Turban tumor syndrome – a case report

Authors

  • Sofia Granito Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal
  • Joana Cortez Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal
  • Karina Gama Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal
  • José Luís Andrade Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal
  • Manuela Lélis Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal
  • Michelle Cordeiro Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal
  • Maria da Luz Brazão Serviços de Medicina Interna e Anatomia Patológica do Hospital Central do Funchal

Keywords:

Familial cylindromatosis, turban tumor, Brooke-Spiegler syndrome

Abstract

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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Nogueira E, Pimenta C, Freitas A, Cruvinel S, Pedrosa C. Cilindromas múltiplos-tumor em turbante- relato de caso. Rev Soc Bras Cir Plást São Paulo 1999; 4(1): 17-22.

Carlson RM et al. Brooke-Spiegler syndrome with associated pegged teeth. Cutis 2008;82(5): 345-349.

Hu G et al. a novel missense mutation in CYLD in a family with Brooke-Spiegler syndrome. J Invest dermatol 2003; 121(4): 732-734.

Stoll C, Alembik Y, Wilk A, Grosshans E. Familial cylindromatosis. Genet Couns 2004;15(2): 175-182.

Parren LJ, Bauer B, Hamm H, Frank J. Brooke-Spiegler syndrome complicated by unilateral hearing loss. Int J Dermatol 2008; 47 Suppl 1: 56-59

Additional Files

Published

2011-09-30

How to Cite

1.
Granito S, Cortez J, Gama K, Andrade JL, Lélis M, Cordeiro M, Brazão M da L. Turban tumor syndrome – a case report. RPMI [Internet]. 2011 Sep. 30 [cited 2025 Jan. 5];18(3):151-4. Available from: https://revista.spmi.pt/index.php/rpmi/article/view/1326

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Case Reports

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