caa a22 4500 445332956 CHVBK 20180317142743.0 cr unu---uuuuu 170323e20110101xx s 000 0 eng 10.1245/s10434-010-1339-y doi (NATIONALLICENCE)springer-10.1245/s10434-010-1339-y Medullary Thyroid Carcinoma: Long-Term Outcomes of Surgical Treatment [Elektronische Daten] [Deepak Abraham, Tsu-Hui Low, Marinella Messina, Nicole Jackson, Anthony Gill, Angela Chou, Leigh Delbridge, Diana Learoyd, Bruce Robinson, Stan Sidhu, Mark Sywak] Background: Medullary thyroid carcinoma (MTC) accounts for 5 to 10% of all thyroid cancers but is responsible for a disproportionate number of deaths. Methods: We performed a retrospective review to describe clinical outcomes in patients with medullary thyroid carcinoma, screening a subset of patients for somatic mutations in the RET and p18 genes and performing genotype-phenotype correlation in a tertiary-care referral hospital from 1967 to 2009. Results: We studied a total of 94 patients identified from a prospectively maintained thyroid cancer database. Data gathered included patient demographics, serum calcitonin, clinical outcomes, histopathology, genetic analysis, and status at final follow-up. A subset cohort (n=50) was screened for somatic mutations in the RET gene and the three exons of the p18 gene. The subset cohort was composed of hereditary medullary thyroid carcinoma (HMTC) (n=19, index patients=10, screen detected=9) and sporadic medullary thyroid carcinoma (SMTC) (n=31). There were no mutations in the p18 gene in the subset cohort. Conclusions: A total of 67 SMTC and 27 (28.7%) HMTC cases identified. SMTC were older at initial presentation (52 vs. 34, P=0.003), had higher preoperative serum calcitonin levels (7968 vs. 1346ng/L, P=0.008), and had lymph node recurrence (P=0.001) compared to HMTC. The tumors were smaller in HMTC (P=0.038). Overall 10-year survival in SMTC versus HMTC was 69 versus 93% (P=0.12). On multivariate analysis, vascular invasion (hazard ratio 6.4, P=0.019) was an adverse predictor for disease-free survival. HMTC in the era of RET analysis presents with a smaller primary tumor, lower preoperative serum calcitonin levels, and lower rates of lymph node metastasis. Mutations in the p18 gene were not a major factor in medullary thyroid carcinoma tumorigenesis. Society of Surgical Oncology, 2010 Abraham Deepak Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut Low Tsu-Hui Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut Messina Marinella Cancer Genetics Unit, Kolling Institute of Medical Research, University of Sydney, Sydney, Australia aut Jackson Nicole Cancer Genetics Unit, Kolling Institute of Medical Research, University of Sydney, Sydney, Australia aut Gill Anthony Department of Anatomical Pathology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut Chou Angela Department of Anatomical Pathology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut Delbridge Leigh Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut Learoyd Diana Department of Endocrinology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut Robinson Bruce Department of Endocrinology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut Sidhu Stan Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut Sywak Mark Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut Annals of Surgical Oncology Springer-Verlag 18/1(2011-01-01), 219-225 1068-9265 18:1<219 2011 18 10434 https://doi.org/10.1245/s10434-010-1339-y text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1245/s10434-010-1339-y text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Abraham Deepak Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut NATIONALLICENCE 700 1- Low Tsu-Hui Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut NATIONALLICENCE 700 1- Messina Marinella Cancer Genetics Unit, Kolling Institute of Medical Research, University of Sydney, Sydney, Australia aut NATIONALLICENCE 700 1- Jackson Nicole Cancer Genetics Unit, Kolling Institute of Medical Research, University of Sydney, Sydney, Australia aut NATIONALLICENCE 700 1- Gill Anthony Department of Anatomical Pathology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut NATIONALLICENCE 700 1- Chou Angela Department of Anatomical Pathology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut NATIONALLICENCE 700 1- Delbridge Leigh Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut NATIONALLICENCE 700 1- Learoyd Diana Department of Endocrinology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut NATIONALLICENCE 700 1- Robinson Bruce Department of Endocrinology, Royal North Shore Hospital, University of Sydney, Sydney, Australia aut NATIONALLICENCE 700 1- Sidhu Stan Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut NATIONALLICENCE 700 1- Sywak Mark Department of Surgery, University Clinic, Royal North Shore Hospital, University of Sydney Endocrine Surgical Unit, St Leonards, Sydney, NSW, Australia aut NATIONALLICENCE 773 0- Annals of Surgical Oncology Springer-Verlag 18/1(2011-01-01), 219-225 1068-9265 18:1<219 2011 18 10434 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer