Respiratory epithelial adenomatoid hamartoma – case report and literature review

portuguesO Hamartoma Adenomatoide Epitelial Respiratorio (REAH) e um tipo distinto de hamartoma encontrado na cavidade nasal e seios perinasais. O REAH e raro e tem um comportamento benigno. A etiologia desta lesao permanece por esclarecer. O REAH apresenta-se habitualmente com obstrucao nasal, rinorreia, hiposmia/anosmia ou epistaxis. A observacao histologica mostra uma proliferacao glandular bem diferenciada limitada por epitelio pseudoestratificado ciliado, com um crescimento auto-limitado. A resseccao completa da lesao e curativa e a recorrencia inesperada. Apresentamos um caso clinico de um homem de 63 anos de idade com historia de obstrucao nasal de longa duracao. A tomografia computadorizada demonstrou uma neoformacao polipoide bilateral surgindo da fenda olfactiva. A lesao foi removida por cirurgia endoscopica nasossinusal. A analise histopatologica confirmou REAH. O seguimento de seis meses nao mostrou evidencia de recorrencia. Esta entidade deve ser considerada no diagnostico diferencial de lesoes nasossinusais e distinguida de polipose nasal, papiloma invertido e adenocarcinoma. A identificacao correcta do REAH e importante para evitar tratamentos mais agressivos. EnglishThe Respiratory Epithelial Adenomatoid Hamartoma (REAH) is a distinct kind of hamartoma found in the nasal cavity and paranasal sinuses. REAH is rare and it has a benign behavior. The etiology of this lesion remains unclear. REAH usually presents with nasal obstruction, rhinorrhea, hyposmia/anosmia or epistaxis. The histological observation shows a well-differentiated glandular proliferation limited by pseudostratified ciliated epithelium, with a self-limited growth. Complete resection of the lesion is curative and the recurrence is unexpected. We report a case of a 63-year-old man, with history of longlasting nasal obstruction. Computed tomography (CT) scans demonstrated a bilateral polypoid mass arising from olfactory recess. The lesion was removed through endoscopic sinus surgery. The histopathologic analysis confirmed REAH. Follow-up after six months showed no evidence of recurrence. This entity must be considered in the differential diagnosis of sinonasal lesions and distinguished from nasal polyposis, inverted papilloma and adenocarcinoma. The correct identification of REAH is important to avoid more aggressive treatments.