Thyroglossal duct remnant carcinoma: Beyond the Sistrunk procedure

Abstract

Thyroglossal duct remnants (TGDRs) account for more than 70% of anterior neck masses in children and 7% in adults; however, cancer is identified in only 1–2% of the cases. The diagnosis of a TGDR is based on clinical manifestation of a painless, anterior neck swelling, which elevates with swallowing. Cytological evaluation with fine needle aspiration and biopsy (FNAB) may facilitate the pre-operative diagnosis of malignancy, as the majority of TGDR cancers are of papillary histotype. The recommended treatment for symptomatic TGDR without evidence of malignancy is a Sistrunk procedure, which entails en bloc resection of the remnant and the mid-portion of the hyoid bone. The optimal management of patients with diagnosed malignancy is controversial, and in the past, additional total thyroidectomy was recommended for all of these patients. The purpose of this study is to review the literature on TGDR carcinomas, present the evidence on the available diagnostic tools, identify the surgical and post-operative medical management strategies, discuss current controversies, and conclude with a management algorithm.

Thyroglossal Duct Cyst Carcinomas: A Clinicopathologic Series of 22 Cases with Staging Recommendations

“…Unlike nonneoplastic TGDCs, which show no gender predilection [2][3][4], TGDCCa affect women more frequently than men with a 3:1 female to male ratio observed in the present series, or 2.3:1 overall when including the literature ( Most patients present with a mobile midline neck mass, clinically indistinguishable from benign TGDC [2,13,17,[19][20][21]. The finding of a fixed mass has been emphasized as a possible indicator of malignancy [3,20,36], but was present in only 2 patients in this series.…”

“…The reported incidence of carcinoma occurring in TGDCs ranges from \1 to 19.6 % [5][6][7][8][9][10][11][12][13][14][15][16], although probably more in the 2 % range when referral and academic bias are removed from consideration [4]. The vast majority of TGDC carcinomas (TGDCCa) are papillary thyroid carcinomas (PTCs), thought to originate from the ectopic thyroid gland tissue which is commonly (approximately 70 % of cases) found in association with TGDCs [2,4,7,9,[16][17][18][19][20][21][22]. The clinical presentation of TGDCCa do not differ significantly from that of nonneoplastic TGDC, although a fixed mass is worrisome, with most cases of malignancy diagnosed postoperatively following pathologic evaluation of the lesion [2,17,20,21].…”

“…The vast majority of TGDC carcinomas (TGDCCa) are papillary thyroid carcinomas (PTCs), thought to originate from the ectopic thyroid gland tissue which is commonly (approximately 70 % of cases) found in association with TGDCs [2,4,7,9,[16][17][18][19][20][21][22]. The clinical presentation of TGDCCa do not differ significantly from that of nonneoplastic TGDC, although a fixed mass is worrisome, with most cases of malignancy diagnosed postoperatively following pathologic evaluation of the lesion [2,17,20,21]. Given the rarity of TGDCCa and the frequent incidental nature of the diagnosis, clinical management of these tumors remains controversial particularly in regards to the need for thyroidectomy and the need for staging of the tumors [3,8,12,16,20,23,24].…”

“…The clinical presentation of TGDCCa do not differ significantly from that of nonneoplastic TGDC, although a fixed mass is worrisome, with most cases of malignancy diagnosed postoperatively following pathologic evaluation of the lesion [2,17,20,21]. Given the rarity of TGDCCa and the frequent incidental nature of the diagnosis, clinical management of these tumors remains controversial particularly in regards to the need for thyroidectomy and the need for staging of the tumors [3,8,12,16,20,23,24].…”

“…Currently, the American Joint Committee on Cancer (AJCC) staging (2010) criteria for cancer of thyroid gland tumors is not consistently applied to these tumors and has not been recommended [6,8,20,22,25,26]. This study of a community practice based series of TGDCCa is presented with analysis of histopathologic features and recommendations of the use of staging to alter therapeutic management.…”