Oligodontia-cancer predisposition syndrome

Orpha code: 300576OMIM code: 608615

Definition

Oligodontia-cancer predisposition syndrome is a rare, genetic, odontologic disease characterized by congenital absence of six or more permanent teeth (excluding the third molars) in association with an increased risk for malignancies, ranging from gastrointestinal polyposis to early-onset colorectal cancer and/or breast cancer. Ectodermal dysplasia (manifesting with sparse hair and/or eyebrows) may also be associated.

Disease data
Classification

Disease

Synonyms
Autosomal dominant ectodermal dysplasia-cancer predisposition syndrome
Zespół autosomalna dominująca dysplazja ektodermalna - predyspozycja do nowotworów
ORPHA code
300576
OMIM code
608615
ICD10 code
C19
ICD11 code
-

No additional description.

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