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Perilobar nephroblastomatosis: natural history and management.

Stabouli S, Printza N, Dotis J, Matis A, Koliouskas D, Gombakis N, Papachristou F - Case Rep Pediatr (2014)

Bottom Line: Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established.Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment.We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.

View Article: PubMed Central - PubMed

Affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.

ABSTRACT
Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established. Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment. We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.

No MeSH data available.


Related in: MedlinePlus

(a) Abdominal ultrasound showing an enlarged right kidney with a large hypoechoic region with no corticomedullary differentiation before chemotherapy treatment; (b) decrease of right kidney large hypoechoic lesion dimensions after 4 months of treatment.
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fig2: (a) Abdominal ultrasound showing an enlarged right kidney with a large hypoechoic region with no corticomedullary differentiation before chemotherapy treatment; (b) decrease of right kidney large hypoechoic lesion dimensions after 4 months of treatment.

Mentions: Some years ago we presented the case of a 23-month-old boy, who did not received any treatment for the initial diagnosis of right NB and developed WTs 24 and 42 months later at the left and the right kidney, respectively, despite regression of initial lesions of NB [3]. Review of the literature on the management of NB revealed one large retrospective study and several case reports describing in most cases adverse outcome in nontreated patients. Thus, our female patient initiated chemotherapy according to SIOP Wilms Tumor/2001 protocol and received vincristine and actinomycin D for 4 weeks. Abdominal ultrasonography at 4 weeks showed decrease of lesion's size (shrinkage of the large right kidney mass volume from 7,56 cm3 to 3,26 cm3) and the patient received further cycles of vincristine and actinomycin D every 14 days for the next 3 months. Follow-up ultrasound at 4 months of treatment showed additional decrease of lesions dimensions (Figure 2). However, the follow-up period is currently too short to allow us to determine the response to treatment with confidence.


Perilobar nephroblastomatosis: natural history and management.

Stabouli S, Printza N, Dotis J, Matis A, Koliouskas D, Gombakis N, Papachristou F - Case Rep Pediatr (2014)

(a) Abdominal ultrasound showing an enlarged right kidney with a large hypoechoic region with no corticomedullary differentiation before chemotherapy treatment; (b) decrease of right kidney large hypoechoic lesion dimensions after 4 months of treatment.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4120796&req=5

fig2: (a) Abdominal ultrasound showing an enlarged right kidney with a large hypoechoic region with no corticomedullary differentiation before chemotherapy treatment; (b) decrease of right kidney large hypoechoic lesion dimensions after 4 months of treatment.
Mentions: Some years ago we presented the case of a 23-month-old boy, who did not received any treatment for the initial diagnosis of right NB and developed WTs 24 and 42 months later at the left and the right kidney, respectively, despite regression of initial lesions of NB [3]. Review of the literature on the management of NB revealed one large retrospective study and several case reports describing in most cases adverse outcome in nontreated patients. Thus, our female patient initiated chemotherapy according to SIOP Wilms Tumor/2001 protocol and received vincristine and actinomycin D for 4 weeks. Abdominal ultrasonography at 4 weeks showed decrease of lesion's size (shrinkage of the large right kidney mass volume from 7,56 cm3 to 3,26 cm3) and the patient received further cycles of vincristine and actinomycin D every 14 days for the next 3 months. Follow-up ultrasound at 4 months of treatment showed additional decrease of lesions dimensions (Figure 2). However, the follow-up period is currently too short to allow us to determine the response to treatment with confidence.

Bottom Line: Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established.Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment.We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.

View Article: PubMed Central - PubMed

Affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.

ABSTRACT
Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established. Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment. We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.

No MeSH data available.


Related in: MedlinePlus