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Table of Contents
Year : 2013  |  Volume : 17  |  Issue : 5  |  Page : 936-938

Malignant thyroglossal duct cyst with synchronous occult thyroid gland papillary carcinoma

Department of Surgical Oncology, IIIrd Floor, Superspecialty Block, JIPMER, Puducherry, India

Date of Web Publication29-Aug-2013

Correspondence Address:
R Aravind
Department of Surgical Oncology, IIIrd Floor, Superspecialty Block, JIPMER, Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2230-8210.117229

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A 52-year-old male was referred to our department with complaints of a painless midline neck swelling. Clinico-radiological evaluation suggested a 6 × 5 cm thyroglossal cyst with non-palpable nodules in isthmus and right lobe of thyroid gland. FNAC of the thyroglossal cyst was suggestive of papillary carcinoma. He underwent Sistrunk's operation, total thyroidectomy, and central compartment neck dissection. Co-existence of papillary carcinoma of thyroid gland and thyroglossal cyst is a rare presentation and in this report, we describe our management and propose an evidence-based algorithm to assist decision-making in the management of these patients in future.

Keywords: Papillary carcinoma, Sistrunk′s operation, thyroglossal duct cyst carcinoma, thyroglossal duct cyst, thyroid cancer

How to cite this article:
Senthilkumar R, Neville J F, Aravind R. Malignant thyroglossal duct cyst with synchronous occult thyroid gland papillary carcinoma. Indian J Endocr Metab 2013;17:936-8

How to cite this URL:
Senthilkumar R, Neville J F, Aravind R. Malignant thyroglossal duct cyst with synchronous occult thyroid gland papillary carcinoma. Indian J Endocr Metab [serial online] 2013 [cited 2021 Sep 24];17:936-8. Available from: https://www.ijem.in/text.asp?2013/17/5/936/117229

   Introduction Top

Thyroglossal duct cyst (TGDC) is the most common anomaly in thyroid development. Thyroid gland descends from foramen caecum to a point below the thyroid cartilage. It leaves an epithelial tract known as thyroglossal tract. The tract disappears during 5 th to 10 th gestational week. Incomplete atrophy of tract forms the basis of origin of the cyst. In this report, we present an adult male with papillary carcinoma of TGDC and occult papillary carcinoma of thyroid gland.

   Case Report Top

A 52-year-old male presented to our department with a painless swelling in front of neck progressing slowly over the past 6 years. He complained of a rapid increase in size over the past 6 months. No history of irradiation. Clinical examination and CT evaluation suggested the presence of an irregularly-shaped complex TGDC measuring 6 × 5 cm with solid and cystic components. There was also evidence of fine calcification within the solid areas [Figure 1]. There was also a mildly enhancing nodule found in right lobe and isthmus of thyroid with fine calcification within the nodules. There was no cervical lymphadenopathy. Ultrasound-guided FNAC from the solid area of the TGDC suggested papillary carcinoma. Sistrunk operation for thyroglossal cyst done using a single transverse neck incision, and per-operative thyroid exploration revealed a hard nodule in the right lobe of thyroid with few enlarged paratracheal nodes. Since there was a strong suspicion of malignant thyroid nodule as well, total thyroidectomy and central compartment dissection was completed in the same sitting. Gross examination revealed TGDC, which was multiloculated and had solid and cystic areas (about 2 cm) [Figure 2]. There was a 0.5 cm nodule in the isthmus and a 1 cm nodule in the right lobe of thyroid gland. Microscopic diagnosis [Figure 3], [Figure 4] and [Figure 5] was multifocal papillary carcinoma of thyroid and thyroglossal cyst with no metastasis in level VI nodes. Post op I 131 scan showed 0.3% uptake in the thyroid bed and no evidence of functioning distant metastasis. Patient is on suppressive thyroxin therapy.
Figure 1: Clinical photograph showing the midline irregular-shaped neck swelling

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Figure 2: Contrast CT images showing the thyroglossal cyst with thick septae and a nodule in the right lobe of thyroid gland

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Figure 3: Photograph of the gross specimen showing the thyroglossal cyst attached to the thyroid gland through the thyroglossal tract

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Figure 4: Light microscope appearance with the "black star" denoting normal cyst wall lining and the "white star" indicating papillary carcinoma of the thyroglossal cyst

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Figure 5: Management algorithm for malignant thyroglossal cyst

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   Discussion Top

TGDC occurs in the midline neck and is diagnosed clinically. Majority is benign, but 1% may be malignant. [1],[2] To date, around 215 cases of malignant TGDC have been reported, 80% of these being papillary carcinoma, 7% mixed papillary and follicular carcinoma, 5% squamous cell carcinoma, 1.7% follicular and adenocarcinoma and 0.9% anaplastic carcinoma. [3]

Since malignant TGDC is rare, diagnosis is made only if there is a strong clinical suspicion. Features that should arouse such suspicion include large or increasing size, hard, fixity, irregular shape, previous exposure to ionizing radiation. There is no role for routine FNAC of TGDC in the absence of suspicious features.

When malignancy is strongly suspected, pre-operative cytological and radiological evaluation aid in planning the extent of surgery. Usually, 2-3 aspirations are required for an adequate sample by FNAC, but yield is positive only in 66% of lesions since dilution by cyst contents can lead to hypocellular smears. [4],[5] Scintigraphy may help differentiating a partially descended thyroid from a TGDC and identifying cold nodules in the thyroid gland. USG and CT help characterize the extent, presence of solid components, calcification, co-existing thyroid nodules, and cervical lymphadenopathy. [6]

In the vast majority of patients, diagnosis of malignant TGDC is made post-operatively after a Sistrunk operation. If histopathology of cyst is negative or shows low risk malignancy features like microscopic focus less than 1 cm without cyst wall invasion, follow up alone is adequate. [6],[7] If there are high risk features like a neoplastic focus more than 1 cm or cyst wall invasion, then a total thyroidectomy is recommended as these lesions are aggressive. Further, total thyroidectomy allows long-term monitoring of thyroglobulin and I 131 studies. [6] It has been shown that as many of 11% of patients with malignant TGDC may harbor foci of papillary carcinoma in the thyroid gland. [3] When both are present, it remains controversial whether the primary is the malignant TGDC or the thyroid gland. [3],[8]

Whenever malignant TGDC is preoperatively diagnosed, pre-operative imaging and careful intra-operative evaluation of thyroid gland is performed to rule out clinically occult neoplastic nodules. [8] Presence of synchronous thyroid nodules warrants hemithyroidectomy and frozen section in addition to the Sistrunk operation. Further surgery is planned based on the frozen section report as one would manage a solitary thyroid nodule. There is no role for prophylactic lateral neck dissection. [8] Postoperative management of synchronous malignant TGDC and thyroid carcinoma is in line with the guidelines adopted for the management if differentiated thyroid cancer. [8]

To conclude, we present an evidence-based algorithm, constructed based on literature review, to aid decision-making in management of malignant TGDC.

   References Top

1.Miccoli P, Minuto MN, Galleri D, Puccini M. Extend of surgery in TGDC carcinoma reflections on a series of 18 cases. Thyroid 2004;14:121-3.  Back to cited text no. 1
2.Luna-Ortiz K, Hurtado-Lopez LM, Valderrama-Landaeta JL, Ruiz-Vega A.Thyroglossal duct cyst with papillary carcinoma: What must be done. Thyroid 2004;14:363-6.  Back to cited text no. 2
3.Weiss SD, Orlich CC. Primary papillary carcinoma of a thyroglossal duct cyst: Report of a case and literature review. Br J Surg 1991;78:87-9.  Back to cited text no. 3
4.Yang YJ, Haghir S, Wanamaker JR, Powers CN. Diagnosis of papillary carcinoma in a thyroglossal duct cyst by fine-needleaspiration biopsy. Arch Pathol Lab Med 2000;124:139-42.  Back to cited text no. 4
5.Agarwal K, Puri V, Singh S. Critical appraisal of FNAC in the diagnosis of primary papillary carcinoma arising in thyroglossal cyst: A case report with review of the literature on FNAC and its diagnostic pitfalls. J Cytol 2010;27:22-5.  Back to cited text no. 5
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6.Park MH, Yoon JH, Jegal YJ, Lee JS. Papillary thyroglossal duct cyst carcinoma with synchronous occult papillary thyroid microcarcinoma. Yonsei Med J 2010;51:609-11.  Back to cited text no. 6
7.Basu S, Shet T, Borges AM. Outcome of primary papillary carcinoma of thyroglossal duct cyst with local infiltration to soft tissues and uninvolved thyroid. Indian J Cancer 2009;46:169-70.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.Doshi SV, Cruz RM, Hilsinger RL Jr. Thyroglossal duct carcinoma: A large case series. Ann Otol Rhinol Laryngol 2001;110:734-8.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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