Esthesioneuroblastoma

ESTHESIONEUROBLASTOMA = OLFACTORY NEUROBLASTOMA–,   ESTHESIONEUROEPTHELIOMA OLFACTIF, NEUROESTHESIOMA
ONLY -300 CASES REPORTED
IN DIFFERENTIAL FOR CONG NASAL MASS
SEE A RED POLYPOID MASS ORIGINATING HIGH IN THE NASAL CAVITY
WOULD GET CT/MRI—HYPO/ISO DENSE ON T1—HYPERINTENSE ON T2
GET NS CONSULT
OF NEUROECTODERMAL ORIGIN (NEUROCREST CELLS)—OLFACTORY EPITHILIUM WITHIN THE SUP NASAL VAULT
NEUROSECRETORY GRANULES BY E.M. AND LIGHT MICROSCOPY
+ S100, +NEURON SPECIFIC ENOLASE (NSE), SYNAPTOPHYSIS, CHROMAGEN, NEG FOR EPITHELIAL MARKERS, GLIAL FIBRILLARY ACIDIC PROTIEN, NEUROFIBRILLARY PROT.
HISTO–PSEUDOROSETTES(SMALL ROUND CELLS ARRANGED IN A ROSSETTE PATTERN), SHEETS, OR CLUSTERS—HOMER WRIGHT ROSETTE = PATHOGNEUMONIC
PRESENT WITH SINUSITIS LIKE SYMPTOMS—-EPISTAXIS AND NAO WITH SOME PAIN AND HYPOSMIA
VERY VARIABLY AGRESSIVE—-LN METS < 20%
MALE = FEMALE
BIMODAL INCIDENCE (11-20, 51-60)
BARNES (HISTO CLASSIFICATION)
40% NEUROCYTOMA–SHEETS OF CLUSTERS
40% NEUROEPITHELIOMA—TRUE ROSETTES
20% NEUROBLASTOMA–PSEUDOROSETTES
GRADES I-IV
KADISH (CLINICAL CLASSIFICATION)/ TNM
A–NC ONLY   100% 5 YEAR SURVIVAL
B–NC AND PARANASAL SINUSES   75% 5 YEAR SURVIVAL
C–BEYOND   10-20% 5 YEAR SURVIVAL
Rx SURGERY (CRANIOFACIAL RESECTION) AND POST OP RT +/- CHEMO(CISPLATIN, ETOPOSIDE)
TREAT THE RARE CLINICALLY + NECK ONLY
WILL NEED SERIAL MRI SCANS FOR SURVEILLENCE

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