BILATERAL WILMS TUMOR AND INHERITANCE, PURPOSE OF A CASE

TUMOR DE WILMS BILATERAL Y HERENCIA, A PROPÓSITO DE UN CASO

Abstract

Pediatric cancer is considered rare and accounts for approximately 2% of all cancers. Wilms tumor accounts for approximately 6% of all pediatric cancers and in turn is the most common malignant renal tumor in childhood. Most are diagnosed before 5 years of age, with an average age at the time of diagnosis between 3 and 5 years, it is not common in those over 7 years and is rare in adults.

More than 90% are unilateral, 2% have synchronous multifocal lesions and 7% are bilateral. When this entity affects both kidneys simultaneously they are called synchronous, and when it occurs in the contralateral organ some time after the initial diagnosis, it is called metachronic. This malignant neoplasm is formed at the expense of embryonic kidney debris. It originates from the primitive metanephric blastema, precursor of normal renal tissue, although histologically they may contain different tissue remains, such as skeletal muscle, cartilage and squamous epithelium.

The familiar presentation of Wilms' Tumor as a single entity is rare. Only 2% of cases have an affected relative and the predisposition to the tumor is segregated as an autosomal dominant trait. However, there are several syndromes associated with a predisposition to develop this tumor. Among them are the WAGR syndromes (Wilms tumor, Aniridia, genitourinary abnormalities and mental retardation), Denys-Drash (DDS), Beckwith-Wiedeman (BWS), Frasier, Simpson-Golabi-Behmel (SGBS) and Wilms family tumor.