Tags

Type your tag names separated by a space and hit enter

Peripheral primitive neuroectodermal tumour of the left maxillary sinus.
Acta Otolaryngol. 2003 Aug; 123(6):776-8.AO

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+Show Affiliations

Rhinology Unit, Department of Otorhinolaryngology, Hospital Clinic, Barcelona, Spain. 32874iao@comb.esNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

12953782

Citation

Alobid, Isam, et al. "Peripheral Primitive Neuroectodermal Tumour of the Left Maxillary Sinus." Acta Oto-laryngologica, vol. 123, no. 6, 2003, pp. 776-8.
Alobid I, Bernal-Sprekelsen M, Alós L, et al. Peripheral primitive neuroectodermal tumour of the left maxillary sinus. Acta Otolaryngol. 2003;123(6):776-8.
Alobid, I., Bernal-Sprekelsen, M., Alós, L., Benítez, P., Traserra, J., & Mullol, J. (2003). Peripheral primitive neuroectodermal tumour of the left maxillary sinus. Acta Oto-laryngologica, 123(6), 776-8.
Alobid I, et al. Peripheral Primitive Neuroectodermal Tumour of the Left Maxillary Sinus. Acta Otolaryngol. 2003;123(6):776-8. PubMed PMID: 12953782.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral primitive neuroectodermal tumour of the left maxillary sinus. AU - Alobid,Isam, AU - Bernal-Sprekelsen,Manuel, AU - Alós,Llucia, AU - Benítez,Pedro, AU - Traserra,Jose, AU - Mullol,Joaquim, PY - 2003/9/5/pubmed PY - 2003/10/29/medline PY - 2003/9/5/entrez SP - 776 EP - 8 JF - Acta oto-laryngologica JO - Acta Otolaryngol VL - 123 IS - 6 N2 - 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. SN - 0001-6489 UR - https://www.unboundmedicine.com/medline/citation/12953782/Peripheral_primitive_neuroectodermal_tumour_of_the_left_maxillary_sinus_ L2 - https://www.tandfonline.com/doi/full/10.1080/00016480310001213 DB - PRIME DP - Unbound Medicine ER -