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Dermpath-India

Pathology of Cylindroma           

Dr Sampurna Roy MD        

 

Cylindroma is a benign adnexal tumour.

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.

Clinical presentation: 

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.

Pathology Photo of the Day -Turban Tumor (Cylindroma) and Brooke-Spiegler syndrome

Dermatopathology patterns that remind us of something else 

Hyaline - Value of a descriptive word in General Pathology and Dermatopathology

Microscopic features: 

 

Histologically cylindroma is a circumscribed, non-encapsulated , dermal tumour without attachment to the epidermis.

The lesion is composed of numerous oval and polygonal nests arranged in an interlocking 'jigsaw-like' pattern.

There are two cell types, the peripheral cells are small and basophilic and central cells are larger and pale stained.

Small ductal lumina may be present.

Thick PAS positive hyaline bands surround tumour islands.

Hyaline droplets may be present within the nests.

Differential Diagnosis:

Spiradenoma - Lack of lymphoid tissue in cylindroma and presence of variable number of dendritic Langerhans cells in the tumour. These cells are CD1a positive.

Malignant Cylindroma:

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.

 

Further reading:

Identification of a large rearrangement in CYLD as a cause of familial cylindromatosis.

Expression of p53 and TP53 mutational analysis in malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma, sporadic or associated with Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma and eccrine spiradenoma.

Blaschkoid distribution of cylindromas in a germline CYLD mutation carrier.

Brooke-Spiegler syndrome: report of two cases not associated with a mutation in the CYLD and PTCH tumor-suppressor genes.

Five novel germline function-impairing mutations of CYLD in Italian patients with multiple cylindromas.

Malignant cylindroma of the scalp arising in a setting of multiple cylindromatosis: a case report.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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