Primary orbito-cranial adenoid cystic carcinoma with torcular metastasis: a case report and review of the literature.

Adenoid cystic carcinoma is a rare neoplasm arising from the exocrine glands such as salivary glands, lachrymal glands, upper respiratory tract, breast or uterine cervix. Intracranial involvement is commonly from direct skull base involvement, although metastasis may rarely be seen. The predisposition of the adenoid cystic carcinoma for perineural and perivascular invasion is the prime reason for the locally invasive character of the tumour, often extending into the cranium via foramina at the skull base. The authors report a case of primary orbito-cranial extradural adenoid cystic carcinoma and cranial metastasis away from the primary site. This patient initially had a local excision, and later an exenteration of the right eye followed by with radiotherapy. Within months she presented with an extradural cranial recurrence, distant torcular metastasis without any neurological deficit. Craniotomy and radical excision was undertaken as these tumours have slow growth rates, and long term survival of the se patients even in the presence of local recurrence and metastasis has been well documented. Copyright 2001 Harcourt Publishers Ltd.