Nasopharyngeal Angiofibroma



Nasopharyngeal Angiofibroma


Lester Thompson, MD

Cyril Fisher, MD, DSc, FRCPath





Key Facts


Terminology



  • Benign, highly cellular, and richly vascularized mesenchymal neoplasm arising in nasopharynx in males


Clinical Issues



  • Recurrent, spontaneous epistaxis


  • Nasopharynx is nearly always affected


  • Patients < 20 years old


  • Males exclusively


  • Recurrences in ˜ 20% of patients


  • Up to 22 cm in size, mean: 4 cm


Image Findings



  • Anterior bowing of posterior wall of maxillary sinus with posterior displacement of pterygoid plates (Holman-Miller sign)


  • Angiography identifies feeding vessel(s) and allows for presurgical embolization


  • Tumor blush is characteristic


Microscopic Pathology



  • Submucosal proliferation of vascular component within fibrous stroma


  • Many variably sized disorganized vessels


  • Fibrous stroma consists of plump spindle, angular, or stellate-shaped cells


  • Variable amounts of fine and coarse collagen fibers


  • Elastic tissue is not identified within stroma


Top Differential Diagnoses



  • Lobular capillary hemangioma


  • Antrochoanal polyp


  • Inflammatory polyp






Low magnification shows an intact surface with a wide variety of vessels in a fibrous stroma. Some of the vessels have smooth muscle, and others do not. Patulous and compressed vessels are noted.






Smooth-muscle-walled vessels image lie close to vessels without smooth muscle image. There are also numerous capillaries in the fibrous stroma. The lesion is moderately cellular and lacks atypia.


TERMINOLOGY


Abbreviations



  • Juvenile angiofibroma (JNA)


  • Angiofibroma (AF)


Synonyms



  • Angiomyofibroblastoma-like tumor


  • Angiofibroma


  • Fibroangioma


  • Fibroma


Definitions



  • Benign, highly cellular, and richly vascularized mesenchymal neoplasm arising in nasopharynx in males


ETIOLOGY/PATHOGENESIS


Hormonal



  • Testosterone-dependent puberty-induced growth can be blocked with estrogen &/or progesterone therapy


Genetic



  • Reported association with familial adenomatous polyposis


CLINICAL ISSUES


Epidemiology



  • Incidence



    • < 1% of all nasopharyngeal tumors


    • < 0.1% of all head and neck neoplasms


  • Age



    • < 20 years old



      • Adolescents to young men


      • Peak in 2nd decade of life


  • Gender



    • Males exclusively


    • If diagnosed in female, studies of sex chromosomes required to confirm gender


  • Ethnicity



    • Worldwide distribution


    • Higher frequency in Caucasians



      • Favors fair-skinned, red-haired individuals


Site



  • Nasopharynx usually affected


  • Pterygoid region usually affected


  • May expand to involve surrounding structures (30% of cases)



    • Anterior: Nasal cavity and maxillary sinus via roof of nasopharynx


    • Lateral: Temporal and infratemporal fossae via pterygomaxillary fissure, resulting in cheek or intraoral buccal mass


    • Posterior: Middle cranial fossa


    • Superior: Pterygopalatine fossa and orbit via inferior and superior orbital fissures resulting in proptosis


    • Medial: Contralateral side


Presentation



  • Nasal obstruction


  • Recurrent, spontaneous epistaxis


  • Nasal discharge


  • Facial deformity (proptosis), exophthalmia, diplopia


  • Rhinolalia, sinusitis


  • Otitis media, tinnitus, deafness


  • Headaches


  • Rarely, anosmia or pain


  • Symptoms present for 12-24 months (nonspecific presentation)


Treatment

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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Nasopharyngeal Angiofibroma

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