Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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March 31, 2022
Respiratory Epithelial Adenomatoid Hamartoma (REAH)
- Background:
- Respiratory epithelial adenomatoid hamartomas (REAHs) are often incidental, benign, inflammatory, glandular neoplasms that are lined with respiratory epithelium.
- REAH can occur in isolation or in association with other paranasal sinus pathology such as polyps, inverted papilloma, or sinonasal adenocarcinomas.
- Clinical Presentation:
- Presenting symptoms are nonspecific and may include nasal airway obstruction, hyposmia, facial pain, rhinorrhea, and postnasal drip.
- Key Diagnostic Features:
- On CT, these lesions present as soft tissue arising from and widening the anterior olfactory clefts.
- On MRI, they demonstrate T2-hyperintense signal, avid enhancement, and a discoid shape that is better appreciated on sagittal images.
- Differential Diagnoses:
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Inflammatory polyp: May be bilateral but asymmetric with varying degrees of mass effect, which can result in benign osseous remodeling or erosions; REAH can coexist with inflammatory polyps.
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Sinonasal adenocarcinoma: May arise in the olfactory clefts but is more commonly associated with aggressive osseous destruction and may invade adjacent structures; typically heterogeneous enhancement
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Encephalocele: Will demonstrate association with a skull base defect and may be expansile
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Esthesioneuroblastoma: Dumbbell-shaped mass centered in the ethmoid, characteristically with mass effect and osseous destruction and hyperostosis/bony remodeling; may have peritumoral cysts at the brain interface and rare calcification
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Treatment:
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REAH is considered a self-limiting disease; however, endoscopic resection is thought to be curative with a low recurrence rate.
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