International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 4 , Pages 274-278, December 2006

Papillary carcinoma of the thyroid in a 2-year old: Case report with review of the literature

  • S. Srikumar

      Affiliations

    • Department of ENT and Head & Neck Surgery, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK
    • ,
  • F.O. Agada

      Affiliations

    • Department of ENT and Head & Neck Surgery, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK
    • Corresponding Author InformationCorresponding author at: 10 Pavilion Way, Newton Hill, Wakefield WF1 3AJ, UK. Tel.: +44 1924 374054; fax: +44 7789 815212.
    • ,
  • S.V. Picton

      Affiliations

    • Department of Paediatric Oncology and Haematology, St. James University Hospital, Leeds, UK
    • ,
  • R. Squire

      Affiliations

    • Department of Paediatric Surgery, St. James University Hospital, Leeds, UK
    • ,
  • L.C. Knight

      Affiliations

    • Department of ENT and Head & Neck Surgery, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK

Received 5 May 2006; received in revised form 21 July 2006; accepted 23 July 2006.

Article Outline

Summary 

Thyroid carcinoma is rare in children and adolescents but tends to behave in a more biologically aggressive manner when compared with the disease in adults. Despite this, the prognosis has been reported to be very good in several series. Papillary carcinoma is the commonest type encountered. We report and discuss a case of a 2-year-old child with papillary thyroid carcinoma, which is the youngest reported case from Great Britain. We discussed the difficulty in making diagnosis and the need for early multidisciplinary involvement in management.

Keywords: Thyroid carcinoma, Papillary, Child, Paediatric

 

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1. Introduction 

Differentiated thyroid carcinoma constitutes 0.4–3% of all paediatric malignancies [1], [2]. It has aggressive clinical behaviour in children, with regional nodal metastasis occurring in up to 60–80% at presentation and with 10–20% of cases having distant metastasis [3], [4]. We describe a case of papillary thyroid carcinoma presenting in a 2-year old, which we believed is the youngest case ever reported in the UK, and also highlight the diagnostic difficulties and preferred management.

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2. Case report 

A 2-year-8-month-old male child presented with a painless but rapidly progressive anterior neck swelling of 4 weeks duration. There was no associated dysphagia, dysphonia or breathing difficulties.

Clinical examination revealed a 3cm×4cm cystic mass in the low anterior neck with no clinically palpable cervical lymphadenopathy.

The blood counts, thyroid functions tests and calcium levels were normal. Ultrasound scan of the neck revealed a complex cystic mass in the anterior neck apparently replacing the thyroid gland.

CT scan of neck revealed a 4.5cm complex cystic mass replacing the thyroid gland (Fig. 1) with superior extension towards the pyriform sinus on the right, and inferiorly extending beyond the thoracic inlet (Fig. 2). There was a minimal degree of tracheal compression to the left. There was no apparent normal thyroid tissue but small volume cervical lymphadenopathy was apparent in the posterior triangle superior to the mass. This was considered clinically and radiologically to be insignificant. The chest X-ray was normal.

A technetium uptake scan showed decreased and non-homogenous uptake of radioisotope in the thyroid area consistent with a complex cystic mass replacing the majority of the thyroid (Fig. 3).

  • View full-size image.
  • Fig. 3. 

    Demonstrates decreased and non-homogenous uptakes of radioisotope in the thyroid area on technetium uptake scan. This is consistent with a complex cystic mass replacing the majority of the thyroid.

Fine needle aspiration cytology from several areas of the mass was diagnostically inconclusive.

The neck was thus explored and revealed an encapsulated tumour mass replacing the thyroid. A near total thyroidectomy was undertaken saving 5mm of macroscopically apparently normal left upper pole of thyroid tissue and the left parathyroid glands. Both recurrent laryngeal nerves were identified and preserved.

Postoperative recovery was uneventful and normal calcium levels were maintained.

The histology confirmed papillary carcinoma with tumour extending to the superior left upper pole margin. The patient therefore subsequently received iodine (131I) ablation therapy and has remained well. Follow up duration however is currently under 1 year.

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3. Discussion 

Thyroid cancer is rare in children and represents 0.5–1.5% of all childhood malignancies [1]. The reported incidence in Great Britain is about 0.4% of all childhood malignancies [2]. A total of 82 cases of thyroid cancers in children less than 15 years old were registered in Great Britain between 1993 and 2002, of these 45 cases were of papillary carcinomas.1 Previously only one other case in an under 5 year old had been recorded in the past 25 years (a 3 year and 2 month old) (see footnote 1).

Diagnosis of thyroid swellings in children of all age groups is difficult because they are rare. However, because papillary carcinomas are extremely rare in very young children of this age group (<5 years), diagnosis can be particularly difficult. Diagnosis is often as a result of a combination of ultrasound, CT scan, fine needle aspiration cytology in the older child and lobectomy for the younger children. In young children the positive predictive value of FNA is low and therefore is not always recommended and should not delay definitive surgical procedure [5], [6].

In our case ultrasound showed a cystic mass in the anterior neck without the definitive demonstration of any separate thyroid gland. A CT scan subsequently showed the presence of a complex cystic mass in the region of the thyroid gland, and a radioiodine scan showed no defined area of normal glandular activity. This case illustrates the difficulty in distinguishing thyroid tumour from other complex cystic masses within the anterior neck using standard radiological examination. Surgical exploration may thus be required ideally supported by per operative frozen section histology sampling to assist the extent of surgery, although this was not used in our case.

Differentiated thyroid carcinomas have a more aggressive clinical behaviour in young children with a reported rate of lymph node metastasis ranging between 60% and 80% and lung metastasis present in around 20% cases at diagnosis [7]. Our patient had no clinically significant regional lymph node enlargement at presentation and no evidence of lung metastasis.

The paradox in differentiated thyroid carcinomas in children is the good prognosis reported despite the apparent aggressive clinical behaviour. The apparent aggressive clinical behaviour does not appear to relate to the biological nature of the tumour. Biologically the tumour seems to be indolent. This is best illustrated by the report from Memorial Sloan-Kettering Cancer Center, New York [3], where out of 327 patients under 21 years with differentiated thyroid cancer 83 had distant metastasis; 90% of this group had regional nodal metastasis and 48% had extrathyroidal extension and all had distant metastasis to the lung at presentation. However, despite the aggressive clinical characteristic of differentiated thyroid carcinoma in their series survival rate was 100% after 10 years [3].

The prognosis is generally excellent for differentiated thyroid cancer in children. Several reports have attributed excellent survival outcomes of up to95% survival at 20 years [3]. There is currently 100% survival for all registered UK cases (National Registry of Childhood Tumours) [5].

Thyroid cancers in general in children are more common during the teenage years with an increased incidence found in girls. Previous exposure to radiation is a definitive risk factor and has been well documented by Sigurdson's study in which a 30-fold increase in thyroid cancers in girls under 14 years of age in Belarus, who had been exposed to radioactive fallout after the Chernobyl nuclear-reactor explosion, was demonstrated [8]. Papillary carcinoma was the most commonly occurring histological variant reported.

Research has also indicated mutations in ret-proto-oncogene and p53 genes in radiation induced thyroid cancers [9], [10].

Predictive factors for recurrence includes; younger age (<16 years), regional lymph node involvement or distant metastasis at diagnosis and some histological characteristics such as a diffuse sclerosing papillary variant, noted frequently in children [11].

The lack of prospective and randomized controlled trials for well-differentiated cancers of the thyroid and the slow progression with excellent survival rates, result in continued debate as to what the ideal operative strategy should be. Many studies recommend total or near total thyroidectomy as the procedure of choice for papillary thyroid carcinoma in children [4], [12], [13], the indication for total thyroidectomy being the presence of multicentric tumour occurrence and bilateral lobe involvement. A useful monitoring tool, the thyroglobulin assay is more specific after an initial radical thyroidectomy than when there is recurrence in a remnant thyroid stump [14], [15].

However, arguments against total thyroidectomy are mainly based on occurrence of higher complication rates. Studies have also shown that extent of initial surgical intervention did not significantly influence survival outcomes [12], [16].

The other issue in the management of children with thyroid cancers is the role and extent of neck dissection. Neck dissection is indicated for palpable metastatic neck nodes but there is no proven efficacy for prophylactic neck dissection in the absence of initial palpable or radiological disease [14]. Standard curative regional neck dissection involves clearance of ipsilateral Levels II to V lymph nodes with pre and paratracheal lymph node clearance.

Radioiodine ablation with 30–150mCi of 131I to destroy any remaining functional thyroid tissue 4 weeks after surgery have been recommended, especially in children under 16 years [17].

Patients require regular follow up and their thyroglobulin levels are monitored, in addition were necessary a diagnostic 131I whole body scanning performed annually [15].

It is our view that there should be early involvement of a multidisciplinary team (MDT), when a neck mass raises the suspicion of a thyroid mass in children. This would facilitate the best management approach for each particular case from the diagnosis stage through management and follow up.

The MDT approach to managing tumour is well established in the UK. It has the advantage that it provides a broad view of the problem and the various experts can contribute, therefore providing the patient and the family a considered opinion on the optimal multimodality evaluation, treatment and follow up plan. MDT guidance is particularly valuable in children with differentiated thyroid cancer where there are uncertainties regarding the role of FNA, frozen section, extent of surgery, and radioiodine therapy.

In this case the MDT for endocrine malignancy collaborated with the paediatric oncology MDT, and included surgeons, pathologists, radiologists and specialist nurses. The MDT recommended ablating the residual thyroid tissue post initial surgery with radioiodine as completion surgery was considered to carry a significant risk to parathyroid and recurrent laryngeal nerve. The patient continues to be followed up regularly in the paediatric MDT clinic.

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4. Conclusion 

Thyroid lumps in children should be assumed to be cancer until proven otherwise as benign swellings (nodular goitres) are extremely rare. Thyroid cancers in children are uncommon but papillary carcinoma is the commonest variant. Despite their aggressive clinical behaviour, prognosis is excellent.

Optimal surgical management for papillary thyroid cancer is debatable. Surgical management often includes selective neck dissection (if necessary) and this is followed by 131I ablation.

Regular and long-term follow up is recommended for the identification of long-term consequences. A multidisciplinary team approach provides the best opportunity for such children.

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Acknowledgement 

Dr Charles Stiller, Childhood Cancer Research Group Department of Paediatrics. University Of Oxford, UK.

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References 

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  • 1 C. Stiller, Personal communication, Childhood Cancer Research Group, Department of Paediatrics, University of Oxford, April 26, 2005.

PII: S1871-4048(06)00076-1

doi:10.1016/j.pedex.2006.07.005

International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 4 , Pages 274-278, December 2006

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