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Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Nasal Cavity, Paranasal Sinuses and Skull Base

  • Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors
  • Published:
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Abstract

The World Health Organization Classification of Head and Neck Tumours recently published the 5th edition. There are new entities, emerging entities, and significant updates to the taxonomy and characterization of tumor and tumor-like lesions, specifically in this article as it relates to nasal cavity, paranasal sinuses and skull base. Importantly, the number of diagnostic entries has been reduced by creating category-specific chapters for soft tissue, hematolymphoid, melanocytic, neuroectodermal, and metastatic tumors. Bone and salivary gland tumors are also not separately reported in the sinonasal tract, but included in the jaw and salivary gland sections, respectively. Repetition of characteristic entities in each anatomic site was also reduced, instead highlighting only the unique features in each anatomic site. Two new entities (SWI/SNF complex-deficient sinonasal carcinomas and HPV-related multiphenotypic sinonasal carcinoma) will be highlighted in this review, with a discussion of several emerging entities. There is a short description of updated information for all 24 diagnostic entities included in this edition to allow the reader a snapshot of current state of knowledge, but to encourage more investigation and further broaden understanding of these diverse and rare entities.

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References

  1. Bishop JA, Loney EL, Thompson LDR. Nasal cavity, paranasal sinuses, and skull base tumours. In: Board WCTE, editor. Head and neck tumours. 5th ed. Lyon: International Agency for Research on Cancer; 2022.

    Google Scholar 

  2. Ozolek JA, Hunt JL. Tumor suppressor gene alterations in respiratory epithelial adenomatoid hamartoma (REAH): comparison to sinonasal adenocarcinoma and inflamed sinonasal mucosa. Am J Surg Pathol. 2006;30:1576–80.

    Article  PubMed  Google Scholar 

  3. Baneckova M, Michal M, Laco J, et al. Immunohistochemical and genetic analysis of respiratory epithelial adenomatoid hamartomas and seromucinous hamartomas: are they precursor lesions to sinonasal low-grade tubulopapillary adenocarcinomas? Hum Pathol. 2020;97:94–102.

    Article  CAS  PubMed  Google Scholar 

  4. Lee JT, Garg R, Brunworth J, Keschner DB, Thompson LD. Sinonasal respiratory epithelial adenomatoid hamartomas: series of 51 cases and literature review. Am J Rhinol Allergy. 2013;27:322–8.

    Article  PubMed  Google Scholar 

  5. Daniel A, Wong E, Ho J, Singh N. Chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH): case report and literature review. Case Rep Otolaryngol. 2019;2019:5247091.

    PubMed  PubMed Central  Google Scholar 

  6. Khan RA, Chernock RD, Lewis JS Jr. Seromucinous hamartoma of the nasal cavity: a report of two cases and review of the literature. Head Neck Pathol. 2011;5:241–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Weinreb I, Gnepp DR, Laver NM, et al. Seromucinous hamartomas: a clinicopathological study of a sinonasal glandular lesion lacking myoepithelial cells. Histopathology. 2009;54:205–13.

    Article  PubMed  Google Scholar 

  8. Fleming KE, Perez-Ordonez B, Nasser JG, Psooy B, Bullock MJ. Sinonasal seromucinous hamartoma: a review of the literature and a case report with focal myoepithelial cells. Head Neck Pathol. 2012;6:395–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Schultz KA, Yang J, Doros L, et al. DICER1-pleuropulmonary blastoma familial tumor predisposition syndrome: a unique constellation of neoplastic conditions. Pathol Case Rev. 2014;19:90–100.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Mason KA, Navaratnam A, Theodorakopoulou E, Chokkalingam PG. Nasal Chondromesenchymal Hamartoma (NCMH): a systematic review of the literature with a new case report. J Otolaryngol Head Neck Surg. 2015;44:28.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Schultz KAP, Williams GM, Kamihara J, et al. DICER1 and associated conditions: identification of at-risk individuals and recommended surveillance strategies. Clin Cancer Res. 2018;24:2251–61.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Ozolek JA, Carrau R, Barnes EL, Hunt JL. Nasal chondromesenchymal hamartoma in older children and adults: series and immunohistochemical analysis. Arch Pathol Lab Med. 2005;129:1444–50.

    Article  PubMed  Google Scholar 

  13. Priest JR, Williams GM, Mize WA, Dehner LP, McDermott MB. Nasal chondromesenchymal hamartoma in children with pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry registry. Int J Pediatr Otorhinolaryngol. 2010;74:1240–4.

    Article  PubMed  Google Scholar 

  14. Yang B, Li J, Dong J. MR imaging and CT features of oncocytic papilloma of the sinonasal tract with comparison to inverted papilloma. Br J Radiol. 2018;91:20170957.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Rooper LM, Bishop JA, Westra WH. Transcriptionally active high-risk human papillomavirus is not a common etiologic agent in the malignant transformation of inverted schneiderian papillomas. Head Neck Pathol. 2017;11:346.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Mehrad M, Stelow EB, Bishop JA, et al. Transcriptionally active HPV and targetable EGFR mutations in sinonasal inverted papilloma: an association between low-risk HPV, condylomatous morphology, and cancer risk? Am J Surg Pathol. 2020;44:340–6.

    Article  PubMed  Google Scholar 

  17. Udager AM, Rolland DCM, McHugh JB, et al. High-frequency targetable EGFR mutations in sinonasal squamous cell carcinomas arising from inverted sinonasal papilloma. Cancer Res. 2015;75:2600–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Wang H, Li H, Hu L, et al. EGFR and KRAS mutations in Chinese patients with sinonasal inverted papilloma and oncocytic papilloma. Histopathology. 2019;75:274–81.

    Article  PubMed  Google Scholar 

  19. Udager AM, McHugh JB, Goudsmit CM, et al. Human papillomavirus (HPV) and somatic EGFR mutations are essential, mutually exclusive oncogenic mechanisms for inverted sinonasal papillomas and associated sinonasal squamous cell carcinomas. Ann Oncol. 2018;29:466–71.

    Article  CAS  PubMed  Google Scholar 

  20. Brown NA, Plouffe KR, Yilmaz O, et al. TP53 mutations and CDKN2A mutations/deletions are highly recurrent molecular alterations in the malignant progression of sinonasal papillomas. Mod Pathol. 2021;34:1133–42.

    Article  CAS  PubMed  Google Scholar 

  21. Rajalingam K, Schreck R, Rapp UR, Albert S. Ras oncogenes and their downstream targets. Biochim Biophys Acta. 2007;1773:1177–95.

    Article  CAS  PubMed  Google Scholar 

  22. Maisch S, Mueller SK, Traxdorf M, et al. Sinonasal papillomas: a single centre experience on 137 cases with emphasis on malignant transformation and EGFR/KRAS status in “carcinoma ex papilloma.” Ann Diagn Pathol. 2020;46:151504.

    Article  PubMed  Google Scholar 

  23. Buchwald C, Franzmann MB, Jacobsen GK, Lindeberg H. Human papillomavirus (HPV) in sinonasal papillomas: a study of 78 cases using in situ hybridization and polymerase chain reaction. Laryngoscope. 1995;105:66–71.

    Article  CAS  PubMed  Google Scholar 

  24. Syrjanen K, Syrjanen S. Detection of human papillomavirus in sinonasal papillomas: systematic review and meta-analysis. Laryngoscope. 2013;123:181–92.

    Article  PubMed  Google Scholar 

  25. Glatre R, De Kermadec H, Alsamad IA, et al. Exophytic sinonasal papillomas and nasal florid papillomatosis: a retrospective study. Head Neck. 2018;40:740–6.

    Article  PubMed  Google Scholar 

  26. Mafee MF, Peyman GA, Grisolano JE, et al. Malignant uveal melanoma and simulating lesions: MR imaging evaluation. Radiology. 1986;160:773–80.

    Article  CAS  PubMed  Google Scholar 

  27. Blumberg JM, Escobar-Stein J, Vining EM, Prasad ML. Low-grade, nonintestinal nonsalivary sinonasal adenocarcinoma associated with an exophytic schneiderian papilloma: a case report. Int J Surg Pathol. 2015;23:662–6.

    Article  CAS  PubMed  Google Scholar 

  28. Lewis JS Jr, Westra WH, Thompson LD, et al. The sinonasal tract: another potential “Hot Spot” for carcinomas with transcriptionally-active human papillomavirus. Head Neck Pathol. 2013;8:241.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Bishop JA, Guo TW, Smith DF, et al. Human papillomavirus-related carcinomas of the sinonasal tract. Am J Surg Pathol. 2013;37:185–92.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Nudell J, Chiosea S, Thompson LD. Carcinoma ex-Schneiderian papilloma (malignant transformation): a clinicopathologic and immunophenotypic study of 20 cases combined with a comprehensive review of the literature. Head Neck Pathol. 2014;8:269–86.

    Article  PubMed  PubMed Central  Google Scholar 

  31. McCuiston A, Bishop JA. Usefulness of NKX2.2 immunohistochemistry for distinguishing Ewing sarcoma from other sinonasal small round blue cell tumors. Head Neck Pathol. 2017;12:89–91.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Bishop JA, Alaggio R, Zhang L, Seethala RR, Antonescu CR. Adamantinoma-like Ewing family tumors of the head and neck: a pitfall in the differential diagnosis of basaloid and myoepithelial carcinomas. Am J Surg Pathol. 2015;39:1267–74.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Haack H, Johnson LA, Fry CJ, et al. Diagnosis of NUT midline carcinoma using a NUT-specific monoclonal antibody. Am J Surg Pathol. 2009;33:984–91.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Larque AB, Hakim S, Ordi J, et al. High-risk human papillomavirus is transcriptionally active in a subset of sinonasal squamous cell carcinomas. Mod Pathol. 2014;27:343–51.

    Article  CAS  PubMed  Google Scholar 

  35. Laco J, Sieglova K, Vosmikova H, et al. The presence of high-risk human papillomavirus (HPV) E6/E7 mRNA transcripts in a subset of sinonasal carcinomas is evidence of involvement of HPV in its etiopathogenesis. Virchows Arch. 2015;467:405–15.

    Article  CAS  PubMed  Google Scholar 

  36. El-Mofty SK, Lu DW. Prevalence of high-risk human papillomavirus DNA in nonkeratinizing (cylindrical cell) carcinoma of the sinonasal tract: a distinct clinicopathologic and molecular disease entity. Am J Surg Pathol. 2005;29:1367–72.

    Article  PubMed  Google Scholar 

  37. Alos L, Moyano S, Nadal A, et al. Human papillomaviruses are identified in a subgroup of sinonasal squamous cell carcinomas with favorable outcome. Cancer. 2009;115:2701–9.

    Article  PubMed  Google Scholar 

  38. Chowdhury N, Alvi S, Kimura K, et al. Outcomes of HPV-related nasal squamous cell carcinoma. Laryngoscope. 2017;127:1600–3.

    Article  CAS  PubMed  Google Scholar 

  39. Jiromaru R, Yamamoto H, Yasumatsu R, et al. HPV-related sinonasal carcinoma: clinicopathologic features, diagnostic utility of p16 and Rb immunohistochemistry, and EGFR copy number alteration. Am J Surg Pathol. 2020;44:305–15.

    Article  PubMed  Google Scholar 

  40. Bishop JA, Gagan J, Paterson C, McLellan D, Sandison A. Nonkeratinizing squamous cell carcinoma of the sinonasal tract with DEK-AFF2: further solidifying an emerging entity. Am J Surg Pathol. 2021;45:718–20.

    Article  PubMed  Google Scholar 

  41. Rooper LM, Agaimy A, Dickson BC, et al. DEK-AFF2 carcinoma of the sinonasal region and skull base: detailed clinicopathologic characterization of a distinctive entity. Am J Surg Pathol. 2021;45:1682–93.

    Article  PubMed  Google Scholar 

  42. Kuo YJ, Lewis JS Jr, Zhai C, et al. DEK-AFF2 fusion-associated papillary squamous cell carcinoma of the sinonasal tract: clinicopathologic characterization of seven cases with deceptively bland morphology. Mod Pathol. 2021;34:1820–30.

    Article  CAS  PubMed  Google Scholar 

  43. Stelow EB, Bellizzi AM, Taneja K, et al. NUT rearrangement in undifferentiated carcinomas of the upper aerodigestive tract. Am J Surg Pathol. 2008;32:828–34.

    Article  PubMed  Google Scholar 

  44. Lee T, Cho J, Baek CH, et al. Prevalence of NUT carcinoma in head and neck: analysis of 362 cases with literature review. Head Neck. 2020;42:924–38.

    Article  PubMed  Google Scholar 

  45. French CA, Rahman S, Walsh EM, et al. NSD3-NUT fusion oncoprotein in NUT midline carcinoma: implications for a novel oncogenic mechanism. Cancer Discov. 2014;4:928–41.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Alekseyenko AA, Walsh EM, Zee BM, et al. Ectopic protein interactions within BRD4-chromatin complexes drive oncogenic megadomain formation in NUT midline carcinoma. Proc Natl Acad Sci USA. 2017;114:E4184–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Shiota H, Elya JE, Alekseyenko AA, et al. “Z4” complex member fusions in NUT carcinoma: implications for a novel oncogenic mechanism. Mol Cancer Res. 2018;16:1826–33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Wang R, Liu W, Helfer CM, et al. Activation of SOX2 expression by BRD4-NUT oncogenic fusion drives neoplastic transformation in NUT midline carcinoma. Cancer Res. 2014;74:3332–43.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Chau NG, Ma C, Danga K, et al. An anatomical site and genetic-based prognostic model for patients with nuclear protein in testis (NUT) midline carcinoma: analysis of 124 patients. JNCI Cancer Spectr. 2020;4:94.

    Article  Google Scholar 

  50. Tilson MP, Bishop JA. Utility of p40 in the differential diagnosis of small round blue cell tumors of the sinonasal tract. Head Neck Pathol. 2014;8:141–5.

    Article  PubMed  Google Scholar 

  51. Matsuda K, Kashima J, Yatabe Y. The isoform matters in NUT carcinoma: a diagnostic pitfall of p40 immunohistochemistry. J Thorac Oncol. 2020;15:e176–8.

    Article  CAS  PubMed  Google Scholar 

  52. Bishop JA, Antonescu CR, Westra WH. SMARCB1 (INI-1)-deficient carcinomas of the sinonasal tract. Am J Surg Pathol. 2014;38:1282–9.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Agaimy A, Weichert W. SMARCA4-deficient sinonasal carcinoma. Head Neck Pathol. 2017;11:541–5.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Agaimy A, Koch M, Lell M, et al. SMARCB1(INI1)-deficient sinonasal basaloid carcinoma: a novel member of the expanding family of SMARCB1-deficient neoplasms. Am J Surg Pathol. 2014;38:1274–81.

    Article  PubMed  PubMed Central  Google Scholar 

  55. Shah AA, Jain D, Ababneh E, et al. SMARCB1 (INI-1)-deficient adenocarcinoma of the sinonasal tract: a potentially under-recognized form of sinonasal adenocarcinoma with occasional yolk sac tumor-like features. Head Neck Pathol. 2019;14:465.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Agaimy A, Hartmann A, Antonescu CR, et al. SMARCB1 (INI-1)-deficient sinonasal carcinoma: a series of 39 cases expanding the morphologic and clinicopathologic spectrum of a recently described entity. Am J Surg Pathol. 2017;41:458–71.

    Article  PubMed  PubMed Central  Google Scholar 

  57. Agaimy A, Jain D, Uddin N, Rooper LM, Bishop JA. SMARCA4-deficient sinonasal carcinoma: a series of 10 cases expanding the genetic spectrum of SWI/SNF-driven sinonasal malignancies. Am J Surg Pathol. 2020;44:703–10.

    Article  PubMed  Google Scholar 

  58. Zong Y, Liu K, Zhong B, Chen G, Wu W. Epstein-Barr virus infection of sinonasal lymphoepithelial carcinoma in Guangzhou. Chin Med J (Engl). 2001;114:132–6.

    CAS  Google Scholar 

  59. Jeng YM, Sung MT, Fang CL, et al. Sinonasal undifferentiated carcinoma and nasopharyngeal-type undifferentiated carcinoma: two clinically, biologically, and histopathologically distinct entities. Am J Surg Pathol. 2002;26:371–6.

    Article  PubMed  Google Scholar 

  60. Wenig BM. Lymphoepithelial-like carcinomas of the head and neck. Semin Diagn Pathol. 2015;32:74–86.

    Article  PubMed  Google Scholar 

  61. Rytkonen AE, Hirvikoski PP, Salo TA. Lymphoepithelial carcinoma: two case reports and a systematic review of oral and sinonasal cases. Head Neck Pathol. 2011;5:327–34.

    Article  PubMed  PubMed Central  Google Scholar 

  62. Reiersen DA, Pahilan ME, Devaiah AK. Meta-analysis of treatment outcomes for sinonasal undifferentiated carcinoma. Otolaryngol Head Neck Surg. 2012;147:7–14.

    Article  PubMed  Google Scholar 

  63. Xu CC, Dziegielewski PT, McGaw WT, Seikaly H. Sinonasal undifferentiated carcinoma (SNUC): the Alberta experience and literature review. J Otolaryngol Head Neck Surg. 2013;42:2.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Dogan S, Chute DJ, Xu B, et al. Frequent IDH2 R172 mutations in undifferentiated and poorly-differentiated sinonasal carcinomas. J Pathol. 2017;242:400–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  65. Jo VY, Chau NG, Hornick JL, Krane JF, Sholl LM. Recurrent IDH2 R172X mutations in sinonasal undifferentiated carcinoma. Mod Pathol. 2017;30:650–9.

    Article  CAS  PubMed  Google Scholar 

  66. Mito JK, Bishop JA, Sadow PM, et al. Immunohistochemical detection and molecular characterization of IDH-mutant sinonasal undifferentiated carcinomas. Am J Surg Pathol. 2018;42:1067–75.

    Article  PubMed  Google Scholar 

  67. Riobello C, Lopez-Hernandez A, Cabal VN, et al. IDH2 mutation analysis in undifferentiated and poorly differentiated sinonasal carcinomas for diagnosis and clinical management. Am J Surg Pathol. 2020;44:396–405.

    Article  PubMed  Google Scholar 

  68. Birkeland AC, Burgin SJ, Yanik M, et al. Pathogenetic analysis of sinonasal teratocarcinosarcomas reveal actionable beta-catenin overexpression and a beta-catenin mutation. J Neurol Surg B Skull Base. 2017;78:346–52.

    Article  PubMed  PubMed Central  Google Scholar 

  69. Rooper LM, Uddin N, Gagan J, et al. Recurrent loss of SMARCA4 in sinonasal teratocarcinosarcoma. Am J Surg Pathol. 2020;44:1331–9.

    Article  PubMed  Google Scholar 

  70. Bishop JA, Andreasen S, Hang JF, et al. HPV-related multiphenotypic sinonasal carcinoma: an expanded series of 49 cases of the tumor formerly known as HPV-related carcinoma with adenoid cystic carcinoma-like features. Am J Surg Pathol. 2017;41:1690–701.

    Article  PubMed  PubMed Central  Google Scholar 

  71. Bishop JA, Ogawa T, Stelow EB, et al. Human papillomavirus-related carcinoma with adenoid cystic-like features: a peculiar variant of head and neck cancer restricted to the sinonasal tract. Am J Surg Pathol. 2013;37:836–44.

    Article  PubMed  PubMed Central  Google Scholar 

  72. Bishop JA, Westra WH. Human papillomavirus-related multiphenotypic sinonasal carcinoma: an emerging tumor type with a unique microscopic appearance and a paradoxical clinical behaviour. Oral Oncol. 2018;87:17–20.

    Article  PubMed  Google Scholar 

  73. Rooper LM, McCuiston AM, Westra WH, Bishop JA. SOX10 immunoexpression in basaloid squamous cell carcinomas: a diagnostic pitfall for ruling out salivary differentiation. Head Neck Pathol. 2018;13:543–7.

    Article  PubMed  PubMed Central  Google Scholar 

  74. Antony VM, Kakkar A, Sikka K, et al. p16 Immunoexpression in sinonasal and nasopharyngeal adenoid cystic carcinomas: a potential pitfall in ruling out HPV-related multiphenotypic sinonasal carcinoma. Histopathology. 2020;77:989–93.

    Article  PubMed  Google Scholar 

  75. Choussy O, Ferron C, Vedrine PO, et al. Adenocarcinoma of ethmoid: a GETTEC retrospective multicenter study of 418 cases. Laryngoscope. 2008;118:437–43.

    Article  PubMed  Google Scholar 

  76. Donhuijsen K, Kollecker I, Petersen P, Gassler N, Wolf J, Schroeder HG. Clinical and morphological aspects of adenocarcinomas of the intestinal type in the inner nose: a retrospective multicenter analysis. Eur Arch Otorhinolaryngol. 2016;273:3207–13.

    Article  CAS  PubMed  Google Scholar 

  77. Fiaux-Camous D, Chevret S, Oker N, et al. Prognostic value of the seventh AJCC/UICC TNM classification of intestinal-type ethmoid adenocarcinoma: systematic review and risk prediction model. Head Neck. 2017;39:668–78.

    Article  PubMed  Google Scholar 

  78. Barnes L. Intestinal-type adenocarcinoma of the nasal cavity and paranasal sinuses. Am J Surg Pathol. 1986;10:192–202.

    Article  CAS  PubMed  Google Scholar 

  79. Franchi A, Massi D, Palomba A, Biancalani M, Santucci M. CDX-2, cytokeratin 7 and cytokeratin 20 immunohistochemical expression in the differential diagnosis of primary adenocarcinomas of the sinonasal tract. Virchows Arch. 2004;445:63–7.

    CAS  PubMed  Google Scholar 

  80. Kennedy MT, Jordan RC, Berean KW, Perez-Ordonez B. Expression pattern of CK7, CK20, CDX-2, and villin in intestinal-type sinonasal adenocarcinoma. J Clin Pathol. 2004;57:932–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  81. Sjostedt S, Schmidt AY, Vieira FG, et al. Major driver mutations are shared between sinonasal intestinal-type adenocarcinoma and the morphologically identical colorectal adenocarcinoma. J Cancer Res Clin Oncol. 2021;147:1019–27.

    Article  CAS  PubMed  Google Scholar 

  82. Perrone F, Oggionni M, Birindelli S, et al. TP53, p14ARF, p16INK4a and H-ras gene molecular analysis in intestinal-type adenocarcinoma of the nasal cavity and paranasal sinuses. Int J Cancer. 2003;105:196–203.

    Article  CAS  PubMed  Google Scholar 

  83. Holmila R, Bornholdt J, Heikkila P, et al. Mutations in TP53 tumor suppressor gene in wood dust-related sinonasal cancer. Int J Cancer. 2010;127:578–88.

    Article  CAS  PubMed  Google Scholar 

  84. Perez-Escuredo J, Martinez JG, Vivanco B, et al. Wood dust-related mutational profile of TP53 in intestinal-type sinonasal adenocarcinoma. Hum Pathol. 2012;43:1894–901.

    Article  CAS  PubMed  Google Scholar 

  85. Garcia-Inclan C, Lopez F, Perez-Escuredo J, et al. EGFR status and KRAS/BRAF mutations in intestinal-type sinonasal adenocarcinomas. Cell Oncol (Dordr). 2012;35:443–50.

    Article  CAS  Google Scholar 

  86. Lopez F, Garcia Inclan C, Perez-Escuredo J, et al. KRAS and BRAF mutations in sinonasal cancer. Oral Oncol. 2012;48:692–7.

    Article  CAS  PubMed  Google Scholar 

  87. Franchi A, Innocenti DR, Palomba A, et al. Low prevalence of K-RAS, EGF-R and BRAF mutations in sinonasal adenocarcinomas. Implications for anti-EGFR treatments. Pathol Oncol Res. 2014;20:571–9.

    Article  CAS  PubMed  Google Scholar 

  88. Heffner DK, Hyams VJ, Hauck KW, Lingeman C. Low-grade adenocarcinoma of the nasal cavity and paranasal sinuses. Cancer. 1982;50:312–22.

    Article  CAS  PubMed  Google Scholar 

  89. Neto AG, Pineda-Daboin K, Luna MA. Sinonasal tract seromucous adenocarcinomas: a report of 12 cases. Ann Diagn Pathol. 2003;7:154–9.

    Article  CAS  PubMed  Google Scholar 

  90. Skalova A, Cardesa A, Leivo I, et al. Sinonasal tubulopapillary low-grade adenocarcinoma. Histopathological, immunohistochemical and ultrastructural features of poorly recognised entity. Virchows Arch. 2003;443:152–8.

    Article  CAS  PubMed  Google Scholar 

  91. Storck K, Hadi UM, Simpson R, Ramer M, Brandwein-Gensler M. Sinonasal renal cell-like adenocarcinoma: a report on four patients. Head Neck Pathol. 2008;2:75–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  92. Shen T, Shi Q, Velosa C, et al. Sinonasal renal cell-like adenocarcinomas: robust carbonic anhydrase expression. Hum Pathol. 2015;46:1598–606.

    Article  CAS  PubMed  Google Scholar 

  93. Stelow EB, Jo VY, Mills SE, Carlson DL. A histologic and immunohistochemical study describing the diversity of tumors classified as sinonasal high-grade nonintestinal adenocarcinomas. Am J Surg Pathol. 2011;35:971–80.

    Article  PubMed  Google Scholar 

  94. Purgina B, Bastaki JM, Duvvuri U, Seethala RR. A subset of sinonasal non-intestinal type adenocarcinomas are truly seromucinous adenocarcinomas: a morphologic and immunophenotypic assessment and description of a novel pitfall. Head Neck Pathol. 2015;9:436–46.

    Article  PubMed  PubMed Central  Google Scholar 

  95. Andreasen S, Skalova A, Agaimy A, et al. ETV6 gene rearrangements characterize a morphologically distinct subset of sinonasal low-grade non-intestinal-type adenocarcinoma: a novel translocation-associated carcinoma restricted to the sinonasal tract. Am J Surg Pathol. 2017;41:1552–60.

    Article  PubMed  Google Scholar 

  96. Villatoro TM, Mardekian SK. Two cases of sinonasal non-intestinal-type adenocarcinoma with squamoid morules expressing nuclear beta-catenin and CDX2: a curious morphologic finding supported by molecular analysis. Case Rep Pathol. 2018;2018:8741017.

    PubMed  PubMed Central  Google Scholar 

  97. Abraham SC, Montgomery EA, Giardiello FM, Wu TT. Frequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas. Am J Pathol. 2001;158:1073–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  98. Sanchez-Romero C, Carlos R, Diaz Molina JP, Thompson LDR, de Almeida OP, Rumayor PA. Nasopharyngeal angiofibroma: a clinical, histopathological and immunohistochemical study of 42 cases with emphasis on stromal features. Head Neck Pathol. 2018;12:52–61.

    Article  PubMed  Google Scholar 

  99. Carlos R, Thompson LD, Netto AC, et al. Epstein-Barr virus and human herpes virus-8 are not associated with juvenile nasopharyngeal angiofibroma. Head Neck Pathol. 2008;2:145–9.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Hwang HC, Mills SE, Patterson K, Gown AM. Expression of androgen receptors in nasopharyngeal angiofibroma: an immunohistochemical study of 24 cases. Mod Pathol. 1998;11:1122–6.

    CAS  PubMed  Google Scholar 

  101. Lasota J, Felisiak-Golabek A, Aly FZ, Wang ZF, Thompson LD, Miettinen M. Nuclear expression and gain-of-function beta-catenin mutation in glomangiopericytoma (sinonasal-type hemangiopericytoma): insight into pathogenesis and a diagnostic marker. Mod Pathol. 2015;28:715–20.

    Article  CAS  PubMed  Google Scholar 

  102. Haller F, Bieg M, Moskalev EA, et al. Recurrent mutations within the amino-terminal region of beta-catenin are probable key molecular driver events in sinonasal hemangiopericytoma. Am J Pathol. 2015;185:563–71.

    Article  CAS  PubMed  Google Scholar 

  103. Suzuki Y, Ichihara S, Kawasaki T, et al. beta-catenin (CTNNB1) mutation and LEF1 expression in sinonasal glomangiopericytoma (sinonasal-type hemangiopericytoma). Virchows Arch. 2018;473:235–9.

    Article  CAS  PubMed  Google Scholar 

  104. Thompson LD, Miettinen M, Wenig BM. Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation. Am J Surg Pathol. 2003;27:737–49.

    Article  PubMed  Google Scholar 

  105. Sangoi AR, Bishop JA. Variability of CD34 expression in sinonasal glomangiopericytoma: a potential diagnostic pitfall. Head Neck Pathol. 2020;14:459–64.

    Article  PubMed  Google Scholar 

  106. Cottone L, Eden N, Usher I, et al. Frequent alterations in p16/CDKN2A identified by immunohistochemistry and FISH in chordoma. J Pathol Clin Res. 2020;6:113–23.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  107. Bai J, Shi J, Li C, et al. Whole genome sequencing of skull-base chordoma reveals genomic alterations associated with recurrence and chordoma-specific survival. Nat Commun. 2021;12:757.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  108. Hasselblatt M, Thomas C, Hovestadt V, et al. Poorly differentiated chordoma with SMARCB1/INI1 loss: a distinct molecular entity with dismal prognosis. Acta Neuropathol. 2016;132:149–51.

    Article  PubMed  Google Scholar 

  109. Vujovic S, Henderson S, Presneau N, et al. Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol. 2006;209:157–65.

    Article  CAS  PubMed  Google Scholar 

  110. Jo VY, Hornick JL, Qian X. Utility of brachyury in distinction of chordoma from cytomorphologic mimics in fine-needle aspiration and core needle biopsy. Diagn Cytopathol. 2014;42:647–52.

    Article  PubMed  Google Scholar 

  111. Schafer DR, Thompson LD, Smith BC, Wenig BM. Primary ameloblastoma of the sinonasal tract: a clinicopathologic study of 24 cases. Cancer. 1998;82:667–74.

    Article  CAS  PubMed  Google Scholar 

  112. Kurppa KJ, Caton J, Morgan PR, et al. High frequency of BRAF V600E mutations in ameloblastoma. J Pathol. 2014;232:492–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  113. Brown NA, Rolland D, McHugh JB, et al. Activating FGFR2-RAS-BRAF mutations in ameloblastoma. Clin Cancer Res. 2014;20:5517–26.

    Article  CAS  PubMed  Google Scholar 

  114. Momota H, Ichimiya S, Ikeda T, et al. Immunohistochemical analysis of the p53 family members in human craniopharyngiomas. Brain Tumor Pathol. 2003;20:73–7.

    Article  CAS  PubMed  Google Scholar 

  115. Xin W, Rubin MA, McKeever PE. Differential expression of cytokeratins 8 and 20 distinguishes craniopharyngioma from rathke cleft cyst. Arch Pathol Lab Med. 2002;126:1174–8.

    Article  CAS  PubMed  Google Scholar 

  116. Thimsen V, John N, Buchfelder M, et al. Expression of SRY-related HMG Box Transcription Factors (Sox) 2 and 9 in craniopharyngioma subtypes and surrounding brain tissue. Sci Rep. 2017;7:15856.

    Article  PubMed  PubMed Central  Google Scholar 

  117. Buslei R, Holsken A, Hofmann B, et al. Nuclear beta-catenin accumulation associates with epithelial morphogenesis in craniopharyngiomas. Acta Neuropathol. 2007;113:585–90.

    Article  CAS  PubMed  Google Scholar 

  118. Goschzik T, Gessi M, Dreschmann V, et al. Genomic alterations of adamantinomatous and papillary craniopharyngioma. J Neuropathol Exp Neurol. 2017;76:126–34.

    CAS  PubMed  Google Scholar 

  119. Apps JR, Stache C, Gonzalez-Meljem JM, et al. CTNNB1 mutations are clonal in adamantinomatous craniopharyngioma. Neuropathol Appl Neurobiol. 2020;46:510–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  120. Beaty NB, Ahn E. Images in clinical medicine. Adamantinomatous craniopharyngioma containing teeth. N Engl J Med. 2014;370:860.

    Article  PubMed  Google Scholar 

  121. Thompson LD, Gyure KA. Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature. Am J Surg Pathol. 2000;24:640–50.

    Article  CAS  PubMed  Google Scholar 

  122. Rushing EJ, Bouffard JP, McCall S, et al. Primary extracranial meningiomas: an analysis of 146 cases. Head Neck Pathol. 2009;3:116–30.

    Article  PubMed  PubMed Central  Google Scholar 

  123. Liu Y, Wang H, Shao H, Wang C. Primary extradural meningiomas in head: a report of 19 cases and review of literature. Int J Clin Exp Pathol. 2015;8:5624–32.

    PubMed  PubMed Central  Google Scholar 

  124. Menke JR, Raleigh DR, Gown AM, Thomas S, Perry A, Tihan T. Somatostatin receptor 2a is a more sensitive diagnostic marker of meningioma than epithelial membrane antigen. Acta Neuropathol. 2015;130:441–3.

    Article  PubMed  Google Scholar 

  125. Silva CB, Ongaratti BR, Trott G, et al. Expression of somatostatin receptors (SSTR1-SSTR5) in meningiomas and its clinicopathological significance. Int J Clin Exp Pathol. 2015;8:13185–92.

    CAS  PubMed  PubMed Central  Google Scholar 

  126. Wu W, Zhou Y, Wang Y, et al. Clinical significance of somatostatin receptor (SSTR) 2 in meningioma. Front Oncol. 2020;10:1633.

    Article  PubMed  PubMed Central  Google Scholar 

  127. Cleary JO, Yeung J, McMeekin H, Wilhelm T, Wagner T. The significance of incidental brain uptake on 68Ga-DOTATATE PET-CT in neuroendocrine tumour patients. Nucl Med Commun. 2016;37:1197–205.

    Article  CAS  PubMed  Google Scholar 

  128. Parghane RV, Talole S, Basu S. Prevalence of hitherto unknown brain meningioma detected on (68)Ga-DOTATATE positron-emission tomography/computed tomography in patients with metastatic neuroendocrine tumor and exploring potential of (177)Lu-DOTATATE peptide receptor radionuclide therapy as single-shot treatment approach targeting both tumors. World J Nucl Med. 2019;18:160–70.

    Article  PubMed  PubMed Central  Google Scholar 

  129. Juratli TA, Thiede C, Koerner MVA, et al. Intratumoral heterogeneity and TERT promoter mutations in progressive/higher-grade meningiomas. Oncotarget. 2017;8:109228–37.

    Article  PubMed  PubMed Central  Google Scholar 

  130. Mirian C, Duun-Henriksen AK, Juratli T, et al. Poor prognosis associated with TERT gene alterations in meningioma is independent of the WHO classification: an individual patient data meta-analysis. J Neurol Neurosurg Psychiatry. 2020;91:378–87.

    Article  PubMed  Google Scholar 

  131. Rostomily RC, Elias M, Deng M, et al. Clinical utility of somatostatin receptor scintigraphic imaging (octreoscan) in esthesioneuroblastoma: a case study and survey of somatostatin receptor subtype expression. Head Neck. 2006;28:305–12.

    Article  PubMed  Google Scholar 

  132. Hasan OK, Ravi Kumar AS, Kong G, et al. Efficacy of peptide receptor radionuclide therapy for esthesioneuroblastoma. J Nucl Med. 2020;61:1326–30.

    Article  CAS  PubMed  Google Scholar 

  133. Gains JE, Aldridge MD, Mattoli MV, et al. 68Ga-DOTATATE and 123I-mIBG as imaging biomarkers of disease localisation in metastatic neuroblastoma: implications for molecular radiotherapy. Nucl Med Commun. 2020;41:1169–77.

    Article  CAS  PubMed  Google Scholar 

  134. Cracolici V, Wang EW, Gardner PA, et al. SSTR2 expression in olfactory neuroblastoma: Clin. Head Neck Pathol. 2021;15:1185–91.

    Article  PubMed  Google Scholar 

  135. Gallagher KK, Spector ME, Pepper JP, McKean EL, Marentette LJ, McHugh JB. Esthesioneuroblastoma: updating histologic grading as it relates to prognosis. Ann Otol Rhinol Laryngol. 2014;123:353–8.

    Article  PubMed  Google Scholar 

  136. Tajudeen BA, Arshi A, Suh JD, St John M, Wang MB. Importance of tumor grade in esthesioneuroblastoma survival: a population-based analysis. JAMA Otolaryngol Head Neck Surg. 2014;140:1124–9.

    Article  PubMed  Google Scholar 

  137. Saade RE, Hanna EY, Bell D. Prognosis and biology in esthesioneuroblastoma: the emerging role of Hyams grading system. Curr Oncol Rep. 2015;17:423.

    Article  PubMed  Google Scholar 

  138. Thompson LD. Olfactory neuroblastoma. Head Neck Pathol. 2009;3:252–9.

    Article  PubMed  PubMed Central  Google Scholar 

  139. Holbrook EH, Wu E, Curry WT, Lin DT, Schwob JE. Immunohistochemical characterization of human olfactory tissue. Laryngoscope. 2011;121:1687–701.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  140. Bishop JA, Thompson LD, Cardesa A, et al. Rhabdomyoblastic differentiation in head and neck malignancies other than rhabdomyosarcoma. Head Neck Pathol. 2015;9:507–18.

    Article  PubMed  PubMed Central  Google Scholar 

  141. Thompson LD. Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach. Mod Pathol. 2017;30:S1–26.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Head and Neck Pathology Consultations, 22543 Ventura Boulevard, Ste 220 PMB1034, Woodland Hills, CA, 91364, USA

    Lester D. R. Thompson

  2. University of Texas Southwestern Medical Center, Clements University Hospital, UH04.250, 6201 Harry Hines Blvd., Dallas, TX, 75390, USA

    Justin A. Bishop

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Thompson, L.D.R., Bishop, J.A. Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Nasal Cavity, Paranasal Sinuses and Skull Base. Head and Neck Pathol 16, 1–18 (2022). https://doi.org/10.1007/s12105-021-01406-5

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