JNA (JUVENILE NASOPHARYNGEAL ANGIOFIBROMA)
PRIMARY VASCULAR MASS OF THE NC
DESCRIBED IN 1940 BY FRIEDBERG
RARE, BENIGN, VASCULAR NEOPLASM
ALMOST EXCLUSIVELY IN ADOLESCENT MALES—IF FEMALE DO A KARYOTYPE
USUALLY AT PUBERTY—AGE 7-21—–CYTOSOL FRACTIONALTION FOR DHT AND TESTOSTERONE RECEPTORS—-RARELY MANIFEST AFTER 20
? AGE OF SAFETY
NON-METASTASIZING BUT LOCALLY AGGRESSIVE—50% BONEY DESTRUCTION
VERY VASCULAR—DO NOT BX IN THE OFFICE
ARISE IN THE NP OR LAT NASAL WALL AT THE POST MID TURB
TEND TO INVADE POST ASPECT OF CHOANAE—PTERYGOPALATINE FOSSA—NP RECESS OR ANT WALL OF THE SPHENOID
BOWING OF THE POST MAXILLARY SINUS WALL—a ptyerygomaxillary space mass that enhances and “bowes” forward the posterior maxillary sinus wall “Holman-Miller” sign = JNA juvenille nasoangiofibroma in adult young males
BLOOD SUPPLY FROM THE ECA, IMA AND SP ARTERY OR THE ASC. PHARYNGEAL A
Hx AND PE
ANOSMIA, HYPONASAL SPEECH, NAO, SINUSITIS, CHL
INTERMITTENT EPISTAXIS—-IDA, RHINOLELIA CLAUSA, OME
PROPTOSIS, OPTIC ATROPHY
PURPLISH, REDDISH, GREY NC MASS—MUCOSAL COVERED, OVOID, FIRM, LOBULATED, SESSILE, SMOOTH
OCCASIONAL DEFORMITY OF THE CHEEK—-OFTEN COVERED WITH ULCERATIONS
STAGHORN HISTOLOGIC APPEARANCE, MULTIPLE VASCULAR CHANNELS SURROUNDED BY TOUGH FIBROUS CT
Dx: MRI FOR INTRACRANIAL SPREAD, ?MRA
CT WITH CONTRAST FOR VASCULARITY AND BONEY INVOLVEMENT
ARTERIOGRAM OF CAROTID SYSTEM IS PATHOGNEUMONIC
NO NEED FOR BX
CHANDLER STAGING SYSTEM
I NP ONLY
II NC +/- SPHENOID
III + OTHER SINUSES
IV INTRACRANIAL
Rx: PRE OP EMBOLIZATION AT TIME OF ARTERIOGRAM—USUALLY 48-72 HOURS PRE-OP
SURGICAL RESECTION—-A 2 AVENUE APPROACH PROVIDES THE BEST RESULTS
WEBER-FERGUSSON, TRANSPALATAL, LEFORT I OSTEOTOMY WITH FACIAL DEGLOVING
RT LIMITED TO PTS WITH EXTENSIVE INTRACRANIAL Dz (UNRESECTABLE Dz)
50% RECURRENCE RATE—ALMOST ALL WITHIN 2 YEARS