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Esthesioneuroblastoma
uncommon neoplasm 3%olfactory epithelium high in the nasal vault invades the skull base, cranial vault, and orbit
PATHOLOGYlobular architecturedense neurofibrillary backgroundround to oval nucleiscant, poorly defined cytoplasm.olfactory rosettes or pseudorosettes
Haematoxylin & Eosin stainimmunohistochemical staining
neuron-specific enolase (NSE)neurofilamentsynaptophysinchromograninLeu-7
S-1 00mostvariably positiveNeuron Specific Enolase
Ki67 Stain positive
less commoncommonfleshy, friable nasal mass
Males =females
3 to 78 years
second and fifth decades
Metastasis 17% to 48%
No geographic, environmental or , lifestyle risk factorsMales =females
3 to 78 years
second and fifth decades
Metastasis 17% to 48%
No geographic, environmental or , lifestyle risk factors
Cervical lymph nodesLungBoneLiverMediastinumAdrenal glandOvarySpleenParotidCNSSpinal epidural space
MR T1 sequence. Low signal mass with lateral displacement of the left optical nerve suggestive of left temporal lobule infiltration
radiology
MR T1+GD sequence. Infiltrative mass poorly enhancement.
MR T1+GD in coronal view. Apparent intradural invasion. Central necrotic or cystic degeneration areas.
MR T2 sequence. Partial occupation of the frontal and sphenoid sinuses
Coronal T2Axial T2Coronal T1 + GD
PATIENT EVALUATION
TREATMENTsurgeryRadiotherapyChemotherapyRadiosurgeryendoscopyopenRadiotherapyEarly lesions (Kadish stage A or B)inoperable casesadvanced or metastatic diseasetumor massPreoperative radiotherapylocal tumor disseminationdistant metastasesPostoperative radiothrapy50 to 60 Gy,Decrease recurrenceChemotherapyneuroblastomasmall cell lung carcinomaprimitive neuroectodermal
Cyclophosphamide , doxorubicineVincristin , platinum-based therapyGross total tumor resection +
chemotherapyBetter outcomeChemoradiation Therapy decrease in total tumor volume of greater than 50%
reduction in intracranial tumor mass of greater than 90%
Kadish stage A or B
45To 50 Gr preoperativelyKadish stage C
45 To 50 Gr
Six cycle of cyclophosphamidevincristine
Radiosurgeryrecurrent or residual disease
difficulty of determining an accurate target in the setting of postoperativechanges
optic nerves and chiasm
Surgical Resectionmainstay of therapy for esthesioneuroblastomaantibiotic nafcillin and ceftriaxone preoperativelybetween 7 and 10 days until the nasal packing is removedlumbar cerebrospinal fluid (CSF) drainageBolus and maintained on anticonvulsantsENT , neurosurgeon, ophthalmologist
SURGICAL TECHNIQUEcraniofacial resection
supine position
abdomen and lateral aspect of the thigh are prepared for grafting of fat and fascia
bicoronal incision.
Preoperative MRI scan
Postoperative CT scanCOMPLICATIONSSurgical complicationsCerebral infarction and contusionCSF leakageepidural abscessmeningitisbone flap infection requiring flap removalVisual complications
Blindness,loss of acuty)Diplopia,(Exophthalmos,Chemotoxic complicationsbone marrow suppression,Vocal cord paralysis
peripheral neuropathy,herpes zoster infectionsymptomatic pneumocephaluspercutaneous aspirationTrendelenburg position.
100% oxygenHyams' grade II86% 5-year survivalgrade III58% 5-year survivalTumor stage has been proposed as a prognostic factor as well
disease beyond the cervical lymph nodes rarely survive more than 1 year
No strong correlation between initial symptoms and outcome has been made
not found histologic gradeto be of more prognostic significance than disease stage
Recurrence of disease after gross total surgical resectionis common. 14%
reoperationaverage time to recurrence is 2 to 6 yearslong-term follow-upOUTCOMES
Esthesioneuroblastoma: Mansoura University Hospitals experience with multimodality therapy in 10 years
Conclusions: ENB can be a highly curable sinonasal malignancy. Combined modality treatment is considered an integral part of management protocol
Survival 5.3 8.75 years- 35 imaging . . 94 ( type C : +
) type C : ) type D :) type B : + 41- 35 imaging . . 94 ( type C : +
) type C : ) type D :) type B : +