Primary XXXX of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report.

@entity1 @entity2828 @entity227 @entity5644 @entity2940 @entity5473 @entity5 @entity1415 @entity189 @entity3778 @entity3508 @entity2277 @entity10
UNASSIGNED: @entity2828 ( @entity2828 ) is an aggressive @entity5 that may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is the case report of a @entity2828 of the ethmoid sinus with intracranial and orbital extension in a 33-year-old male @entity1 who presented with anosmia, @entity1415 , @entity227 and @entity10 . On otorhinolaryngological clinical examination and biopsy via flexible endoscope, the lesion was misdiagnosed as ethmoid @entity189 . The subsequent magnetic resonance imaging (MRI) of the brain revealed a large mass (6*7 cm) eroding the ethmoid and sphenoid sinuses, extending beyond the orbits and occupying the anterior cranial fossa with a maximum extension of ~5 cm. The @entity1 underwent surgical resection and the microscopic examination of the specimen established the diagnosis of @entity2828 (immunohistochemically positive for @entity3778 , @entity3508 , @entity2940 , @entity5473 , pancytokeratin, low-molecular weight cytokeratins and @entity2277 . The @entity5644 Schiff stain exhibited strong intracytoplasmic block positivity and fluorescence in situ hybridization revealed a t(22;11) translocation. First-line chemotherapy was administered for 3 cycles; however, on restaging MRI, local disease progression was diagnosed. The @entity1 received radiotherapy and second-line chemotherapy for 6 cycles. At 15 months after the diagnosis, the @entity1 remains recurrence-free and maintains a good functional status and quality of life.
