Manifestações extracolônicas da polipose adenomatosa familiar: incidência e impacto na evolução da doença

Campos, Fábio Angioluci Diniz; Habr‐Gama, Angelita; Kiss, Desidério Roberto; Atui, Fabio Cesar; Katayama, Fábio; Gama-Rodrigues, Joaquim

doi:10.1590/s0004-28032003000200006

Cited by 19 publications

(5 citation statements)

References 31 publications

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“…6) they may be the hallmark of Gardner syndrome (GS), a cancer‐prone syndrome associating (benign) cutaneous tumours and visceral carcinomas. This syndrome was described by Gardner in 1953 38 as the association of gastrointestinal polyposis, mandible and skull osteomas and (sub)cutaneous tumours; it is nowadays considered a phenotypic variant of Familial Adenomatous Polyposis (FAP), rather than a truly distinct subtype of the disease, 39–41 and accounts for about 20–40% of patients with FAP 1,42 …”

Section: Hair Follicle Cysts and Gardner Syndrome/familial Adenomatoumentioning

confidence: 99%

“…This syndrome was described by Gardner in 1953 38 as the association of gastrointestinal polyposis, mandible and skull osteomas and (sub)cutaneous tumours; it is nowadays considered a phenotypic variant of Familial Adenomatous Polyposis (FAP), rather than a truly distinct subtype of the disease, [39][40][41] and accounts for about 20-40% of patients with FAP. 1,42 Skin manifestations of GS include: 38,43 • multiple epidermoid, trichilemmal, hybrid or pilomatrical cysts or pilomatricomas, 44 developing on the face, the scalp and the limbs in 25-50% of patients;…”

Section: Hair Follicle Cysts and Gardner Syndrome/familial Adenomatoumentioning

confidence: 99%

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Adnexal tumours of the skin as markers of cancer‐prone syndromes

Kanitakis¹

2010

Acad Dermatol Venereol

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Adnexal tumours of the skin are benign, more rarely malignant, primary skin tumours that originate from, or differentiate towards, hair follicles, sebaceous and sweat glands. Although they are usually encountered as single, sporadic tumours, they may occasionally be multiple, hereditary; in that case, they may herald complex genetic syndromes that comprise visceral cancers. Dermatologists should be aware of these adnexal skin tumours, the diagnosis of which may contribute to an early detection of a cancer-prone syndrome with a potentially lethal outcome. The main tumours falling into this category and their associated syndromes are reviewed here.

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Section: Hair Follicle Cysts and Gardner Syndrome/familial Adenomatoumentioning

confidence: 99%

Section: Hair Follicle Cysts and Gardner Syndrome/familial Adenomatoumentioning

confidence: 99%

Adnexal tumours of the skin as markers of cancer‐prone syndromes

Kanitakis¹

2010

Acad Dermatol Venereol

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“…3 The malignant potential of non-treated patients requires prophylactic colectomy and familiar screening to minimize cancer risk. 4 Main surgical options are represented either by colectomy with ileorectal anastomosis (IRA) or restorative proctocolectomy with ileal pouch-anal anastomosis (RPC). Indications of proctocolectomy and ileostomy (PCI) are reserved for patients with advanced low rectal cancer, sphincter dysfunction, or impossibility to perform RPC (mesenteric desmoid).…”

Section: Introductionmentioning

confidence: 99%

Rectal and Pouch Recurrences After Surgical Treatment for Familial Adenomatous Polyposis

Campos

Imperiale

Seid

et al. 2009

Journal of Gastrointestinal Surgery

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(1) Within the present series, FAP patients presented a high incidence of associated CRC and diagnosis was generally established after the third decade of life; (2) operative complications occurred in about one third of the patients, being more frequent after the confection of an ileal reservoir; (3) rectal cancer after IRA was detected in 16.6% of patients and it was associated with greater age and previous colonic carcinoma; (4) both continuous and long-term surveillance of the rectal stump and ileal pouch are necessary during follow-up.

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“…Em aproximadamente 20% dos casos, a doença pode ser decorrente de mutações genéticas. O tratamento consiste em remoção das manifestações clínicas apresentadas pela síndrome, como remoção dos osteomas e remoção total do intestino preventivamente 6 . A síndrome de Cowden é uma enfermidade autossômica dominante, sendo que a mutação ocorre no gene PTEN (fosfatase e tensina homólo-gas deletadas no cromossomo 10) 7 .…”

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Síndromes Paraneoplásicas – O que o Cirurgião-Dentista Precisa Saber?

Silva¹,

Crivelini²,

Moraes³

et al. 2010

Odonto

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25Resumo Introdução: o termo paraneoplasia é um conjunto de distúrbios clínicos, bioquímicos, hormonais, neurológicos e/ou hematológicos associados com neoplasias malignas, mas não diretamente relacionados com invasão tumoral primária ou metástase. As síndromes paraneoplásicas podem ser o primeiro sinal de uma malignidade.Revisão de literatura: as síndromes que estão mais comumente relacionadas com a prática odontológica são: de Lambertt-Eaton, de Gardner, de Cowden, de Peutz-Jeghers, de Sjögren, a neoplasia endócrina múltipla, a neurofibromatose múltipla de Von Recklinghausen, o carcinoma nevóide de células basais, a acantose nigrans e o pênfigo paraneoplásico.Conclusão: o diagnóstico precoce das neoplasias malignas favorece o seu prognóstico e as síndromes paraneoplásicas auxiliam neste diagnóstico. É de suma importância que os Cirurgiões-dentistas conheçam tais síndromes de modo a diagnosticá-las o mais rápido possível e encaminhar os pacientes ao tratamento especializado. Palavras-chave: Síndromes Paraneoplásicas; Diagnóstico Precoce; Tratamento.AbstRACt Introduction: the term paraneoplastic is a clinical, biochemical, hormonal, neurological and/or associated disorder with hematologic malignancies, but not directly related to primary tumor invasion and metastasis. Paraneoplatic syndromes may be the first sign of a malignancy.Review of literature: the syndromes that are most commonly related to dentistry are of lambertt-Eaton, Gardner, Cowden disease, Peutz-Jeghers, Sjögren, multiple endocrine neoplasic, multiple neurofibromatosis of Von Recklinghausen, nevoid basal cell carcinoma, acanthosis nigrans and pemphigus paraneoplastic.Conclusion: early diagnosis of malignant neoplasms favors prognosis and paraneoplastic syndromes assist in diagnosis. It is important that surgeons-dentists know these events in order to diagnose them as soon as possible and refer these patients to specialized treatment.

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Manifestações extracolônicas da polipose adenomatosa familiar: incidência e impacto na evolução da doença

Abstract: Extracolonic manifestations incidence is high (40%) and may affect disease's outcome and patient's quality of life. For these reasons, the research, prevention and adequate treatment of extracolonic manifestations turn into vital importance in familial adenomatous polyposis patients.

Cited by 19 publications

References 31 publications

Adnexal tumours of the skin as markers of cancer‐prone syndromes

Adnexal tumours of the skin as markers of cancer‐prone syndromes

Rectal and Pouch Recurrences After Surgical Treatment for Familial Adenomatous Polyposis

Síndromes Paraneoplásicas – O que o Cirurgião-Dentista Precisa Saber?

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