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An Adolescent with Papillary Thyroid Carcinoma and Locally Metastatic Disease but No Distant Metastases

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Thyroid Cancer

Abstract

Thyroid cancer is rare in the pediatric population, but the incidence appears to be increasing, especially in adolescents. Papillary thyroid carcinoma (PTC), the most common type of thyroid cancer, represents 90% or more of cases in patients ≤18 years of age. Children with PTC are highly likely to present with extra-thyroidal extension and regional lymph node metastases. Despite often having advanced cervical disease at presentation, pediatric patients have very favorable outcomes with long-term survival decades after diagnosis, especially in the absence of distant metastases. Current data suggest that a comprehensive initial surgery performed by a high-volume thyroid surgeon is the most important factor in improving long-term disease-free survival. With the increasing acceptance that routine radioactive iodine (RAI) ablation may no longer be necessary in lower-risk patients who have had surgery by a high-volume surgeon, and given knowledge that patients naïve to RAI can still be effectively followed with serum thyroglobulin (Tg) levels and neck ultrasound (US), 131I is no longer routinely being given to all children with PTC, even in the presence of lymph node metastases at diagnosis. The use of RAI in pediatric PTC is also evolving due to concerns about possible late effects such as secondary malignancies. In 2015, the American Thyroid Association (ATA) published guidelines specifically for the management of pediatric PTC, and these guidelines try to balance the risks and benefits of 131I therapy while also advocating that each child be treated at centers of excellence where there are multidisciplinary teams experienced in the management of pediatric thyroid cancer.

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References

  1. Howlader N, Noone AM, Krapcho M, Miller D, Brest A, Yu M, et al. SEER Cancer Statistics Review, 1975–2016, based on November 2018 SEER data submission, posted to the SEER web site, April 2019. Bethesda: National Cancer Institute; [cited 2020 March 31]. Available from: https://seer.cancer.gov/csr/1975_2016/.

  2. Waguespack SG, Wasserman JD. Pediatric differentiated thyroid carcinoma. In: Mallick U, Harmer C, Mazzaferri EL, Kendall-Taylor P, editors. Practical management of thyroid cancer: a multidisciplinary approach. 2nd ed: Springer International Publishing; 2018. p. 273–94.

    Google Scholar 

  3. Zimmerman D, Hay ID, Gough IR, Goellner JR, Ryan JJ, Grant CS, et al. Papillary thyroid carcinoma in children and adults: long-term follow-up of 1039 patients conservatively treated at one institution during three decades. Surgery. 1988;104(6):1157–66.

    CAS  PubMed  Google Scholar 

  4. Frankenthaler RA, Sellin RV, Cangir A, Goepfert H. Lymph node metastasis from papillary-follicular thyroid carcinoma in young patients. Am J Surg. 1990;160(4):341–3.

    Article  CAS  PubMed  Google Scholar 

  5. Wada N, Sugino K, Mimura T, Nagahama M, Kitagawa W, Shibuya H, et al. Treatment strategy of papillary thyroid carcinoma in children and adolescents: clinical significance of the initial nodal manifestation. Ann Surg Oncol. 2009;16(12):3442–9.

    Article  PubMed  Google Scholar 

  6. Machens A, Lorenz K, Nguyen Thanh P, Brauckhoff M, Dralle H. Papillary thyroid cancer in children and adolescents does not differ in growth pattern and metastatic behavior. J Pediatr. 2010;157(4):648–52.

    Article  PubMed  Google Scholar 

  7. Newman KD, Black T, Heller G, Azizkhan RG, Holcomb GW 3rd, Sklar C, et al. Differentiated thyroid cancer: determinants of disease progression in patients <21 years of age at diagnosis: a report from the Surgical Discipline Committee of the Children’s Cancer Group. Ann Surg. 1998;227(4):533–41.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Popovtzer A, Shpitzer T, Bahar G, Feinmesser R, Segal K. Thyroid cancer in children: management and outcome experience of a referral center. Otolaryngol Head Neck Surg. 2006;135(4):581–4.

    Article  PubMed  Google Scholar 

  9. Savio R, Gosnell J, Palazzo FF, Sywak M, Agarwal G, Cowell C, et al. The role of a more extensive surgical approach in the initial multimodality management of papillary thyroid cancer in children. J Pediatr Surg. 2005;40(11):1696–700.

    Article  PubMed  Google Scholar 

  10. Byeon HK, Kim SB, Oh HS, Kim HK, Choi IH, Kim H, et al. Clinical analysis of pediatric thyroid cancer: a single medical institution experience of 18 years. Ann Otol Rhinol Laryngol. 2019;128(12):1152–7. https://doi.org/10.1177/0003489419868251.

    Article  PubMed  Google Scholar 

  11. Pawelczak M, David R, Franklin B, Kessler M, Lam L, Shah B. Outcomes of children and adolescents with well-differentiated thyroid carcinoma and pulmonary metastases following (1)(3)(1)I treatment: a systematic review. Thyroid. 2010;20(10):1095–101.

    Article  CAS  PubMed  Google Scholar 

  12. Vassilopoulou-Sellin R, Klein MJ, Smith TH, Samaan NA, Frankenthaler RA, Goepfert H, et al. Pulmonary metastases in children and young adults with differentiated thyroid cancer. Cancer. 1993;71(4):1348–52.

    Article  CAS  PubMed  Google Scholar 

  13. Bal CS, Kumar A, Chandra P, Dwivedi SN, Mukhopadhyaya S. Is chest x-ray or high-resolution computed tomography scan of the chest sufficient investigation to detect pulmonary metastasis in pediatric differentiated thyroid cancer? Thyroid. 2004;14(3):217–25.

    Article  CAS  PubMed  Google Scholar 

  14. Demidchik YE, Demidchik EP, Reiners C, Biko J, Mine M, Saenko VA, et al. Comprehensive clinical assessment of 740 cases of surgically treated thyroid cancer in children of Belarus. Ann Surg. 2006;243(4):525–32.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Handkiewicz-Junak D, Wloch J, Roskosz J, Krajewska J, Kropinska A, Pomorski L, et al. Total thyroidectomy and adjuvant radioiodine treatment independently decrease locoregional recurrence risk in childhood and adolescent differentiated thyroid cancer. J Nucl Med. 2007;48(6):879–88.

    Article  CAS  PubMed  Google Scholar 

  16. Golpanian S, Perez EA, Tashiro J, Lew JI, Sola JE, Hogan AR. Pediatric papillary thyroid carcinoma: outcomes and survival predictors in 2504 surgical patients. Pediatr Surg Int. 2016;32(3):201–8.

    Article  PubMed  Google Scholar 

  17. Hay ID, Gonzalez-Losada T, Reinalda MS, Honetschlager JA, Richards ML, Thompson GB. Long-term outcome in 215 children and adolescents with papillary thyroid cancer treated during 1940 through 2008. World J Surg. 2010;34(6):1192–202.

    Article  PubMed  Google Scholar 

  18. Hay ID, Johnson TR, Kaggal S, Reinalda MS, Iniguez-Ariza NM, Grant CS, et al. Papillary thyroid carcinoma (PTC) in children and adults: comparison of initial presentation and long-term postoperative outcome in 4432 patients consecutively treated at the Mayo Clinic during eight decades (1936-2015). World J Surg. 2018;42(2):329–42.

    Article  PubMed  Google Scholar 

  19. Vassilopoulou-Sellin R, Goepfert H, Raney B, Schultz PN. Differentiated thyroid cancer in children and adolescents: clinical outcome and mortality after long-term follow-up. Head Neck. 1998;20(6):549–55.

    Article  CAS  PubMed  Google Scholar 

  20. Hogan AR, Zhuge Y, Perez EA, Koniaris LG, Lew JI, Sola JE. Pediatric thyroid carcinoma: incidence and outcomes in 1753 patients. J Surg Res. 2009;156(1):167–72.

    Article  PubMed  Google Scholar 

  21. Bal CS, Garg A, Chopra S, Ballal S, Soundararajan R. Prognostic factors in pediatric differentiated thyroid cancer patients with pulmonary metastases. J Pediatr Endocrinol Metab. 2015;28(7-8):745–51.

    Article  PubMed  Google Scholar 

  22. Alzahrani AS, Alswailem M, Moria Y, Almutairi R, Alotaibi M, Murugan AK, et al. Lung metastasis in pediatric thyroid cancer: radiological pattern, molecular genetics, response to therapy, and outcome. J Clin Endocrinol Metab. 2019;104(1):103–10.

    Article  PubMed  Google Scholar 

  23. Samuel AM, Rajashekharrao B, Shah DH. Pulmonary metastases in children and adolescents with well-differentiated thyroid cancer. J Nucl Med. 1998;39(9):1531–6.

    CAS  PubMed  Google Scholar 

  24. Francis GL, Waguespack SG, Bauer AJ, Angelos P, Benvenga S, Cerutti JM, et al. Management guidelines for children with thyroid nodules and differentiated thyroid cancer. Thyroid. 2015;25(7):716–59.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Niedziela M, Handkiewicz-Junak D, Malecka-Tendera E, Czarniecka A, Dedecjus M, Lange D, et al. Diagnostics and treatment of differentiated thyroid carcinoma in children - Guidelines of Polish National Societies. Endokrynol Pol. 2016;67(6):628–42.

    Article  PubMed  Google Scholar 

  26. Cancer Genome Atlas Research N. Integrated genomic characterization of papillary thyroid carcinoma. Cell. 2014;159(3):676–90.

    Article  CAS  Google Scholar 

  27. Bauer AJ. Molecular genetics of thyroid cancer in children and adolescents. Endocrinol Metab Clin N Am. 2017;46(2):389–403.

    Article  Google Scholar 

  28. Prasad ML, Vyas M, Horne MJ, Virk RK, Morotti R, Liu Z, et al. NTRK fusion oncogenes in pediatric papillary thyroid carcinoma in Northeast United States. Cancer. 2016;122(7):1097–107.

    Article  CAS  PubMed  Google Scholar 

  29. Picarsic JL, Buryk MA, Ozolek J, Ranganathan S, Monaco SE, Simons JP, et al. Molecular characterization of sporadic pediatric thyroid carcinoma with the DNA/RNA ThyroSeq v2 next-generation sequencing assay. Pediatr Dev Pathol. 2016;19(2):115–22.

    Article  PubMed  Google Scholar 

  30. Nikita ME, Jiang W, Cheng SM, Hantash FM, McPhaul MJ, Newbury RO, et al. Mutational analysis in pediatric thyroid cancer and correlations with age, ethnicity, and clinical presentation. Thyroid. 2016;26(2):227–34.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Cordioli MI, Moraes L, Bastos AU, Besson P, Alves MT, Delcelo R, et al. Fusion oncogenes are the main genetic events found in sporadic papillary thyroid carcinomas from children. Thyroid. 2017;27(2):182–8.

    Article  CAS  PubMed  Google Scholar 

  32. Vanden Borre P, Schrock AB, Anderson PM, Morris JC 3rd, Heilmann AM, Holmes O, et al. Pediatric, adolescent, and young adult thyroid carcinoma harbors frequent and diverse targetable genomic alterations, including kinase fusions. Oncologist. 2017;22(3):255–63.

    Article  CAS  Google Scholar 

  33. Pekova B, Dvorakova S, Sykorova V, Vacinova G, Vaclavikova E, Moravcova J, et al. Somatic genetic alterations in a large cohort of pediatric thyroid nodules. Endocr Connect. 2019;8:796.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Mostoufi-Moab S, Labourier E, Sullivan L, LiVolsi V, Li Y, Xiao R, et al. Molecular testing for oncogenic gene alterations in pediatric thyroid lesions. Thyroid. 2018;28(1):60–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Cordioli MI, Moraes L, Cury AN, Cerutti JM. Are we really at the dawn of understanding sporadic pediatric thyroid carcinoma? Endocr Relat Cancer. 2015;22(6):R311–24.

    Article  CAS  PubMed  Google Scholar 

  36. Ballester LY, Sarabia SF, Sayeed H, Patel N, Baalwa J, Athanassaki I, et al. Integrating molecular testing in the diagnosis and management of children with thyroid lesions. Pediatr Dev Pathol. 2016;19(2):94–100.

    Article  PubMed  Google Scholar 

  37. Alzahrani AS, Murugan AK, Qasem E, Alswailem M, Al-Hindi H, Shi Y. Single point mutations in pediatric differentiated thyroid cancer. Thyroid. 2017;27(2):189–96.

    Article  CAS  PubMed  Google Scholar 

  38. Sisdelli L, Cordioli M, Vaisman F, Moraes L, Colozza-Gama GA, Alves PAG Jr, et al. AGK-BRAF is associated with distant metastasis and younger age in pediatric papillary thyroid carcinoma. Pediatr Blood Cancer. 2019;66(7):e27707.

    Article  PubMed  CAS  Google Scholar 

  39. Chakravarty D, Santos E, Ryder M, Knauf JA, Liao XH, West BL, et al. Small-molecule MAPK inhibitors restore radioiodine incorporation in mouse thyroid cancers with conditional BRAF activation. J Clin Invest. 2011;121(12):4700–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Baumgarten HD, Bauer AJ, Isaza A, Mostoufi-Moab S, Kazahaya K, Adzick NS. Surgical management of pediatric thyroid disease: complication rates after thyroidectomy at the Children's Hospital of Philadelphia high-volume Pediatric Thyroid Center. J Pediatr Surg. 2019;54:1969.

    Article  PubMed  Google Scholar 

  41. Tuggle CT, Roman SA, Wang TS, Boudourakis L, Thomas DC, Udelsman R, et al. Pediatric endocrine surgery: who is operating on our children? Surgery. 2008;144(6):869–77; discussion 77

    Article  PubMed  Google Scholar 

  42. Klein Hesselink MS, Nies M, Bocca G, Brouwers AH, Burgerhof JG, van Dam EW, et al. Pediatric differentiated thyroid carcinoma in the Netherlands: a nationwide follow-up study. J Clin Endocrinol Metab. 2016;101(5):2031–9.

    Article  PubMed  CAS  Google Scholar 

  43. Wu SY, Chiang YJ, Fisher SB, Sturgis EM, Zafereo ME, Nguyen S, et al. Risks of hypoparathyroidism after total thyroidectomy in children: a 21-year experience in a high-volume cancer center. World J Surg. 2020;44(2):442–51.

    Article  PubMed  Google Scholar 

  44. Zobel MJ, Long R, Gosnell J, Sosa JA, Padilla BE. Postoperative hypoparathyroidism after total thyroidectomy in children. J Surg Res. 2020;252:63–8.

    Article  PubMed  Google Scholar 

  45. Jarzab B, Handkiewicz Junak D, Wloch J, Kalemba B, Roskosz J, Kukulska A, et al. Multivariate analysis of prognostic factors for differentiated thyroid carcinoma in children. Eur J Nucl Med. 2000;27(7):833–41.

    Article  CAS  PubMed  Google Scholar 

  46. Sywak M, Cornford L, Roach P, Stalberg P, Sidhu S, Delbridge L. Routine ipsilateral level VI lymphadenectomy reduces postoperative thyroglobulin levels in papillary thyroid cancer. Surgery. 2006;140(6):1000–5; discussion 5–7.

    Article  PubMed  Google Scholar 

  47. Jain NK, Mostoufi-Moab S, Hawkes CP, Nelson ND, Surrey LF, Jones ZS, et al. Extrathyroidal extension is an important predictor of regional lymph node metastasis in pediatric differentiated thyroid cancer. Thyroid. 2020;30(7):1037–43.

    Article  CAS  PubMed  Google Scholar 

  48. Spinelli C, Tognetti F, Strambi S, Morganti R, Massimino M, Collini P. Cervical lymph node metastases of papillary thyroid carcinoma, in the central and lateral compartments, in children and adolescents: predictive factors. World J Surg. 2018;42(8):2444–53.

    Article  CAS  PubMed  Google Scholar 

  49. Jeon MJ, Kim YN, Sung TY, Hong SJ, Cho YY, Kim TY, et al. Practical initial risk stratification based on lymph node metastases in pediatric and adolescent differentiated thyroid cancer. Thyroid. 2018;28(2):193–200.

    Article  PubMed  Google Scholar 

  50. Lazar L, Lebenthal Y, Segal K, Steinmetz A, Strenov Y, Cohen M, et al. Pediatric thyroid cancer: postoperative classifications and response to initial therapy as prognostic factors. J Clin Endocrinol Metab. 2016;101(5):1970–9.

    Article  CAS  PubMed  Google Scholar 

  51. Lee KE, Chung IY, Kang E, Koo do H, Kim KH, Kim SW, et al. Ipsilateral and contralateral central lymph node metastasis in papillary thyroid cancer: patterns and predictive factors of nodal metastasis. Head Neck. 2013;35(5):672–6.

    Article  PubMed  Google Scholar 

  52. Verburg FA, Van Santen HM, Luster M. Pediatric papillary thyroid cancer: current management challenges. Onco Targets Ther. 2017;10:165–75.

    Article  CAS  PubMed  Google Scholar 

  53. Wu X, Gu H, Gao Y, Li B, Fan R. Clinical outcomes and prognostic factors of radioiodine ablation therapy for lymph node metastases from papillary thyroid carcinoma. Nucl Med Commun. 2018;39(1):22–7.

    Article  CAS  PubMed  Google Scholar 

  54. Albano D, Bertagna F, Panarotto MB, Giubbini R. Early and late adverse effects of radioiodine for pediatric differentiated thyroid cancer. Pediatr Blood Cancer. 2017;64.

    Google Scholar 

  55. Iyer NG, Morris LG, Tuttle RM, Shaha AR, Ganly I. Rising incidence of second cancers in patients with low-risk (T1N0) thyroid cancer who receive radioactive iodine therapy. Cancer. 2011;117(19):4439–46.

    Article  PubMed  Google Scholar 

  56. Durante C, Montesano T, Attard M, Torlontano M, Monzani F, Costante G, et al. Long-term surveillance of papillary thyroid cancer patients who do not undergo postoperative radioiodine remnant ablation: is there a role for serum thyroglobulin measurement? J Clin Endocrinol Metab. 2012;97(8):2748–53.

    Article  CAS  PubMed  Google Scholar 

  57. Evans C, Tennant S, Perros P. Thyroglobulin in differentiated thyroid cancer. Clin Chim Acta. 2015;444:310–7.

    Article  CAS  PubMed  Google Scholar 

  58. Ajdari SE, Shafiei B, Motazedian M, Qutbi M, Esmaeilzadeh P, Asli IN, et al. Shifting paradigms in the management of pediatric differentiated thyroid cancer from static to dynamic risk stratification: a step forward toward precision medicine. Nuklearmedizin. 2019;58(3):249–57.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Endocrine Neoplasia & Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Sarah J. Bottomley & Steven G. Waguespack

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Correspondence to Steven G. Waguespack .

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Editors and Affiliations

  1. Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy

    Giorgio Grani

  2. Division of Endocrinology, Diabetes, and Metabolism, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    David S. Cooper

  3. Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy

    Cosimo Durante

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Bottomley, S.J., Waguespack, S.G. (2021). An Adolescent with Papillary Thyroid Carcinoma and Locally Metastatic Disease but No Distant Metastases. In: Grani, G., Cooper, D.S., Durante, C. (eds) Thyroid Cancer. Springer, Cham. https://doi.org/10.1007/978-3-030-61919-0_11

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  • DOI: https://doi.org/10.1007/978-3-030-61919-0_11

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