(1) Department of Periodontology, Service of Odontology, Rothschild Hospital, AP-HP; U.F.R. of Odontology, Paris 7- Denis Diderot University, Paris, France,
(2) Department of Dentomaxillofacial Radiology and Imaging, School of Dentistry, Lebanese University, Lebanon.
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Primary Sinonasal Small Cell Carcinomas, Neuroendocrine type (SCCNET) are rare tumours. Few cases have been reported in the head and neck region of adult patients. They have a fair response to multimodality therapy; but the prognosis remains poor.
We report here a case of SCCNET in a 5 year old child. He presented with swelling in his right eye with diminution of vision and right sided neck swelling. Cervical lymph node biopsy revealed poorly differentiated metastatic neuroendocrine carcinoma; confirmed by immunohistochemistry. 18 FDG- PET CT scan revealed a nasopharyngeal mass extending to other paranasal sinuses and base of frontal lobe with bilateral extensive cervical lymphadenopathies. He was treated with neoadjuvant platinum based combination chemotherapy followed by radical radiotherapy. At 17 months post diagnosis he is alive but on palliative care.
SCCNETs have an unfavourable outcome. Further improvements in our understanding of the pathology and treatment strategies are needed to combat the disease.