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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 2  |  Page : 85-88

Thyroglossal duct cyst papillary carcinoma: A treatment puzzle yet to be solved!


1 Department of Otorhinolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Date of Submission18-May-2019
Date of Acceptance29-Sep-2019
Date of Web Publication25-Nov-2019

Correspondence Address:
Dr. Sourabha Kumar Patro
Department of Otorhinolaryngology and Head and Neck Surgery, 408, 4th Floor, A Block, ENT OPD, OPD Complex, All India Institute of Medical Sciences, Basni, Jodhpur - 342 005, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aiao.aiao_7_19

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  Abstract 


Thyroglossal duct cyst (TGDC) is the most common congenital aberration of the thyroid gland. On the contrary, TGDC malignancies are rare events constituting <1% of all cases of thyroid carcinoma reported in the world. The rarity of the lesion precludes the formulation of clear guidelines and makes management decisions difficult. We describe a case of a young male who presented with a midline neck swelling and underwent radical Sistrunk's procedure and total thyroidectomy for primary papillary carcinoma in a TGDC. The need of total thyroidectomy in high-risk cases needs clinical suspicion and careful consideration of this differential.

Keywords: Sistrunk's procedure, thyroglossal duct cyst, thyroglossal duct cyst carcinoma, total thyroidectomy in thyroglossal duct cyst carcinoma


How to cite this article:
Shakrawal N, Varshney B, Verma AK, Khera S, Goyal A, Patro SK. Thyroglossal duct cyst papillary carcinoma: A treatment puzzle yet to be solved!. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2019;3:85-8

How to cite this URL:
Shakrawal N, Varshney B, Verma AK, Khera S, Goyal A, Patro SK. Thyroglossal duct cyst papillary carcinoma: A treatment puzzle yet to be solved!. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2019 [cited 2019 Dec 14];3:85-8. Available from: http://www.aiaohns.in/text.asp?2019/3/2/85/271602




  Introduction Top


Thyroglossal duct cysts (TGDCs) are well-recognized lesions arising from the congenital remnants of the thyroglossal duct formed during the embryogenesis of thyroid gland[1] and are known to be present as midline neck masses. The incidence varies greatly with age from 70% in children to 7% in adults. Only 1% of the TGDCs convert into carcinomas;[2],[3] the first description of this was given by Brentano and Uchermann in the 20th century.[4] Of the various histological variants described, papillary carcinoma (75%–80%) is the most common primary variant with mixed papillary, follicular carcinomas (7%), squamous cell carcinoma (5%), follicular carcinoma, Hürthle cell carcinoma, and anaplastic carcinoma encountered in the decreasing order of incidences. The variant of anaplastic carcinoma carries the worst prognosis.[5],[6]

The principal concern in these diseases is deciding an adequate surgical plan which involves a total thyroidectomy in high-risk cases in view of the need of the postoperative radioiodine therapy to the surgical field which is away from the thyroid gland and would not concentrate iodine in the presence of the thyroid glands. Hence, clinical and cytological suspicious features need to be stressed on for proper management and surgical planning. Clinical features such as dysphagia, dysphonia, progressive weight loss, rapid increase in size, and recent-onset restriction in mobility and fixity to the surrounding structures are the alarming signs indicating possible ominous malignant changes in TGDC. However, these obvious clinical features are rarely or never present in cases of primary carcinomas of thyroglossal cysts. High false-negative rates in fine-needle aspirations (FNAs)[1],[7] are the primary reason for the diagnostic postsurgical histopathologic surprises often seen in primary papillary carcinoma of TGDC. In the present case, as a rare scenario, calcification, infiltrative radiological margins, and nuclear features helped us to diagnose the case preoperatively with the help of FNA cytology.

For low-risk cases which do not require postoperative adjuvant treatment, Sistrunk's procedure including excision of the cyst along with the whole thyroglossal tract and its branches including body of the hyoid (due to strong association between thyroglossal tract and body of the hyoid) suffices. However for high-risk cases which might require postoperative adjuvant treatment, total thyroidectomy needs to be considered, though the indications of thyroidectomy in papillary carcinoma of TGDC are still controversial. Literature favors thyroidectomy in cases with (a) nodular thyroid gland, with a positive radioiodine uptake scan, (b) presence of enlarged lymph nodes, and (c) history of radiation exposure.[8] The rarity of this type of carcinoma precludes the formulation of any fixed protocol for the management.

Distant metastases in this carcinoma have never been noted[1],[9] in primary cancers of the TGDC. However, cervical metastases are noted in 7.7%–15% of cases.[9] The involvement of skin around the cyst or intraoperative rupture of the cyst is the proposed etiology for postoperative recurrence in cases of carcinoma of TGDC. The overall prognosis of primary papillary carcinoma in TGDC is impressive with a 95% 5-year survival rate.[7]

We report this case encountered by us to emphasize the need for earlier suspicion and proper management of this entity by the surgeons and clinicians.


  Case Report Top


A 38-year-old male patient was referred to our tertiary care center with a painless, progressively enlarging midline neck swelling for the past 5 years without any history voice change or difficulty in breathing or swallowing. Examination revealed a cystic, ~5 cm × 3 cm swelling at the level of the hyoid moving with deglutition and tongue protrusion. No other swelling, thyroid gland, or lymph nodes were palpable in the neck. Primary clinical diagnosis of benign TGDC was made considering long slow-growing history and absence of any suspicious clinical features. Biochemical investigations and thyroid profile were normal.

Ultrasound (US) examination of the neck revealed a midline cystic suprahyoid lesion with intracystic solid areas along with an isoechoic well-defined lesion in the right lobe of the thyroid gland. Contrast-enhanced computed tomography (CT) showed a ~ 4 cm × 3 cm midline complex cystic lesion of heterogeneous densities with tiny specks of calcification with a significantly enhancing posterolateral intracystic nodular compartment abutting the hyoid bone on the left side with adjacent blurred margins [Figure 1]. Magnetic resonance imaging of the neck showed a midline complex cystic lesion in the upper anterior neck with altered signal intensities of nonspecific nature in the right lobe of the thyroid. Thyroid scan revealed normal thyroid function without any hypo/hyperfunctioning nodules, and the mid-line swelling did not show any uptake.
Figure 1: Primary papillary carcinoma of the thyroglossal duct cyst in the CT scan as soft tissue density midline mass (a) at infrahyoid level, (b) at the level ofthyroid cartilage

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US-guided aspiration cytology from midline lesion favored nuclear features of papillary carcinoma in a background of extensive cystic degeneration (Bethesda Category V) and from right thyroid swelling showed thin colloid with features of an adenomatous nodule with cystic degeneration (Bethesda Category II). In view of suspicious nuclear features seen in FNA cytology, infiltrating margins, microcalcifications, and mixed densities in the radiologic investigations, a radical Sistrunk's procedure including cuff of surrounding muscles along with total thyroidectomy was planned and performed [Figure 2].
Figure 2: (a and b) Preoperative appearance as a midline mass in front of the neck. (c-e) Intraoperative steps of Sistrunk operation and total thyroidectomy exposing the cyst and thyroid gland. (f) Excised mass including thyroglossal duct cyst carcinoma and total thyroidectomy

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Postoperative calcium and Parathormone (PTH) were normal with functional vocal cords. Gross examination of the surgical specimen of the thyroid gland showed a cystic nodule measuring 1.5 cm × 1.3 cm × 0.8 cm in the right lobe of the thyroid, while the left lobe was unremarkable. The primary specimen was TGDC measuring 4 cm × 3.5 cm × 2.5 cm with attached hyoid bone which revealed a papillary growth measuring 1.0 cm × 1.2 cm × 0.8 cm attached to the cyst wall on cut section. The thickness of the cyst wall ranged from 0.2 to 0.3 cm.

Histopathology displayed tumor in the thyroglossal cyst wall which was arranged in papillae and follicular pattern lined with cuboidal cells, having nuclear overlapping, optically clear chromatin, nuclear grooves, and intranuclear cytoplasmic inclusions. It also showed focal areas of dystrophic calcification [Figure 3]. Histology of the attached hyoid bone was unremarkable. Thyroidectomy specimen did not reveal any malignant features in histology. The right lobe of the thyroid showed collections of small follicles projecting into the lumen of large actively secreting follicles forming Sanderson's polsters. The left lobe of the thyroid was unremarkable.
Figure 3: (a) Photomicrograph showing thyroglossal duct cyst wall and papillary carcinoma arising from it, predominantly follicular pattern (H and E staining, ×40). (b) Cells displaying optic clearing of nuclei and nuclear grooves (H and E staining, ×400)

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The final diagnosis of papillary carcinoma of TGDC was made, and the patient was then sent for radioiodine ablation in view of high-risk features.


  Discussion Top


TGDCs primarily affect children and young adults till the third decade of life. However, TGDC carcinomas which constitute <1% of the TGDCs have a median age for TGDC as 39.5 years at presentation with sex predilection toward females with a ratio of 2.9:1.

The origin of these cancerous lesions in TGDC has often fascinated the scientists. The proposed theories of origin include a de novo origin from rests of thyroid tissue which is supported by the finding of the presence of thyroid tissue in 62% of cases of TGDCs operated with Sistrunk's surgery, which becomes more logical in the context of no reported cases of a medullary carcinoma in the TGDC.[10] The second proposed metastatic theory suggests it to be a metastasis from the occult thyroid gland primaries of papillary carcinoma.[11] The only tumor that arises from the duct lining of TGDC is squamous cell carcinoma[6] which is theoretically the only true carcinoma as well as the only primary thyroglossal cyst tumor.

Presentation, diagnosis, and treatment of primary papillary carcinoma in TGDC are more aggressive and difficult to deal in comparison to those which arise primarily within the thyroid as TGDC cancers may also involve the thyroid gland and cervical lymph nodes at presentation requiring careful surgical planning. The most common presentation of TGDC carcinoma is an asymptomatic neck mass (95.1%) with an incidental diagnosis (73.3%),[12] and hence, workup includes imaging in addition to clinical evaluation. Although ultrasonography (USG) clues of microcalcifications along the wall[13] and similar CT findings of calcifications around the wall with enhancement[14] can help suspect the diagnosis, preoperative radiological investigations are often within the normal limits. Contrast to the 85% sensitivity of FNA biopsy in primary thyroid cancers, TGDC carcinomas have low sensitivity rates of 56%–62% with positive predictive value of 69%[15] due to the aspiration of cystic fluid along with the FNA. Peretz et al.[16] suggest clinical features similar to TGDCs, similar USG features between TGDC carcinoma and inflammatory TGDCs, and true positive rate of 53% by FNA biopsy to be the possible causes for preoperative diagnosis. On the contrary, in the present case, radiological presentation of nodular solid areas with microcalcifications along the cyst wall with indistinct adjacent margins and suspicious nuclear features in USG-guided FNA helped us to suspect TGDC papillary carcinoma preoperatively and perform a single setting radical Sistrunk surgery including a cuff of surrounding tethered strap muscles along with total thyroidectomy.

Anticipation and suspicion of the possibility of a carcinoma which can involve thyroid and neck nodes has to be considered at the presentation for adequate surgical planning. Controversies still exist concerning its management of TGDC cancers due to its rarity. Although Sistrunk's procedure is accepted as the primary management in all cases, the role of total thyroidectomy and radioiodine therapy is controversial with the presence of contrasting opinions present in the current literature, and protocols are yet to be made.[17] Alatsakis et al.[17] describe a case of primary papillary carcinoma of TGDC in a 27-year-old female where preoperative radiological and pathological evaluation of the thyroid gland was normal, and they only performed a Sistrunk's surgery. Patel et al. in 2002 described 100% 5-year survival rate for primary cancers of TGDC and concluded that adjunction of total thyroidectomy to Sistrunk's procedure does not add to the outcome.[18] On the contrary, recent literature conveys a different opinion as documented by Roehlen et al.,[1] who performed total thyroidectomy along with Sistrunk's surgery followed by radioiodine ablation in a 21-year-old female in view of infiltration of carcinoma and bulky disease. Similarly, Verma et al.[19] also performed total thyroidectomy and radioiodine ablation in a case of TGDC carcinoma in a 48-year-old female due to increased age and greater cyst size. Normal thyroid morphology on US scan is the factor leading to the dilemma for performing a total thyroidectomy among the surgeons encountering cases of primary cancers of TGDC. Male sex, age >45 years, size >4 cm, extracystic invasion, lymph node metastases, history of neck irradiation, and the presence of nodules in the thyroid gland on thyroid scan have been considered as high-risk factors by various authors for deciding on performing a total thyroidectomy in such cases.[20] Similarly, comprehensive neck dissection including mid-jugular clearance is considered for the presence of neck nodes. Forty-three percent chance of missing a cancer in the thyroid glands in USG performed in cases of suspected TGDC cancers[21] and 60% incidence of microcarcinomas in total thyroidectomies done in cases of TGDC carcinoma[20] also support total thyroidectomy in cases of TGDCs with suspicious features. Few of the authors also opine further aggressive treatment with total thyroidectomy in all cases of TGDCs with high-risk features irrespective of the status of the thyroid gland as it assists in appropriate staging and eases the detection of metastases.[2]

In the presented case, male sex, TGDC size >4 cm, presence of solid areas within the cyst having microcalcifications, indistinct adjacent margins, suspicious nuclear features in FNA biopsy, and a coexisting right-sided thyroid nodule led us to perform total thyroidectomy along with the Sistrunk's surgery followed by radioiodine ablation. Postoperative histology also proved the diagnosis primary papillary carcinoma of TGDC. Total thyroidectomy might be avoided in cases with low-risk features such as age <45 years, size <4 cm, absence of extracapsular infiltration, history of irradiation exposure, and distant or lymphatic metastases with adequate consent and compliance for adequate and timely follow-up visits. Re-assessment and USG surveillance of the neck are to be done every 6 months during the 1st year and annually after that.[22]


  Conclusion Top


To sum up, performing primary/completion total thyroidectomy along with Sistrunk's procedure in every case of suspected TGDC carcinoma is based on the (a) presence of concurrent suspicious lesion in thyroid gland, (b) high-risk factors requiring the feasibility of radioactive iodine as an adjunctive therapy, and (c) the levels of raising serum thyroglobulin in cases with initial Sistrunk's surgery. This case serves as an illustration for the clinicians and surgeons to suspect primary cancers of TGDC in patients with atypical presentations and suspicious clinicoradiologic features to avoid a diagnostic miss ensuring proper and adequate treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Roehlen N, Takacs S, Ebeling O, Seufert J, Laubner K. Ectopic papillary thyroid carcinoma within a thyroglossal duct cyst: A case report. Medicine (Baltimore) 2017;96:e8921.  Back to cited text no. 1
    
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Dedivitis RA, Guimarães AV. Papillary thyroid carcinoma in thyroglossal duct cyst. Int Surg 2000;85:198-201.  Back to cited text no. 3
    
4.
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. 4
    
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Kalyani R, Hebbar A, Murthy S. Primary papillary carcinoma arising in thyroglossal duct cyst: A rare case report. Nat J Lab Med 2015;4:27-9.  Back to cited text no. 5
    
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Shah S, Kadakia S, Khorsandi A, Andersen A, Iacob C, Shin E, et al. Squamous cell carcinoma in a thyroglossal duct cyst: A case report with review of the literature. Am J Otolaryngol 2015;36:460-2.  Back to cited text no. 6
    
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Patel NS, Sheykholeslami K. Papillary carcinoma in thyroglossal duct cyst: Two case reports and review of the literature. Ear Nose Throat J 2016;95:E36-8.  Back to cited text no. 7
    
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Tew S, Reeve TS, Poole AG, Delbridge L. Papillary thyroid carcinoma arising in thyroglossal duct cysts: Incidence and management. Aust N Z J Surg 1995;65:717-8.  Back to cited text no. 11
    
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Cignarelli M, Ambrosi A, Marino A, Lamacchia O, Cincione R, Neri V. Three cases of papillary carcinoma and three of adenoma in thyroglossal duct cysts: Clinical-diagnostic comparison with benign thyroglossal duct cysts. J Endocrinol Invest 2002;25:947-54.  Back to cited text no. 13
    
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Branstetter BF, Weissman JL, Kennedy TL, Whitaker M. The CT appearance of thyroglossal duct carcinoma. AJNR Am J Neuroradiol 2000;21:1547-50.  Back to cited text no. 14
    
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Shahin A, Burroughs FH, Kirby JP, Ali SZ. Thyroglossal duct cyst: A cytopathologic study of 26 cases. Diagn Cytopathol 2005;33:365-9.  Back to cited text no. 15
    
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Peretz A, Leiberman E, Kapelushnik J, Hershkovitz E. Thyroglossal duct carcinoma in children: Case presentation and review of the literature. Thyroid 2004;14:777-85.  Back to cited text no. 16
    
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Alatsakis M, Drogouti M, Tsompanidou C, Katsourakis A, Chatzis I. Invasive thyroglossal duct cyst papillary carcinoma: A case report and review of the literature. Am J Case Rep 2018;19:757-62.  Back to cited text no. 17
    
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Pellegriti G, Lumera G, Malandrino P, Latina A, Masucci R, Scollo C, et al. Thyroid cancer in thyroglossal duct cysts requires a specific approach due to its unpredictable extension. J Clin Endocrinol Metab 2013;98:458-65.  Back to cited text no. 20
    
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Bakkar S, Biricotti M, Stefanini G, Ambrosini CE, Materazzi G, Miccoli P, et al. The extent of surgery in thyroglossal cyst carcinoma. Langenbecks Arch Surg 2017;402:799-804.  Back to cited text no. 21
    
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