title: Peripheral primitive neuroectodermal tumour of the left maxillary sinus.
abstract: Peripheral primitive neuroectodermal tumours (pPNETs) are highly malignant, small-cell neoplasms found mainly in children and young adults. Recent advances in immunohistochemistry and genetic typing have led to reports of a close relationship between pPNET and the previously difficult-to-classify Ewing's sarcoma. We report a case of pPNET involving the left maxillary sinus in a 23-year-old female who presented with a 2-month history of unilateral left-sided nasal obstruction, rhinorrhoea and recurrent bloody nasal discharge. A CT scan of the paranasal sinuses showed a large mass (10 x 7 x 3 cm3) arising from the left maxillary sinus, with signs of bone destruction and invasion of the left orbital floor and pterygomaxillary fossa. MRI revealed a heterogeneous hyperintense signal on a T2-weighted image in the left maxillary sinus. The tumour was surgically removed by means of external lateral rhinotomy. Pathological examination showed a sheet of small cells with irregular nuclei. Immunohistochemical studies demonstrated positive immunoreactivity for neurone-specific enolase, synaptophysin, chromogranin, vimentin, S-100 protein and p30-32 MIC-2 gene product. The patient was treated with chemotherapy consisting of cyclophosphamide, vincristine, adriamycin and actinomycin D, together with radiotherapy to a total tumour dose of 60 Gy. After 59 months of follow-up, the patient remained free of disease and a repeat MRI scan was normalized, with no sign of residual tumour.
authors.affiliation: Rhinology Unit, Department of Otorhinolaryngology, Hospital Clinic, Barcelona, Spain. 32874iao@comb.es
authors.forename: Isam
authors.initials: I
authors.lastname: Alobid
authors.forename: Manuel
authors.initials: M
authors.lastname: Bernal-Sprekelsen
authors.forename: Llucia
authors.initials: L
authors.lastname: Alós
authors.forename: Pedro
authors.initials: P
authors.lastname: Benítez
authors.forename: Jose
authors.initials: J
authors.lastname: Traserra
authors.forename: Joaquim
authors.initials: J
authors.lastname: Mullol
mesh_terms: D000328:Adult; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008444:Maxillary Sinus Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000628:therapy*; D013517:Otorhinolaryngologic Surgical Procedures / Q000379:methods; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome