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Is Wilms Tumor Congenital?
The exact cause or pathogenesis of Wilms ’ neoplasm ( WT ) is not bang clearly , but it can be due to genetic and non - genetic crusade . Wilms ’ tumor develop from embryologic kidney cell which have not developed simultaneously with the other embryonic cells . These cells tend to have some connection with the course of the disease .
Studies have not found any tie-up between Wilms ’ tumor and environmental factor exposed during pregnancy or after delivery . Most type of Wilms ’ neoplasm are sporadic . 1 - 2 % of tumors are genetic and there is a family history of a unaired congeneric diagnosed with Wilms ’ tumour .
12 - 15 % of cases or 1 in 10 with Wilms ’ tumor are associated with congenital nascency defect and most of these birth defects are associated with a syndrome .
Wilms ’ neoplasm is linked up with some birth defects which do not admit into a syndrome such as :
Aniridia – complete or fond departure of the iris of the eye
Hemihypertrophy – outsize branch or arm either on one side of the body
cryptorchism – failure of the testicles to come down to the scrotum
Hypospadias – the urethral opening is on the underside of the penis.(1 )
The common syndromes Wilms ’ tumor is associated are :
This association between Wilms ’ neoplasm and birth flaw and inborn syndromes suggests it can be a congenital disease . The unnatural cells in the kidney might be present from birth and these cubicle develop and procreate which leads to the manifestation of symptom and diagnosing of Wilms ’ tumor .
There have been few case reports of Wilms ’ tumour diagnose during the maternity menses . One caseful news report was on a 20 - year - former woman who presented at 28 weeks with a fetal intraabdominal tumour . There was fetal hydrops fetalis , well - capsulated mass in the remaining upper abdomen and the left kidney was not capable to identify in the scan . Then a transdermal ultrasound - guided biopsy was perform , after the procedure , the shape of the fetus was not unchanging therefore , an emergency cesarean section was perform but unluckily , the fetus was deadened at birth . The post-mortem examination examination revealed a left kidney replace by an capsule neoplasm , bicornuate womb , and biventricular hypertrophy was present in the heart . There were not any dysmorphic feature article and the other organs were normal . The histological test confirmed a inborn Wilms ’ tumor.(2 ) ( 3 )
Congenital Wilms ’ neoplasm is associated with polyhydramnios ( increase amniotic fluid levels ) and hydrops fetalis . It is rather difficult to identify the tumor through anultrasound scanas it extend with the kidney . Usually , congenital Wilms ’ tumor is capsulize but it is not well - delineate and it combine in with the continue normal kidney .
This demo that Wilms ’ tumor can be inborn but there are not many case reports or studies involved in fetuses with congenital Wilms ’ tumor . It might be due to the want of diagnosing during the pregnancy period because the tumor is pocket-sized and it will not be discover by an echography scan . The fetus that develop large Wilms ’ tumors will get dropsy fetalis and polyhydramnios therefore , these foetus will be notice but most of these foetus will not dwell after birth.(3 ) ( 4 )
Conclusion
The precise cause of Wilms tumor is not known understandably but it is associated with some of the birth defects such as aniridia , hemihypertrophy , cryptorchidism , hypospadias ; and with congenital syndromes such as WAGR syndrome , Denys - Drash syndrome , Frasier syndrome , and Beckwith - Wiedemann syndrome . Therefore , Wilms ’ tumor can be a congenital circumstance . Although Wilms ’ tumor commonly pass in children mature 3 - 7 year it might be present from birth but the symptoms evidence later in life . This is support further by the sheath reports published on meaning women with fetuses name with congenital Wilms ’ tumor .
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