1.Esthesioneuroblastoma in a 42-year old Filipino: A case report.
Reynold A WONG ; Marianne S. SAJULGA ; Marivic GUIEB-PREYSLER
Philippine Journal of Neurology 2009;13(2):45-45
INTRODUCTION: Olfactory esthesioneuroblastoma also known as olfactory neuroblastoma or esthesioneuroblastoma is an uncommon neuroectodermal tumor that originates from the olfactory sensory epithelium in the upper nasal fossa at the level of the cribriform plate. It represents up to 5% of malignant tumors of the nasal cavity and 20-30% of its metastasis involves the CNS.
CASE REPORT: We present a case of a 42-year-old female who presented with an eleven (11)-year history of the evolution and recurrence of a nasal mass which initially presented as recurrent nasal congestion. Until she presented with profuse epastaxis where she was worked up and MRI of the brain showed a nasal mass extending to bilateral frontal lobes (the official report was unavailable). She underwent maxillofacial surgery and frontal craniotomy with removal of the maxillary and dural masses. Histopathology reported metastatic esthesioneuroblastoma. There was recurrence of a mass in the left submandibular area followed by another mass in the right maxillary area and a third mass in the right submandibular area. She was advised excision of the mass but was lost to follow up. She was admitted for generalized tonic-clonic seizure.
DISCUSSION: The case presented started with a mass in the nasal cavity which has spread to the cranial cavity. Differentials for nasal cavity lesions should include more common malignancies of the nasal cavity such as squamous cell carcinoma, adenocystic carcinoma, and adenoid carcinomas. Definite diagnosis is obtained through histopathology. No standardized treatment protocol is available at the time of this writing. This is followed by radiotherapy for primary low- to moderate-grade lesions with the addition of chemotherapy for patients with advanced, recurrent or metastatic disease (i.e. cyclophosphamide and vincristine). The 5-year survival rate of ENB is 45% for surgery and radiotherapy. Prognosis depends on the stage and grade of the disease.
SUMMARY: When we are presented with a history of nasal obstruction or epistaxis, the differential diagnosis should always include a mass lesion in the nasal cavity. Among the mass lesions, though not so common, esthesioneuroblastoma should always be ruled out. Early diagnosis and early intervention may delay or arrest the progression of the tumor.
Human ; Female ; Adult (a Person 19-44 Years Of Age) ; Humans ; Female ; Adult ; Esthesioneuroblastoma, Olfactory ; Nasal Cavity ; Nasal Obstruction ; Vincristine ; Epistaxis ; Survival Rate ; Carcinoma, Adenoid Cystic ; Adenoids ; Ethmoid Bone ; Diagnosis, Differential ; Early Intervention (education) ; Lost To Follow-up ; Magnetic Resonance Imaging ; Prognosis ; Cyclophosphamide ; Brain ; Carcinoma, Squamous Cell ; Frontal Lobe ; Clinical Protocols ; Early Diagnosis ; Craniotomy ; Surgery, Oral ; Writing ; Seizures
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