التفاصيل البيبلوغرافية
| العنوان: |
Pediatric Medullary Thyroid Carcinoma: Clinical Presentations and Long-Term Outcomes in 144 Patients Over 6 Decades. |
| المؤلفون: |
Hensley, Sarah G, Hu, Mimi I, Bassett, Roland L, Ying, Anita K, Zafereo, Mark E, Perrier, Nancy D, Busaidy, Naifa L, Hyde, Samuel M, Grubbs, Elizabeth G, Waguespack, Steven G |
| المصدر: |
Journal of Clinical Endocrinology & Metabolism; Sep2024, Vol. 109 Issue 9, p2256-2268, 13p |
| مصطلحات موضوعية: |
MEDULLARY thyroid carcinoma, SYMPTOMS, OLDER patients, OVERALL survival, FAMILY history (Medicine), CHILD patients |
| مستخلص: |
Context Sporadic medullary thyroid carcinoma (sMTC) rarely occurs in childhood and no studies have specifically focused on this entity. Objective To describe the clinical presentations and long-term outcomes of a large cohort of children and young adults with sMTC compared with hereditary MTC (hMTC). Methods Retrospective study of 144 patients diagnosed with MTC between 1961 and 2019 at an age ≤ 21 years and evaluated at a tertiary referral center. Results In contrast to hMTC (n = 124/144, 86%), patients with sMTC (n = 20/144, 14%) are older (P <.0001), have larger tumors (P <.0001), a higher initial stage grouping (P =.001) and have more structural disease (P =.0045) and distant metastases (DM) (P =.00084) at last follow-up, but are not more likely to die from MTC (P =.42). Among 77 patients diagnosed clinically, not by family history (20/20 sMTC and 57/124 hMTC), there was no difference in the initial stage (P =.27), presence of DM at diagnosis (P = 1.0), disease status at last follow-up (P =.13), overall survival (P =.57), or disease-specific survival (P =.87). Of the 12 sMTC tumors that underwent somatic testing, 11 (91%) had an identifiable alteration: 10 RET gene alterations and 1 ALK fusion. Conclusion sMTC is primarily a RET-driven disease that represents 14% of childhood-onset MTC in this cohort. Pediatric sMTC patients are older, present with clinical disease at a more advanced TNM classification, and have more persistent disease at last follow-up compared with hMTC, but these differences disappear when comparing those presenting clinically. Somatic molecular testing should be considered in sMTC patients who would benefit from systemic therapy. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
Complementary Index |
