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Turban Tumor (Cylindroma) and Brooke-Spiegler syndrome
Dr Sampurna Roy MD May 2016
A Blue Turban with Pink border
A patient with a turban-like tumour (cylindromas) was first reported by Ancell.
The clinicopathological features of cylindroma were described in detail by Spiegler and Brooke.
Multiple cylindromas are inherited in an autosomal dominant manner.
At an advanced stage cylindromas present as multiple potato-like tumours (numerous pink, red, or occasionally bluish nodules) on the head and neck.
When they coalesce on the scalp, they resemble a turban,and are referred to as a "turban tumor". The term "turban tumour" is used to describe the location of the tumour and its enormous size. It can cover the whole scalp causing severe disfigurement.
Visit: Pathology of Cylindroma
Brooke-Spiegler syndrome is a rare autosomal dominant disorder described first by Spiegler and Brooke.
It is characterized by the development of multiple cylindromas, trichoepitheliomas, and occasional spiradenomas.
Visit: Pathology of Spiradenoma
Usually cylindromas occur on the scalp while trichoepitheliomas develop on the face, particulary around the nose.
Other lesions reported in Brooke-Spiegler syndrome include parotid basal cell adenomas, organoid nevi, syringomas, and basal cell carcinomas.
Brooke-Spiegler syndrome as well as sporadic cylindromas have been shown to result from mutations leading to loss of both alleles of the cylindromatosis gene (CYLD1).
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