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Pediatrics: Renal and Adrenal Oncology: Difference between revisions

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=== Congenital Mesoblastic Nephroma ===
=== Congenital Mesoblastic Nephroma ===
* '''Most common renal tumor in infants'''
 
==== Epidemiology ====
*'''<span style="color:#ff0000">Most common renal tumor in infants'''
** Most common renal tumor diagnosed on pre-natal US
** Most common renal tumor diagnosed on pre-natal US
** Most common tumor in infants > 4 months of age
** Most common tumor in infants > 4 months of age
* '''Mean age at diagnosis of 3.5 months (in contrast, the median age for diagnosis of a Wilms' tumor is 3.5 years)'''
* '''Mean age at diagnosis of 3.5 months (in contrast, the median age for diagnosis of a Wilms' tumor is 3.5 years)'''
* '''Typically infiltrative on imaging (whereas Wilms' tumors displace and compress renal architecture)'''
* '''Subtypes (2)'''
*# '''Classic type'''
*#* Far more common
*#* '''Rarely recur,''' provided surgical margins are negative
*# '''Cellular variant'''
*#* Consists of atypical spindle cells with frequent mitotic figures (25-30/10 hpf) and necrosis
*#* Considered a variant of a '''fibrosarcoma'''
*#* Associated with both '''local recurrence and widespread metastasis.'''
* '''Management'''
** '''Radical nephrectomy'''
*** '''Biopsy and partial nephrectomy are not recommended''' since the tumour occasionally extends into the hilum or perirenal soft tissue; complete excision is important to prevent local recurrence.
* Follow-up
** Surveillance by interval 6 month abdominal US for the first 2 years is usually recommended for the classic variant and more aggressive follow-up with interval CT or MRI scans of the lungs and abdomen are recommended at 3-6 intervals for the first two years for the cellular variant


=== Solitary Multilocular Cyst and Cystic Partially Differentiated Nephroblastoma ===
==== Subtypes (2) ====
* Solitary multilocular cyst, or multilocular cystic nephroma, is an uncommon, benign renal tumor
# '''Classic type'''
#* Far more common
#* '''Rarely recur,''' provided surgical margins are negative
# '''Cellular variant'''
#* Consists of atypical spindle cells with frequent mitotic figures (25-30/10 hpf) and necrosis
#* Considered a variant of a '''fibrosarcoma'''
#* Associated with both '''local recurrence and widespread metastasis.'''
 
==== Diagnosis and Evaluation ====
*'''Imaging'''
**'''Typically infiltrative (whereas Wilms' tumors displace and compress renal architecture)'''
 
==== Management ====
* '''<span style="color:#ff0000">Radical nephrectomy'''
** '''Biopsy and partial nephrectomy are not recommended''' since the tumour occasionally extends into the hilum or perirenal soft tissue; complete excision is important to prevent local recurrence.


==== Follow-up ====
* Surveillance by interval 6 month abdominal US for the first 2 years is usually recommended for the classic variant and more aggressive follow-up with interval CT or MRI scans of the lungs and abdomen are recommended at 3-6 intervals for the first two years for the cellular variant
=== Renal Cell Carcinoma ===
=== Renal Cell Carcinoma ===
* '''RCC is the most likely diagnosis in a child age >12 with a renal mass'''
* '''RCC is the most likely diagnosis in a child age >12 with a renal mass'''
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=== Metanephric Adenofibroma ===
=== Metanephric Adenofibroma ===
=== Solitary Multilocular Cyst and Cystic Partially Differentiated Nephroblastoma ===
* Solitary multilocular cyst, or multilocular cystic nephroma, is an uncommon, benign renal tumor


== References ==
== References ==
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