Sinonasal respiratory epithelial adenomatoid hamartoma
Sinonasal respiratory epithelial adenomatoid hamartoma (REAH) is a rare benign glandular proliferation of the sinonasal cavities. It is most commonly encountered within the olfactory clefts.
Epidemiology
It is most often encountered in middle-aged adults with no definite gender predilection .
Clinical presentation
Clinical presentation is nonspecific with symptoms related to obstruction of the sinonasal cavities . The most common symptoms include nasal airway obstruction (~60%), hyposmia (~18%), facial pain (~14%), rhinorrhea ( ~14%), and postnasal drip ( ~8%) .
Pathology
It is a benign, non-neoplastic, inflammatory polypoid lesion lined with ciliated respiratory epithelium most commonly found in the olfactory clefts or posterior nasal cavities with nasopharyngeal extensions .
There is some controversy as to whether it represents an isolated (REAHi) or an incidental pathologic finding as it is often encountered with sinonasal polyposis (~70%, REAHsnp) . It has also been described in association with inverted papilloma and low-grade sinonasal adenocarcinoma . As such, its clinical significance, especially when coexisting with sinonasal polyposis, is uncertain.
Radiographic features
REAHi is described as a hypoattenuating homogeneous mass arising from and widening the olfactory clefts that mimic other solitary polypoid sinonasal lesion .
REAHsnp is difficult to distinguish from sinonasal polyposis, but the widening of the olfactory clefts (> 10 mm) should raise suspicion.
In one series, REAH has been described as bilateral in up to ~40% of cases .
History and etymology
It was first described as a distinct entity in 1995 by Wenig and Heffner .
Differential diagnosis
General differentials consideration include:
- sinonasal polyposis (REAHsnp) and inflammatory polyp (REAHi)
- esthesioneuroblastoma: associated with a skull-base defect
- encephalocele: associated with a skull-base defect
- sinonasal adenocarcinoma