M Saleem Marfani, Shabbir Kanjee, Azam H Yousifani.
Olfactory Neuroblastoma A Case Report.
J Surg Pak Jan ;6(3):40-1.

A case of a 8 year old female who presented with a polipoidal mass protruding from the nose and oral cavity and a malar swelling is reported. The patient had experienced nose bleeds for several months. The diagnosis of olfactory neuroblastoma was established on histopathology, which revealed presence of small round cells forming pseudo-rosette. The patient was treated by surgical resection followed by radiotherapy and is well on the 12th month followup.

An 8 year old female patient from Hyderabad Sindh, presented with a painless massive swelling involving the left side of face and a polypoidal mass protruding from the nose and oral cavity On examination a friable red fungating mass was seen protruding from the left nostril and the oral cavity. The surface of the tumor was necrotic and covered with blood clots. There was a foul smelling bloody discharge from the nasal cavity. The swelling or the left cheek measured 10x8 cms involving the maxilla. It was soft in consistency, non tender and fluctuant. A similar swelling measuring 5x5 cms was seen on the left zygomatic region. Regional lymph nodes were not enlarged. There was ocular hypertelorism but ocular movements were not restricted. Investigations carried out included complete blood counts and urinalysis which were normal, expect for the haemoglobin level of 5gm. Liver and renal function tests were within normal limits. Chest radiograph (abdomen) were normal. CT scan showed heterogenous enhancing mass with multiple cystic areas involving left side of the face causing extensive bony destruction with extension into the paranasal sinuses, oral and nasal cavities. The tumor mass was extending into the nasopharynx and pushing the orbital contents of the left side Biopsy was consistent with olfactory neuroblastoma showing round to oval cells with hyperchromatic nuclei and scanty cytoplasm separated by highly vascularized fibrous tissue. Pseudorosette formation was also seen. The patient was operated in two stages, in the first stage wide surgical excision was performed through a lip splitting Weber Ferguson incision and the growth was excised from maxilla, infraorbital region and the nasal cavity. The growth from the zygomatic area was removed after 4 weeks. The patient was advised radiothereapy two weeks after surgery. An obturator was placed over the palatal defect which was produced by the removal of the hard palate. The patient was followed regularly at two weekly intervals. At 12 months post surgical followup the patient was free of local recurrence and no sign of distant metastasis.

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