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Medullary thyroid cancer

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https://www.readbyqxmd.com/read/27904849/medullary-thyroid-carcinoma-a-30-year-experience-at-one-institution-in-korea
#1
Cho Rok Lee, Sohee Lee, Haiyoung Son, Eunjeong Ban, Sang-Wook Kang, Jandee Lee, Jong Ju Jeong, Kee-Hyun Nam, Woong Youn Chung, Cheong Soo Park
PURPOSE: The objective of this study was to review the clinical outcome and prognosis of patients with sporadic and hereditary medullary thyroid cancer (MTC) who were treated at a single tertiary hospital in Korea. METHODS: We retrospectively reviewed the case files of 85 patients treated from August 1982 to February 2012. RESULTS: In all, 65 patients (76.5%) had sporadic MTC and 20 patients (23.5%) had hereditary MTC. Patients in the sporadic group were older than in the hereditary group (P < 0...
December 2016: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/27900756/a-rare-case-of-medullary-carcinoma-thyroid-metastasizing-to-bilateral-breast-parenchyma
#2
Aamna Hassan, Maimoona Siddiqui, Sidra Jahangir
Medullary carcinoma of the thyroid (MTC) commonly spreads through the lymphatics to distant sites such as lung, liver and bone. Spread to the breast is rare. We report a case of metastatic MTC which progressed to develop nodal metastases to cer-vical and mediastinal regions, visceral metastases to the liver, lung and ultimately to bilateral breasts. Clinically it is important to distinguish metastatic breast lesions from primary breast cancer as each is managed differently. Both cytological and radio-logical investigations were done followed by excision biopsy...
2016: Nuclear Medicine Review. Central & Eastern Europe
https://www.readbyqxmd.com/read/27899191/genetic-predisposition-to-endocrine-tumors-diagnosis-surveillance-and-challenges-in-care
#3
REVIEW
Elisabeth Joye Petr, Tobias Else
Endocrine tumor syndromes, eg, multiple endocrine neoplasia types 1 and 2, were among the first recognized hereditary predisposition syndromes to tumor development. Over time, the number of endocrine tumor syndromes has significantly expanded, eg, with the recent inclusion of hereditary paraganglioma syndromes. Associations of non-endocrine tumors with hereditary endocrine tumor syndromes and endocrine tumors with non-classical endocrine tumor syndromes have emerged. These findings have certainly expanded the scope of care, necessitating a multidisciplinary approach by a team of medical professionals and researchers, integrating shared patient decision-making at every step of surveillance, diagnosis, and treatment...
October 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/27873108/flushing-in-neuro-endocrinology
#4
REVIEW
Fady Hannah-Shmouni, Constantine A Stratakis, Christian A Koch
Cutaneous flushing is a common presenting complaint in endocrine disorders. The pathophysiology of flushing involves changes in cutaneous blood flow triggered by multiple intrinsic factors that are either related to physiology or disease. Flushing can be divided into episodic or persistent causes. Episodic flushing is mediated by the release of endogenous vasoactive mediators or medications, while persistent flushing results in a fixed facial erythema with telangiectasia and cyanosis due to slow-flowing deoxygenated blood in large cutaneous blood vessels...
November 21, 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27872141/differential-roles-of-ret-isoforms-in-medullary-and-papillary-thyroid-carcinomas
#5
Eric Y Lian, Sarah M Maritan, Jessica G Cockburn, Katayoon Kasaian, Mathieu Jf Crupi, David Hurlbut, Steven Jones, Sam Wiseman, Lois M Mulligan
The RET receptor tyrosine kinase mediates cell proliferation, survival and migration in embryogenesis, and is implicated in transformation and tumour progression in multiple cancers. RET is frequently mutated and constitutively activated in familial and sporadic thyroid carcinomas. As a result of alternative splicing, RET is expressed as two protein isoforms, RET9 and RET51, which differ in their unique C-terminal amino acids. These isoforms have distinct intracellular trafficking and associated signaling complexes, but functional differences are not well defined...
November 21, 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27869424/-study-of-medullary-thyroid-carcinoma-from-a-proband
#6
Laura Morlán Herrador, Antonio de Arriba, Gloria Miguel, Marta Ferrera, José I Labarta
Thyroid cancer is an uncommon type of cancer, accounting less than 1% of all cancers in adults, and 0.5-3% of all cancers in children. There are four different types: papillary carcinoma (80-90% of cases), follicular (5-10%), medullary (5%) and anaplastic cell (2-3%). Eighty per cent of cases of medullary thyroid cancer are sporadic, but 20% are associated with an inherited syndrome that is divided into three groups: multiple endocrine neoplasia type 1, multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27856495/clinical-outcomes-of-a-cohort-of-patients-with-central-nervous-system-metastases-from-thyroid-cancer
#7
Joana Simões-Pereira, Daniel Macedo, Maria João Bugalho
INTRODUCTION: Metastases to central nervous system (M1-CNS) are rarely reported in thyroid cancer (TC) patients. We aimed to characterize patients with M1-CNS from TC followed in our department. METHODS: Review of the medical records of 27 patients with TC-related M1-CNS. RESULTS: Mean age at TC diagnosis was 56.9 ± 19.1 years. Papillary TC (55.6%) was the commonest histological type, followed by poorly differentiated (18.5%), medullary (11...
November 2016: Endocrine Connections
https://www.readbyqxmd.com/read/27843809/effect-of-an-outreach-programme-on-vandetanib-safety-in-medullary-thyroid-cancer
#8
Lars Bastholt, Michael C Kreissl, Dagmar Führer, Ana L Maia, Laura D Locati, Léa Maciel, Yi Wu, Kevin N Heller, Alan Webster, Rossella Elisei
OBJECTIVES: Effective management of adverse events (AEs) following vandetanib treatment is important to maximize clinical benefits. We examined whether more frequent contact with vandetanib-treated patients reduced AEs of CTCAE grade 2 or higher. STUDY DESIGN: In this open-label, multicentre, phase III study, patients with locally advanced or metastatic medullary thyroid cancer were randomized to a patient outreach programme (outreach) or a standard AE monitoring schedule (vandetanib control) for 52 weeks...
September 2016: European Thyroid Journal
https://www.readbyqxmd.com/read/27842913/trends-in-the-presentation-treatment-and-survival-of-patients-with-medullary-thyroid-cancer-over-the-past-30%C3%A2-years
#9
Reese W Randle, Courtney J Balentine, Glen E Leverson, Jeffrey A Havlena, Rebecca S Sippel, David F Schneider, Susan C Pitt
BACKGROUND: The impact of recent medical advances on disease presentation, extent of operation, and disease-specific survival for patients with medullary thyroid cancer is unclear. METHODS: We used the Surveillance, Epidemiology, and End Results registry to compare trends over 3 time periods, 1983-1992, 1993-2002, and 2003-2012. RESULTS: There were 2,940 patients diagnosed with medullary thyroid cancer between 1983 and 2012. The incidence of medullary thyroid cancer increased during this time period from 0...
November 11, 2016: Surgery
https://www.readbyqxmd.com/read/27837029/digitalis-like-compounds-facilitate-non-medullary-thyroid-cancer-redifferentiation-through-intracellular-ca2-fos-and-autophagy-dependent-pathways
#10
Marika H Tesselaar, Thomas Crezee, Herman G Swarts, Danny Gerrits, Otto C Boerman, Jan B Koenderink, Hendrik G Stunnenberg, Mihai G Netea, Jan Wa Smit, Romana T Netea-Maier, Theo S Plantinga
Up to 20-30% of patients with metastatic non-medullary thyroid cancer have persistent or recurrent disease resulting from tumour dedifferentiation. Tumour redifferentiation to restore sensitivity to radioactive iodide (RAI) therapy is considered a promising strategy to overcome RAI resistance. Autophagy has emerged as an important mechanism in cancer dedifferentiation. Here, we demonstrate the therapeutic potential of autophagy activators for redifferentiation of thyroid cancer cell lines. Five autophagy activating compounds, all known as digitalis-like compounds, restored hNIS expression and iodide uptake in TC cell lines...
November 11, 2016: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/27834132/surgical-management-of-medullary-thyroid-carcinoma-in-pediatric-age
#11
Claudio Spinelli, Leonardo Rossi, Silvia Strambi, Jessica Piscioneri, Rossella Elisei, Maura Massimino, Paolo Miccoli
Medullary thyroid carcinoma (MTC) is a rare, accounting for 5% of thyroid malignancies. It is a neuroendocrine tumor wich origins from thyroid parafollicular cells. It may be sporadic, mostly in adult patients, or inherited as autosomal dominant pattern, mostly in pediatric patients. As familial cancer, MTC may presented isolated as familial medullary thyroid carcinoma (FMTC) - 10% of cases - or, most often, as part of multiple endocrine neoplasm type 2 (MEN 2A or MEN 2B) syndromes - 90% of cases. The therapy for sporadic or hereditary MTC is surgical resection and consists in total thyroidectomy associated with central compartment lymph nodal dissection; the radicality of this intervention is fundamental to obtain a definitive cure...
November 11, 2016: Current Pediatric Reviews
https://www.readbyqxmd.com/read/27824322/68ga-dotanoc-focal-pulmonary-activity-with-no-corresponding-ct-abnormality
#12
Emmanouil Panagiotidis, Nagabhushan Seshadri, Rashika Fernando, Priya Healey, Sobhan Vinjamuri
A 28-year-old woman with medullary thyroid cancer treated with total thyroidectomy and bilateral central and right lateral neck nodal resection postoperatively had increasing calcitonin and carcinoembryonic antigen values. A Ga-DOTANOC PET/CT study performed showed tracer-avid disease in the neck, mediastinal, and hilar lymph nodes. However, there were 3 tracer-avid foci in the lungs bilaterally, suggestive of lung metastases but with no corresponding abnormalities on low-dose CT. A negative contrast-enhanced CT of the lungs along with clinical and radiological follow-up confirmed that the lung activity was artifactual, demonstrating the possibility of Ga-DOTANOC-avid iatrogenic pulmonary microembolism...
December 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27819766/safety-and-tolerability-of-vandetanib-in-japanese-patients-with-medullary-thyroid-cancer-a-phase-i-ii-open-label-study
#13
Keita Uchino, Masato Komoda, Junichi Tomomatsu, Takahiro Okamoto, Kiyomi Horiuchi, Akihito Tsuji, Yasuhiro Ito, Takuya Todo, Ki Rito, Shunji Takahashi
OBJECTIVE: In a Phase III trial, Western patients with medullary thyroid cancer (MTC) treated with the oral multikinase inhibitor vandetanib showed significantly improved progression-free survival (PFS) and objective response rate (ORR) compared with placebo. The biology of MTC and pharmacokinetics (PK) are similar for Japanese and Western patients; therefore, similar clinical benefit is anticipated in the Japanese population. This study evaluated the safety and tolerability of vandetanib in Japanese patients with unresectable locally advanced or metastatic MTC...
November 7, 2016: Endocrine Practice
https://www.readbyqxmd.com/read/27808580/recurrence-of-phaeochromocytoma-in-pregnancy-in-a-patient-with-multiple-endocrine-neoplasia-2a-a-case-report-and-review-of-literature
#14
Efterpi Tingi, Angelos Kyriacou, Lynda Verghese
Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant inherited condition with a prevalence of one in 40 000 individuals. It causes the development of tumours in endocrine glands, such as medullary thyroid cancer, pheochromocytomas, as well as primary hyperparathyroidism. MEN 2A in pregnancy is very rare with only 29 cases reported in the literature. The presence of pheochromocytoma is a rare cause of hypertension during pregnancy with an incidence of 0.007% of all pregnancies. This has severe implications on both mother and the foetus...
November 3, 2016: Gynecological Endocrinology
https://www.readbyqxmd.com/read/27807061/familial-non-medullary-thyroid-cancer-unraveling-the-genetic-maze
#15
REVIEW
Samantha Peiling Yang, Joanne Ngeow
Familial non-medullary thyroid cancer (FNMTC) constitutes 3-9% of all thyroid cancers. Out of all FNMTC cases, only 5% in the syndromic form has well-studied driver germline mutations. These associated syndromes include Cowden syndrome, familial adenomatous polyposis, Gardner syndrome, Carney complex type 1, Werner syndrome and DICER1 syndrome. It is important for the clinician to recognize these phenotypes so that genetic counseling and testing can be initiated to enable surveillance for associated malignancies and genetic testing of family members...
December 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27807060/m918v-ret-mutation-causes-familial-medullary-thyroid-carcinoma-study-of-8-affected-kindreds
#16
M Cecília Martins-Costa, Lucas L Cunha, Susan C Lindsey, Cleber P Camacho, Renata P Dotto, Gilberto K Furuzawa, M Sharmila A Sousa, Teresa S Kasamatsu, Ilda S Kunii, Márcio M Martins, Alberto L Machado, João R M Martins, Magnus R Dias-da-Silva, Rui M B Maciel
Germline mutations in codon 918 of exon 16 of the RET gene (M918T) are classically associated with multiple endocrine neoplasia type 2B (MEN 2B) with highly aggressive medullary thyroid cancer (MTC), pheochromocytoma and a unique phenotype. The objectives of this study are to describe the rare M918V RET mutation discovered in 8 MTC kindreds from Brazil lacking the MEN 2B phenotype classically observed in M918T patients and to investigate the presence of a founder effect for this germline mutation. Eight apparently sporadic MTC cases were diagnosed with the germline M918V RET mutation...
December 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27804856/a-papillary-thyroid-carcinoma-in-pediatric-age-an-example-of-a-rare-tumour-managed-within-a-cooperative-comprehensive-project
#17
Claudio Spinelli, Maura Massimino, Giovanna Sironi, Andrea Ferrari, Marta Podda, Stefano Chiaravalli
Though rare in childhood, thyroid cancers represent the most frequent tumours of endocrine glands in childhood and adolescence. Papillary thyroid carcinoma is a non-medullary, follicular-derived differentiated tumour of the thyroid. There is still controversy concerning the therapeutic approach of PTCs in the paediatric population; regarding which should be the extent of the surgical approach, and the need for radioactive iodine (RAI) therapy. The cooperative TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]) was launched in Italy in 2000 (under the patronage of AIEOP - Associazione Italiana Ematologia Oncologia Pediatrica) with a view to improving both research the clinical management on the less common paediatric cancers...
October 31, 2016: Current Pediatric Reviews
https://www.readbyqxmd.com/read/27799794/lenvatinib-and-other-tyrosine-kinase-inhibitors-for-the-treatment-of-radioiodine-refractory-advanced-and-progressive-thyroid-cancer
#18
REVIEW
Loredana Lorusso, Letizia Pieruzzi, Agnese Biagini, Elena Sabini, Laura Valerio, Carlotta Giani, Paolo Passannanti, Benedetta Pontillo-Contillo, Valentina Battaglia, Salvatore Mazzeo, Eleonora Molinaro, Rossella Elisei
Lenvatinib is a small oral molecule able to inhibit three of the extracellular and intracellular molecules involved in the modulation of angiogenesis and lymphangiogenesis: vascular endothelial growth factor receptor 1-3, fibroblast growth factor receptor 1-4, and platelet-derived growth factor receptor alpha. Since it is also able to inhibit the REarranged during Transfection oncogene and the protooncogene c-KIT, this drug can also be used to control tumor cell proliferation. The maximum tolerated dose, as demonstrated in Phase I studies, is 25 mg daily...
2016: OncoTargets and Therapy
https://www.readbyqxmd.com/read/27799362/animal-models-of-medullary-thyroid-cancer-state-of-the-art-and-view-to-the-future
#19
Giovanni Vitale, Germano Gaudenzi, Luisa Circelli, Marco Federico Manzoni, Andrea Bassi, Niccolò Fioritti, Antongiulio Faggiano, Annamaria Al Colao
Medullary thyroid carcinoma is a neuroendocrine tumour originating from parafollicular C cells accounting for 5-10% of thyroid cancers. Increased understanding of disease-specific molecular targets of therapy has led to the regulatory approval of two drugs (vandetanib and cabozantinib) for the treatment of medullary thyroid carcinoma. These drugs increase progression free survival, however, they are often poorly tolerated and most treatment responses are transient. Animal models are indispensable tools for investigating the pathogenesis, mechanisms for tumour invasion and metastasis, and new therapeutic approaches for cancer...
October 31, 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27798860/histone-deacetylase-inhibitors-a-novel-therapeutic-weapon-against-medullary-thyroid-cancer
#20
REVIEW
Christos Damaskos, Serena Valsami, Eleftherios Spartalis, Efstathios A Antoniou, Periklis Tomos, Stefanos Karamaroudis, Theofano Zoumpou, Vasilios Pergialiotis, Konstantinos Stergios, Constantinos Michaelides, Konstantinos Kontzoglou, Despina Perrea, Nikolaos Nikiteas, Dimitrios Dimitroulis
BACKGROUND/AIM: Medullary thyroid cancer (MTC) is highly malignant, metastatic and recurrent, remaining generally incurable, and responsible for approximately 14% of all thyroid carcinoma-related deaths. MTC can metastasize to lymph nodes, trachea and distant organs, such as brain, lungs, liver and bones. MTC cells are resistant to chemotherapy and traditional external therapies are not showing definite clinical benefits. Scientists are trying to understand the molecular background of carcinogenesis and histone deacetylase (HDAC) seems to play a potential role to gene transcription...
October 2016: Anticancer Research
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