SUMMARY: REAH is a rare benign lesion of the sinonasal tract. The nasal cavity, particularly the posterior nasal septum, is the most common site of involvement. It usually occurs unilaterally and can be cured with conservative surgical resection. We present an unusual case of adenomatoid hamartoma involving bilateral olfactory recesses and discuss the importance of distinguishing this from other neoplastic processes that may lead to overly aggressive treatment.ABBREVIATIONS: H&E ϭ hematoxylin-eosin; IP ϭ inverted papilloma; REAH ϭ respiratory epithelial adenomatoid hamartoma; SNAC ϭ sinonasal adenocarcinoma A hamartoma is a malformation of tissue that is indigenous to the involved anatomic site. It has no capacity for unimpeded proliferation and causes symptoms mainly related to mass effect on adjacent structures. Hamartomas can occur anywhere in the body, but involvement of the head and neck is exceedingly uncommon.A particular subset known as REAH was first characterized in 1995 and represents a rare lesion of the sinonasal tract.1 It is important to distinguish REAH from more aggressive neoplastic processes because REAH is a benign entity that can be cured with conservative surgical resection.
Case ReportAn otherwise healthy 60-year-old woman presented with headaches of 2 weeks' duration. She also complained of nasal congestion, an altered sense of smell, and stuffiness in both ears. Her medical history was notable for environmental allergies and chronic sinusitis. Findings of physical examination and laboratory work-up were unremarkable.MR and CT imaging were performed, which revealed an enhancing soft-tissue mass in the anterior left nasal cavity involving the olfactory recess and a similar-appearing smaller mass in the right olfactory recess (Fig 1AϪC). There were no destructive bone changes or intracranial extension. The uninvolved paranasal sinuses were clear. Imaging findings were most consistent with a primary neoplasm of the sinonasal cavity.Endoscopic biopsy revealed a REAH, which was subsequently resected endoscopically. On examination, the masses involved the anterior frontal skull base and bilateral olfactory recesses, left greater than right, and extended to the root of the middle turbinate. The masses were completely excised by using an endoscopic approach.The masses measured 1.5 (left) and 0.8 cm (right) and were microscopically similar (Fig 1D, -E). They contained many enlarged glands lined by ciliated respiratory epithelium. The periglandular connective tissue was attenuated and fibrotic, consistent with basement membrane thickening. Intervening stroma resembled that of inflammatory sinonasal polyps (ie, loose connective tissue with edema), vascular congestion, and moderate numbers of chronic inflammatory cells. Stains for  catenin and smooth muscle actin showed positivity in the stromal cells, identical to those of a typical sinonasal polyp. Stains for S-100, c-kit, and CD34 were negative in the stromal cells. Notably, lobulated seromucinous glands were absent. These characteristic histolog...