esthesioneuroblastoma


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es·the·si·o·neu·ro·blas·to·ma

(es-thē'zē-ō-nū'ō-blas-tō'mă),
A neoplasm of immature, poorly differentiated neuronal cells believed to arise from neuroepithelial precursors.
[esthesio- + neuroblastoma]

esthesioneuroblastoma

A rare malignant neuroectodermal tumour of the nasal cavity in the retrobulbar region, middle cranial fossa, near the cribriform plate. It is thought to arise from the olfactory membrane of the sinonasal tract.

Clinical findings
Bimodal age distribution, affecting those in the 2nd and 6th decades of life; presents as a unilateral nasal obstruction, epistaxis, anosmia and headache, accompanied by nonspecific symptoms similar to other intranasal lesions—i.e., congestion, rhinorrhoea.
 
DiffDx
Small round cell malignancies that occur in the sinonasal tract—e.g., sinonasal undifferentiated carcinoma, lymphoma, rhabdomyosarcoma and neuroendocrine carcinomas.

Prognosis
5-year survival 50–60%; late recurrence is common. Hyams’ grading system is used for grading; it incorporates architecture, rosette formation, mitoses, necrosis and pleomorphism, and correlates with survival.

esthesioneuroblastoma

Olfactory neuroblastoma ENT A tumor of the nasal cavity, retrobulbar region or middle cranial fossa Clinical Nonspecific, similar to other intranasal lesions, ie congestion, rhinorrhea DiffDx Lymphoma, plasmacytoma, embryonal rhabdomyosarcoma Prognosis 5-yr survival 50-60%; late recurrence is common

esthesioneuroblastoma

see olfactory neuroblastoma.
References in periodicals archive ?
Two patients with chordoma died from tumor aggression and two patients with esthesioneuroblastoma died from metastasis.
Olfactory neuroblastoma (ONB), also known as Esthesioneuroblastoma, is a rare but distinct malignant neuroectodermal tumor arising from the olfactory epithelium of roof of nasal cavity and cribriform plate.
Some histologies are staged using other systems, for example, the Kadish system is used for staging esthesioneuroblastoma.
SNUC can be distinguished from esthesioneuroblastomas by the histologic occurrence of intercellular fibrils and periodic Homer Wright rosettes in esthesioneuroblastomas.
Prognostic utility of Hyams histological grading and Kadish-Morita staging systems for esthesioneuroblastoma outcomes.
The differential diagnosis of lesions with this clinical apperance is very broad and includes epithelial neoplasm, esthesioneuroblastoma, meningioma, hemangiopericytoma, lymphoma, schwannoma, leiomyoma, angiofibroma, fibromatosis, malignant fibrous histiocytoma and fibrosarcoma.
Esthesioneuroblastoma is not a member of the primitive peripheral neuroectodermal tumour-Ewing's group.
DISCUSSION: Olfactory neuroblastoma also called as Esthesioneuroblastoma is a relatively uncommon intranasal tumour that originates from the olfactory epithelium of the upper nasal cavity in the region of cribriform plate.
Lesions included adnexal neoplasm, ameloblastic fibro-odontoma, basal cell carcinoma, benign fibrous histiocytoma, blue nevus, chondrosarcoma, compound nevus, epithelioma adenoides cysticum, esthesioneuroblastoma, Ewing sarcoma, fibrosarcoma, giant cell granuloma, granulocytic sarcoma, hemangioma, hemangiopericytoma, Langerhans cell histiocytosis, lymphangioma, lymphoma, melanoma, neuroblastoma, neurofibroma, ossifying osteofibroma, osteochondroma, osteosarcoma, port wine stain, rhabdomyosarcoma, Spitz nevus, and xanthogranuloma.
DISCUSSION: Esthesioneuroblastoma (olfactory neuroblastoma) is a rare neuroectodermal tumour, (1) which is challenging for multidisciplinary management, as so far there is no known accepted standard therapy.
1 It may be mistaken for esthesioneuroblastoma because of its neuroectodermal histologic component and features of cribriform plate transgression.