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ABSTRACT
This is case where an elderly woman referred to our clinic with history of recurrent epistaxis from right nostril for one year duration associated with persistent right nasal blockage, hyposmia, loss of weight and loss of appetite.
On examination noted polypoidal mass at right sphenoethmoidal recess with evidence of previous surgery. Patient underwent endoscopic removal of the mass and histopathologic examination was reported as malignant small round blue cells tumors from right olfactory cleft and right ethmoidal air cells. Based on immunohistochemical examination, it was consistent with primitive neuroectodermal tumour (PNET).
Key Words: Primitive neuroectodermal tumor, PNET, nasal cavity, small round cell tumor
Introduction
Primitive neuroectodermal tumors (PNETs) are highly malignant small round cell tumors of neuroectodermal origin which affect soft tissues and bone. Their clinical manifestation exhibit great diversity and pathologic similarities with other small round cell tumors. Betsakis et al. (1) in 1996 divided these tumors in three group based on tissue of origin which is
- Central nervous system (CNS) PNETs which derived from CNS.
- Neuroblastoma which derived from autonomic nervous system (ANS)
- Peripheral PNETs derived from tissues outside the CNS and ANS
The incidence of PNETs in the head and neck region is around 2-70% where mandible and skull base are the two most common site. Its incidence in sinonasal region can be considered as rare (2,3) and very limited cases been reported due to its rareness.
In this case report we discuss a case of PNET originating from olfactory cleft in the nasal cavity.
Case report
A 72 years old lady with background history of Diabetes mellitus and hypertension complained of recurrent epistaxis from right nostril for one year duration, which occured on blowing her nose. Each time the epistaxis was moderate amount and stopped spontaneously. The above symptoms were associated with persistent right nasal blockage, hyposmia, loss of weight and loss of appetite. However she denied other nasal symptoms including facial pain or swelling, blurring of vision or diplopia, headache or ear symptoms.
She initially presented elsewhere and was noted to have multiple polypoidal mass in the right nasal cavity involving right maxillary sinus, middle meatus and ethmoids. The mass was noted to be arising from right posterior superior nasal septum. She underwent endoscopic endonasal...