A case of respiratory epithelial adenomatoid hamartoma

doi:10.1016/j.amjoto.2006.09.013

American Journal of Otolaryngology

Volume 28, Issue 4, July–August 2007, Pages 277-279

https://doi.org/10.1016/j.amjoto.2006.09.013 Get rights and content

Abstract

We report a case of a 70-year-old man diagnosed with a respiratory epithelial adenomatoid hamartoma (REAH) of the nasal cavity after a past sinus surgery and associated with nasal polyposis. REAH is a recently described pathologic entity that can present with nasal obstruction, congestion, rhinorrhea, epistaxis, hyposmia, and headaches. It is a rare lesion of nasal and paranasal sinuses, but should be considered in the differential diagnosis because it is a benign lesion and complete surgical resection is curative.

Introduction

Hamartomas are benign malformations or inborn errors of tissue development. The term “hamartoma” was first described by Albrect in 1904. A hamartoma is composed of disorganized tissue indigenous to the particular site, but with an excess of one or more of the tissue types. Although the cellular elements are mature and identical to those found in the remainder of the organ, they do not reproduce the normal architecture of the surrounding tissue. Hamartomas can occur in any area of the body, with a predilection for the lung, kidney, and intestine [1]. Hamartomas of the head and neck region, in particular the nasal cavity and paranasal sinuses, are very rare [2]. One particular type of hamartoma, first described by Wenig and Heffner in 1995 [3], is the respiratory epithelial adenomatoid hamartoma (REAH). Among sinonasal hamartomas, the REAH subgroup of hamartomas represents a rare entity that has been described in only a handful of case reports.

Section snippets

Case reports

A 70-year-old white man with a history of nasal obstruction and nasal polyposis initially presented with chronic symptoms of anosmia and difficulty breathing through the nose. His symptoms have worsened over a 10-month period despite oral steroid and antibiotic medications. He denies any seasonal allergies, and has a 50-pack-year smoking history. Endoscopic evaluation demonstrated right septal deviation and nasal polyposis bilaterally. The patient underwent bilateral endoscopic nasal polyectomy

Discussion

Respiratory epithelial adenomatoid hamartomas are a rare type of hamartoma found in the nasal cavity and paranasal sinuses. Wenig and Heffner [3] first described this distinct lesion in 1995 by identifying 31 cases from the files of the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology. The patients included 27 men and 4 women aged 27 to 81 years, with a mean age of 58 years. Presenting symptoms in the cases included obstruction, nasal stuffiness, deviated septum, epistaxis,

References (10)

R. Athre et al.
Frontal sinus hamartomas
Am J Otolaryngol
(2005)
H.P. Kessler et al.
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
(2004)
C. Delbrouck et al.
Respiratory epithelial adenomatoid hamartoma associated with nasal polyposis
Am J Otolaryngol
(2004)
R. Flavin et al.
Chondro-osseous respiratory epithelial adenomatoid hamartoma of the nasal cavity: a case report
Int J Pediatr Otorhinolaryngol
(2005)
E. Choi et al.
Chondro-osseous respiratory epithelial hamartoma of the sinonasal tract
Otolaryngol Head Neck Surg
(2006)

There are more references available in the full text version of this article.

Cited by (27)

Sinonasal respiratory epithelial adenomatoid hamartoma: An overlooked entity
2017, Egyptian Journal of Ear, Nose, Throat and Allied Sciences
Citation Excerpt :
This correlates with our patient discussed. Albeit myriad mechanisms has been postulated regarding REAH, of the most plausible is inflammatory process as this entity is more pertinent amongst patients with rhinosinusitis, inflammatory polyposis and sinus surgery.6 Some authors have also linked REAH with smoking and asthma.7
Respiratory epithelial adenomatoid hamartoma is an unusual benign glandular proliferation arising from the respiratory epithelium mostly involving the posterior nasal septum. Herein, we report a classic presentation of chronic rhinosinusitis with bilateral nasal polyposis which turns out to be respiratory epithelial adenomatoid hamartoma. Albeit benign, awareness of this entity is judicious as it may masquerade a more aggressive lesion causing patients to succumb to unnecessary procedure.
Glandular Neoplasia of the Sinonasal Tract
2017, Surgical Pathology Clinics
Citation Excerpt :
They identified 31 lesions with consistent histologic features that they named REAH. Nearly 90% of patients with REAHs are men and the reported patient ages have ranged from 27 to 81 years (median, 58).2–6 Symptoms include obstruction, stuffiness, epistaxis, postnasal drainage, and chronic rhinosinusitis.
Glandular lesions that cannot be diagnosed readily as salivary gland tumors occur uncommonly in the upper aerodigestive tract. They occur only with some frequency within the sinonasal tract. Well-characterized lesions at this site include the respiratory epithelial adenomatoid hamartoma, seromucinous hamartoma, and intestinal and non–intestinal-type adenocarcinomas. This article reviews the clinicopathologic features of these fascinating lesions.
The importance of the differential diagnosis in rhinology: Respiratory epithelial adenomatoid hamartoma of the sinonasal tract
2012, Acta Otorrinolaringologica Espanola
El Hamartoma respiratorio adenoepitelial (HRA) es una lesión poco común de la nariz y los senos paranasales caracterizada por la formación anormal de tejido glandular. Fue descrita por primera vez por Wenig y cols., en 1995 y, desde entonces, tan sólo se ha publicado unos 60 casos.
En este trabajo, presentamos el estudio de seis casos de hamartoma de cavidad nasal, los cuales fueron diagnosticados y tratados en nuestro Centro entre los años 2005 y 2010. Asimismo, realizamos un estudio completo del diagnóstico diferencial de esta patología, tanto a nivel clínico como anatomopatológico.
En estos pacientes, la razón hombre:mujer fue 5:1, con una media de edad de 63,1 años (DE = 16,9). Todos los sujetos presentaron afectación de la fosa nasal y, en tres casos, ocupación de los senos maxilar, frontal y etmoidal. Los síntomas más frecuentes fueron obstrucción nasal, rinorrea y pérdida de olfato. Todos los casos fueron tratados mediante cirugia endoscópica nasosinusal sin evidencia de recirrencia en el periodo de seguimimento (28,2 meses, DE = 11,5). Las características más importantes del HRA son: edema estromal, proliferación glandular seromucosa, proliferación fibroblástica y vascular, infiltrado inflamatorio mixto, incremento de la proliferación adenomatoide, acúmulo de material glandular y cambios atróficos del epitelio.
Sugerimos que el diagnóstico diferencial debe realizarse con la poliposis nasosinusal, pólipo antrocoanal, papiloma invertido y adenocarcinoma. Un diagnóstico incorrecto podría dar lugar a un tratamiento demasiado agresivo. Esta revisión de los casos publicados, con énfasis en el diagnóstico diferencial es de interés tanto para otorrinolarigólogos como anatomopatólogos.
Respiratory epithelial adenomatoid hamartoma (REAH) is an uncommon lesion of the nasal and paranasal sinuses. It was first described in 1995 by Wenig and Heffner, and only about 60 cases have been published since then.
We present 6 case studies of nasal cavity hamartoma diagnosed and treated at our institution between 2005 and 2010. We also conducted a literature review and comprehensive study of the differential diagnosis of this condition, both clinical and pathological.
The male-female ratio was 5:1, with a mean age of 63.1 years. The most frequent symptoms were nasal obstruction, rhinorrhea and loss of smell. All cases were treated with endoscopic sinus surgery, without recurrences in the follow-up period (28.2 months; SD = 11.5 months).
We suggest that differential diagnosis should be made on the basis of nasal polyps, antrochoanal polyps, inverted papilloma and low-grade adenocarcinoma. This review of published cases is of equal interest to both pathologists and otorhinolaryngologists.
Nasal chondromesenchymal hamartoma: Case report
2011, International Journal of Pediatric Otorhinolaryngology Extra
Citation Excerpt :
Adjuvant therapy is unnecessary. The predominant components of the hamartomas can be either epithelial [9–11] or mesenchymal tissues [1,2,6–8,12–14] or a mixture of both. Mesenchymal predominant hamartomas are poorly represented in the literature, particularly in the nose and nasopharynx [2].
We report the case of an 18-month-old boy with a large heterogeneous and lobulated soft-tissue mass, filling completely the right nasal cavity and anterior ethmoid, displacing the nasal septum, eroding the lamina papyracea, compressing the orbit and extending to the endocranium. There were multiple intralesional foci of calcification. The tumor was completely removed via an endonasal endoscopic approach. The definitive histopathologic diagnosis was that of a nasal chondromesenchymal hamartoma (NCMH). This is an exceedingly rare benign tumor of the upper respiratory tract that shares similarities to the mesenchymal hamartoma arising from the chest wall in neonates. Moreover as some NCMHs are associated with a “pleuropulmonary blastoma”, they can be part of the heredofamilial disease complex associated with PPB and linked to mutations of gene DICER1.
Respiratory epithelial adenomatoid hamartoma of bilateral olfactory clefts associated with nasal polyposis: Three cases report and literature review
2010, Auris Nasus Larynx
We report three cases of patients with respiratory epithelial adenomatoid hamartoma (REAH) of bilateral olfactory clefts associated with nasal polyposis. REAH is a kind of rare lesion which commonly occurs in nasal cavity, paranasal sinuses, and nasopharynx. Our cases were all localized in bilateral olfactory clefts which is very rare since the most common site reported in the past in nasal cavity is the posterior nasal septum and unilateral. Furthermore, our presented cases all associated with nasal polyposis which can provide greater evidence that inflammation can induce REAH development. Although REAH is benign lesion it can probably continue developing after operation if REAH was not completely resected. So during endoscopic sinus surgery (ESS), complete resection of the lesions is key point in successfully treatment of REAH. Although REAH arising from the rhinosinusal region is very rare rhinolaryngologists should know this entity thoroughly in order to differentiate it from inverted papilloma and adenocarcinoma since misinterpretation of this lesion may result in aggressive surgery for a benign lesion which could greatly affect patient's quality of life after operation.
A Case of Respiratory Epithelial Adenomatoid Hamartoma Treated as a Case of Chronic Sinusitis with a Nasal Polyp
2023, Practica Otologica, Supplement

View all citing articles on Scopus

View full text

Case reportA case of respiratory epithelial adenomatoid hamartoma

Abstract

Introduction

Section snippets

Case reports

Discussion

Am J Otolaryngol

Oral Surg Oral Med Oral Pathol Oral Radiol Endod

Am J Otolaryngol

Int J Pediatr Otorhinolaryngol

Otolaryngol Head Neck Surg

Case report
A case of respiratory epithelial adenomatoid hamartoma