Turban tumor syndrome – a case report
Keywords:
Familial cylindromatosis, turban tumor, Brooke-Spiegler syndromeAbstract
Familial cylindromatosis is a rare benign neoplasm with a dominant autosomal transmission, characterized by the existence of
multiple cylindroma spread mainly on the scalp, neck or forehead,
risking disfiguration and underlying malignancy. When the entire
scalp is attained we call it “turban tumor”.
We report a clinical case of a 74 year-old man, attending the
emergency department with a condition compatible with respiratory infection. On physical examination, we detected multiple
tumors on his face, scalp, back and both legs evolving for 30 years.
We referred him to the Internal Medicine to evaluate his clinical
condition. After the Dermatology and Plastic Surgery cooperation,
it was possible to diagnose the existence of multiple cylindroma
tumors. We also managed several other conditions diagnosed
in the visit: arterial hypertension, diabetes mellitus and mixed
dyslipidaemia. The authors would like to emphasize the scope
and impact such disease can have on an individual’s life. Due
to its aspect, he isolated himself, allowing tumors to evolve and
emerging diseases remaining undiagnosed. Although it is a rare
nosological entity, it can become malignant and it deserves, also
for this, our special attention to the patient and the family.
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References
AllixT. et al. Management of whole-scalp tumour: The Poncet-Spiegler cylindroma or turban tumour. Rev Stomatol. Chir Maxilofac 2009; 110 (2): 109-112.
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