Respiratory epithelial adenomatoid hamartoma (REAH) is an uncommon lesion of the upper aerodigestive tract first described by Wenig and Heffner in 1995 as prominent glandular proliferations lined by ciliated respiratory epithelium originating from the surface epithelium. Case Report: We report a case of 48-year-old women with nasal polyposis history, which consults for nasal obstruction, with suspicion of malignancy on CT. Surgical resection showed a respiratory epithelial adenomatoid hamartoma (REAH) of the nasal cavity. Conclusion: REAH is a recently described pathologic entity that can present 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.
The respiratory epithelial adenomatoid hamartoma (REAH) was first described as a distinct lesion by Wenig and Heffner [
We report a case of 48-year-old women with nasal polyposis history, which consults for nasal obstruction. His symptoms have worsened over a 10-month period despite oral steroid and antibiotic medications. The CT of nasal cavity and sinus showed soft tissue density (
The REAH is a rare lesion limited in its site of occurrence to the nasal cavity, paranasal sinuses, and nasopharynx [1-4]. Its rarity is evidenced by the extremely limited number of journal article references found in a search of the National Library of Medicine database, [1,5,6] with 2
of these 3 citations being single case reports [5,6] The lesion goes unmentioned in most textbooks of general pathology, and its discussion in texts limited to head and neck pathology typically consists of less than a single page of text [3,4,7]. Therefore, it seems reasonable to presume that the REAH is a lesion that most pathologists may never have encountered. Involvement of the maxillary sinus by REAH is particularly rare. The original 31 cases reported by Wenig and Heffner1 did not include any lesions involving the maxillary sinus, and only one other report of a REAH involving the maxillary sinus was identified [
On microscopic examination, sinonasal adenocarcinomas show a wide range of differentiation and patterns. Intestinal type adenocarcinomas are of high grade. The most common architecture is the tubulopapillary type but goblet cell, signet ring, and mucinous types have been described [10,11]. Differentiating the intestinal type sinonasal adenocarcinoma from REAH is usually not difficult as the cell types, high grade features, and greatly increased mitoses are features of adenocarcinoma [
Respiratory epithelial adenomatoid hamartoma truly is a lesion in its infancy. Described only a decade ago, it is an uncommon entity with distinctive morphologic features. Clinically, it is an expansive mass which causes upper respiratory symptoms and discomfort mainly in adult men, although cases in women and children have been reported. Distinctive histologic features include a glandular component which originates from the overlying surface respiratory epithelium and polypoid growth as a result of respiratory epithelial adenomatoid proliferation. The columnar cells lining the glands are ciliated, further illustrating the benign nature of the lesion. Diagnostic misinterpretation is a serious issue regarding this lesion. Pathologists must be aware of this entity in order to avoid overdiagnosis and excessive surgical procedures for the patient.