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Results: 4

1.

Wilms tumor 1

MedGen UID:
447509
Concept ID:
CN033288
Disease or Syndrome
2.

Wilms tumor 3

Wilms tumor (nephroblastoma), an embryonal malignancy of the kidney, is the most common renal tumor of childhood. Wilms tumor usually presents as an abdominal mass in an otherwise apparently healthy child. Wilms tumor has the potential for both local and distant spread. Approximately 5%-10% of children with Wilms tumor have bilateral or multicentric tumors. The average age at presentation is 42-47 months for children with unilateral Wilms tumor and 30-33 months for those with bilateral Wilms tumor. Current models of Wilms tumor development propose that a genetic mutation predisposes to nephrogenic rests (benign foci of embryonal kidney cells that persist abnormally into postnatal life). Nephrogenic rests are characterized as intralobar or perilobar. In general, intralobar rests (usually single lesions within the renal lobe, renal sinus, or calyceal walls) are associated with the WAGR (Wilms tumor-aniridia-genital anomalies-retardation) syndrome and Denys-Drash syndrome (DDS). In general, perilobar rests (usually multiple lesions in the periphery of the renal lobe) are associated with Beckwith-Wiedemann syndrome (BWS) and hemi-hyperplasia. However, the association between type of nephrogenic rest and predisposition syndrome is not absolute. [from GeneReviews]

MedGen UID:
349770
Concept ID:
C1860265
Disease or Syndrome
3.

Wilms tumor 4

Wilms tumor (nephroblastoma), an embryonal malignancy of the kidney, is the most common renal tumor of childhood. Wilms tumor usually presents as an abdominal mass in an otherwise apparently healthy child. Wilms tumor has the potential for both local and distant spread. Approximately 5%-10% of children with Wilms tumor have bilateral or multicentric tumors. The average age at presentation is 42-47 months for children with unilateral Wilms tumor and 30-33 months for those with bilateral Wilms tumor. Current models of Wilms tumor development propose that a genetic mutation predisposes to nephrogenic rests (benign foci of embryonal kidney cells that persist abnormally into postnatal life). Nephrogenic rests are characterized as intralobar or perilobar. In general, intralobar rests (usually single lesions within the renal lobe, renal sinus, or calyceal walls) are associated with the WAGR (Wilms tumor-aniridia-genital anomalies-retardation) syndrome and Denys-Drash syndrome (DDS). In general, perilobar rests (usually multiple lesions in the periphery of the renal lobe) are associated with Beckwith-Wiedemann syndrome (BWS) and hemi-hyperplasia. However, the association between type of nephrogenic rest and predisposition syndrome is not absolute. [from GeneReviews]

MedGen UID:
318623
Concept ID:
C1832426
Neoplastic Process
4.

Wilms tumor 2

Wilms tumor (nephroblastoma), an embryonal malignancy of the kidney, is the most common renal tumor of childhood. Wilms tumor usually presents as an abdominal mass in an otherwise apparently healthy child. Wilms tumor has the potential for both local and distant spread. Approximately 5%-10% of children with Wilms tumor have bilateral or multicentric tumors. The average age at presentation is 42-47 months for children with unilateral Wilms tumor and 30-33 months for those with bilateral Wilms tumor. Current models of Wilms tumor development propose that a genetic mutation predisposes to nephrogenic rests (benign foci of embryonal kidney cells that persist abnormally into postnatal life). Nephrogenic rests are characterized as intralobar or perilobar. In general, intralobar rests (usually single lesions within the renal lobe, renal sinus, or calyceal walls) are associated with the WAGR (Wilms tumor-aniridia-genital anomalies-retardation) syndrome and Denys-Drash syndrome (DDS). In general, perilobar rests (usually multiple lesions in the periphery of the renal lobe) are associated with Beckwith-Wiedemann syndrome (BWS) and hemi-hyperplasia. However, the association between type of nephrogenic rest and predisposition syndrome is not absolute. [from GeneReviews]

MedGen UID:
151968
Concept ID:
C0694899

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