EsthesioneuroblastomaBy mahyarHistory: 24 Y/ Male with left eye proptosis Findings:
:: MRI 1 ::Axial T2W- non contrast: nasal cavity mass with extension into left orbit and ethmoid sinuses
:: MRI 2 ::sagittal T2W, non contrast: Dumbbell shape mass with upper portion in intracranial fossa and lower portion in upper nasal cavity
:: MRI 3 ::sagittal T1W + Gd: The lesion enhances diffusely.
:: MRI 4 ::Axia l FLAIR Discussion: Esthesioneuroblastoma is an uncommon malignant tumour that arises from bipolar sensory receptor cells in the olfactory mucosa. These cells originate in the neural crest and differentiate into the olfactory sensory elements. Esthesioneuroblastomas are histologically similar to adrenal or sympathetic ganglionic neuroblastomas and retinoblastomas. It was originally descried by Bergen et al. in 1924 as esthesioneuroepithelioma olfactif . Incidence peaks once in 11-20 years of age group (16.8% of all tumors) and again in 50-60 years age group (22.8% of all tumors); however, age of these patients ranges from 3-88 years. It occurs equally in men and women. "Some references:slight female predominance". Synonyms include olfactory esthesioneuroma, neuroesthesioma and olfactory neurocystoma. The main symptoms are nasal occlusion, proptosis, epistaxis, headache, excessive lacrimation, anosmia and visual disturbances. Because sensory nerves of smell originates in olfactory bulb and pass through cribriform plate to olfactory area of nasal mucosa which is located in the most superior part of both nasal fosse, usual primary sites of occurrence include superior nasal cavity or nasal septum, the turbinates, the ethmoid, or the cribriform plate, although an extra nasal site of origin has been suggested. Even if the lesion can not be demonstrated radiographically above the cribriform plate, involvement of both sided of the plate is presumed and surgical approaches include removal of this area. This polypoid tumor may be of variable consistency and may bleed profusely on biopsy. It is almost always unilateral and only in highly neglected cases, they appear as a bilateral nasal fosse mass. The mass may be relatively slow growing for malignant tumour and cause some expansion and remodeling of bony structures. Hyperostosis may be an inflammatory reaction related o obstructed neighboring air cells. This neoplasm is locally aggressive and cause metastasis by lymphatic and hematogenous routes. Local recurrence has been reported in up to 57% of patients. A metastatic rate of 20% to 60% is reported with the most common site being the cervical lymph node. Other sites include the parotid glands, skin, lungs, bone, liver, orbit, spinal cord and spinal canal. The diagnosis and evaluation of staging of esthesioneuroblastoma can be done by CT which provides the best information about the tumor invasion into bony structures. The protocols for CT scanning include axial and coronal scans of 1 to 5 mm thick slices with IV contrast. The tumour is presented as homogenous density mass, equal to or greater than surrounding soft tissues. Contrast enhancement is usually moderate and homogenous. They commonly extend to ipsilateral ethmoid and maxillary sinuses and only rarely involve sphenoid sinuses. When large, they can extend to involve both sides of nasal cavity and the paranasal sinuses. Intralesional calcification and presence of cysts along the intracranial margins in case of intracranial extension yield a definite diagnosis. Obstruction of sinus draining ostia results in an accumulation of nasal secretions which tend to difficult to differentiate from tumour tissue when viewed by a CT scan. Bone erosion is frequent and usually accompanied by modeling of bone. Staging, determined by extension and critical for treatment decisions, is well evaluated by CT. On T1W MR Images, esthesioneuroblastoma presents as homogenously enhancing tumour with intermediate signal intensity, where as T2W images, the original intensity is increased. MRI can delineate intraorbital and intracerebral extension. The tumor appears hypo to gray matter on T1WI and iso or hyper to gray matter on T2WI. Gadolinium enhanced MR images help to differentiate tumour from obstructed secretions in paranasal sinuses, determining meningeal and extradural spread and to detect perineural spread. Angiography demonstrates tumour blush in majority of cases. Scintigraphy: Since most ENBs express somatostatin receptors, the use of scintigraphy with a radiolabeled somatostatin analog (111 In-pentoctreotide [111 In-DTPA-D-pheoctreotide]; Octreoscan) has been proposed. A preliminary study of this technique found it to be clinically useful, especially for discriminating between postoperative changes and residual or recurrent tumor after extensive skull base surgery. The sensitivity and specificity remain unclear, however. Differential Diagnoses: Lymphoma, Non-Hodgkin Malignant Melanoma Metastatic Cancer, Unknown Primary Site Plasmacytoma, Extramedullary Nasal and paranasal squamous cell carcinoma Sinonasal polyposis Choanal polyp Juvenile angiofibroma Neuroendocrine carcinoma Embryonal rhabdomyosarcoma Undifferentiated sinonasal carcinoma Ewing sarcoma In 1992, Dulguerov and Calceterra proposed a classification based on the tumor, node, metastasis (TNM) system, which is predicated on CT and MRI findings that can be identified before treatment.15 although this classification system has gained popularity, and attempts have been made to further modify the Kadish system for ENB. Differential diagnosis: Differential Diagnoses: Lymphoma, Non-Hodgkin Malignant Melanoma Metastatic Cancer, Unknown Primary Site Plasmacytoma, Extramedullary Nasal and paranasal squamous cell carcinoma Sinonasal polyposis Choanal polyp Juvenile angiofibroma Neuroendocrine carcinoma Embryonal rhabdomyosarcoma Undifferentiated sinonasal carcinoma Ewing sarcoma Diagnosis confirmation: Surgery / Histo Category: Neuro Region / Organ: Head-Nose Etiology: neoplastic References: 1)TSENG J, Michel MA, Loehrl TA, Peripheral cysts: a distinguishing feature of esthesioneuroblastoma with intracranial extension. Ear Nose Throat J. 2009; 88:E14. 2) Khia S, Patel K. Esthesioneuroblastoma. Indian J Radiol Imaging 2006;16:669-72 3) Rostomily RC, Elias M, Deng M, Elias P, Born DE, Muballe D, et al. Clinical utility of somatostatin receptor scintigraphic imaging (octreoscan) in esthesioneuroblastoma: a case study and survey of somatostatin receptor subtype expression. Head Neck. Apr 2006; 28(4):305-12. 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