Você conhece esta Síndrome?

Abstract

A síndrome de Brooke-Spiegler é uma doença autossômica dominante, caracterizada pelo aparecimento de neoplasias de anexos cutâneos, habitualmente tricoepiteliomas e cilindromas. Ocorre, em geral, na segunda e terceira décadas de vida. A histopatologia revela uma ampla gama de tumores, com diferenciação écrina, apócrina, folicular e sebácea. O tratamento pode ser feito por excisão cirúrgica, laser, crioterapia, eletrofulguração e dermabrasão. Em razão do risco de malignidade, há necessidade de um bom acompanhamento clínico e aconselhamento genético.

Cylindroma: an update

“…Cylindroma occurs typically in a sporadic form in mid-age and elderly patients, while multiple tumors are found in hereditary disorders. 23 There are reports of malignant transformation of dermal cylindromas and the possibility of metastases to the lymph nodes, thyroid, liver, lungs, and bones. 22 Despite growth being slow, compressive and cosmetic symptoms are remarkable.…”

“…In those patients, the presentation occurs early and progressive growth occurs during the lifespan requiring repeated surgical interventions. 23 They may even infiltrate the skull, causing hemorrhage and meningitis. The nodules can coalesce on the scalp resulting in a turban-like aspect.…”

“…1 Association with this syndrome and adenoma and carcinoma of the parotids, sebaceous nevus, basocellular carcinomas, milium, xeroderma pigmentosus, hypo-and hyperchromia, polycystosis of the lungs, kidneys, breast, and multiple fibromas were identified. 23 There are reports of malignant transformation of dermal cylindromas and the possibility of metastases to the lymph nodes, thyroid, liver, lungs, and bones. 23 They may even infiltrate the skull, causing hemorrhage and meningitis.…”

“…23 There are reports of malignant transformation of dermal cylindromas and the possibility of metastases to the lymph nodes, thyroid, liver, lungs, and bones. 23 They may even infiltrate the skull, causing hemorrhage and meningitis. Therefore, a systemic follow-up of these patients is important.…”