Respiratory Epithelial Adenomatoid Hamartoma (REAH) in the Olfactory Cleft: Often Masked by Bilateral Nasal Polyps

Abstract

Respiratory epithelial adenomatoid hamartoma (REAH) is a distinct non-neoplastic entity originating from anterior olfactory cleft in the nasal cavity, often going unnoticed. Clinically, REAH presents as unilateral or bilateral nasal polyps. Our aim is to expand the understanding of bilateral REAH associated with nasal polyposis with respect to clinical, radiological and histopathological features for better clinical outcomes. Our analysis includes patients presenting as bilateral nasal polyps, whose CT-PNS showed opacity in olfactory clefts. During endoscopic sinus surgery, the lesions in the olfactory cleft (medial-to-middle turbinate) were identified and the specimens from olfactory cleft and ethmoid sinus cavity were subjected separately to histopathological analysis. Six patients (average age 50 years, 83% male) of bilateral REAH with nasal obstruction of > 3 years were analysed. On nasal endoscopy, the polypoid masses in the olfactory cleft and in the ethmoids did not show any gross differences. However, polypoidal masses from the olfactory cleft bled more during biopsy and excision. Histopathological study of these masses revealed the closely arranged round to oval glands (with few dilated glands) lined by ciliated columnar epithelium in mildly edematous stroma, confirming the presence of REAH. REAH is an often overlooked lesion in the nasal cavity, arising from olfactory cleft. The presence of nasal polyposis obscures this lesion, resulting in under diagnosis. The prompt identification with high index of suspicion by the otorhinolaryngologists helps in accurate histopathological diagnosis thereby improving clinical outcomes.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2
Fig. 3

References

  1. 1.

    Terris MH, Billman GF, Pransky SM (1993) Nasal hamartoma: case report and review of the literature. Int J Pediatr Otorhinolaryngol 28:83–88

    CAS  Article  Google Scholar 

  2. 2.

    Jain RK (2003) Hamartoma of the head and neck. Indian J Otolaryngol Head Neck Surg 9:76–78. https://doi.org/10.1016/B978-0-7234-3261-6.50020-7

    Article  Google Scholar 

  3. 3.

    Liang J, O’Malley BW, Feldman M, Newman JG (2007) A case of respiratory epithelial adenomatoid hamartoma. Am J Otolaryngol 28:277–279. https://doi.org/10.1016/j.amjoto.2006.09.013

    Article  PubMed  Google Scholar 

  4. 4.

    Ake Y, Kouame J, Moh E et al (2014) Skin hamartoma in children: diagnosis and therapeutic issues. J Pediatr Surg Case Rep 2:319–321. https://doi.org/10.1016/j.epsc.2014.06.007

    Article  Google Scholar 

  5. 5.

    Wenig BM, Heffner DK, Dennis Heffner CK et al (1995) Respiratory epithelial adenomatoid hamartomas of the sinonasal tract and nasopharynx: a clinicopathologic study of 31 cases. Ann Otol Rhinol Laryngol 104:639–645. https://doi.org/10.1177/000348949510400809

    CAS  Article  PubMed  Google Scholar 

  6. 6.

    Wenig BM (2005) Respiratory epithelial adenomatoid hamartoma. In: Barnes L, Eveson J, Reichart P, Sidransky D (eds) WHO classification of tumors pathology and genetics of head and neck tumors. IARC Press, France, p 33

    Google Scholar 

  7. 7.

    Park I-H, Lee H-MHC, Lee H-MHC et al (2013) Respiratory epithelial adenomatoid hamartoma originating from nasal septum. Clin Exp Otorhinolaryngol 6:45. https://doi.org/10.3342/ceo.2013.6.1.45

    Article  PubMed  Google Scholar 

  8. 8.

    Himi Y, Yoshizaki T, Sato K, Furukawa M (2002) Respiratory epithelial adenomatoid hamartoma of the maxillary sinus. J Laryngol Otol 116:317–318. https://doi.org/10.1258/0022215021910672

    Article  PubMed  Google Scholar 

  9. 9.

    Kessler HP, Unterman B (2004) Respiratory epithelial adenomatoid hamartoma of the maxillary sinus presenting as a periapical radiolucency: a case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 97:607–612. https://doi.org/10.1016/j.tripleo.2003.09.013

    Article  PubMed  Google Scholar 

  10. 10.

    Metselaar RM, Stel HV, Van Der Baan S (2005) Respiratory epithelial adenomatoid hamartoma in the nasopharynx. J Laryngol Otol 119:476–478. https://doi.org/10.1258/0022215054273124

    CAS  Article  PubMed  Google Scholar 

  11. 11.

    Di Carlo R, Rinaldi R, Ottaviano G, Pastore A (2006) Respiratory epithelial adenomatoid hamartoma of the maxillary sinus: case report. Acta Otorhinolaryngol Ital 26:225–227. https://doi.org/10.1258/0022215021910672

    Article  PubMed  PubMed Central  Google Scholar 

  12. 12.

    Lee JT, Garg R, Brunworth J et al (2013) Sinonasal respiratory epithelial adenomatoid hamartomas: series of 51 cases and literature review. Am J Rhinol Allergy 27:322–328. https://doi.org/10.2500/ajra.2013.27.3905

    Article  PubMed  Google Scholar 

  13. 13.

    Nagy AA, Trombitas V, Vlad D, Albu S (2014) Respiratory epithelial adenomatoid hamartoma: a few considerations and a review of literature. Rom J Rhinol 4(15):135–140

    Google Scholar 

  14. 14.

    Di I, Chirurgia OE, Pastore A (2015) Respiratory epithelial adenomatoid hamartoma of the maxillary sinus: case Respiratory epithelial adenomatoid hamartoma of the maxillary sinus: case report. Int J Case Rep Images 7:346. https://doi.org/10.5348/ijcri-201662-CR-10650

    Article  Google Scholar 

  15. 15.

    Mulazimoglu S, Islamoglu Y, Beton S et al (2015) Respiratory epithelial adenomatoid hamartoma of the maxillary sinus presenting as an antrochoanal polyp. Egypt J Ear Nose, Throat Allied Sci 16:185–187. https://doi.org/10.1016/j.ejenta.2015.02.006

    Article  Google Scholar 

  16. 16.

    Shukla A, Kakad T, Shah S, Bhaduri AS (2017) Hamartomatous polyp of the nasopharynx: a rare case report. Indian J Otolaryngol Head Neck Surg. https://doi.org/10.1007/s12070-017-1077-6

    Article  Google Scholar 

  17. 17.

    Seol JG, Livolsi VA, Malley BWO et al (2010) Respiratory epithelial adnomatoid hamartoma of the bilateral olfactory recesses: a neoplastic mimic? Am J Neuroradiol 31:277–279. https://doi.org/10.3174/ajnr.A1755

    CAS  Article  PubMed  Google Scholar 

  18. 18.

    Nguyen DT, Nguyen-Thi PL, Gauchotte G et al (2014) Predictors of respiratory epithelial adenomatoid hamartomas of the olfactory clefts in patients with nasal polyposis. Laryngoscope 124:2461–2465. https://doi.org/10.1002/lary.24778

    Article  PubMed  Google Scholar 

  19. 19.

    Falco JJ, Peine BS, Clark DW (2017) Bilateral respiratory epithelial adenomatoid hamartomas originating from the anterior olfactory clefts. Baylor Univ Med Cent Proc 30:221–223. https://doi.org/10.1080/08998280.2017.11929594

    Article  Google Scholar 

  20. 20.

    Delbrouck C, Fernandez Aguilar S, Choufani G, Hassid S (2004) Respiratory epithelial adenomatoid hamartoma associated with nasal polyposis. Am J Otolaryngol Head Neck Med Surg 25:282–284. https://doi.org/10.1016/j.amjoto.2004.02.005

    Article  Google Scholar 

  21. 21.

    Endo R, Matsuda H, Takahashi M et al (2002) Respiratory epithelial adenomatoid hamartoma in the nasal cavity. Acta Otolaryngol 122:398–400. https://doi.org/10.1080/00016480260000085

    Article  PubMed  Google Scholar 

  22. 22.

    Saniasiaya J, Md Shukri N, Ramli RR et al (2017) Sinonasal respiratory epithelial adenomatoid hamartoma: an overlooked entity. Egypt J Ear Nose Throat Allied Sci 18:191–193. https://doi.org/10.1016/J.EJENTA.2016.12.014

    Article  Google Scholar 

  23. 23.

    Perić A, Bijelić D, Vukomanović-Đurđević B, Perić AV (2016) Huge respiratory epithelial adenomatoid hamartoma originating from the inferior nasal turbinate: a case report. Indian J Otolaryngol Head Neck Surg 68:100–103. https://doi.org/10.1007/s12070-015-0849-0

    Article  PubMed  Google Scholar 

  24. 24.

    Cao ZW, Gu ZW, Yang J, Jin MZ (2010) Respiratory epithelial adenomatoid hamartoma of bilateral olfactory clefts associated with nasal polyposis: three cases report and literature review. Auris Nasus Larynx 37:352–356. https://doi.org/10.1016/j.anl.2009.10.003

    Article  PubMed  Google Scholar 

  25. 25.

    Lorentz C, Marie B, Vignaud JM, Jankowski R (2012) Respiratory epithelial adenomatoid hamartomas of the olfactory clefts. Eur Arch Oto Rhino Laryngol 269:847–852. https://doi.org/10.1007/s00405-011-1713-9

    Article  Google Scholar 

  26. 26.

    Hawley KA, Ahmed M, Sindwani R (2013) CT findings of sinonasal respiratory epithelial adenomatoid hamartoma: a closer look at the olfactory clefts. Am J Neuroradiol 34:1086–1090. https://doi.org/10.3174/ajnr.A3345

    CAS  Article  PubMed  Google Scholar 

  27. 27.

    Vira D, Bhuta S, Wang MB (2011) Respiratory epithelial adenomatoid hamartomas. Laryngoscope 121:2706–2709. https://doi.org/10.1002/lary.22399

    Article  PubMed  Google Scholar 

  28. 28.

    Gauchotte G, Marie B, Gallet P et al (2013) Respiratory epithelial adenomatoid hamartoma a poorly recognized entity with mast cell recruitment and frequently associated with nasal polyposis. Am J Surg Pathol 37(11):1678–1685

    Article  Google Scholar 

  29. 29.

    Prasad ML, Perez-Ordonez B (2009) Nonsquamous lesions of the nasal cavity, paranasal sinuses, and nasopharynx. In: Gnepp DR (ed) Diagnostic surgical pathology of the head and neck. Saunders-Elsevier, Philadelphia, pp 148–154

    Google Scholar 

  30. 30.

    Mills S (2009) The nose, paranasal sinuses, and nasopharynx. In: Mills S, Carter D, Greenson J et al (eds) Sternberg’s diagnostic surgical pathology, 5th edn. Lippincott Williams & Wilkins, Philadelphia

    Google Scholar 

Download references

Acknowledgements

We acknowledge the support of staff and PG students of Department of ENT, Department of Radiology and Department of Pathology, SDM College of Medical Sciences and Hospital, Dharwad.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Raghunath Shanbag.

Ethics declarations

Conflict of interest

All the authors declare that there are no potential conflicts of interests regarding the publication of this manuscript.

Ethical Approval

All procedures performed in studies involving human participants followed the ethical standards of the institution.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Shanbag, R., Patil, P., Rani, S.H. et al. Respiratory Epithelial Adenomatoid Hamartoma (REAH) in the Olfactory Cleft: Often Masked by Bilateral Nasal Polyps. Indian J Otolaryngol Head Neck Surg 71, 2121–2126 (2019). https://doi.org/10.1007/s12070-018-1562-6

Download citation

Keywords

  • Respiratory epithelial adenomatoid hamartoma
  • Nasal polyp
  • Olfactory cleft
  • Endoscopic surgery
  • Histopathological analysis