scholarly journals ACTH-secreting medullary thyroid cancer: a case series

2017 ◽  
Vol 2017 ◽  
Author(s):  
Ayanthi A Wijewardene ◽  
Sarah J Glastras ◽  
Diana L Learoyd ◽  
Bruce G Robinson ◽  
Venessa H M Tsang
Keyword(s):  
Thyroid Cancer ◽  
Cushing’S Syndrome ◽  
Medullary Thyroid Cancer ◽  
Kinase Inhibitors ◽  
Neuroendocrine Tumour ◽  
Therapeutic Option ◽  
Rare Complication ◽  
Cushing's Syndrome ◽  
Ectopic Acth ◽  
Medullary Thyroid

Summary Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour that originates from the parafollicular cells of the thyroid gland. The most common presentation of MTC is with a single nodule; however, by the time of diagnosis, most have spread to the surrounding cervical lymph nodes. Cushing’s syndrome is a rare complication of MTC and is due to ectopic adrenocorticotrophic hormone (ACTH) secretion by tumour cells. Cushing’s syndrome presents a challenging diagnostic and management issue in patients with MTC. Tyrosine kinase inhibitors (TKI) previously used for the management of metastatic MTC have become an important therapeutic option for the management of ectopic ACTH in metastatic MTC. The article describes three cases of ectopic ACTH secretion in MTC and addresses the significant diagnostic and management challenges related to Cushing’s syndrome in metastatic MTC. Learning points: Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour. Cushing’s syndrome is a rare complication of MTC that has a significant impact on patients’ morbidity and mortality. Tyrosine kinase inhibitors (TKI) provide an important therapeutic option for the management of ectopic ACTH in metastatic MTC.

Ectopic Cushing's syndrome due to advanced medullary thyroid cancer: a case report

2021 ◽  
Author(s):  
Andrea Corsello ◽  
Vittoria Ramunno ◽  
Rosa Maria Paragliola ◽  
Giovanni Pacini ◽  
Crea Carmela De ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
Case Report ◽  
Cushing’S Syndrome ◽  
Medullary Thyroid Cancer ◽  
Cushing's Syndrome ◽  
Medullary Thyroid ◽  
Ectopic Cushing’S Syndrome

scholarly journals Increased therapeutic effect on medullary thyroid cancer using a combination of radiation and tyrosine kinase inhibitors

PLoS ONE ◽  
2020 ◽  
Vol 15 (5) ◽  
pp. e0233720
Author(s):  
Viktor Sandblom ◽  
Johan Spetz ◽  
Emman Shubbar ◽  
Mikael Montelius ◽  
Ingun Ståhl ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
Tyrosine Kinase ◽  
Tyrosine Kinase Inhibitors ◽  
Therapeutic Effect ◽  
Medullary Thyroid Cancer ◽  
Kinase Inhibitors ◽  
Medullary Thyroid

First Line Sorafenib Treatment for Metastatic Medullary Thyroid Cancer: Efficacy and Safety Analysis

2018 ◽  
Vol 127 (04) ◽  
pp. 240-246 ◽  
Author(s):  
Judit Kocsis ◽  
Éva Szekanecz ◽  
Ali Bassam ◽  
Andrea Uhlyarik ◽  
Zsuzsanna Pápai ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
Tyrosine Kinase ◽  
Disease Control ◽  
Tyrosine Kinase Inhibitors ◽  
Phase Ii ◽  
Medullary Thyroid Cancer ◽  
Kinase Inhibitors ◽  
Efficacy And Safety ◽  
First Line ◽  
Medullary Thyroid

Abstract Background Medullary thyroid cancer (MTC) is a rare disease, the prognosis of advanced and metastatic disease is poor and few therapeutic options are available in this setting. Based on the results of phase II and III studies with sorafenib in differentiated thyroid cancer and the lack of availability of registered tyrosine kinase inhibitors, vandetabin and cabozantinib in Hungary, we designed a uncontrolled, prospective efficacy and safety study of patients with metastatic MTC treated with first-line sorafenib in five Hungarian oncology centers. Methods Ten consecutive patients with progressive or symptomatic metastatic MTC were included and started sorafenib 400  mg twice a day between June 2012 and March 2016. The primary end point was median progression-free survival (mPFS). Secondary endpoints included disease control rate, biochemical response, symptomatic response and toxicity. Results Four patients achieved partial remission (40%) according to RECIST 1.1 evaluation. Five patients had stable disease beyond 12 months (50%) and one patient had progressive disease (10%). Median PFS was 19.1 months. The disease control rate was 90%. Association between radiologic response and biochemical or symptomatic response was inconsistent. Most common side effects were Grade 1-2 fatigue (60%), palmar-plantar erythrodysesthesia, rash/dermatitis 50-50%, alopecia 40%. Conclusions In our prospective case series in patients with MTC first-line sorafenib showed at least similar efficacy as in other small phase II trials and case reports. Based on comparable efficacy with registered tyrosine kinase inhibitors and it’s manageable toxicity profile, we believe that sorafenib has role in the sequential treatment of MTC.

scholarly journals A primer on the genetics of medullary thyroid cancer

2019 ◽  
Vol 26 (6) ◽  
Author(s):  
V. Larouche ◽  
A. Akirov ◽  
C. M. Thomas ◽  
M. K. Krzyzanowska ◽  
S. Ezzat
Keyword(s):  
Thyroid Cancer ◽  
Medullary Thyroid Cancer ◽  
Neuroendocrine Tumour ◽  
Hirschsprung Disease ◽  
Cancer Syndrome ◽  
Rare Type ◽  
Medullary Thyroid ◽  
Sporadic Cases ◽  
Cutaneous Lichen Amyloidosis

Medullary thyroid cancer is a rare type of neuroendocrine tumour that arises from the parafollicular cells (C cells) of the thyroid gland. It accounts for 3%–5% of thyroid cancer cases. Close to 25% of cases are familial, and 75% are considered sporadic. Familial cases are associated with a germline RET mutation; 43%–65% of sporadic cases harbour a somatic event in the gene. Germline RET mutations are associated with the autosomal-dominant inherited multiple endocrine neoplasia (men) 2a and 2b syndromes and the isolated familial medullary thyroid cancer syndrome. More than 100 RET codon mutations have been reported to date, with genotype–phenotype correlations that include the extent and aggressiveness of the medullary thyroid cancer and the presence of other features of the men2 syndromes. The latter include pheochromocytoma–paraganglioma, hyperparathyroidism, cutaneous lichen amyloidosis, and Hirschsprung disease.   In this narrative review, we focus on RET proto-oncogene physiology and pathogenesis induced by germline and somatic RET mutations, the genotype–phenotype correlation, and the management and follow-up of patients with germline-mutated medullary thyroid cancer.

An Unusual Case of Hypopituitarism as an Adverse Effect of Vandetanib and Remission of Breast Metastases in a Patient with Medullary Thyroid Cancer

2021 ◽  
pp. 1-5
Author(s):  
Rosa M. García-Moreno ◽  
Óscar Moreno-Domínguez ◽  
Beatriz Castelo-Fernández ◽  
Laura Yébenes-Gregorio ◽  
Isabel Torres-Sánchez ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
Adrenal Insufficiency ◽  
Medullary Thyroid Cancer ◽  
Kinase Inhibitors ◽  
Hypogonadotropic Hypogonadism ◽  
Complete Disappearance ◽  
Secondary Adrenal Insufficiency ◽  
Medullary Thyroid ◽  
Metastatic Lesions ◽  
Breast Metastases

<b><i>Introduction:</i></b> Tyrosine kinase inhibitors have been a breakthrough in the treatment of advanced medullary thyroid cancer (MTC), and they can prolong progression-free survival (PFS). <b><i>Case Presentation:</i></b> A patient with MTC and metastatic spread to the lymph nodes, lungs, bones, breast, and cerebellum started treatment with vandetanib. During treatment, she developed secondary adrenal insufficiency and hypogonadotropic hypogonadism. After 9 years of vandetanib therapy, the disease has not progressed and the patient maintains a complete response of the breast metastases and a partial response of the other metastatic lesions. <b><i>Conclusion:</i></b> To our knowledge, this is the first reported case of secondary adrenal insufficiency and hypogonadotropic hypogonadism related to therapy with vandetanib. Moreover, the prolonged PFS and the complete disappearance of some of the metastatic lesions in this patient are truly unusual.

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