
==== Front
Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00934-9
10.1016/j.ijscr.2024.110153
110153
Case Report
Very rare tumor of the nasal cavity, hamartome adenomatoid respiratory epithelial chondro-osseous (COREAH): About a case
Hachemi M. Hachemi_doc@hotmail.com
⁎
Oukil N.
Touarigt F.Z.
Hasbellaoui M.
University of Medicine of Algiers I, Chu Bab El Oued, Algiers, Algeria
⁎ Corresponding author. Hachemi_doc@hotmail.com
10 8 2024
10 2024
10 8 2024
123 1101536 6 2024
5 8 2024
8 8 2024
© 2024 Published by Elsevier Ltd on behalf of IJS Publishing Group Limited.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction and importance

Chondroosseous respiratory adenomatous epithelial hamartoma (COREAH) is a rare benign lesion of the nose and sinuses, with some cases reported in the literature.

Clinical case

We present here a case of COREAH, in a woman whose clinical examination finds a polypoid mass in the left nasal cavity. Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the presence of a large mass in the anterior nasal cavity extended to the cranial cavity through the screened blade on the right side. The mass was completely resected by endoscopy. Histological examination confirmed the nature of the mass as COREAH. A hamartoma can be considered a proliferation of endogenous cells or tissues at this anatomical site.

Clinical discussion

Several hamartomatous lesions have been recognized in the sinonasal region. The majority are mesenchymal, most often vascular lesions. Pure chondromatous hamartomas of the nasal cavity, although rare, also occur.

Conclusion

COREAH is a benign hamartomatous proliferation of the respiratory epithelium, submucosal glands and chondroosseous mesenchyma. Differential clinical diagnoses include benign and malignant tumors of the nasal cavity and sinuses. Properly recognizing this lesion as radiologically benign is essential to avoid radical surgery.

Highlights

• Chondroosseous respiratory adenomatous epithelial hamartoma (COREAH) is a rare benign lesion of the nose and sinuses.

• With some cases reported in the literature less than 10 cases to date.

• Clinical examination finds a polypoid mass in the left nasal cavity.

• Confusion with relatively aggressive tumors (adenocarcinoma, inverted papilloma)

Keywords

COREAH
Nasal cavity
Nasosinusian tumors
Meningocelle
Case report
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pmc1 Introduction

Hamartomas of the head and neck in general, as well as the nasosinusian tract in particular, are very rare. Respiratory epithelial adenomatous hamartoma (REAH) and chondrobony respiratory epithelial adenomatoid hamartoma (COREAH) are examples of hamartomas comprising both epithelial and mesenchymal components. Although benign, these lesions can lead to confusion with relatively aggressive tumors (adenocarcinoma, inverted papilloma). We report here a case of COREAH of the nasal cavity, which was confused with a meningocelle highlighting its clinicopathological characteristics and the importance of recognizing and differentiating it from other potentially more aggressive lesions for, in order to have a more precise support.

2 Clinical case

A 58-year-old patient with no history who consulted our service for progressive left nasal obstruction without further signs for 5 years, in which the clinical and endoscopic examination finds a whitish polypoid mass located behind the head of the middle cone seeming to originate from the roof of the nasal cavity arriving at the floor thereof.

Computed tomography (CT) and magnetic resonance imaging (MRI) showed a bone defect of the roof of the anterior ethmoid with meningeal structures in its breast imprinting on the upper part of the nasal septum extended to the left nasal floor (Fig. 1) without involvement of the posterior ethmoid or sphenoid.Fig. 1 (a) Sagittal and (b) coronal CT scan image showing large polypoid lesion in left nasal cavity with bone lysis. (c) T2-weighted coronal MRI showing CSF-like signal formation in ethmoid anterior and nasal cavity.

Fig. 1

The patient underwent endoscopic excision of the mass. At endoscopy, a large whitish mass of elastic consistency was identified from the roof of the ethmoid (Fig. 2) with lysis of its roof of one cm the mass was resected to its root without associated meningeal involvement. Control examination at 3.6 and 12 month revealed no residual or recurrent lesions.Fig. 2 Endoscopic image of the mass outside the middle cone.

Fig. 2

Descriptive analysis: the number and proportion of case reports reporting each of the SCARE items.

Pathological anatomy: 02vials were addressed to us Flacon 1: medium cornet .A tissue fragment of (3 × 1 × 0.3) cm, of elastic consistency, greyish white with hemorrhagic alterations, has sometimes epithelium Formation histology, cellular, nodular cylindrical after containing inclusion consistent pseudostratified vessels, Elastic, with respiratory white wall finds thick, a surmontant translucent sometimes formation S chorion kystisée polypoide and sometimes of the cedemateux upholstery, trabecules of a Vial 2: mass of the right nasal cavity metaplastic looking bones.

Seromucosal glands are noted lobulated, sitting even between bone trabecules, absence of dysplasia or signs of malignancy.

Conclusion: Morphological aspect of an epithelial hamartoma adenomyoid chondro-osseous nasal (COREAH), Absence of dysplasia or malignant neoplasia.

3 Discussion

A hamartoma can be considered a proliferation of endogenous cells or tissues at this anatomical site. Several hamartomatous lesions have been recognized in the sinonasal region. The majority are mesenchymal, most often vascular lesions [1].

Although rare pure chondromatous hamartomes of the nasal cavity may exist [2]. More complex lesions have also been described. Until the early 1990s, the majority of nasosinusian hamartomas were mesenchymal, most often vascular [3]. Epithelial hamartomas have also been described and include seromucinous hamartomas and salivary gland blank tumor [4]. Wenig and Heffner [5] describe an additional epithelial hamartoma, with a respiratory rather than salivary or seromucinous epithelium. These adenomatous hamartomas respiratory epithelial (REAH) look roughly a inflammatory polyps but microscopic examination confirms the characteristic glandular profiles bordered by the ciliated respiratory epithelium from the surface epithelium. Hamartomas with a mixture of mesenchymal and epithelial elements have also been reported, but not in detail. The original description of REAH included a reference to a single case of bone metaplasia [5]. The same group of authors then published an abstract describing a REAH subgroup with a mesenchymal component, called chondro-osseous respiratory adenomatous hamartoma (COREAH) [6].

The morphological overlap that exists between some of these entities was recognized in the original NCMH description and one of the cases included in this report had an identical appearance to this current lesion [7]. Indeed, the authors proposed that COREAH could represent part of a spectrum of hamartomatous lesions, ranging from a purely epithelial hamartoma (REAH) on one side to a purely mesenchymal hamartoma (NCMH) on the other [7].

Two hypotheses have been proposed for the aetiology of REAH (and by extrapolation COREAH). The aetiology of COREAH is unclear and may be secondary to embryological malformation, inflammation. However, COREAH is not associated with other genetic disease processes and often presents at a later stage in life, making a congenital aetiology less likely. Our patient presented at age 58 with only 5 years of symptoms, which supports a non congenital cause for COREAH [5]. REAH has been reported to be related to chronic sinusitis and nasal polyps, which has led some authors to suggest an inflammatory aetiology for COREAH given the close histological characteristics. Roffman et al. [8].

Clinically, it has been observed that REAH appears lateral nasal wall in the majority of cases [5]. However, other sites, including nasopharynx and ethmoidal, maxillary and frontal sinuses, have been described [8]. Symptoms include nasal obstruction and congestion, chronic sinusitis, rhinorrhea, hyposmia, and headaches [9].

COREAH lesions reported in the literature were also localized in the nasal cavity, fleshy to firm and polypoids, with, smooth exterior surfaces and solid cut surfaces [10]. Under the microscope, they resembled REAH, with features including sub-proliferationmainly glandular mucosa with round to oval glands ranging from small to clearly dilated and lined with ciliated respiratory epithelium from the superficial mucosa. These glands were separated by stromal tissue without any signs of complex growth or cribriform architecture. Mucinous metaplasia with mucoid or amorphous material filling the glandular lights was sometimes observed. Other histological features included changes generally observed in association with inflammatory nasosinusian polyps, namely stromal edema, polypoid growth, increased number of seromucinous glands, vascular and fibroblastic proliferation and a mixture of acute and chronic inflammatory cells [10].

Nevertheless, despite these reported molecular alterations, REAH and COREAH are completely benign lesions.

REAH and COREAH can be distinguished from inflammatory polyps for clinical and histological reasons. Clinically, most adenomatoid epithelial hamartoma are solitary masses appearing in the posterior nasal septum [5]. The presence of adenomatoid proliferation and stromal hyalinization distinguish the histological characteristics of REAH as opposed to pauciglandular loose estrogen from inflammatory polyps.

Inverted papillomas are known to be locally aggressive tumors. Their clinical evolution, unlike that of REAH, is characterized by bone destruction, extension along mucosal surfaces, invasion into adjacent structures, local recurrence and malignant transformation potential [5].

REAH and low-grade nasosinusian adenocarcinoma may have similar clinical presentations. However, the histological distinction is rarely difficult, unless the biopsy is exceptionally small. Features such as cribriform architecture, absence of ciliated epithelium lining the glands, desmoplasic stroma and high mitotic rate would strongly promote adenocarcinoma [9]. Unlike inverted papilloma and nasosinusian adenocarcinoma, REAH/COREAH is either treated by complete local excision [11,12]. Cases followed for up to 5 years showed no signs of recurrence [1,2].

4 Conclusion

COREAH is an extremely rare benign lesion of the nasosinusitis region that can be confused with benign or malignant tumors frequently found in the nasal cavities. Knowledge of this pathology radiologically and clinically could greatly help the clinician to correctly identify COREAH in order to avoid unnecessary aggressive treatments.

Consent

Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

The study is exempt from ethnical approval.

Funding

No study sponsors.

Author contribution

Study concept: HACHEMI MOHAMED.

Data collection: Naila Oukil.

Data analysis: Fatma Zohra Touarigt.

Writing papaer: Hasbellaoui Mokhtar.

Guarantor

HACHEMI MOHAMED.

Research registration number

N/A.

Conflict of interest statement

No conflicts of interest.
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