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THE “-OMAS” and “-OPIAS”:  Targeted and Philosophical Considerations Regarding Hamartomas, Choristomas, Teratomas, Ectopias, and Heterotopias in Pediatric Otorhinolaryngologic Pathology

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Abstract

The spectrum of “developmental” lesions that occur in the head and neck predominantly congenital in origin and arising at birth and/or discovered in childhood is broad and fascinating. These have been grouped into categories such as “ectopias”, “heterotopias”, “hamartomas”, and “choristomas”. On a philosophical and consequently systematic level, these lesions, mostly benign tumors seem to lack a true understanding of the pathogenetic foundation on which to base a more unified taxonomic designation. In this review, we will consider some of these select tumors as they represent syndromic associations (nasal chondromesenchymal hamartoma and DICER1 syndrome), the lingual choristoma from the perspective of its nomenclature and classification, lesions with ectopic meningothelial elements, and teratomas and the enigmatic “hairy polyp” in reference to a broader discussion of pathogenesis and pluripotent cells in the head and neck. A consistent thread will be how these lesions are designated with some final thoughts on future directions regarding the investigation of their pathogenesis and taxonomic nomenclature.

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References

  1. Leider M, Rosenblum M. A philological note on a certain classical suffix commonly used in dermatologic and general medical nomenclature. J Invest Dermatol. 1947;9(4):213–8. https://doi.org/10.1038/jid.1947.90.

    Article  CAS  PubMed  Google Scholar 

  2. Batsakis JG. Pathology consultation. Nomenclature of developmental tumors. Ann Otol Rhinol Laryngol. 1984;93(1):98–9. https://doi.org/10.1177/000348948409300122.

    Article  CAS  PubMed  Google Scholar 

  3. Ozolek JA. Selective pathologies of the head and neck in children: a developmental perspective. Adv Anat Pathol. 2009;16(5):332–58. https://doi.org/10.1097/PAP.0b013e3181b50571.

    Article  CAS  PubMed  Google Scholar 

  4. 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(12):1576–80. https://doi.org/10.1097/01.pas.0000213344.55605.77.

    Article  PubMed  Google Scholar 

  5. Tekkesin MS, Chiosea SI, Ozolek JA. Hamartoma of the oral cavity with ectopic meningothelial elements in infants: a rare entity with report of two cases. Head Neck Pathol. 2020;14(1):268–71. https://doi.org/10.1007/s12105-019-01024-2.

    Article  PubMed  Google Scholar 

  6. Batsakis JG, El-Naggar AK, Hicks MJ. Epithelial choristomas and teratomas of the tongue. Ann Otol Rhinol Laryngol. 1993;102(7):567–9. https://doi.org/10.1177/000348949310200715.

    Article  CAS  PubMed  Google Scholar 

  7. Enoz M, Suoglu Y. Salivary gland choristoma of the middle ear. Laryngoscope. 2006;116(6):1033–4. https://doi.org/10.1097/01.mlg.0000217250.08455.d3.

    Article  PubMed  Google Scholar 

  8. Gorini E, Mullace M, Migliorini L, Mevio E. Osseous choristoma of the tongue: a review of etiopathogenesis. Case Rep Otolaryngol. 2014;2014:373104. https://doi.org/10.1155/2014/373104.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Halfpenny W, Odell EW, Robinson PD. Cystic and glial mixed hamartoma of the tongue. J Oral Pathol Med. 2001;30(6):368–71. https://doi.org/10.1034/j.1600-0714.2001.300607.x.

    Article  CAS  PubMed  Google Scholar 

  10. Tekkesin MS, Ozer N, Olgac V. Sublingual dermoid cyst: a case report. Turk Patoloji Derg. 2016;32(2):135–8. https://doi.org/10.5146/tjpath.2014.01236.

    Article  PubMed  Google Scholar 

  11. Prasad MS, Charney RM, Garcia-Castro MI. Specification and formation of the neural crest: perspectives on lineage segregation. Genesis. 2019;57(1):e23276. https://doi.org/10.1002/dvg.23276.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Sambasivan R, Kuratani S, Tajbakhsh S. An eye on the head: the development and evolution of craniofacial muscles. Development. 2011;138(12):2401–15. https://doi.org/10.1242/dev.040972.

    Article  CAS  PubMed  Google Scholar 

  13. Arora V, Kim UR, Khazei HM. Delleman oorthuys syndrome: “oculocerebrocutaneous syndrome.” Indian J Ophthalmol. 2009;57(5):387–9. https://doi.org/10.4103/0301-4738.55074.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Takeyama J, Hayashi T, Sanada T, Shimanuki Y, Saito M, Shirane R. Rhabdomyomatous mesenchymal hamartoma associated with nasofrontal meningocele and dermoid cyst. J Cutan Pathol. 2005;32(4):310–3. https://doi.org/10.1111/j.0303-6987.2005.00312.x.

    Article  PubMed  Google Scholar 

  15. Schultz KAP, Rednam SP, Kamihara J, Doros L, Achatz MI, Wasserman JD, et al. PTEN, DICER1, FH, and their associated tumor susceptibility syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res. 2017;23(12):e76–82. https://doi.org/10.1158/1078-0432.CCR-17-0629.

    Article  CAS  PubMed  Google Scholar 

  16. Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, et al. DICER1 and associated conditions: identification of at-risk individuals and recommended surveillance strategies. Clin Cancer Res. 2018;24(10):2251–61. https://doi.org/10.1158/1078-0432.CCR-17-3089.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Stewart DR, Best AF, Williams GM, Harney LA, Carr AG, Harris AK, et al. Neoplasm risk among individuals with a pathogenic germline variant in DICER1. J Clin Oncol. 2019;37(8):668–76. https://doi.org/10.1200/JCO.2018.78.4678.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Stewart DR, Messinger Y, Williams GM, Yang J, Field A, Schultz KA, et al. Nasal chondromesenchymal hamartomas arise secondary to germline and somatic mutations of DICER1 in the pleuropulmonary blastoma tumor predisposition disorder. Hum Genet. 2014;133(11):1443–50. https://doi.org/10.1007/s00439-014-1474-9.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Behery RE, Bedrnicek J, Lazenby A, Nelson M, Grove J, Huang D, et al. Translocation t(12;170) (q24.1;q21) as the sole anomaly in a nasal chondromesenchymal hamartoma arising in a patient with pleuropulmonary blastoma. Pediatr Dev Pathol. 2012;15(3):249–53. https://doi.org/10.2350/11-11-1121-CR.1.

    Article  PubMed  Google Scholar 

  20. Li Y, Yang QX, Tian XT, Li B, Li Z. Malignant transformation of nasal chondromesenchymal hamartoma in adult: a case report and review of the literature. Histol Histopathol. 2013;28(3):337–44. https://doi.org/10.14670/HH-28.337.

    Article  PubMed  Google Scholar 

  21. 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. https://doi.org/10.1186/s40463-015-0077-3.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Avci H, Comoglu S, Ozturk E, Bilgic B, Kiyak OE. Nasal chondromesenchymal hamartoma: a rare nasal benign tumor. Kulak Burun Bogaz Ihtis Derg. 2016;26(5):300–3. https://doi.org/10.5606/kbbihtisas.2016.70852.

    Article  PubMed  Google Scholar 

  23. Cui YQ, Wang LL, Huang G, Zhao LP. Computed tomography and magnetic resonance imaging findings of nasal chondromesenchymal hamartoma in a young child: a case report. J Int Med Res. 2020;48(8):300060520920431. https://doi.org/10.1177/0300060520920431.

    Article  PubMed  Google Scholar 

  24. Golbin DA, Ektova AP, Demin MO, Lasunin N, Cherekaev VA. Nasal chondromesenchymal hamartoma with skull base and orbital involvement: case presentation. Cureus. 2018;10(6):e2892. https://doi.org/10.7759/cureus.2892.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Kitayama T, Akaki S, Hisazumi K, Yoshio K, Inoue D, Tajiri N, et al. An adult case of nasal chondromesenchymal hamartoma: imaging characteristics including diffusion-weighted images. Acta Med Okayama. 2019;73(6):529–32. https://doi.org/10.18926/AMO/57718.

    Article  PubMed  Google Scholar 

  26. Lee CH, Park YH, Kim JY, Bae JH. Nasal chondromesenchymal hamartoma causing sleep-disordered breathing in an infant. Int J Clin Exp Pathol. 2015;8(8):9643–6.

    PubMed  PubMed Central  Google Scholar 

  27. Mirchia K, Naous R. Nasal chondromesenchymal hamartoma: rare case report in an elderly patient and brief review of literature. Case Rep Pathol. 2018;2018:5971786. https://doi.org/10.1155/2018/5971786.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Nakaya M, Yoshihara S, Yoshitomi A, Baba S. Endoscopic endonasal excision of nasal chondromesenchymal hamartoma with intracranial extension. Eur Ann Otorhinolaryngol Head Neck Dis. 2017;134(6):423–5. https://doi.org/10.1016/j.anorl.2016.05.014.

    Article  CAS  PubMed  Google Scholar 

  29. Unal A, Kum RO, Avci Y, Unal DT. Nasal chondromesenchymal hamartoma, a rare pediatric tumor: Case report. Turk J Pediatr. 2016;58(2):208–11. https://doi.org/10.24953/turkjped.2016.02.014.

    Article  PubMed  Google Scholar 

  30. 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(11):1444–50. https://doi.org/10.1043/1543-2165(2005)129[1444:NCHIOC]2.0.CO;2.

    Article  PubMed  Google Scholar 

  31. Chai RL, Ozolek JA, Branstetter BF, Mehta DK, Simons JP. Congenital choristomas of the oral cavity in children. Laryngoscope. 2011;121(10):2100–6. https://doi.org/10.1002/lary.21758.

    Article  PubMed  Google Scholar 

  32. Jain P, Rathee M. Embryology, tongue. Treasure Island: StatPearls; 2020.

    Google Scholar 

  33. Ratajczak MZ, Shin DM, Kucia M. Very small embryonic/epiblast-like stem cells: a missing link to support the germ line hypothesis of cancer development? Am J Pathol. 2009;174(6):1985–92. https://doi.org/10.2353/ajpath.2009.081143.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Ratajczak MZ, Zuba-Surma EK, Wysoczynski M, Ratajczak J, Kucia M. Very small embryonic-like stem cells: characterization, developmental origin, and biological significance. Exp Hematol. 2008;36(6):742–51. https://doi.org/10.1016/j.exphem.2008.03.010.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Adhikari BR, Sato J, Morikawa T, Obara-Itoh J, Utsunomiya M, Harada F, et al. Osseous choristoma of the tongue: two case reports. J Med Case Rep. 2016;10:59. https://doi.org/10.1186/s13256-016-0840-8.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Sibley DA, Cooper PH. Rudimentary meningocele: a variant of “primary cutaneous meningioma.” J Cutan Pathol. 1989;16(2):72–80. https://doi.org/10.1111/j.1600-0560.1989.tb00014.x.

    Article  CAS  PubMed  Google Scholar 

  37. Thompson LDR, Fanburg-Smith JC. Update on select benign mesenchymal and meningothelial sinonasal tract lesions. Head Neck Pathol. 2016;10(1):95–108. https://doi.org/10.1007/s12105-016-0697-6.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Suster S, Rosai J. Hamartoma of the scalp with ectopic meningothelial elements. A distinctive benign soft tissue lesion that may simulate angiosarcoma. Am J Surg Pathol. 1990;14(1):1–11. https://doi.org/10.1097/00000478-199001000-00001.

    Article  CAS  PubMed  Google Scholar 

  39. Chen D, Dedhia K, Ozolek J, Mehta D. Case series of congenital heterotopic neuroglial tissue in the parapharyngeal space. Int J Pediatr Otorhinolaryngol. 2016;86:77–81. https://doi.org/10.1016/j.ijporl.2016.04.026.

    Article  PubMed  Google Scholar 

  40. Ozolek JA, Losee JE, Lopes TC, Galambos C. Temporal soft tissue glioneuronal heterotopia in a child with a seizure disorder: case report and review of the literature. Pediatr Dev Pathol. 2005;8(6):673–9. https://doi.org/10.1007/s10024-005-0007-x.

    Article  PubMed  Google Scholar 

  41. Barksdale EM Jr, Obokhare I. Teratomas in infants and children. Curr Opin Pediatr. 2009;21(3):344–9. https://doi.org/10.1097/MOP.0b013e32832b41ee.

    Article  PubMed  Google Scholar 

  42. Bernbeck B, Schneider DT, Bernbeck B, Koch S, Teske C, Lentrodt J, et al. Germ cell tumors of the head and neck: report from the MAKEI study group. Pediatr Blood Cancer. 2009;52(2):223–6. https://doi.org/10.1002/pbc.21752.

    Article  PubMed  Google Scholar 

  43. De Backer A, Madern GC, Pieters R, Haentjens P, Hakvoort-Cammel FG, Oosterhuis JW, et al. Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors. Eur J Pediatr Surg. 2008;18(1):1–6. https://doi.org/10.1055/s-2007-989399.

    Article  PubMed  Google Scholar 

  44. Tapper D, Lack EE. Teratomas in infancy and childhood. A 54-year experience at the Children’s Hospital Medical Center. Ann Surg. 1983;198(3):398–410. https://doi.org/10.1097/00000658-198309000-00016.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Schmoll HJ. Extragonadal germ cell tumors. Ann Oncol. 2002;13(Suppl 4):265–72. https://doi.org/10.1093/annonc/mdf669.

    Article  PubMed  Google Scholar 

  46. Le Douarin NM, Creuzet S, Couly G, Dupin E. Neural crest cell plasticity and its limits. Development. 2004;131(19):4637–50. https://doi.org/10.1242/dev.01350.

    Article  CAS  PubMed  Google Scholar 

  47. Hauser S, Widera D, Qunneis F, Muller J, Zander C, Greiner J, et al. Isolation of novel multipotent neural crest-derived stem cells from adult human inferior turbinate. Stem Cells Dev. 2012;21(5):742–56. https://doi.org/10.1089/scd.2011.0419.

    Article  CAS  PubMed  Google Scholar 

  48. Ozolek JA, Cohen DE, Kool M, Pfister SM, Korshunov A, Bukowinski AJ, et al. Medulloepithelioma with peculiar clinical presentation, stem cell phenotype and aberrant DNA-methylation profile. CNS Oncol. 2015;4(4):203–12. https://doi.org/10.2217/cns.15.9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Oosterhuis JW, Looijenga LHJ. Human germ cell tumours from a developmental perspective. Nat Rev Cancer. 2019;19(9):522–37. https://doi.org/10.1038/s41568-019-0178-9.

    Article  CAS  PubMed  Google Scholar 

  50. Oosterhuis JW, Stoop H, Honecker F, Looijenga LH. Why human extragonadal germ cell tumours occur in the midline of the body: old concepts, new perspectives. Int J Androl. 2007;30(4):256–63. https://doi.org/10.1111/j.1365-2605.2007.00793.x (discussion 63–4).

    Article  PubMed  Google Scholar 

  51. Planas S, Ferreres JC, Ortega A, Higueras T, Carreras E, Ramon YCS, et al. Association of congenital hypothalamic hamartoma and hairy polyp. Ultrasound Obstet Gynecol. 2009;33(5):609–10. https://doi.org/10.1002/uog.6314.

    Article  CAS  PubMed  Google Scholar 

  52. Tariq MU, Din NU, Bashir MR. Hairy polyp, a clinicopathologic study of four cases. Head Neck Pathol. 2013;7(3):232–5. https://doi.org/10.1007/s12105-013-0433-4.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Rothova M, Thompson H, Lickert H, Tucker AS. Lineage tracing of the endoderm during oral development. Dev Dyn. 2012;241(7):1183–91. https://doi.org/10.1002/dvdy.23804.

    Article  PubMed  Google Scholar 

  54. Lee J, Rabbani CC, Gao H, Steinhart MR, Woodruff BM, Pflum ZE, et al. Hair-bearing human skin generated entirely from pluripotent stem cells. Nature. 2020;582(7812):399–404. https://doi.org/10.1038/s41586-020-2352-3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Coons SW, Rekate HL, Prenger EC, Wang N, Drees C, Ng YT, et al. The histopathology of hypothalamic hamartomas: study of 57 cases. J Neuropathol Exp Neurol. 2007;66(2):131–41. https://doi.org/10.1097/nen.0b013e3180302090.

    Article  PubMed  Google Scholar 

  56. Steinbach TJ, Reischauer A, Kunkemoller I, Mense MG. An oral choristoma in a foal resembling hairy polyp in humans. Vet Pathol. 2004;41(6):698–700. https://doi.org/10.1354/vp.41-6-698.

    Article  CAS  PubMed  Google Scholar 

  57. Ozolek J, Castro C. Teratomas derived from embryonic stem cells as models for embryonic development, disease, and tumorigenesis. In: Kallos MS, editor. Embryonic stem cells: basic biology to bioengineering. London: IntechOpen; 2011. p. 231–62.

    Google Scholar 

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Ozolek, J.A., Tekkesin, M.S. THE “-OMAS” and “-OPIAS”:  Targeted and Philosophical Considerations Regarding Hamartomas, Choristomas, Teratomas, Ectopias, and Heterotopias in Pediatric Otorhinolaryngologic Pathology. Head and Neck Pathol 15, 25–40 (2021). https://doi.org/10.1007/s12105-020-01251-y

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