CLINICAL
AND
LABORATORY OBSERVATIONS
An Adolescent With a Rare Midline Neck Tumor: Thyroid Carcinoma in a Thyroglossal Duct Cyst Evangeline Vassilatou, MD, PhD,* Konstantinos Proikas, MD,w Niki Margari, MD, PhD,z Nikolaos Papadimitriou, MD, PhD,w Dimitrios Hadjidakis, MD, PhD,* and George Dimitriadis, MD, PhDy
Summary: Occurrence of malignancy in a thyroglossal duct cyst (TDC) in children and adolescents is very rare, preoperative diagnosis is a challenge, and appropriate management is still debated. We report a 19-year-old male patient referred for a midline neck mass diagnosed as an atypical TDC after initial and subsequent investigations. Ultrasound-guided fine-needle aspiration (FNA) of the mass was diagnostic for papillary thyroid carcinoma. Sistrunk procedure and total thyroidectomy were performed. Histologic analysis confirmed the presence of papillary thyroid carcinoma within TDC, infiltrating surrounding soft tissues. Postoperatively, radioiodine ablation treatment was administered, followed by TSH suppression therapy. Key Words: thyroglossal duct cyst carcinoma, papillary thyroid cancer, Sistrunk procedure, fine-needle aspiration
(J Pediatr Hematol Oncol 2014;36:407–409)
T
hyroglossal duct cyst (TDC) is the most common developmental anomaly of the thyroid gland and the most common midline neck mass in childhood and adolescence.1 Malignant tumors in TDCs arising either from ectopic thyroid follicular cells or ductal epithelium are reported in approximately 1.5% of surgically treated TDCs1–3 and are usually diagnosed postoperatively. Prognosis of these tumors varies depending on the histologic type: the best is for well-differentiated thyroid carcinoma, poorer for squamous cell carcinoma, and the worst for anaplastic thyroid carcinoma.2–8 The vast majority of these tumors are well-differentiated thyroid carcinomas, most commonly of papillary type. Because of rarity of this cancer, diagnostic and therapeutic recommendations are based on small series and single case reports. The role of fineneedle aspiration (FNA) for the diagnostic evaluation of TDCs is controversial and appropriate management of these rare tumors concerning the role of total thyroidectomy, prophylactic neck dissection, and postoperative
Received for publication March 18, 2013; accepted January 8, 2014. From the *Endocrine Unit; y2nd Department of Internal Medicine— Propaedeutic; w2nd Department of Otorhinolaryngology; and zDepartment of Cytopathology, Athens University Medical School, “Attikon” University Hospital, Athens, Greece. The authors declare no conflict of interest. Reprints: Evangeline Vassilatou, MD, PhD, Endocrine Unit, 2nd Department of Internal Medicine—Propaedeutic, Athens University Medical School, “Attikon” University Hospital, 1 Rimini st., Haidari, Athens 12462, Greece (e-mail: [email protected]). Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Website, www. jpho-online.com. Copyright r 2014 by Lippincott Williams & Wilkins
J Pediatr Hematol Oncol
Volume 36, Number 5, July 2014
radioiodine therapy is still debated. A recent review on ectopic thyroid cancer concluded that management should be based on individual risk stratification.9 We present an adolescent with papillary thyroid carcinoma in a TDC that was diagnosed preoperatively by FNA.
CASE REPORT A 19-year-old male patient presented with a 6-year history of a painless midline neck mass. Initial evaluation at the age of 16 years showed a 1.23.2 cm midline neck mass with solid and multicystic components and calcifications (Fig. 1) and a multinodular goiter on neck ultrasound. Thyroid function tests were normal. Subsequent neck computed tomography (CT) showed a 2.1 2.7 3.8 cm heterogenous mass with similar characteristics, extending from the base of the tongue to the thyroid cartilage, positioned in front of the hyoid bone, compatible with an atypical TDC, a multinodular goiter, and no evidence of cervical lymphadenopathy. FNA of the mass detected atypical epithelial cells. Parents refused the recommended surgery. Two years later, a neck CT scan showed a slight enlargement of the mass (2.1 3.7 3.8 cm), mainly because of increased cystic component, similar thyroid imaging findings, and, again, no cervical lymphadenopathy. The patient was then referred to us. His past medical history was unremarkable. On clinical examination he was euthyroid. A midline, nontender, firm, B4.0 cm mass was evident in the neck and movable upon palpation (Fig. 2A). Thyroid gland was palpable, and there were no palpable cervical lymph nodes. Laboratory evaluation was normal, including normal thyroid function tests (T3: 1.1 ng/mL [range, 0.8 to 2.0 ng/mL]; T4: 10.8 mg/dL [range, 5.1 to 14.1 ng/mL]; TSH: 1.78 mIU/mL [range, 0.27 to 4.20 ng/mL]), and negative antithyroid antibodies (anti-TPO: 6.5 IU/mL [< 34.0 IU/mL], anti-Tg 10 IU/ mL [< 115 IU/mL]). Ultrasound-guided FNA of the mass was diagnostic for papillary thyroid carcinoma. Chest x-ray was normal. A neck magnetic resonance imaging showed similar findings (Fig. 2B) with the last CT scan. The patient underwent the Sistrunk procedure and total thyroidectomy (Supplementary Figures: 1A, 1B; Supplemental Digital Content 1, http://links.lww.com/JPHO/A66). Excised 4.3 cm mass contained a main cystic area of 1.8 cm maximal diameter. Excised thyroid gland weighed 54 g. Histologic analysis showed papillary thyroid carcinoma extending in the cystic area, infiltrating surrounding soft tissues, without hyoid bone involvement. No vascular invasion was documented. Colloid nodules were detected in the enlarged thyroid. Postoperatively, the patient received 70 mCi I-131 after thyroxine withdrawal. Hormonal evaluation before radioiodine remnant ablation showed serum TSH > 100 mIU/mL (range, 0.27 to 4.20 mIU/mL), free-T4
AND
LABORATORY OBSERVATIONS
An Adolescent With a Rare Midline Neck Tumor: Thyroid Carcinoma in a Thyroglossal Duct Cyst Evangeline Vassilatou, MD, PhD,* Konstantinos Proikas, MD,w Niki Margari, MD, PhD,z Nikolaos Papadimitriou, MD, PhD,w Dimitrios Hadjidakis, MD, PhD,* and George Dimitriadis, MD, PhDy
Summary: Occurrence of malignancy in a thyroglossal duct cyst (TDC) in children and adolescents is very rare, preoperative diagnosis is a challenge, and appropriate management is still debated. We report a 19-year-old male patient referred for a midline neck mass diagnosed as an atypical TDC after initial and subsequent investigations. Ultrasound-guided fine-needle aspiration (FNA) of the mass was diagnostic for papillary thyroid carcinoma. Sistrunk procedure and total thyroidectomy were performed. Histologic analysis confirmed the presence of papillary thyroid carcinoma within TDC, infiltrating surrounding soft tissues. Postoperatively, radioiodine ablation treatment was administered, followed by TSH suppression therapy. Key Words: thyroglossal duct cyst carcinoma, papillary thyroid cancer, Sistrunk procedure, fine-needle aspiration
(J Pediatr Hematol Oncol 2014;36:407–409)
T
hyroglossal duct cyst (TDC) is the most common developmental anomaly of the thyroid gland and the most common midline neck mass in childhood and adolescence.1 Malignant tumors in TDCs arising either from ectopic thyroid follicular cells or ductal epithelium are reported in approximately 1.5% of surgically treated TDCs1–3 and are usually diagnosed postoperatively. Prognosis of these tumors varies depending on the histologic type: the best is for well-differentiated thyroid carcinoma, poorer for squamous cell carcinoma, and the worst for anaplastic thyroid carcinoma.2–8 The vast majority of these tumors are well-differentiated thyroid carcinomas, most commonly of papillary type. Because of rarity of this cancer, diagnostic and therapeutic recommendations are based on small series and single case reports. The role of fineneedle aspiration (FNA) for the diagnostic evaluation of TDCs is controversial and appropriate management of these rare tumors concerning the role of total thyroidectomy, prophylactic neck dissection, and postoperative
Received for publication March 18, 2013; accepted January 8, 2014. From the *Endocrine Unit; y2nd Department of Internal Medicine— Propaedeutic; w2nd Department of Otorhinolaryngology; and zDepartment of Cytopathology, Athens University Medical School, “Attikon” University Hospital, Athens, Greece. The authors declare no conflict of interest. Reprints: Evangeline Vassilatou, MD, PhD, Endocrine Unit, 2nd Department of Internal Medicine—Propaedeutic, Athens University Medical School, “Attikon” University Hospital, 1 Rimini st., Haidari, Athens 12462, Greece (e-mail: [email protected]). Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Website, www. jpho-online.com. Copyright r 2014 by Lippincott Williams & Wilkins
J Pediatr Hematol Oncol
Volume 36, Number 5, July 2014
radioiodine therapy is still debated. A recent review on ectopic thyroid cancer concluded that management should be based on individual risk stratification.9 We present an adolescent with papillary thyroid carcinoma in a TDC that was diagnosed preoperatively by FNA.
CASE REPORT A 19-year-old male patient presented with a 6-year history of a painless midline neck mass. Initial evaluation at the age of 16 years showed a 1.23.2 cm midline neck mass with solid and multicystic components and calcifications (Fig. 1) and a multinodular goiter on neck ultrasound. Thyroid function tests were normal. Subsequent neck computed tomography (CT) showed a 2.1 2.7 3.8 cm heterogenous mass with similar characteristics, extending from the base of the tongue to the thyroid cartilage, positioned in front of the hyoid bone, compatible with an atypical TDC, a multinodular goiter, and no evidence of cervical lymphadenopathy. FNA of the mass detected atypical epithelial cells. Parents refused the recommended surgery. Two years later, a neck CT scan showed a slight enlargement of the mass (2.1 3.7 3.8 cm), mainly because of increased cystic component, similar thyroid imaging findings, and, again, no cervical lymphadenopathy. The patient was then referred to us. His past medical history was unremarkable. On clinical examination he was euthyroid. A midline, nontender, firm, B4.0 cm mass was evident in the neck and movable upon palpation (Fig. 2A). Thyroid gland was palpable, and there were no palpable cervical lymph nodes. Laboratory evaluation was normal, including normal thyroid function tests (T3: 1.1 ng/mL [range, 0.8 to 2.0 ng/mL]; T4: 10.8 mg/dL [range, 5.1 to 14.1 ng/mL]; TSH: 1.78 mIU/mL [range, 0.27 to 4.20 ng/mL]), and negative antithyroid antibodies (anti-TPO: 6.5 IU/mL [< 34.0 IU/mL], anti-Tg 10 IU/ mL [< 115 IU/mL]). Ultrasound-guided FNA of the mass was diagnostic for papillary thyroid carcinoma. Chest x-ray was normal. A neck magnetic resonance imaging showed similar findings (Fig. 2B) with the last CT scan. The patient underwent the Sistrunk procedure and total thyroidectomy (Supplementary Figures: 1A, 1B; Supplemental Digital Content 1, http://links.lww.com/JPHO/A66). Excised 4.3 cm mass contained a main cystic area of 1.8 cm maximal diameter. Excised thyroid gland weighed 54 g. Histologic analysis showed papillary thyroid carcinoma extending in the cystic area, infiltrating surrounding soft tissues, without hyoid bone involvement. No vascular invasion was documented. Colloid nodules were detected in the enlarged thyroid. Postoperatively, the patient received 70 mCi I-131 after thyroxine withdrawal. Hormonal evaluation before radioiodine remnant ablation showed serum TSH > 100 mIU/mL (range, 0.27 to 4.20 mIU/mL), free-T4
