While thyroid function, assessed by the measurement of free triiodothyronine (fT3), free thyroxine (fT4), and thyroid stimulating hormone (TSH), is commonly normal, serum CT is elevated, and sometimes this finding can represent the first suspicion of the presence of MTC, thus requiring further diagnostic procedures[34]. 80. Octreotide was administered subcutaneously in a starting dose of 0.6 to 1.0 mg/day by automatic pump (Travax ASH6, … This result was confirmed for all RET mutation profiles, including V804M. Thyroid 1996;6:305-10. In advanced cases, carcinoembryonic antigen (CEA) can also be elevated. If you are ready to make an appointment, select a button on the right. The next-generation RET inhibitor TPX-0046 is active in drug-resistant and naïve RET-driven cancer models.
Purpose: Two tyrosine kinase inhibitors (TKIs), vandetanib and cabozantinib, have been approved for recurrent/metastatic (R/M) medullary thyroid carcinoma (MTC). Nocera M, Baudin E, Pellegriti G, et al. According to its role in REToma carcinogenesis, RET protein has been considered an ideal target for highly selective drugs. 115. Clinical Trials: The NCCN recommends cancer patient participation in clinical trials as the gold standard for treatment. 63. 79. Vandetanib in children and adolescents with multiple endocrine neoplasia type 2B associated medullary thyroid carcinoma. 7. When an early diagnosis is performed, and the tumor is still intrathyroidal, 90% of patients can survive up to 35 years[29,31]. Patients were randomized 1:1 to receive vandetanib 150 or 300 mg/daily for a maximum time of 14 months (Part A). Furthermore, these drugs are susceptible to induce peculiar resistance mechanisms, and further studies are needed to completely understand the ideal clinical and molecular scenario to use these drugs. When choosing the drug, it should also be considered that cabozantinib has been tested as second line treatment after other TKIs and showed advantages in prolongation of PFS, while this information is unavailable for vandetanib.
However, most of these studies, although with a limited number of patients, showed that a stabilization of the disease (43%-100%) was the main clinical response obtained after PRRT treatments with Y90 or 177Lu[136,140,142,148]. Cabanillas ME, Hu MI, Jimenez C. Medullary thyroid cancer in the era of tyrosine kinase inhibitors: to treat or not to treat--and with which drug--those are the questions. In large, progressive liver metastases or metastases associated with symptoms, local treatment is required. Oncol Rep 2009;21:1585-92. The B-Cell Lymphoma Moon Shot is revolutionizing the conventional medical research approach to rapidly translate findings into patient treatment options and develop personalized therapeutic strategies. Boostrom SY, Grant CS, Thompson GB, Farley DR, Richards ML, Hoskin TL, et al. If the tumor invades the vital structures of the neck and if patients experienced radiation treatment of the neck or mediastinum, they carry a higher risk for hemorrhages and fistula formation, which is a life-threatening AE[94]. Schlessinger J. Children can only be affected by inherited MTC, and the more aggressive is the syndrome (i.e., MEN IIB), the younger is the affected child[11-14]. Oncotarget 2016;7:32318-28. The presence of mucosal neurinomas of the tongue or conjunctivas, in particular if associated to marfanoid habitus and/or skeletal alterations, should immediately suggest the diagnosis of MEN IIB[11]. Comments must be written in English. Moreover, RAS mutated MTC cases are apparently less aggressive that RET mutated ones. Oto- 64. Paepegaey AC, Cochand-Priollet B, Louiset E, et al. Carlomagno F, Vitagliano D, Guida T, et al. Langenbecks Arch Surg 2019;404:411-9. Treatment of Medullary Thyroid Carcinoma Surgical Therapy . 15. Because MTC is one of the amine precursor uptake and decarboxylation (APUD) tumors, treatment was … Thyroid 2014;24:1385-93. B. Grossman, Department of Endocrinology, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK. 83. Pacini F, Basolo F, Elisei R, Fugazzola L, Cola A, Pinchera A. Medullary thyroid cancer. Pitoia F, Bueno F, Schmidt A, Lucas S, Cross G. Rapid response of hypercortisolism to vandetanib treatment in a patient with advanced medullary thyroid cancer and ectopic Cushing syndrome. Therapy goals should be distinguished between curative and palliative. Head Neck 2020;42:401-16. Br J Pharmacol 2018;175:3504-15. All the other authors declared that there have no conflicts of interest. 8. Thus, a second generation of highly selective RET inhibitors have been developed both to maintain a significant anti-tumor efficacy, similar to vandetanib and cabozantinib, and to improve the safety profile. Romei C, Ciampi R, Elisei R. A comprehensive overview of the role of the RET proto-oncogene in thyroid carcinoma. A 41-year-old female, who presented with a breast mass, was initially diagnosed with invasive ductal carcinoma. Background: Medullary thyroid carcinoma (MTC) is a rare disease.
Review of adjuvant therapies in renal cell carcinoma: evidence to date. Forssell-Aronsson E, Fjälling M, Nilsson O, et al. 106. Stage IV thyroid cancer—also called metastatic disease is cancer that has spread beyond the thyroid to the soft tissues of the neck, lymph nodes in the neck, or distant locations in the body. Similarly, the detection of an interscapular cutaneous itchy lesion, defined as cutaneous lichen amyloidosis, is highly suspicious of MEN IIA[33], since this lesion is almost exclusively found in this syndrome. Comparison of octreotide scintigraphy and conventional imaging in medullary thyroid carcinoma. It is more diffuse in ATC patients[114], and its level is higher in BRAFV600E PTC compared to BRAF wild-type tumors[115]. 144. 2.
It is given as a pill once a day. Angell TE, Lechner MG, Jang JK, Correa AJ, LoPresti JS, Epstein AL. "Rare cancers get intense focus here, so they aren't rare to us," says Steven Sherman, M.D., chair of Endocrine Neoplasia and Hormonal Disorders. Hum Mol Genet 1994;3:237-41. N Engl J Med 2020;383:825-35. Five-year survival rates vary from 62% to 87% according to the different series, and the 10-year survival could decrease to 50%[24-29]. 72. 1990;23:453–473. Several TKIs have been tested for the treatment of advanced progressive MTC, including imatinib [ 57], axitinib [ 58], motesanib [ 59], sorafenib [ 60], sunitinib [ 61], pazopanib [ 62], ponatinib [ 63], lenvatinib [ 64], and anlotinib [ 65]. Mato E, MatÃas-Guiu X, Chico A, et al. iScience 2019;20:324-36. Calcitonin is the main secretory product and marker for diagnosis and follow-up. 121. In MEN IIA, the most frequently detected RET point mutation is located at codon 634[53]; conversely, in MEN IIB and most sporadic cases, it is at codon 918 (M918T)[11,15]. Ann Oncol 2017;28:2813-9. Safety and efficacy of two starting doses of vandetanib in advanced medullary thyroid cancer. Sanso GE, Domene HM, Garcia R, et al. Background: Therapeutic options for treating advanced or metastatic medullary thyroid carcinoma (MTC) and anaplastic thyroid carcinoma (ATC) are still limited in Japan, even though vandetanib for MTC and lenvatinib for MTC and ATC have been approved. 65. Similarly, mutations occurring in the MAPK and PI3K pathways can promote tumorigenesis. Bottici V,
It has been shown that a subgroup of patients with differentiated thyroid cancer (DTC) and medullary thyroid carcinoma (MTC) would progress to advanced stages of thyroid cancer. Follicular carcinoma is … Solomon BJ, Tan L, Lin JJ, et al. Therefore, radioactive iodine therapy is not useful for the treatment … These results were further confirmed in animal models and clinical studies[101,102], in which a relevant tumor shrinkage was shown. [71], in a retrospective study on a small subgroup of advanced MTC (n = 11), confirmed that a partial response was obtained in 36% of the study group. Bongiovanni et al. Correspondence to: Dr. Antonio Matrone, Department of Clinical and Experimental Medicine, Endocrine Unit, University Hospital of Pisa, Via Paradisa 2, Pisa 56124, Italy. It is a rare aggressive tumor, known to metastasize to lymph nodes, liver, bones, and lungs. Romei C, Pardi E, Cetani F, Elisei R. Genetic and clinical features of multiple endocrine neoplasia types 1 and 2. Vandetanib and cabozantinib are two cytostatic drugs, therefore they inhibit cellular growth but do not kill the neoplastic cells. J Clin Oncol 2020;38:2773-81. MTC was first characterized in 1959. According to the Common Terminology Criteria for Adverse Events (CTCAE)[75], AEs are classified into grades of severity. Patient Findings: A 64-year-old male was diagnosed with MTC in January 2010. Hu M, Subbiah M, Wirth LJ, et al. Valkema R, De Jong M, Bakker WH, et al. 87. Moreover, several clinical data indicate that patients on TKIs treatment will eventually develop a mechanism of resistance to the drug, associated with progression of the disease. Moreover, selpercatinib and pralsetinib inhibited the proliferation of cells harboring many kinds of RET mutations, both gene fusions and point mutations, including the V804M mutation which, as mentioned above, showed in vitro resistance to vandetanib[95]. 116. 123. Hum Mol Genet 1993;2:851-6. Schlumberger M, Jarzab B, Cabanillas ME, et al. 149. Best Pract Res Clin Endocrinol Metab 2017;31:319-34. 146. Overcoming MET-dependent resistance to selective RET inhibition in patients with RET fusion-positive lung cancer by combining selpercatinib with crizotinib. Spam, offensive content, impersonation, and private information will not be permitted. Protein kinase inhibitors for the treatment of advanced and progressive radiorefractory thyroid tumors: from the clinical trials to the real life. THE PROGNOSIS of patients with differentiated or medullary thyroid carcinoma is generally favorable, with median survivals measured in decades. J Clin Oncol 2020;38:3616. 52. Hereditary MTC (occurring in about 25% of cases) is caused by a germline mutation in the rearranged during transfection (RET) gene. There is a limited role for external beam radiotherapy. Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with 131 I‐meta‐iodobenzylguanidine (131 I‐mIBG) J. J. Mukherjee. Our previous single-arm phase 2 ALTN/MTC trial (NCT01874873) has demonstrated that anlotinib has a durable antitumor activity with a manageable adverse event profile in locally advanced or metastatic medullary thyroid carcinoma (MTC). Need for a revised staging consensus in medullary thyroid carcinoma. Metastatic medullary thyroid carcinoma presenting as ectopic Cushing's syndrome. Arao T, Matsumoto K, Furuta K, et al. RET gene mutations (genotype and phenotype) of multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma. Elisei R. Routine serum calcitonin measurement in the evaluation of thyroid nodules. PFS after 18 months was 71% in patients previously treated with other TKIs and 85% in treatment naïve patients. COSMIC: the catalogue of somatic mutations in cancer. Yamaga LYI, Cunha ML, Campos Neto GC, et al. Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A.
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