Br. J. Surg. 1991. Vol. 78, January, 87-89

S. D.Weiss and C. C . Orlich* Department of Surgery, Contra Costa County Hospital, Martinez, California, and *General Surgery Service, Hospital San Juan de Dios, San Jose, Costa Rica Correspondence to: Stephen D.Weiss, 322 Primrose Place, Pleasant Hill, 1CA 94523, USA

Primary papillary carcinoma of a thyroglossal duct cyst: report of a case and literature review Thyroglossal duct cysts are the most common anomaly in thyroid development. They are twice asfrequent as branchial cleft abnormalities and, in children, are second only to enlarged cervical lymph nodes as the cause of neck mass. Generally, duct cysts are benign, but 1 per cent of cases may be malignant. From the world literature, 114 cases of malignant thyroglossal cysts were availablefor review. With the addition of our own case, we discuss 115 instances of duct cysts. The different types of neoplasia described included thyroid papillary carcinoma in 81.7 per cent, mixed papillary-follicular carcinoma in 6-9 per cent, squamous cell carcinoma in 5.2 per cent, follicular and adenocarcinoma in 1.7 per cent each, and malignant struma, epidermoid carcinoma and anaplastic carcinoma in 0.9 per cent each. Of the 115 cases surveyed, 35 thyroid glands were examined microscopically; of these, four (11.4 per cent) contained malignant foci. Whether these are primary malignancies of the thyroglossal duct cysts or metastases is discussed.

In 1911, Brentano relported the first case of a neoplasm arising in a thyroglossal ducl: cyst. Since that date, increasing numbers of cases have been re:ported as a result of a greater awareness of the malignant potential of thyroglossal duct cysts, and a more thorough search of the pathology specimens. During the first week of embryological development, the thyroid gland attains its final normal position in the neck by downward migration of a median anlage arising from the foramen caecum in the base of the tongue. The pyramidal lobe of the thyroid is formed by descending through the second branchial arch to just in front of the second to sixth tracheal rings. The second branchial arch gives rise to the tissue that later forms the hyoicl bone, which may surround the duct or pass it anteriorly or Ordinarily, the duct itself disappears at the sixth or seventh week of fetal life3, but occasionally it can persist. In this case, thyroid cells seeded during the process of descent may later proliferate and become functional. The persistent thyroglossal tract may then dilate to form a cyst. This is the most common anomaly in thyroid development. Thyroglossal duct remnants are twice as frequent as branchial cleft abnormalities4 and are second only to enlarged cervical lymph nodes as the cause of masses in the neck in children5. Positive identification of a thyroglossal duct cyst is accomplished by demonstrating epithelial lining of the duct or cyst and normal thyroid follicles in the wall of the ~ y s t ~Wide ,~. variations are seen in the epithelial lining of such cysts. In one series, the most ccmmon lining was stratified squamous epithelium, followed by pseudostratified, simple cuboidal and transitional epithelium’. Moreover, typical mucous and seromucinous salivary glands have been identified in the cyst wa113,7,9-l I . Althouglh gastrointestinal epithelium is common in branchogenic and dorsal enteric mediastinal cysts, it is extremely rare in thyroglossal duct remnants. Such a cyst with epithelium from the small intestine has been described”. Furthermore, a cyst containing gastric epithelium in its lining has been reported with special reference to its potential for acid and pepsin producti’on, which in turn may produce peptic ulceration, tracheal penetration, massive haemorrhage and death”. _

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Benign thyroglossal duct cysts are treated surgically. In 1928, Sistrunk described a procedure to excise these cysts. This included resection of the cyst and tract, extending to the foramen caecum at the base of the tongue in continuity with the midportion of the hyoid bone. More than 60 years later, this procedure has not been modified.

Case report A 41-year-old man was seen at the Hospital San Juan de Dios in San Jose, Costa Rica, for a neck mass he had noted incidentally 1 month earlier. He was asymptomatic. On physical examination, a mass of 4 x 5 crn was evident in the anterior midline of the neck. The mass was rubbery, was not tender, and was easily displaced by palpation. Vertical motion of the mass was present upon deglutition or protrusion of the tongue. The thyroid gland was normal and there was no palpable cervical lymphadenopathy. The patient was admitted with the diagnosis of a thyroglossal duct cyst and had en bloc excision as described by Sistrunk. The thyroid was examined at operation and was normal. A pathological examination of the thyroglossal cyst found a small, warty excrescence within the lumen. This was identified as a thyroid papillary carcinoma on histological sectioning (Figure I ). Normal epithelium and normal ectopic thyroid tissue were also identified within the cyst (Figure 2). The patient was readmitted and had near total thyroidectomy. The regional lymph nodes were palpated and found to be normal, and no biopsy was deemed necessary. Serial sections of the entire gland revealed a normal thyroid with no evidence of neoplastic foci.

Review of reported cases With the addition of our own case to 114 published cases available for re vie^^.^.^.*-' o.12-15 , t h e total number of patients discussed is 115. Of the 115 patients, the fema1e:male ratio was 3 2 . The median age was 38 (range 6-84) years. The excised cysts were 1-12 cm in size. More than 80 per cent of the cysts were 2-5 cm in size. In most cases, the thyroglossal duct cysts became manifest as an asymptomatic mass. Occasional symptoms included dysphagia, dysphonia, a draining sinus and recent change in

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Carcinoma of thyroglossal duct cyst: S. D. Weiss and C. C. Orlich

Figure 1 Phofomicrogruph showing papillary Carcinoma originaling in cyst wall

Figure 2 Photomicrograph showing ectopic thyroid tissue in cyst

size of the mass. Nearly 90 per cent of the cysts had been present for more than 1 month. The pathological diagnoses of the malignancies found within the cysts included papillary carcinoma in 94 patients (81.7 per cent), mixed papillary-follicular carcinoma in eight patients (6.9 per cent), squamous cell carcinoma in six patients (5.2 per cent), follicular carcinoma in two patients (1.7 per cent), adenocarcinoma in two patients (1.7 per cent), and single occurrences (0.9 per cent each) of malignant struma, epidermoid carcinoma and anaplastic carcinoma. Twenty-four patients had local invasion through the cyst wall and into surrounding tissues (including strap muscles) o r vascular invasion. Thirteen patients had one or more metastatic lymph nodes.

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Thirty-five thyroids were serially examined microscopically, 33 from thyroidectomies (including our case) and two from autopsies. Only four (11.4 per cent) of the 35 thyroids contained malignant foci (three had papillary carcinoma and one had mixed papillary-follicular carcinoma). One additional patient who manifested a thyroid nodule 6 years after the initial Sistrunk procedure had thyroidectomy and was found to have a papillary carcinoma’. Twenty-eight patients had thyroid scintiscans either before or after removal of their thyroglossal duct cysts. All were normal except two who showed ‘cold’ nodules. These two had thyroidectomies: one, who had a squamous cell carcinoma diagnosed within the cyst, was found to have a benign

Br. J. Surg., Vol. 78, No. 1. January1991

Carcinoma of thyroglossal duct cyst: S. D. Weiss and C. C. Orlich

multinodular gland; the other, who had an anaplastic carcinoma in the cyst, was shown to have a benign follicular adenoma of the thyroid gland. The management of each patient varied widely. Seventy-five patients had an initial Sistrunk procedure, 25 had local excision, and the remainder had other procedures. Twenty-nine patients had subsequent procedures such as thyroidectomy, re-excision or cervical lymph node dissection. Almost half of the patients who had some form of lymph node dissection (‘berry picking’ or sampling, radical or modified radical, unilateral or bilateral) had metastatic disease Adjuvant medical management included suppression with exogenous thyroid supplementation in 24 patients, radio-iodine treatment in 10 patients and external radiation therapy in six patients. The follow-up period also varied widely and was reported to be as short as 2 rnonths and as long as 28 years. Most patients were well fox the period during which they were followed.

non-metastatic disease. Thyroidectomy is recommended only if there is objective suspicion of a mass or nodule in the gland (e.g. ‘cold’ nodule on scan, palpable mass or history of radiation). Radical or modified radical neck dissection is indicated only in the presence of positive lymph nodes. Because of the prolonged course of papillary carcinoma (20 years or more), long-term follow-up is recommended.

References 1. 2. 3. 4. 5.

Discussion

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Primary carcinoma of the thyroglossal duct should conform to the following diagnostic criteria: (1) localization of the carcinoma to a clearly demonstrable thyroglossal duct as defined previously, and (2) no carcinoma at histopathological examination of the thyroid glandI6. Whether carcinomas in the thyroglossal remnants originated primarily in this tissue or represented metastasis from the thyroid gland is an iinportant issue. A papillary carcinoma separate from the main thyroid gland raises the possibility of an occult primary carcinoma in the thyroid7.’ 7 - 2 0 . Papillary carcinoma of the thyroid may indeed metastasize without a detectable lesion. in the gland itself”. Crile’’ believed that the thyroglossal duct coulad act as a natural conduit for spread of thyroid carcinomas. Juddz2 and Nutta1lZ3 believed the metastatic theory. The rather frequent finding of normal thyroid follicles in association with carcinomas in thyroglossal duct cysts suggests that they arise primarily from the cysts. Of the cases discussed here in which the thyroid gland was microscopically examined, only 11.4per cent had foci of carcinoma present, which suggests a primary origin for the thyroglossal duct carcinoma in 88.6 per cent. The cases found to have neoplastic foci in the thyroid gland may represent metastasis from a primary carcinoma in the cyst, two independent primary carcinomas (multicentric), or indeed a primary thyroid carcinoma. In most of the cases reviewed, as well as in our own, the diagnosis of malignancy was not suspected before operation. Carcinoma was identified only at pathological examination. They were indistinguishable clinically. Patients were asymptomatic in most cases. Clinical correlation between size and change in size as an indicator of carcinoma is not a reliable finding4v9.Nevertheless, carcinoma should be suspected in any thyroglossal duct cyst which is hard, fixed, irregular or has undergone recent change24. The presence of enlarged palpable lymph nodes in the neck should suggest malignan~y~,’~. Metastasis to regional lymph nodes was present in 11.3 per cent of cases reviewed (13 of 115), and about half of these were suspected before operation. A history of irradiation to the neck or mediastinum during childhood or adolescence should arouse suspicion of carcinoma because this is a known risk factor for papillary thyroid Carcinoma of the thyroid is a slow growing, malignant tumour that may progress for 20 years or more, spreading infrequently to regionad lymph nodes before it metastasizes to distant sites such as lung, liver or bone2-4-’3-16*19 . P rimary papillary carcinoma of a thyroglossal duct cyst should not be expected to behave differently. Treatment by the Sistrunk procedure is suggested for

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8. 9. 10. 11.

12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25.

Joseph TJ, Komorowski RA. Thyroglossal duct carcinoma. Hum Pathol 1975; 6 : 717-29. Bottsford JE Jr, Weir AF. Papillary adenocarcinoma of a thyroglossal duct cyst. South Med J 1977; 70: 123940. Saharia PC. Carcinoma arising in thyroglossal duct remnant: case reports and review of the literature. Br J Surg 1975; 62: 689-91. Trail ML, Zeringue GP, Chicola JP. Carcinoma in thyroglossal duct remnants. Laryngoscope 1977; 87: 1685-91. Davies MRQ, Cywes S. Children with thyroid carcinomas. S Afr Med J 1977; 52: 223-6. Villet WT, Kemp CB. Thyroglossal duct carcinoma: a case report and review of the literature. S Afr Med J 1981; 60:795-6. Roses DF, Snively SL, Phelps RG, Cohen N, Blum M. Carcinoma ofthe thyroglossal duct. Am JSurg 1983; 145: 266-9. Stone AR, Miln DC. Thyroglossal cyst carcinoma: case presentation and a review of normal thyroglossal cysts. Br J CIin Pract 1978; 32: 233-1. Bhagavan BS, Rao DRG, Wein berg T. Carcinoma of thyroglossal duct cyst: case reports and review of the literature. Surgery 1970; 67: 281-92. Benveniste GL, Hunter R, Cook MG. Squamous carcinoma of thyroglossal duct remnants: a case report and review of the literature. Aust NZ J Surg 1980; 50: 53-5. Ranadive NU, Shroff CP. Thyroglossal cyst (a clinicopathological evaluation with special reference to its malignant potential). J Postgrad Med 1984; 30: 175-8. Chandrasoma P, Janssen M. A thyroglossal cyst lined by gastric epithelium. JAMA 1982; 247: 1406. Jaques DA, Chambers RG, Oertel JE. Thyroglossal tract carcinoma: a review of the literature and addition of eighteen cases. Am J Sury 1970; 120: 43946. Stanley DG, Robinson FW. Thyroid carcinoma in thyroglossal duct cyst: a case report and literature review. Am Surg 1970; 36: 581-2. Zamrazil V, N&nec J, ProkS C, Niederle B, Mafiak J, Zeman K. Primary carcinoma of the thyroglossal duct: report on a case treated by radioiodine. Neoplasma 1971; 18: 421-6. Widstrom A, Magnusson P, Hallberg 0, Hellqvist H, Riiber H. Adenocarcinoma originating in the thyroglossal duct. Ann Otol Rhino1 Laryngol 1976; 85: 286-90. LiVolsi VA, Perzin KH, Savetsky L. Carcinoma arising in median ectopic thyroid (including thyroglossal duct tissue). Cancer 1974; 34: 1303-15. Nussbaum M, Buchwald RP, Ribner A, Mori K, Litwing J. Anaplastic carcinoma arising from median ectopic thyroid (thyroglossal duct remnant). Cancer 1981; 48: 27248. Hans SS, Lee PT, Proctor B. Carcinoma arising in thyroglossal duct remnants. Ann Surg 1976; 42: 773-1. Blum M, Roses DF, Cohen N. Thyroglossal duct carcinoma. JAMA 1982; 248: 924. Crile G Jr. Papillary carcinoma of the thyroid and lateral cervical region: so called ‘lateral aberrant thyroid’. Sury Gynecol Obstet 1947; 85: 757-66. Judd ES. Thyroglossal-duct cysts and sinuses. Surg Clin North Am 1963;43: 1023-32. Nuttall FQ. Cystic metastases from papillary adenocarcinoma of the thyroid with comments concerning carcinoma associated with thyroglossal remnants. Am J Surg 1965; 109: 5 W 5 . Sohn N, Gumport SL, Blum M. Thyroglossal duct carcinoma. NY State J Med 1974; 74: 2004-5. Page CP, Kemmerer WT, Haff RC, Mazzaferri EL. Thyroid carcinomas arising in thyroglossal ducts. Ann Surg 1974; 180: 799-803.

Paper accepted 21 June 1990

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Primary papillary carcinoma of a thyroglossal duct cyst: report of a case and literature review.

Thyroglossal duct cysts are the most common anomaly in thyroid development. They are twice as frequent as branchial cleft abnormalities and, in childr...
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