Olfactory Neuroblastoma

Pv of uniform, small, undifferentiated, primitive neuro-ectodermal cells (Fig. 2.13a) [149]. The great majority of these tumours will react strongly with antibodies against the MIC-2 (CD99) protein product (Fig. 2.13b). This marker is of considerable value, but it is by no means specific. A growing number of other neoplasms expressing this protein have been documented. Among these are T-cell lymphomas [263]. The standard translocation t(11; 22) (q24; q12) of PNET [260] results in the fusion of the EWS-FLI1 genes. The detection of the chimeric transcript by techniques of molecular biology confirms the diagnosis [238]. We have seen one example of PNET arising from the maxillary antrum [39], which ultrastructurally showed rudimentary neuritic differentiation, as well as scanty microtubule formation. This raised the differential diagnostic dilemma of "ectopic olfactory neuroblastoma"; nevertheless, the tumour cells were positive for MIC-2 and showed the t(11;22)(q24;q12) translocation, findings that are characteristically negative in ONB [13].

Fig. 2.12. a Olfactory neuroblastoma: polypoid mass of fleshy consistency and pink colour. b Well-differentiated olfactory neuroblastoma: nests of small round neuroblasts embedded in a neurofibrillary matrix are surrounded by delicate networks of capillary blood vessels

metastases account for the rest [70]. Distant metastases mainly involve bone and lung [68]. Complete surgical excision, often followed by radiation therapy and/or chemotherapy, seems to be the treatment of choice [14, 179]. In advanced ONB, high-dose chemotherapy and autologous bone marrow transplantation has been used [68, 188].

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