SQU Med J, December 2009, Vol. 9, Iss. 3, pp. 328-332, Epub. 19th Dec 2009 Submitted - 21st mar 09 Revision Req 30th June 09, Revision Recd. 20th July 09 Accepted - 7th September 09 Thyroid carcinoma presenting with cervical lymphadenopathy as a sole presentation is uncommon.1,2,3 As metastatic cervical lymph nodes could potentially arise from several primary sources in the head and neck region, management could pose problems. Investigations may fail to reveal a primary carcinoma in the thyroid gland and hence fine needle aspiration cytology (FNAC) or biopsy is of the primary importance to establish the origin of primary tumour.5,6 Once established, management should include complete resection of the primary and metastatic carcinoma, post-operative radioactive iodine scanning and possible radioactive iodine treatment and lifelong replacement thyroxine. Case Report A 62 year-old lady presented with a 5 month history of an enlarging right neck mass [Figure 1]. She had no associated symptoms of change in voice, dysphagia, stridor, epistaxis, haemoptysis or haematemesis. Examination revealed a firm, non- tender swelling at the anterior border of the right sternomastoid muscle which measured 3 x 3cms, with restricted mobility. A detailed examination Departments of 1Surgery & 2Pathology, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman *To whom correspondence should be addressed. Email: oneilnorman@gmail.com ظهور السرطانة احلُلَيِميَّة للغدة الدرقية كورم يف عقد الرقبة الليمفاوية أسلوب العالج نورمان او مكادو ، براديب ج شوبرا ، عايشة احلمداني امللخص: السرطانة احلُلَيِميَّة للغدة الدرقية غالبا ما تظهر كورم محسوس في الغدة الدرقية ، وقد يصاحبها ألم ، بحة في الصوت ، صفير مع الشهيق ، ألم أثناء البلع و نادرا ما تظهر كورم في العقد الليمفاوية لوحدها مع وجود غدة درقية سليمة. وهذه الصورة قد تسبب مشكلة في ْفط نحيفة التشخيص والعالج. هذا تقرير عن امرأة متوسطة العمر جاءت تشكو من تورم في العقد الليمفاوية وبعد أخذ عينة بواسطة إِبْرَُة شَّ تبني انه سرطانة ُحلَيِميَّة للغدة الدرقية. كان الفحص السريري واألشعة التشخيصية للغدة الدرقية طبيعيا. فحص الرقبة أكد سالمة الغدة الدرقية متاما وتبني أن غدة العنق الليمفاوية متصلة بالَوريُد الِوداِجيُّ الغائر. خضعت املريضة الستئصال الغدة الدرقية الكامل واستئصال الغدد الليمفاوية من جانب واحد واستئصال اجلزء املتصل من الَوريُد الِوداِجيُّ الغائر. الفحص اخللوي املرضي للعينة أكد وجود انتقال سرطاني في العقدة الليمفاوية و سرطانة حليمية صغيرة بحجم 2 مم من الغدة الدرقية املستأصلة. املسح الضوئي باليود املشع بعد العملية أكد عدم انتشار املرض. املريضة أخضعت لعالج الثيروكسني مدى احلياة. ما زالت املريضة في املتابعة الدورية منذ أربع سنوات وهي بصحة جيدة. الكلمات الرئيسية: استئصال الغدة الدرقية ، سرطان الغدة الدرقية ، النقيلة الليمفاوية ،تقرير حالة ، ُعمان. abstract: Papillary carcinoma of thyroid usually presents as a palpable thyroid mass. This could be associated with pain, hoarseness, stridor or dysphagia. Rarely, it presents solely as cervical lympadenopthy in the presence of an otherwise grossly normal thyroid gland. This could pose management problems. We report here a middle-aged lady who presented with cervical lymphadenopathy which on fine needle aspiration cytology was confirmed as metastatic papillary thyroid carcinoma. The thyroid gland was, however, normal on clinical examination and radiological investigations. Neck exploration confirmed a grossly normal thyroid gland; however, the cervical lymph node was found to invade the internal jugular vein. She underwent a total thyroidectomy and unilateral functional block dissection with resection of the involved segment of the internal jugular vein. Histopathology confirmed metastasis in the lymph node and a 2mm sized microcarcinoma in the resected thyroid gland. A radioactive iodine scan in the postoperative period revealed no other metastasis. The patient was prescribed lifelong thyroxine. She is on regular follow-up and 4 years following surgery continues to do well. Key words: Thyroidectomy; Carcinoma of thyroid; Lymphatic metastasis; Case report; Oman. Papillary Carcinoma of theThyroid Presenting Primarily as Cervical Lymphadenopathy An approach to management *Norman O Machado,1 Pradeep J Chopra,1 Aisha Al Hamdani2 case report Norman O Machado, Pradeep J Chopra and Aisha Al Hamdani Case Report | 329 of the head and neck region showed no intraoral lesions. The thyroid gland was not palpable and she had no other cervical lymphadenopathy. The basic investigations, including a complete blood count, electrolytes and thyroid function tests, were all within the normal range. An indirect laryngoscopy was normal. An ultrasound scan (US) of the thyroid showed no abnormality. A FNAC of the neck mass revealed groups of follicular cells exhibiting nuclear overlapping, nuclear grooves and occasional intranuclear inclusions, consistent with metastatic papillary carcinoma of the thyroid [Figure 2]. The patient underwent neck exploration which revealed a grossly normal thyroid gland and an isolated right- sided midjugular lymph node invading the internal jugular vein. A total thyroidectomy and a functional right-sided block dissection were performed with excision of a segment of the involved internal jugular vein, sparing the sternomastoid muscle and spinal accessory nerve [Figure 3]. Histopathological examination confirmed metastatic classic papillary carcinoma in the lymph node and a 2mm sized focus of papillary microcarcinoma in the right lobe of the excised thyroid gland with nuclear features characteristic of papillary carcinoma [Figure 4]. The patient had an uneventful post-operative period. A radionuclide scan performed 6 weeks later to look for other potential metastasis revealed none. She has been on a replacement dose of thyroxine 100μg per day to lower her thyroid stimulating hormone level to below 0.1 IU. When last seen, 4 years after her surgery, she was in good health. Discussion Thyroid carcinoma commonly presents as a dominant thyroid mass in an otherwise asymptomatic patient.1,2,3 Less common presentations include isolated cervical lymphadenopathy, as in our patient, recurrent laryngeal nerve palsy, parapharyngeal masses, cervical cysts, haemoptysis and pulmonary metastasis, all of which could pose a diagnostic challenge.2,3 Even though a lateral neck mass as the initial manifestation of thyroid carcinoma is reported in some series to be present in as much Figure 1: Isolated cervical lymphnode metastasis with no apparent thyroid enlargement. Papillary Carcinoma of Thyroid Presenting Primarily as Cervical Lymphadenopathy An approach to management 330 | SQU Medical Journal, December 2009, Volume 9, Issue 3 as 10-21% of all thyroid carcinomas,1,2,3 they are usually uncommon. Clinical examination of the thyroid gland in such patients may fail to disclose easily a palpable dominant intrathyroidal mass or nodularity.1,3 The majority of the palpable cervical masses are located in the midjugular lymph node (51%) and low jugular lymph node (33%) although supraclavicular (2%), posterior cervical (1.7%) and high jugular lympadenopathy (10%) are also reported.1 These clinical findings are supported by the pattern of distribution and location of the metastatic cervical lymph node demonstrated in the pathology specimen of modified radical neck dissection.1 The diagnosis of metastatic thyroid cancer should be considered in patients presenting with isolated cervical lymphadenopathy despite a clinically normal thyroid gland. However, further evaluation is necessary to exclude other primary tumours of the scalp and head and neck region. The investigations used to assess the cervical mass of thyroid origin include radionuclide scans, US, computed tomography (CT) scans, magnetic resonance imaging (MRI) and FNAC.3-8 The diagnostic usefulness of radionuclide scanning of the thyroid in patients with isolated cervical lymphnode metastasis from thyroid carcinoma has been disappointing as less than 50% of the scans performed revealed a cold nodule in the thyroid.2,4 A combined ultrasonography and radionuclide scan of the thyroid has been reported to demonstrate the mass in 75% of these patients.1 Twenty-five per cent of patients with thyroid malignancy may have normal thyroid imaging and a clinically normal thyroid gland, as in our patient, and hence the clinician should still suspect a papillary carcinoma in the thyroid gland in the presence of lymph node metastasis.1,2 However recently, US has been reported to be very useful in predicting the risk of malignancy in nonpalpable lesions.8, In a recent review to assess the risk of malignancy in nonpalpable thyroid nodules using US and colour Doppler, features including irregular or blurred nodular margins, an intranodular vascular pattern and microcalcification were found to be closely linked to neoplastic lesions.8 On the other hand, a hypoechoic appearance or the presence of solitary lesions were not independent risk factors for malignancy in nonpalpable thyroid nodules.8 The presence of microcalcification presented a higher specificity for malignancy (95%) than the findings of irregular margin (85%) or intranodular vascular images (80.8%), but the predictive value of microcalcifiaction was blunted by their low sensitivity (29%).8 Cytological evaluation of nonpalpable nodules, presenting with a hypoechoic appearance in conjunction with at least one independent US risk factor, succeeded in identifying the vast majority of nonpalpable thyroid tumours. Papini et al. recommended that US guided FNAC should be performed on all 8-15mm hypoechoic nodules with irregular margins, intranodular vascular spots and microcalcifiaction.8 Nonpalpable lesions of the thyroid without sonographic features of risk Figure 2: Fine needle aspiration cytology of cervical mass, pap stain x 100 showing follicular cells with nuclear overlapping, inclusions (white arrow) and grooves (black arrows) suggestive of thyroid papillary metastasis. Figure 3: Operative field view revealing the exposed trachea (long arrow) after completion of total thyroidectomy and the exposed carotid artery (short arrows) and the cut ends of resected internal jugular vein (curved arrows) following functional block dissection Norman O Machado, Pradeep J Chopra and Aisha Al Hamdani Case Report | 331 for malignancy should be followed up by repeating clinical and ultrasound evaluation after 6-12 weeks.8 Scanning by US is also useful for assessing the lateral cervical mass by defining it and for differentiating its solid or cystic nature.5 While cystic swellings in the neck are likely to be of branchial cleft origin, several reports suggest the possibility of papillary carcinoma metastasis in the node when cystic.5 This is particularly so if, on US, the cystic mass reveals an internal lining that is thickened and irregular.5 Both CT and MRI scans, while providing cross-sectional imaging of the neck mass, may also detect other cervical lymphadenopathies particularly those in the retropharyngeal and parapharyngeal spaces.7 The cytological nature of the neck mass and the possibility of metastasis is initially determined by FNAC.1,6 FNAC is well tolerated, minimally invasive, reliable in the hands of an experienced cytologist and will not compromise future neck dissection.1,6 If FNAC is inconclusive and the possibility of metastasis in the node is strong, a thorough search for other possible primary sites in the oropharynx, larynx and head and neck region should be carried out. In the event that an open biopsy is required to establish the diagnosis, care should be taken in placing the incision so that it will not jeopardise the outcome of future neck dissection if required.1 The recommended treatment of patients with thyroid carcinoma presenting predominantly as cervical lymph node metastasis is total thyroidectomy with appropriate ipsilateral and, if necessary, contralateral modified radical neck dissection.1,9,10 The outcome is generally good when surgical management is followed by postoperative radioiodine scanning and lifelong replacement thyroxine.1,10,11 Contralateral lymph node metastasis, though rare, is possible and hence it is important to carry out bilateral lymph node sampling of any enlarged lymph nodes during total thyroidectomy.1,9,10 The primary lesion in the thyroid gland can vary from 2mm to several millimetres in size and, unless multiple thin histologic sections are examined, the primary lesion can be missed.1,12,13 Cervical metastasis, however, from a thyroid carcinoma and in patients who present with occult thyroid carcinoma, appears to be independent of the size of the primary thyroid carcinoma.13 This was the case in our patient who, in spite of significant sized lymph node metastasis, had only a small focus of papillary microcarcinoma of the thyroid gland. To establish a diagnosis of thyroid carcinoma in these small solitary tumours, multiple histological sections of the total thyroidectomy specimen are often necessary so that multicentric lesions can be emonstrated.8 Although small intraglandular tumour foci and cervical lymph node metastasis were found not to effect the survival rate of patients with thyroid carcinoma, considerably higher recurrence rates are observed in patients who undergo conservative operations.10,14,15 In the last 10 years, major improvements and new technologies have been proposed and applied in thyroid surgery. These include minimally invasive thyroidectomy; new devices like ultracision for achieving haemostasis and dissection; regional anaesthesia; intraoperative neuromonitoring of the recurrent laryngeal nerve to avoid its injury; parathyroid hormone assay technology and genetic screening.16 Conclusion A papillary thyroid carcinoma presenting primarily as a cervical lymphadenopathy is rare. The nature of these lesions is confirmed by a combination of radiological investigation and FNAC. The primary lesions, however, could be as small as a few millimetres in size and difficult to detect preoperatively. If cervical lymphadenopthay is Figure 4: Haematoxylin and eosin stain x 100 showing thyroid tissue with colloid filled follicles lined by follicular cells exhibiting nuclear overlapping, clearing (black arrows) and intranuclear grooves (white arrows) Papillary Carcinoma of Thyroid Presenting Primarily as Cervical Lymphadenopathy An approach to management 332 | SQU Medical Journal, December 2009, Volume 9, Issue 3 confirmed to be metastatic thyroid carcinoma, total thyroidectomy and lymph node dissection would be the most appropriate approach. When this is followed up by postoperative radionuclide scanning and a lifelong suppressive dose of thyroxine, a good outcome is most likely. References 1. De Jong SA, Demeter JG, Jarosz H, Lawrence AM, Paloyan E. Primary papillary thyroid carcinoma presenting as cervical lymphadenopathy: the operative approach to the lateral aberrant thyroid: Am Surg 1993; 59:172-7. 2. Maceri DR, Babyak J, Ossakow SJ. Lateral neck mass. Sole presenting sign of metastatic thyroid cancer. Arch Otolaryngol Head Neck Surg 1986; 112:47-9. 3. Park CS, Min JS. Lateral neck mass as the initial manifestation of thyroid carcinoma. Head Neck 1989; 11:410-13. 4. Nussbaum M, Bukachevsky R. Thyroid carcinoma presenting as a regional neck mass. Head Neck 1990; 12:114-17. 5. Park JS, Son KR, Na DG, Kim E, Kim S. Performance of preoperative sonographic staging of papillary thyroid carcinoma based on the sixth edition of AJCC/UICC TNM classification system. AJR Am J Roentgenol 2009; 192:66-72. 6. Kim MJ, Kim EK, Park SI, Kim BM, Kwak JY, Kim JS, et al. US guided fine needle aspiration of thyroid nodule: indications, technique, results. Radiographics 2008; 28:1869-86. 7. Loevner LA, Kaplan SL, Cunnane ME, Moonis G. Cross-sectional imaging of the thyroid gland. Neuroimaging Clin N Am 2008; 18:445-61. 8. Papini E, Guglielmi R, Bianchini A, Crescenzi A, Taccogna S, Nardi F et al. Risk of malignancy in nonpalpable thyroid nodules: Predictive value of ultrasound and color-doppler features. J Clin Endocrinol Metabol 2002; 87:1941-6. 9. Dralle H, Machens A. Surgical approaches in thyroid cancer and lymph node metastases. Best Pract Res Clin Endocrinol Metab 2008; 22:971-87. 10. Naruse T, Koike A, Kanemitsu T, Kato K. Minimal thyroid carcinoma: a report of nine cases discovered by cervical lymph node metastasis. Jpn J Surg 1984; 14:118-21. 11. Hubert JP Jr, Kiernan PD, Bearhs OH, McConahey WM, Woolner LB. Occult papillary carcinoma of the thyroid. Arch Surg 1980; 115:394-8. 12. Mazzaferri EL. Papillary thyroid carcinoma: factors influencing prognosis and current therapy. Semin Oncol 1987; 14:315-32. 13. Block MA. Management of carcinoma of thyroid. Ann Surg 1977; 185:133-44. 14. Clark OH. Total thyroidectomy: the treatment of choice for patients with differentiated thyroid cancer. Ann Surg 1982; 196:361-70. 15. Kuo SF, Chao TC, Chang HY, Hsueh C, Chang YC, Yang CH, et al. The role of radioactive iodine therapy in young patients with papillary thyroid cancer. Clin Nucl Med 2009; 34:4-6. 16. Dionigi G, Bacuzzi A, Bertocchi V, Carrafiello G, Boni L, Rovera F, et al. Safe incorporation of new technologies in thyroid surgery. Expert Rev Med Devices 2008; 5:747-58.