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Peripheral primitive neuroectodermal tumour of the left maxillary sinus.
Isam Alobid1, Manuel Bernal-Sprekelsen, Llucia Alós
1Rhinology Unit, Department of Otorhinolaryngology, Hospital Clinic, Barcelona, Spain. 32874iao@comb.es
Acta Oto-Laryngologica
|September 5, 2003
Summary
Peripheral primitive neuroectodermal tumors (pPNETs) are aggressive small-cell cancers. This case highlights successful treatment of a maxillary sinus pPNET in a young adult, achieving long-term remission.
Area of Science:
- Oncology
- Pathology
- Radiology
Background:
- Peripheral primitive neuroectodermal tumors (pPNETs) are rare, highly malignant small-cell neoplasms primarily affecting children and young adults.
- Recent research suggests a close relationship between pPNET and Ewing's sarcoma, aiding classification.
- Maxillary sinus involvement by pPNET is uncommon, presenting diagnostic and therapeutic challenges.
Observation:
- A 23-year-old female presented with nasal obstruction, rhinorrhoea, and bloody discharge due to a left maxillary sinus mass.
- CT and MRI revealed a large destructive mass invading orbital and pterygomaxillary regions.
- Histopathology confirmed a small-cell neoplasm with characteristic immunohistochemical markers.
Findings:
- Surgical resection via external lateral rhinotomy successfully removed the maxillary sinus pPNET.
- Immunohistochemistry confirmed the diagnosis with positive markers including NSE, synaptophysin, chromogranin, vimentin, S-100, and MIC-2.
- Multimodal treatment including chemotherapy (cyclophosphamide, vincristine, adriamycin, actinomycin D) and radiotherapy (60 Gy) was administered.
Implications:
- This case demonstrates the feasibility of aggressive surgical and adjuvant therapy for maxillary sinus pPNET.
- Long-term disease-free survival (59 months) was achieved, indicating potential for cure with combined modality treatment.
- Accurate diagnosis through immunohistochemistry is crucial for appropriate management of these rare tumors.