-Kurycki ◽  
Omowunmi E. Oluwo ◽  
Lilah F. Morris-Wiseman
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Distant Metastases ◽  
Hereditary Disease ◽  
Patient Specific ◽  
Medullary Thyroid ◽  
Recent Advances ◽  
Initial Surgery ◽  
Biochemical Testing ◽  
Thyroid Malignancies

Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor that represents <5% of all thyroid malignancies and is generally more aggressive than differentiated thyroid cancer. The aim of this study is to provide an update, through review of clinical studies of patients with MTC published between January 1, 2016, and June 1, 2021, on recent advances in the diagnosis and treatment of MTC. This review focuses on updates in biochemical testing, imaging, hereditary disease, surgical management, adjuvant therapies, and prognosis. Recent advances reviewed herein have sought to diagnose MTC at earlier stages of disease, predict when patients with a hereditary syndrome may develop MTC, use functional imaging to assess for distant metastases, perform optimal initial surgery with appropriate lymphadenectomy, employ targeted systemic therapies for patients with progressive metastatic disease, and better predict patient-specific outcomes.

131I-MIBG as a treatment in medullary thyroid carcinoma with distant metastases.

2018 ◽  
Author(s):  
Nerea Utrilla Uriarte ◽  
Pedro Gonzalez Fernandez ◽  
Alba Esteban Figueruelo ◽  
Marina Nevares Herrero ◽  
Javier Santamaria Sandi
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Distant Metastases ◽  
Medullary Thyroid ◽  
131I Mibg

scholarly journals A Review of the Significance in Measuring Preoperative and Postoperative Carcinoembryonic Antigen (CEA) Values in Patients with Medullary Thyroid Carcinoma (MTC)

Medicina ◽  
2021 ◽  
Vol 57 (6) ◽  
pp. 609
Author(s):  
Ioannis Passos ◽  
Elisavet Stefanidou ◽  
Soultana Meditskou-Eythymiadou ◽  
Maria Mironidou-Tzouveleki ◽  
Vasiliki Manaki ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Carcinoembryonic Antigen ◽  
Medullary Thyroid Carcinoma ◽  
Clinical Significance ◽  
Relevant Literature ◽  
Distant Metastases ◽  
Lymph Node Involvement ◽  
C Cells ◽  
Gastrointestinal Malignancies ◽  
Medullary Thyroid

Background and Objectives: Medullary thyroid carcinoma (MTC) accounts for 1–2% of all thyroid malignancies, and it originates from parafollicular “C” cells. Carcinoembryonic antigen (CEA) is a tumor marker, mainly for gastrointestinal malignancies. There are references in literature where elevated CEA levels may be the first finding in MTC. The aim of this study is to determine the importance of measuring preoperative and postoperative CEA values in patients with MTC and to define the clinical significance of the correlation between CEA and the origin of C cells. Materials and Methods: The existing and relevant literature was reviewed by searching for articles and specific keywords in the scientific databases of PubMedCentraland Google Scholar (till December 2020). Results: CEA has found its place, especially at the preoperative level, in the diagnostic approach of MTC. Preoperative CEA values >30 ng/mL indicate extra-thyroid disease, while CEA values >100 ng/mL are associated with lymph node involvement and distant metastases. The increase in CEA values preoperatively is associated with larger size of primary tumor, presence of lymph nodes, distant metastases and a poorer prognosis. The clinical significance of CEA values for the surgeon is the optimal planning of surgical treatment. In the recent literature, C cells seem to originate from the endoderm of the primitive anterior gut at the ultimobranchial bodies’ level. Conclusions: Although CEA is not a specific biomarker of the disease in MTC, itsmeasurement is useful in assessing the progression of the disease. The embryonic origin of C cells could explain the increased CEA values in MTC.

Medullary thyroid carcinoma

2011 ◽  
pp. 625-630
Author(s):  
Friedhelm Raue ◽  
Karin Frank-Raue
Keyword(s):  
Sex Ratio ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Hereditary Disease ◽  
C Cells ◽  
Biochemical Screening ◽  
Medullary Thyroid ◽  
Histological Features ◽  
Sporadic Disease ◽  
Male To Female

Medullary thyroid carcinoma (MTC) is a rare calcitonin-secreting tumour of the parafollicular or C cells of the thyroid. As the C cells originate from the embryonic neural crest, MTC often have the clinical and histological features of neuroendocrine tumours. They account for 8–12% of all thyroid carcinomas and occur in both sporadic and hereditary forms (1). The majority of patients have sporadic MTC (70%), while 30% have hereditary MTC. The sex ratio in sporadic MTC is 1:1.3 (male to female), while both sexes are nearly equally affected in the familial variety (2). The highest incidence of sporadic disease occurs in the fifth decade of life, while hereditary disease can be diagnosed earlier, depending on the possibility of genetic and biochemical screening.

scholarly journals Polymorphisms of cell cycle control genes influence the development of sporadic medullary thyroid carcinoma

2014 ◽  
Vol 171 (6) ◽  
pp. 761-767 ◽  
Author(s):  
R B Barbieri ◽  
N E Bufalo ◽  
R Secolin ◽  
L V M Assumpção ◽  
R M B Maciel ◽  
...  
Keyword(s):  
Cell Cycle ◽  
Regression Analysis ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Cell Cycle Control ◽  
Multivariate Logistic Regression Analysis ◽  
Distant Metastases ◽  
Genetic Profile ◽  
Sporadic Medullary Thyroid Carcinoma ◽  
Medullary Thyroid

BackgroundThe role of key cell cycle regulation genes such as, CDKN1B, CDKN2A, CDKN2B, and CDKN2C in sporadic medullary thyroid carcinoma (s-MTC) is still largely unknown.MethodsIn order to evaluate the influence of inherited polymorphisms of these genes on the pathogenesis of s-MTC, we used TaqMan SNP genotyping to examine 45 s-MTC patients carefully matched with 98 controls.ResultsA multivariate logistic regression analysis demonstrated that CDKN1B and CDKN2A genes were related to s-MTC susceptibility. The rs2066827*GT+GG CDKN1B genotype was more frequent in s-MTC patients (62.22%) than in controls (40.21%), increasing the susceptibility to s-MTC (OR=2.47; 95% CI=1.048–5.833; P=0.038). By contrast, the rs11515*CG+GG of CDKN2A gene was more frequent in the controls (32.65%) than in patients (15.56%), reducing the risk for s-MTC (OR=0.174; 95% CI=0.048–0.627; P=0.0075). A stepwise regression analysis indicated that two genotypes together could explain 11% of the total s-MTC risk. In addition, a relationship was found between disease progression and the presence of alterations in the CDKN1A (rs1801270), CDKN2C (rs12885), and CDKN2B (rs1063192) genes. WT rs1801270 CDKN1A patients presented extrathyroidal tumor extension more frequently (92%) than polymorphic CDKN1A rs1801270 patients (50%; P=0.0376). Patients with the WT CDKN2C gene (rs12885) presented larger tumors (2.9±1.8 cm) than polymorphic patients (1.5±0.7 cm; P=0.0324). On the other hand, patients with the polymorphic CDKN2B gene (rs1063192) presented distant metastases (36.3%; P=0.0261).ConclusionIn summary, we demonstrated that CDKN1B and CDKN2A genes are associated with susceptibility, whereas the inherited genetic profile of CDKN1A, CDKN2B, and CDKN2C is associated with aggressive features of tumors. This study suggests that profiling cell cycle genes may help define the risk and characterize s-MTC aggressiveness.

scholarly journals Medullary Thyroid Carcinoma with Micronodular Lung Metastases: A Case Report with an Emphasis on the Imaging Findings

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Nina Ventura ◽  
Edson Marchiori ◽  
Gláucia Zanetti ◽  
Antonio Muccillo ◽  
Mariana Leite Pereira ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Medullary Thyroid Cancer ◽  
Lung Metastases ◽  
Disease Process ◽  
Distant Metastases ◽  
Diagnostic Tools ◽  
Case Discussion ◽  
Medullary Thyroid ◽  
Rare Malignancy

Medullary thyroid carcinoma is a rare malignancy that arises from calcitonin-producing C-cells and frequently metastasizes to lymph nodes in the neck. Distant metastases may involve bone, lung, and liver. The infrequent number of cases limits the clinical nature and ability to optimize diagnostic tools. Here, we present a case of a micronodular radiographic pattern in metastatic medullary thyroid cancer in order to enhance awareness of the disease process. A case discussion and relevant review of the literature are provided.

scholarly journals Radioactive iodine in the treatment of medullary thyroid carcinoma: a controlled multicenter study

2013 ◽  
Vol 168 (5) ◽  
pp. 779-786 ◽  
Author(s):  
J A A Meijer ◽  
L E H Bakker ◽  
G D Valk ◽  
W W de Herder ◽  
J H W de Wilt ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Multicenter Study ◽  
Radioactive Iodine ◽  
Disease Free Survival ◽  
Distant Metastases ◽  
Medullary Thyroid ◽  
Clinical Appearance ◽  
Significant Difference

ObjectiveRadioactive iodine (RAI) therapy in medullary thyroid carcinoma (MTC) is applied in some centers, based on the assumption that cross-irradiation from thyroid follicular cells may be beneficial. However, no systematic studies on the effect of RAI treatment in MTC have been performed. The aim of this study was to analyze the effect of RAI treatment on survival in MTC patients.DesignRetrospective multicenter study in eight University Medical Centers in The Netherlands.MethodsTwo hundred and ninety three MTC patients without distant metastases who had undergone a total thyroidectomy were included between 1980 and 2007. Patients were stratified by clinical appearance, hereditary stage, screening status, and localization. All patients underwent regular surgical treatment with additional RAI treatment in 61 patients. Main outcome measures were disease-free survival (DFS) and disease-specific survival (DSS). Cure was defined as biochemical and radiological absence of disease.ResultsIn multivariate analysis, stratification according to clinical appearance (P=0.72), hereditary stage (P=0.96), localization (P=0.69), and screening status (P=0.31) revealed no significant effects of RAI treatment on DFS. Multivariate analysis showed no significant difference in DSS for the two groups stratified according to clinical appearance (P=0.14). Owing to limited number of events, multivariate analysis was not possible for DSS in the other groups of stratification.ConclusionsBased on the results of the present analysis, we conclude that RAI has no place in the treatment of MTC.

scholarly journals Medullary thyroid carcinoma and pregnancy

2021 ◽  
Vol 16 (S3) ◽  
pp. 6-9
Author(s):  
Andreea Elena DUMITRU ◽  
◽  
Corina GICĂ ◽  
Anca Marina CIOBANU ◽  
Brînduşa Ana CIMPOCA-RAPTIS ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Relevant Information ◽  
Needle Aspiration ◽  
Hereditary Disease ◽  
Thyroid Tumor ◽  
Medullary Thyroid ◽  
Ultrasound Evaluation ◽  
Regional Metastases

Medullary thyroid carcinoma (MTC) is a rare neuroendocrine tumor of the parafollicular cells of the thyroid gland. The tumor occurs, either sporadically or in a hereditary form, as a component of the type 2 multiple endocrine neoplasia (MEN) syndromes, MEN2A and MEN2B, both with an autosomal dominant transmission. Due to its higher aggressiveness and urgency to treat, it poses a challenge when diagnosed during pregnancy. We performed a literature review searching medical databases as UpToDate and PubMed for relevant information about diagnosis, impact, treatment, and outcome of medullary thyroid carcinoma during pregnancy. Diagnosis of MTC is established by clinical and ultrasound evaluation, followed by a fine needle aspiration biopsy. Further analyses decide whether it is a sporadic or a hereditary disease. Surgery offers the curative solution, but careful postoperative follow-up is needed for the best outcome. The decision for surgery is taken considering the DNA mutation of RET oncogene and pregnancy status. A premature planned delivery is associated with potential complications for the newborn, but it is recommended when maternal status worsens. Conclusions. Complete resection of the thyroid tumor and any local and regional metastases is the only curative solution. Timing of the intervention is extremely important to decrease fetal outcomes. Close follow-up during the postoperative period, by both biochemical and imaging methods, is needed.

scholarly journals The rare intracellular RET mutation p.Ser891Ala in an apparently sporadic medullary thyroid carcinoma: a case report and review of the literature

2012 ◽  
Vol 56 (8) ◽  
pp. 586-591 ◽  
Author(s):  
Carla Brauner Blom ◽  
Lucieli Ceolin ◽  
Mirian Romitti ◽  
Débora Siqueira ◽  
Ana Luiza Maia
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Molecular Analysis ◽  
Hereditary Disease ◽  
C Cells ◽  
Inherited Disease ◽  
Review Of The Literature ◽  
Medullary Thyroid ◽  
Full Analysis ◽  
Characteristic Features

Medullary thyroid carcinoma (MTC) is a malignant tumor originating from parafollicular C-cells and accounts for 4-10% of all thyroid carcinomas. MTC develops in either sporadic (75%) or hereditary form (25%). Mutations in the RET proto-oncogene are responsible for hereditary MTC and the rate of heritable disease among apparently sporadic MTC (sMTC) cases varies from 6 to 15%. RET genetic testing is now considered fundamental in MTC management but the extent of the molecular analysis required to exclude inherited disease is still controversial. While the screening of all known mutation loci is recommended by some authors, the high costs associated with a full analysis should be also taken into consideration. Here, we illustrate and discuss this controversial issue by reporting a patient who present all characteristic features of sMTC, and in whom a standard genetic analysis by restriction enzyme restriction excluded hereditary disease. Nevertheless, an extensive molecular analysis that included all codons was prompted by the diagnosis of thyroid neoplasm in a patient's sister, and identified the rare intracellular RET p.Ser891Ala mutation. Arq Bras Endocrinol Metab. 2012;56(8):586-91

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