Dr Sampurna Roy MD May 2016
|Cylindroma is a benign
The tumour showed apocrine and trichoepitheliomatous differentiation which indicated complex hair follicle (folliculo-sebaceous-apocrine). According to recent studies immunohistochemical examination has shown myoepithelial, apocrine, eccrine, ductal, and secretory features in both cylindromas and spiradenomas.
Brooke-Spiegler syndrome represents an autosomal dominant disease characterized by the occurrence of multiple cylindromas, trichoepitheliomas and (sporadically) spiroadenomas.
Clinically, the tumour usually presents as a solitary, slow growing rubbery lesion.
The multiple form is autosomal dominantly inherited on cyld1 gene on chromosome 16.
This form is also known as 'turban tumour' and may be associated with spiradenoma.
Some of these lesions are painful.
This condition is associated with trichoepitheliomas and milia.
Site: Usually located on the scalp, head and neck or trunk.
Spiradenoma - Lack of lymphoid tissue in cylindroma and presence of variable number of dendritic Langerhans cells in the tumour. These cells are CD1a positive.
Cylindromas occasionally undergo malignant transformation.
Malignant cylindromas are characterised by islands of cells displaying marked nuclear pleomorphism.
There is increase in mitosis and many abnormal forms are identified.
The tumour shows invasion into the surrounding tissue and loss of the delicate hyaline sheath.
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