TY - JOUR
T1 - Multifocality in sporadic medullary thyroid carcinoma
T2 - An international multicenter study
AU - Essig, Garth F.
AU - Porter, Kyle
AU - Schneider, David
AU - Arpaia, Debora
AU - Lindsey, Susan C.
AU - Busonero, Giulia
AU - Fineberg, Daniel
AU - Fruci, Barbara
AU - Boelaert, Kristien
AU - Smit, Johannes W.
AU - Meijer, Johannes Arnoldus Anthonius
AU - Duntas, Leonidas H.
AU - Sharma, Neil
AU - Costante, Giuseppe
AU - Filetti, Sebastiano
AU - Sippel, Rebecca S.
AU - Biondi, Bernadette
AU - Topliss, Duncan J.
AU - Pacini, Furio
AU - Maciel, Rui M.B.
AU - Walz, Patrick C.
AU - Kloos, Richard T.
PY - 2016/11/1
Y1 - 2016/11/1
N2 - Background: Current surgical standard of care in sporadic medullary thyroid carcinoma (sMTC) consists of a minimum of total thyroidectomy with central neck dissection. Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequency of bilateral disease, although this topic has not been thoroughly studied. This study assessed the prevalence of multifocality in sMTC via a large international multi-institutional retrospective review to quantify this prevalence, including the impact of geography, to assess more accurately the risks associated with alternative surgical approaches. Methods: A retrospective chart review of sMTC patients from 11 institutions over 29 years (1983-2011) was undertaken. Data regarding focality, extent of disease, RET germline analysis plus family and clinical history for multiple endocrine neoplasia type 2 (MEN2), and demographic data were collected and analyzed. Results: Patients from four continents and seven countries were included in the sample. Data for 313 patients with documented sMTC were collected. Of these, 81.2% were confirmed with negative RET germline testing, while the remaining 18.8%demonstrated a negative family history and nomanifestations ofMEN2 syndromes other thanMTC. Bilateral disease was identified in 17/306 (5.6%) patients, while multifocal disease was noted in 50/312 (16.0%) sMTC patients. When only accounting for germline negative patients, these rates were not significantly different (5.6% and 17%, respectively). Among them, when disease was unifocal in the ipsilateral lobe and isthmus, bilateral disease was present in 6/212 (2.8%) cases. When disease was multifocal in the ipsilateral lobe or isthmus, then bilateral disease was present in 8/37 (21.6%) cases ( p < 0.001). No geographic differences in focality were identified. Conclusions: The 5.6% prevalence of bilateral foci in sMTC suggests that total thyroidectomy should remain the standard of care for initial surgery, as less complete thyroid surgery may fail to address fully the primary site of disease. Whether ipsilateral tumor focality should be an independent factor determining the need for completion thyroidectomy when sMTC is diagnosed after hemithyroidectomy remains to be determined.
AB - Background: Current surgical standard of care in sporadic medullary thyroid carcinoma (sMTC) consists of a minimum of total thyroidectomy with central neck dissection. Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequency of bilateral disease, although this topic has not been thoroughly studied. This study assessed the prevalence of multifocality in sMTC via a large international multi-institutional retrospective review to quantify this prevalence, including the impact of geography, to assess more accurately the risks associated with alternative surgical approaches. Methods: A retrospective chart review of sMTC patients from 11 institutions over 29 years (1983-2011) was undertaken. Data regarding focality, extent of disease, RET germline analysis plus family and clinical history for multiple endocrine neoplasia type 2 (MEN2), and demographic data were collected and analyzed. Results: Patients from four continents and seven countries were included in the sample. Data for 313 patients with documented sMTC were collected. Of these, 81.2% were confirmed with negative RET germline testing, while the remaining 18.8%demonstrated a negative family history and nomanifestations ofMEN2 syndromes other thanMTC. Bilateral disease was identified in 17/306 (5.6%) patients, while multifocal disease was noted in 50/312 (16.0%) sMTC patients. When only accounting for germline negative patients, these rates were not significantly different (5.6% and 17%, respectively). Among them, when disease was unifocal in the ipsilateral lobe and isthmus, bilateral disease was present in 6/212 (2.8%) cases. When disease was multifocal in the ipsilateral lobe or isthmus, then bilateral disease was present in 8/37 (21.6%) cases ( p < 0.001). No geographic differences in focality were identified. Conclusions: The 5.6% prevalence of bilateral foci in sMTC suggests that total thyroidectomy should remain the standard of care for initial surgery, as less complete thyroid surgery may fail to address fully the primary site of disease. Whether ipsilateral tumor focality should be an independent factor determining the need for completion thyroidectomy when sMTC is diagnosed after hemithyroidectomy remains to be determined.
KW - Humans
KW - Medullary thyroid carcinoma
KW - Multifocality
KW - Surgery
KW - Thyroid neoplasm
UR - http://www.scopus.com/inward/record.url?scp=84994087092&partnerID=8YFLogxK
U2 - 10.1089/thy.2016.0255
DO - 10.1089/thy.2016.0255
M3 - Article
AN - SCOPUS:84994087092
SN - 1050-7256
VL - 26
SP - 1563
EP - 1572
JO - Thyroid
JF - Thyroid
IS - 11
ER -