We present two cases of a previously unreported association between occipital meningocele and Mondini deformity of the cochlea. The probability of the association is supported by the intimate relationship between the development of these structures; the existence of dysembryoplastic factors that can cause both abnormalities; and the universal existence of cochlear abnormalities in anencephalic foetuses. The importance of considering the association is emphasized, in terms of early identification and referral of children with hearing loss, and avoidance of duplication of investigations.

A 20-year-old woman presented with nasal obstruction and slight epistaxis. The obstructing lesion was excised and microscopy showed a neoplasm composed of comparatively uniform undifferentiated cells forming solid nests. The cytoplasm of the cells was clear but poorly demarcated, partly vacuolated and contained much glycogen. Although widespread in the nasal mucosa, the cells did not penetrate into the underlying bone.

The cells expressed the MIC2 gene (using the CD99 marker). Electron microscopy showed simple cells with a small number of mitochondria, many glycogen particles; there were no neurosecretory granules present.

Early surgical treatment followed by chemo- and radiotherapy have greatly improved the prognosis of EWS: extraskeletal Ewing’s sarcoma (EWS/PNET).

Haemangiopericytomas (HPCs) are rare vascular tumours that commonly involve the soft tissues of the trunk and lower extremities. In the head and neck, the most common sites are the nasal cavity and the paranasal sinuses, and unusually, the orbital region, the parotid gland, and the neck. We report a patient with HPC that originated in the infratemporal fossa and involved the pterygopalatine and the middle cranial fossae, apparently the first such case to be reported. Although the patient has undergone resection on three separate occasions, the tumour recurred. We then performed an extended resection using the infratemporal fossa approach type D. The patient has shown no recurrence in the past five years.

Although histopathologic confirmation of this malignancy may be difficult, extensive resection remains the most effective treatment in such cases.