Annals of Medicine and Surgery 46 (2019) 27–30

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Annals of Medicine and Surgery

journal homepage: www.elsevier.com/locate/amsu

An atypical presentation of a Respiratory Epithelial Adenomatoid T

Hamartoma, a case report
Nisreen Al-Musaileema, Imtiaz M. Qazia, Jassem M. Bastakib, Mahmoud A.K. Ebrahima,∗
a
Zain Hospital, Ministry of Health, Kuwait City, Kuwait
b
Al-Sabah Hospital, Ministry of Health, Kuwait City, Kuwait

A R T I C LE I N FO A B S T R A C T

Keywords: Introduction: Respiratory Epithelial Adenomatoid Hamartoma (REAH) is a benign disease that can resemble
Respiratory epithelial adenomatoid hamartoma other malignant entities. Thus, it is essential to diagnose it accurately as the treatment approach differs, from
Olfactory nueroblastoma radical surgeries in malignant cases, to a simple excision in hamartoma. We present an unusual case of bilateral
REAH that was misdiagnosed, and hence it was treated aggressively.
Case report: A 57-year-old male patient presented with anosmia, 2-years history of bilateral nasal obstruction,
and was accompanied with a moderate headache. An impression of olfactory neuroblastoma was made after
history taking physical examination, and imaging studies. The patient underwent Functional Endoscopic Sinus
Surgery (FESS), excisional biopsy of the cribriform plate mass bilaterally, and superior septectomy.
Histopathologic examination of the bilateral masses showed sinonasal polyposis with crypting of surface mucosa
and pseudoglandular formation. A diagnosis of sinonasal polyps with REAH was established. The patient's nasal
obstruction improved, with no recurrence of sinusitis ± polyposis. However, he still complains of anosmia after
2-years follow-up.
Conclusion: Although REAH is a benign disease, it is critical to reach the correct diagnosis, in order to avoid
aggressive treatment. Unfortunately, the preoperative investigations were not consistent with REAH, thus it was
misdiagnosed and treated aggressively.

1. Introduction 2. Case presentation

Hamartomas are benign, non-neoplastic lesions that develop sec- A 57-year-old male patient presented to our Ear, Nose and Throat
ondary to tissue-development anomalies, and are composed of over- (ENT) clinic with the chief complaint of anosmia and 2-years history of
growth of normal and mature cells and tissues that are normally present bilateral nasal obstruction which was reportedly worse on the right
in the affected location, but in a disorganized manner [1,2]. Ha- side, and was associated with non-specific moderate headache. There
martomas were first described by Albrect in 1904 [3]. Although they was no history of epistaxis, rhinorrhea, recurrent sinusitis, or any
can arise anywhere in the body, they are more common in the lung, neurological complaints. He is a non-smoker. The patient's past medical
kidney, liver, spleen, and intestine [1].In 1995, Wenig and Heffner history was only significant for Type 2 diabetes, controlled with oral
described a type of sinonasal tissue anomaly, and designated it as Re- medications. Previously he has had no surgeries and no history of any
spiratory Epithelial Adenomatoid Hamartoma (REAH) [4]. The disease allergies.
is considered a benign entity; however, the histopathological feature ENT examination was unremarkable except for mild non obstructing
can resemble inverted papilloma or well differentiated adenocarcinoma deviated nasal septum to the right. Nasoendoscopy revealed a mass
leading to misdiagnosis [5]. Thus, it is essential not to misdiagnose it as originating from the roof of the nasal cavity, bilaterally, overlying the
a malignant lesion, due to the different treatment approach, from a olfactory epithelial mucosal surface; the surface of the mass was irre-
radical surgery in malignant cases to a simple excision in hamartoma gular. Due to nonavailability of the standardized smell testing
[5]. Herein, we present an unusual case of bilateral REAH, which was (University of Pennsylvania Smell Identification Test) at our institution,
diagnosed as olfactory neuroblastoma thus over treated. This case re- we were unable to perform such specific testing. Therefore, we used
port has been reported in line with the SCARE criteria [6]. nonirritating substances like grounded coffee, vanilla, and lemon,

∗
Corresponding author. Otolaryngology Head and Neck Surgery, Zain Hospital, Ministry of Health, Kuwait City, Kuwait.
E-mail address: [email protected] (M.A.K. Ebrahim).

https://doi.org/10.1016/j.amsu.2019.08.007
Received 11 June 2019; Received in revised form 14 August 2019; Accepted 28 August 2019
2049-0801/ © 2019 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY license
(http://creativecommons.org/licenses/BY/4.0/).
N. Al-Musaileem, et al. Annals of Medicine and Surgery 46 (2019) 27–30

Fig. 1. A & B: Sagittal (A) and Coronal (B) soft tissue window CT sinus showing bilateral polypoidal soft tissue lesion present at the roof of nasal cavity with intact
overlying bony floor of anterior cranial fossa, no intracranial extension on CT basis.

which stimulate only the olfactory nerve, without causing irritation and with pseudoglandular formation isn't a typical feature of sinonasal
stimulation of the trigeminal nerve, as a rudimentary means to test the polyps (Fig. 3). A diagnosis of sinonasal polyps with REAH was thus
patient's smell function. The patient was instructed to sniff separately rendered by a subspecialized Head and Neck pathologist.
from each nostril while blocking the other nostril, during this time the The patient was followed up in the clinic with serial nasoendoscopy,
patient's eyes were kept closed. He couldn't identify the coffee bilat- his nasal obstruction improved gradually; however, he did not regain
erally and anosmia was confirmed. his sense of smell. The patient is under our follow up for more than two
Routine laboratory investigations were within normal range. years, his nasal breathing is good, and there is no recurrence of sinu-
Computed Tomography (CT) scan of the nose and paranasal sinuses was sitis ± polyposis. The patient, however, continues to have anosmia.
carried out which showed bilateral well circumscribed homogenous soft Overall, he is satisfied with his current health status with regards to the
tissue mass at the olfactory recess and polypoid mucosal thickening nasal disease and our intervention.
bilaterally in the maxillary sinuses that was obstructing and dilating the
related osteomeatal complexes. The imaging also showed marked
polypoid mucosal thickening of ethmoid air cells, sphenoid sinuses and 3. Discussion
nasal cavities. Also seen was rarefaction of the ethmoidal bony labyr-
inth and obstruction of the bilateral spheno-ethmoidal recesses as well Hamartomas are considered non-neoplastic and self-limiting;
as an S shaped nasal septum (Fig. 1). The patient was started on topical though, they have no tendency to resolve spontaneously. It is thought
nasal steroid spray and oral prednisolone; however, the symptoms that the development of inflammatory polyps leads to the occurrence of
persisted warranting further investigation of the olfactory mass. The REAH [7]. Additionally, an association between nasal polyposis and
mass was biopsied in the outpatient clinic under topical anesthesia as bilateral REAH of the olfactory cleft has been documented [8]; as is the
the mass was well clearly visualized, and was easily accessible with a experience in our patient. We believe that the presence of longstanding
45°; the patient was cooperative as well. The histopathology result was bilateral nasal polyposis in our patient led to the development of a
inconclusive. Magnetic resonance imaging (MRI) with contrast was bilateral REAH [7]. Complete surgical removal is the standard method
ordered (Fig. 2) which revealed a bilateral olfactory recess expansive of treatment of REAH [9]. Re- emergence of the disease after its re-
soft tissue pathology extending to the skull base with no intra-cranial solution is remarkably uncommon [9]. Furthermore, REAH have no
extension, showing intermediate signal intensity on T1 and T2 and post malignancy potential [9].
contrast enhancement, which was suggestive of nasal roof mass lesion. REAHs are uncommon in the head and neck, but the majority of the
These findings were suggestive of olfactory neuroblastoma. The case cases that develop in this region are located in the posterior nasal
was discussed in the multidisciplinary team (MDT) meeting, and in septum [10]. They typically arise in the third to ninth decades of life,
view of the inconclusive biopsy report and worrisome radiological with a male predominance (3:2), similar to the age and gender of our
findings suggestive of olfactory neuroblastoma, it was unanimously patient, but the location was different in our patient as it originated
decided to subject the patient to excision biopsy rather than a simple from the nasal roof [11]. They also tend to occur unilaterally, unlike
biopsy, under general anesthesia. Subsequently the patient underwent our case in which it was bilateral [12]. Nasal obstruction, rhinorrhea,
functional endoscopic sinus surgery (FESS) and excisional biopsy of the epistaxis, recurrent chronic rhinosinusitis, facial pain, and hyposmia/
bilateral cribriform plate mass. anosmia are some of the symptoms of the disease [9]. Our patient
The procedure was performed under general anesthesia. Nasal en- suffered from nasal obstruction, anosmia, and chronic rhinosinusitis.
doscopy revealed a bilateral nasal roof mass at the superior part of the Two distinctive hypotheses have been proposed regarding the cause
septum and lateral nasal wall, originating from the cribriform plate. of REAH: Congenital and chronic inflammation [13]. According to the
The mass was excised from the cribriform plate by making an incision chronic inflammatory hypothesis, the development of REAH is a result
around it. The incision extended 1 cm from the mass to the lateral of chronic and/or severe inflammation of the nasal mucosa in the area
border of the superior septum and the lateral wall. Although the nasal of the olfactory cleft [8]. We believe, supported by the long history of
septum was intact yet a superior septectomy was performed to facilitate chronic sinusitis, that our patient in fact developed REAH secondary to
en bloc resection of the mass, leaving the cribriform and lateral and the chronicity of his inflammatory process, and thus fits under the
medial walls as bare bone. In addition, bilateral FESS, for all four si- chronic inflammatory hypothesis. Nasoendoscopy findings of REAH are
nuses with removal of the nasal polyps and septoplasty was carried out. usually of a fleshy to firm polypoid mass. Those findings usually re-
Postoperative period was uneventful. Histopathologic examination of semble an inflammatory polyp or olfactory neuroblastoma, especially in
the bilateral masses revealed Sinonasal type respiratory epithelium certain locations, similar to our case [14]. With regards to imaging
exhibiting submucosal edema, chronic inflammatory cell infiltrate and studies in REAH, there are no specific or diagnostic characteristics [15].
thickening of the basal layer. These findings are typically seen in cases Nasal polyposis, inverted papilloma, and sinonasal adenocarcinoma
of sinonasal polyposis; however, prominent crypting of surface mucosa are the major differential diagnosis contenders of REAH [16]. In-
flammatory nasal polyps most often develop from sinus mucosa,

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N. Al-Musaileem, et al. Annals of Medicine and Surgery 46 (2019) 27–30

Fig. 2. A, B, C, & D MRI sinus showing the olfactory recess bilaterally with soft tissue pathology extending to skull base with no intra-cranial extension, showing
intermediate signal intensity on T1 post contrast enhancement (A: sagittal, B:coronal) and T2 (C: coronal, D: Sagittal). suggestive of nasal roof mass lesion query
neuroblastoma for biopsy.

Fig. 3. A & B: A. Low power (40x) histopathologic

photomicrograph showing the surface mucosa
‘crypting’ into the submucosal stroma with gland-like
structures masquerading as an infiltrative glandular
neoplasm (40x; hematoxylin and eosin H&E). B.
Higher power photomicrograph of the lesion illus-
trating the respiratory epithelium lining those (ade-
nomatoid) structures (100x; hematoxylin and eosin
H&E).

whereas REAH usually originate from the nasal septum and olfactory olfactory cleft that looks enlarged because of the suppression of the
cleft [16]. The histopathological findings of REAH are submucosal middle and superior turbinate to the exterior. In contrast to the findings
proliferation of small to medium-sized round to oval glands lined by of nasal polyposis, compression of the middle turbinate can occur
ciliated respiratory epithelium, frequently admixed with mucin-se- against the septum from the exterior [18]. The characteristics of in-
creting cells; while Inflammatory nasal polyps are characterized by verted papilloma's are infoldings and invaginations of the mucosal
stromal edema, epithelial and fibroblast proliferation, and multiple epithelium that are not associated with glandular proliferations, most
epithelial and subepithelial inflammatory cells infiltrates (eosinophils importantly with prominent neutrophillic microabscesses. Regarding
and lymphocytes), usually devoid of such prominent glandular pro- low-grade adenocarcinomas, they appear as a crowded glandular pro-
liferations. In fact, REAH can sometimes be seen in the background of liferation with pleomorphism, mitoses and different degrees of cellular
inflammatory sinonasal polyps [17]. An essential diagnostic method to atypia. In fact, in cases of intestinal type and non-intestinal type ade-
differentiate between REAH and Nasal polyposis is a CT scan. The CT nocarcinomas, both morphology and immunohistochemical studies can
findings in REAH of the olfactory cleft are classically an opacity of the help easily differentiate between the two [19].

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N. Al-Musaileem, et al. Annals of Medicine and Surgery 46 (2019) 27–30

We present an atypical case of bilateral REAH, in which the clinical, Provenance and peer review
nasoendoscopy, imaging, and the office based biopsy was not consistent
with REAH preoperatively. Thus, it was treated aggressively. In hind- Not commissioned, externally peer reviewed.
sight, we believe that the patient should have had a repeat biopsy
preferably under general anesthesia, which might have prevented the Appendix A. Supplementary data
overaggressive treatment that the patient underwent. Ideally, a simple
excision of the mass with a shaver should have been done, instead of Supplementary data to this article can be found online at https://
excising the whole mass with the surrounding tissue with superior doi.org/10.1016/j.amsu.2019.08.007.
septectomy and leaving bare bone. Hence, one should consider REAH as
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[email protected].
Phone: 00965-94445366.

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