Wilm’s tumour may be associated with all except –
a)Genitourinary anomalies
b)Beckwith’s syndrome
c)Aniridia
d)Glaucoma
a)Genitourinary anomalies
b)Beckwith’s syndrome
c)Aniridia
d)Glaucoma
Ans. is ‘d’ i.e., Glaucoma [Ref Patho Robins 7e p. 504, 505 & 6e p. 488 ]
Wilms’ tumor is the most common primary renal tumor of childhood, usually diagnosed between the ages of 2 and 5 years.
Neuroblastoma is one of the most common childhood solid tumors and is the most common tumor diagnosed in infants less than 1 year of age
Neoplasms that exhibit sharp peaks in incidence in children younger than 10 years of age include (1) leukemia (principally acute lymphoblastic leukemia); (2) neuroblastoma; (3) Wilms’ tumor; (4) hepatoblastoma; (5) retinoblastoma; (6) rhabdomyosarcoma; (7) teratoma; (8) Ewing sarcoma; and, finally, posterior fossa neoplasms–principally (9) juvenile astrocytoma, (10) medulloblastoma, and (11) ependymoma
Hemangiomas: (benign) are the most common tumors of infancy.
WILMS TUMOUR—
The risk of Wilms’ tumor is increased in association with at least three recognizable groups of congenital malformations exhibiting aberrations in at least two distinct chromosomal loci.
1.The first group of patients have the WAGR syndrome characterized by aniridia, genital anomalies, and mental retardation and have a 33% chance of developing Wilms’ tumor.
2.A second group of patients at risk for Wilms’ tumor have the Denys-Drash syndrome, which is characterized by gonadal dysgenesis (male pseudohermaphroditism) and nephropathy leading to renal failure. The majority of these patients develop Wilms’ tumors.
3.Clinically distinct from these previous two groups of patients but also having an increased risk of developing Wilms’ tumor are those children withBeckwith-Wiedemann syndrome, characterized by enlargement of body organs, hemihypertrophy, renal medullary cysts, and abnormal large cells in adrenal cortex (adrenal cytomegaly).
The risk of Wilms’ tumor is increased in association with at least three recognizable groups of congenital malformations exhibiting aberrations in at least two distinct chromosomal loci.
1.The first group of patients have the WAGR syndrome characterized by aniridia, genital anomalies, and mental retardation and have a 33% chance of developing Wilms’ tumor.
2.A second group of patients at risk for Wilms’ tumor have the Denys-Drash syndrome, which is characterized by gonadal dysgenesis (male pseudohermaphroditism) and nephropathy leading to renal failure. The majority of these patients develop Wilms’ tumors.
3.Clinically distinct from these previous two groups of patients but also having an increased risk of developing Wilms’ tumor are those children withBeckwith-Wiedemann syndrome, characterized by enlargement of body organs, hemihypertrophy, renal medullary cysts, and abnormal large cells in adrenal cortex (adrenal cytomegaly).
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