Slide 1

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 : +