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Spiradenoma is a benign dermal tumour
occuring in adults.
This lesion often coexists with
cylindromas.
Clinical presentation:
Usually presents as a
painful or tender solitary nodule (less than 1cm in diameter).
May
occasionally present as multiple lesions .
Giant variants, linear
or zosteriform distribution have also been reported.
Site:
Head & neck, sometimes on the scalp and on the trunk. Rarely on
the extremities.
Microscopic features:
Tumour consists of sharply demarcated lobules located
in the dermis without connection to the epidermis ; Eosinophilic
PAS positive basement membrane-like material may be present around the
lobules ;
Two cell types are
present ; Small cells with dark nuclei are present at the periphery
and the large paler cells are at the centre ;
Cells are arranged
around small lumina ; Lumina contains small amounts of granular
eosinophilic material that is PAS- positive and diastase resistant ;
Numerous small
lymphocytes are present throughout the tumour lobules.
Tumour may show prominent degenerative changes, fibrosis and cyst
formation ; Ancient changes (dilated vessels rimmed by sclerosis) ;
Larger tumours
are partially or totally necrotic ;
Immunohistochemistry:
Strands of cells are cytokeratin positive and lumina are CEA positive.
Malignant transformation of spiradenoma (Spiradenocarcinoma)
may occur in rare cases.
Brooke-Spiegler
syndrome is a rare, autosomally dominant disease characterized by the
development of multiple cylindromas, trichoepitheliomas, and occasional
spiradenomas. |