Authored by Eduardo Machado Rossi Monteiro*
Abstract
Hamartoma (from the Greek, Hamartia, meaning fault or defect) is used to describe an overgrowth of normal mature cells and tissues indigenous to the area of its occurrence. It may occur in any organ of the body. Wenig and Heffner described a series of 31 cases of Respiratory Epithelial Adenomatoid Hamartoma (REAH) from what they considered a benign neoplasm. Today the most acceptable hypothesis is that REAH is a hyperplasia of the respiratory epithelium induced by a local chronic inflammation. Since its publishing, 394 cases of the disease have been described and REAH was found in 35-48% of patients operated on for nasal polypose. Some authors believe that REAH is the most important differential diagnosis of nasal polypose. REAH can be observed in two forms: isolated REAH (less frequent) or in association with another inflammatory process. The location in the olfactory cleft is concisely described in literature. Patients with REAH exhibit similar symptoms to those with chronic nasal inflammatory diseases. Endoscopy usually reveals a mass with a slight cerebriform aspect, fleshy to firm, pinkish or sometimes yellowish. The mass typically emerges from the cleft between the nasal septum superior-anterior and the middle turbinates, with a lateralization of the middle turbinates. Computed tomography (CT) scan is essential to diagnosis suspicion, which is increased by a CT scan that shows opacified and widened olfactory clefts. The conclusive diagnosis is only made by biopsies. Surgical treatment of the olfactory cleft is reported to be curative for REAH and the literature have also not described any recurrence or malignization. The knowledge of this lesion, mostly underdiagnosed, will allow the correct and necessary treatment that it takes.
Keywords: Respiratory ephitelial adenomatoid hamartoma; REAH; Differential diagnosis; Nasal polypose
Introduction
The Hamartoma Epithelial Respiratory Adenomatoid (HERA), better known by the initials REAH (Respiratory Epithelial adenomatoid hamartoma) in Anglo-Saxon literature, was first described in 1995 by Wenig and Heffner as a benign tumor of the nose and sinus cavities [1]. As it is an injury rarely described in the national literature, and in order to facilitate future literature searches, the initials adopted in this review to refer to Hamartoma Epithelial Respiratory Adenomatoid will be REAH. What seemed to be a rare injury, in fact it was an injury underdiagnosed and unknown to most of otolaryngologists, radiologists and pathologists. From 1995, year of its description to 2013, 394 cases of the REAH disease have been described and it was observed in 35-48% of patients undergoing nasosinusal polyposis surgery [2]. Some authors believe that the REAH is the most important differential diagnosis of nasosinusal polyposis [2].
Review of A Particular Subject
Clinicopathological findings
The term hamartoma (from the Greek, Hamartia, meaning fault or defect) is used to designate an overgrowth of disorganized tissue indisgenous to the area of its occurrence. They can occur in any tissue in the body [3]. When their series of 31 cases was described, Wenig and Heffner thought it was a benign tumor. Microscopically, they are characterized by a medium-sized pseudoglands proliferation, oval or round, surrounded by interstitial tissue of the respiratory ciliated pseudostratified epithelium, which invaginates the submucosa and continues with the surface of the epithelium. The glands are typically surrounded by a thick basal membrane eosinophilic [2] (Figure 1). Studies suggest that mast cells play an important role in the development of the REAH. Immunohistochemistry is not useful for the diagnosis [4]. In 2005, REAH was added to the World Health Organization tumors index, although there are controversies, today, the most accepted hypothesis is that the REAH is a respiratory epithelial hyperplasia in response to local chronic inflammatory process [5]. In 2006 Lima et al. [6] confirmed the origin of REAH the olfactory cleft, although other locations have also been described. Clinically the symptoms are similar to other inflammatory diseases of the nose, such as nasal obstruction, rhinorrhea, facial pain or pressure, headache, and hyposmia/anosmia [6]. Nasal obstruction and anosmia are the most frequent [7, 8]. Endoscopy usually reveals bilateral, often asymmetrical or unilateral nasal masses, with the lesion a cerebriform aspect, consistent, pinkish to yellowish emerging between the septum and the middle turbinates (Figure 2). In a recent review, Nguyen et al. [2] found an association of REAH and nasosinusal polyposis in 57% of cases [2]. REAH can be observed in two forms: Isolated (less frequent) and in association with other nasal inflammation, most commonly, naso-sinusal polyposis. REAH was found in 35-48% of polyposis surgery. The disease is observed from the third to ninth decade of life, predominantly in the fifth decade, with a male/women ratio of 3: 22.
Etiological findings
There was no association found between REAH and environmental or occupational agents, such as smoking or alcohol consumption. Several studies have defined the association of REAH and nasosinusal polyposis [2]. In a prospective study Nguyen et al. found a highly association between REAH and long duration of nasosinusal polyposis, repeated sinus surgery and asthma [9]. Others authors suggest association REAH with adenoiditis, hereditary hemorrhagic telangiectasia, allergic rinossinusopatias and even malignant naso-sinusal tumors [2].
Radiological findings
The CT scan is indispensable for the diagnostic suspicion. In the absence of drug treatment CT scan can reveal images compatible with nasosinusal polyposis, with major or minor sinus opacities. After drug treatment with antibiotics and steroids, CT can show improvement or even disappearance of sinus opacification, persisting the opacity of the olfactory cleft, which appears enlarged middle turbinate away laterally (Figure 3). In axial sections, especially in the anterior half, the enlargement of the olfactory cleft is evident, dislocating the middle turbinate and ethmoid cells toward orbital wall [10]. In a retrospective analysis of CT scans Lima et al. [6] found a median of 12mm in size of both olfactory slots for REAH against 5mm in nasosinusal polyposis and 4mm in the control group. This homogeneous opacity can mimic other benign or malignant nasal lesions, but in REAH usually is not found defects or erosion of the skull base [6]. In case of doubt, a preoperative biopsy is clarifying and allows better surgical planning. Magnetic resonance imaging helps in the differential diagnosis for isolated and unilaterally REAH with inverted papilloma, meningoencephalocele and malignant tumors. In addition, MRI also helps to distinguish the lesion from mucous retention [2]. However a conclusive diagnosis is only available after histopathological examination.
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