Sinonasal Hamartoma



Sinonasal Hamartoma


Bruce M. Wenig, MD










The histopathologic changes show a glandular proliferation composed of widely spaced, small to medium-sized glands image separated by stromal tissue invaginating downward into the submucosa image.






The glands are round to oval, composed of multilayered ciliated respiratory epithelium with characteristic envelopment of glands by a variably thickened, eosinophilic basement membrane image.


TERMINOLOGY


Abbreviations



  • Respiratory epithelial adenomatoid (READ) hamartoma


  • Chondro-osseous and respiratory epithelial (CORE) hamartomas


  • Nasal chondromesenchymal hamartoma (NCH)


Synonyms



  • Glandular hamartoma


  • Seromucinous hamartoma


  • Nasal hamartoma


Definitions



  • READ hamartoma: Benign acquired nonneoplastic overgrowth of indigenous glands of sinonasal tract and nasopharynx



    • Arising from surface epithelium


    • Devoid of ectodermal, neuroectodermal, &/or mesodermal elements


  • CORE hamartoma



    • Related to READ hamartoma but has additional feature of chondroid tissue


  • NCH: Tumefactive process of sinonasal tract



    • Composed of admixture of chondroid and stromal elements with cystic features analogous to chest wall hamartoma


    • Histologic similarities to READ and CORE hamartomas; may be within spectrum of same lesion type


ETIOLOGY/PATHOGENESIS


Idiopathic



  • No association with any specific etiologic agent


Developmental



  • READ hamartomas arise in setting of inflammatory polyps



    • Raises possible developmental induction secondary to inflammatory process


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare lesions


  • Age



    • READ hamartoma



      • Occurs in adult patients


      • Range from 3rd to 9th decades


      • Reported median in 6th decade


    • CORE hamartoma



      • Ranges from 11-73 years


    • NCH



      • Most of these lesions occur in newborns within 1st 3 months of life but may occur in 2nd decade of life


      • Occasionally in adults


  • Gender



    • READ hamartoma



      • Equal gender distribution


    • CORE hamartoma



      • Equal gender distribution


    • NCH



      • Male > Female


Site



  • Majority occur in nasal cavity, particularly posterior nasal septum



    • Involvement of other intranasal sites occurs less often and may be identified



      • Along lateral nasal wall, middle meatus, and inferior turbinate


  • Other sites of involvement include nasopharynx, ethmoid sinus, and frontal sinus


  • Majority of lesions are unilateral, but occasionally bilateral lesions may occur



Presentation



  • READ hamartoma



    • Clinical presentation may include one or more of the following symptoms



      • Nasal obstruction, nasal stuffiness, epistaxis, and chronic (recurrent) rhinosinusitis; associated complaints include allergies


      • Symptoms may occur over months to years


    • Nondestructive lesion


  • CORE hamartoma



    • Polypoid mass lesion


  • NCH



    • Respiratory difficulty and intranasal mass or facial swelling may be present


    • May erode into cranial cavity (through cribriform plate area), clinically simulating appearance of meningoencephalocele

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Sinonasal Hamartoma

Full access? Get Clinical Tree

Get Clinical Tree app for offline access