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Secretory Carcinoma of the Thyroid Gland: Report of a Highly Aggressive Case Clinically Mimicking Undifferentiated Carcinoma and Review of the Literature

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Abstract

After being described in the salivary glands as a malignancy with features essentially identical to those of the breast, secretory carcinoma (SC) (formerly mammary analogue SC) has now been identified in other sites including the skin, lung, and thyroid gland. In the breast, SC has a relatively favorable prognosis. Likewise when arising in the salivary glands, it is generally considered to be a low to intermediate grade carcinoma; however, there is a range of clinical behavior with occasional patients dying of progressive disease. SCs of the thyroid gland are rare, and reports suggest a relatively aggressive behavior, at least relative to well differentiated carcinomas such as papillary carcinoma and minimally invasive follicular carcinoma. We present a patient with a highly aggressive thyroid gland SC that mimicked undifferentiated carcinoma clinically. The patient had widespread metastatic disease and died rapidly from airway compromise. We also review the literature for reported cases of thyroid gland SC in order to better establish the clinical features and expected clinical course of such tumors occurring at this site.

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References

  1. Lakhani SR, Ellis IO, Schnitt SJ, WHO classification of tumours of the breast. 4th ed. Lyon: International Agency for Research on Cancer (IARC); 2012.

    Google Scholar 

  2. McDivitt RW, Stewart FW. Breast carcinoma in children. JAMA. 1966;195:388.

    Article  CAS  Google Scholar 

  3. Tavassoli FA, Norris HJ. Secretory carcinoma of the breast. Cancer. 1980;45:2404–13.

    Article  CAS  Google Scholar 

  4. Vasudev P, Onuma K. Secretory breast carcinoma: unique, triple-negative carcinoma with a favorable prognosis and characteristic molecular expression. Arch Pathol Lab Med. 2011;135:1606–10.

    Article  Google Scholar 

  5. Skálová A, Vanecek T, Sima R, Laco J, Weinreb I, Perez-Ordonez B, et al. Mammary analogue secretory carcinoma of salivary glands, containing the ETV6-NTRK3 fusion gene: a hitherto undescribed salivary gland tumor entity. Am J Surg Pathol. 2010;34:599–608.

    PubMed  Google Scholar 

  6. Seethala RR, Stenman G. Update from the 4th edition of the World Health Organization classification of head and neck tumours: tumors of the salivary gland. Head Neck Pathol. 2017;11:55–67.

    Article  Google Scholar 

  7. El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. WHO classification of head and neck tumours. 4th ed. Lyon: International Agency for Research on Cancer; 2017.

    Google Scholar 

  8. Chiosea SI, Griffith C, Assaad A, Seethala RR. Clinicopathological characterization of mammary analogue secretory carcinoma of salivary glands. Histopathology. 2012;61:387–94.

    Article  Google Scholar 

  9. Skálová A, Vanecek T, Majewska H, Laco J, Grossman P, Simpson RH, et al. Mammary analogue secretory carcinoma of salivary glands with high-grade transformation: report of 3 cases with the ETV6-NTRK3 gene fusion and analysis of TP53, β-catenin, EGFR, and CCND1 genes. Am J Surg Pathol. 2014;38:23–33.

    Article  Google Scholar 

  10. Bishop JA, Taube JM, Su A, Binder SW, Kaxakov DV, Michal M, et al. Secretory carcinoma of the skin harboring ETV6 gene fusions: a cutaneous analogue to secretory carcinomas of the breast and salivary glands. Am J Surg Pathol. 2017;41:62–6.

    Article  Google Scholar 

  11. Hyrcza MD, Ng T, Crawford RI. Detection of the ETV6-NTRK3 translocation in cutaneous mammary-analogue secretory carcinoma. Diagn Histopathol. 2015;21:481–84.

    Article  Google Scholar 

  12. Huang T, McHugh JB, Berry GJ, Myers JL. Primary mammary analogue secretory carcinoma of the lung: a case report. Hum Pathol. 2018;74:109–13.

    Article  Google Scholar 

  13. Nguyen JK, Bridge JA, Joshi C, McKenney JK. Primary mammary analog secretory carcinoma (MASC) of the vulva with ETV6-NTRK3 fusion: a case report. Int J Gynecol Pathol. 2018.

  14. Baneckova M, Agaimy A, Andreasen S, Vanecek T, Steiner P, Slouka D, et al. Mammary analog secretory carcinoma of the nasal cavity: characterization of 2 cases and their distinction from other low-grad sinonasal adenocarcinomas. Am J Surg Pathol. 2018;42:735–43.

    Article  Google Scholar 

  15. Lurquin E, Jorissen M, Debiec-Rychter M, Hermans R, Hauben E. Mammary analogue secretory carcinoma of the sinus ethmoidalis. Histopathology. 2015;67:749–51.

    Article  Google Scholar 

  16. Stevens TM, Kovalovsky AO, Velosa C, Shi Q, Dai Q, Owen RP, et al. Mammary analog secretory carcinoma, low-grade salivary duct carcinoma, and mimickers: a comparative study. Mod Pathol. 2015;2864:1084–100.

    Article  Google Scholar 

  17. Reynolds S, Shaheen M, Olson G, Barry M, Wu J, Bocklage T. A case of primary mammary analog secretory carcinoma (MASC) of the thyroid masquerading as papillary thyroid carcinoma: potentially more than a one off. Head Neck Pathol. 2016;10:405–13.

    Article  CAS  Google Scholar 

  18. Dogan S, Wang L, Ptashkin RN, Dawson RR, Shah JP, Sherman EJ, et al. Mammary analog secretory carcinoma of the thyroid gland: a primary thyroid adenocarcinoma harboring ETV6-NTRK3 fusion. Mod Pathol. 2016;29:985–95.

    Article  CAS  Google Scholar 

  19. Dettloff J, Seethala RR, Stevens TM, Brandwein-Gensler M, Centeno BA, Otto K, et al. Mammary analog secretory carcinoma (MASC) involving the thyroid gland: a report of the first 3 cases. Head Neck Pathol. 2016;11:124–30.

    Article  Google Scholar 

  20. Rupp AP, Bocklage TJ. Mammary analog secretory carcinoma of thyroid: a case report. Diagn Cytopathol. 2017;45:45–50.

    Article  Google Scholar 

  21. Lei Y, Chiosea SI. Re-evaluating historic cohort of salivary acinic cell carcinoma with new diagnostic tools. Head Neck Pathol. 2012;6:166–70.

    Article  Google Scholar 

  22. Majewska H, Skálová A, Stodulski D, Klimkova A, Steiner P, Stankiewicz C, et al. Mammary analogue secretory carcinoma of salivary glands: a new entity associated with ETV6 gene rearrangement. Virchows Arch. 2015;466:245–54.

    Article  CAS  Google Scholar 

  23. Horowitz DP, Sharma CS, Connolly E, Gidea-Addeo D, Deutsch I. Secretory carcinoma of the breast: results from the survival, epidemiology and end results database. Breast. 2012;21:350–3.

    Article  Google Scholar 

  24. Bishop JA, Taube JM, Su A, Binder SW, Kazakov DV, Michal M, et al. Secretory carcinoma of the skin harboring ETV6 gene fusions: a cutaneous analogue to secretory carcinomas of the breast and salivary glands. Am J Surg Pathol. 2017;41:62–6.

    Article  Google Scholar 

  25. Wu EY, Lebastchi J, Marqusee E, Lorch JH, Krane JF, Barletta JA. A case of primary secretory carcinoma of the thyroid with high-grade features. Histopathology. 2017;71:665–69.

    Article  Google Scholar 

  26. Liao H, Khan A, Miron PM, Cornejo KM. Mammary analogue secretory carcinoma of the thyroid mimicking locally advanced papillary thyroid carcinoma: a rare case report. Int J Surg Pathol. 2018;26:459–63.

    Article  Google Scholar 

  27. Sethi R, Kozin E, Remenschneider A, Meier J, VanderLaan P, Faquin W, et al. Mammary analogue secretory carcinoma: update on a new diagnosis of salivary gland malignancy. Laryngoscope. 2014;124:188–95.

    Article  Google Scholar 

  28. Khalele BA. Systematic review of mammary analog secretory carcinoma of salivary glands at 7 years after description. Head Neck. 2017;39:1243–48.

    Article  Google Scholar 

  29. Bishop JA. Unmasking MASC: bringing to light the unique morphologic, immunohistochemical and genetic features of the newly recognized mammary analogue secretory carcinoma of salivary glands. Head Neck Pathol. 2013;7:35–9.

    Article  Google Scholar 

  30. Simpson RHW, Bacani J, Skalova A. (2018) United States and Canadian Academy of Pathology evening speciality conference—head, neck, & endocrine pathology. In: Evening speciality conference: challenging cases and concepts in head and neck and endocrine pathology. Vancouver, BC, Canada.

  31. Krausz T, Jenkins D, Grontoft O, Pollock DJ, Azzopardi JG. Secretory carcinoma of the breast in adults: emphasis on late recurrence and metastasis. Histopathology. 1989;14:25–36.

    Article  CAS  Google Scholar 

  32. Kralik JM, Kranewitter W, Boesmueller H, Marschon R, Tschurtschenthaler G, Rumpold H, et al. Characterization of a newly identified ETV6-NTRK3 fusion transcript in acute myeloid leukemia. Diagn Pathol. 2011;6:19.

    Article  CAS  Google Scholar 

  33. Tognon C, Knezevich SR, Huntsman D, Roskelley CD, Melnyk N, Mathers JA, et al. Expression of the ETV6-NTRK3 gene fusion as a primary event in human secretory breast carcinoma. Cancer Cell. 2002;2:367–76.

    Article  CAS  Google Scholar 

  34. Eguchi M, Eguchi-Ishimae M, Tojo A, Morishita K, Suzuki K, Sata Y, et al. Fusion of ETV6 to neurotrophin-3 receptor TRKC in acute myeloid leukemia with t(12;15)(p13;q25). Blood. 1999;93:1355–63.

    Article  CAS  Google Scholar 

  35. Rubin BP, Chen CJ, Morgan TW, Xiao S, Grier HE, Kozakewich HP, et al. Congenital mesoblastic nephroma t(12;15) is associated with ETV6-NTRK3 gene fusion: cytogenetic and molecular relationship to congenital (infantile) fibrosarcoma. Am J Pathol. 1998;153:1451–8.

    Article  CAS  Google Scholar 

  36. Knezevich SR, McFadden DE, Tao W, Lim JF, Sorensen PH. A novel ETV6-NTRK3 gene fusion in congenital fibrosarcoma. Nat Genet. 1998;18:184–7.

    Article  CAS  Google Scholar 

  37. Seethala RR, Chiosea SI, Liu CZ, et al. Clinical and morphologic features of ETV6-NTRK3 translocated papillary thyroid carcinoma in an adult population without radiation exposure. Am J Surg Pathol. 2016;41:446–57.

    Article  Google Scholar 

  38. Ito Y, Ishibashi K, Masaki A, Fujii K, Fujiyoshi Y, Hattori H, et al. Mammary analogue secretory carcinoma of salivary glands: a clinicopathologic and molecular study including 2 cases harboring ETV6-X fusion. Am J Surg Pathol. 2015;39:602–10.

    Article  Google Scholar 

  39. Skálová A, Vanecek T, Simpson RH, Laco J, Majewska H, Baneckova M, et al. Mammary analogue secretory carcinoma of salivary glands: molecular analysis of 25 ETV6 gene rearranged tumors with lack of detection of classical ETV6-NTRK3 fusion transcript by standard RT-PCR: report of 4 cases harboring ETV6-X gene fusion. Am J Surg Pathol. 2016;40:3–13.

    Article  Google Scholar 

  40. Prasad ML, Vyas M, Horne MJ, et al. NTRK fusion oncogenes in pediatric papillary thyroid carcinoma in northeast United States. Cancer. 2016. https://doi.org/10.1002/cncr.29887.

    Article  PubMed  Google Scholar 

  41. Otsubo R, Mussazhanova Z, Akazawa Y, et al. Sporadic pediatric papillary thyroid carcinoma harboring the ETV6/NTRK3 fusion oncogene in a 7-year-old Japanese girl: a case report and review of literature. J Pediatr Endocrinol Metab. 2018;31:461–7.

    Article  Google Scholar 

  42. Skalova A, Vanecek T, Martinek P, Weinreb I, Stevens TM, Simpson RHW, et al. Molecular profiling of mammary analog secretory carcinoma revealed a subset of tumors harboring a novel ETV6-RET translocation: report of 10 cases. Am J Surg Pathol. 2018;42:234–46.

    Article  Google Scholar 

  43. Drilon A, Li G, Dogan S, Gounder M, Shen R, Arcila M, et al. What hides behind the MASC: clinical response and acquired resistance to entrectinib after ETV6-NTRK3 identification in a mammary analogue secretory carcinoma (MASC). Ann Oncol. 2016;27:920–6.

    Article  CAS  Google Scholar 

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Correspondence to James S. Lewis Jr..

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Desai, M.A., Mehrad, M., Ely, K. et al. Secretory Carcinoma of the Thyroid Gland: Report of a Highly Aggressive Case Clinically Mimicking Undifferentiated Carcinoma and Review of the Literature. Head and Neck Pathol 13, 562–572 (2019). https://doi.org/10.1007/s12105-018-0995-2

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  • DOI: https://doi.org/10.1007/s12105-018-0995-2

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