UrologySchool.com

Pediatrics: Renal and Adrenal Oncology: Difference between revisions

← Older edit
Visual
 
(22 intermediate revisions by the same user not shown)
Line 1: Line 1:
== Pediatric Abdominal Mass ==
* See [https://www.youtube.com/watch?v=cSsZt8K7Bmo Childhood Renal Tumors Video Lecture] (PedUroFLO 2020)


* '''<span style="color:#ff0000">Differential diagnosis of pediatric abdominal mass (9):'''
== Differential Diagnosis of Pediatric Abdominal Mass (9) ==
*# '''<span style="color:#ff0000">Neuroblastoma'''
 
*#* '''<span style="color:#ff0000">Most common malignant tumour in infants'''
# '''<span style="color:#ff0000">Neuroblastoma'''
*#* '''Most common extracranial solid tumour in children'''
#* '''<span style="color:#ff0000">Most common malignant tumour in infants'''
*# '''<span style="color:#ff0000">Wilms tumour'''
#* '''Most common extracranial solid tumour in children'''
*#* '''<span style="color:#ff0000">Most common primary malignant renal tumour in children'''
# '''<span style="color:#ff0000">Wilms tumour'''
*# '''<span style="color:#ff0000">Congenital mesoblastic nephroma'''
#* '''<span style="color:#ff0000">Most common primary malignant renal tumour in children'''
*#* '''<span style="color:#ff0000">Most common renal tumour in infants'''[http://www.pathologyoutlines.com/topic/kidneytumormesoblastic.html §]
# '''<span style="color:#ff0000">Congenital mesoblastic nephroma'''
*#'''<span style="color:#ff0000">Renal cell carcinoma'''
#* '''<span style="color:#ff0000">Most common renal tumour in infants'''[http://www.pathologyoutlines.com/topic/kidneytumormesoblastic.html §]
*# '''<span style="color:#ff0000">Clear cell sarcoma'''
#'''<span style="color:#ff0000">Renal cell carcinoma'''
*# '''<span style="color:#ff0000">Rhabdoid tumour'''
# '''<span style="color:#ff0000">Clear cell sarcoma'''
*# '''<span style="color:#ff0000">Other rare tumours'''
# '''<span style="color:#ff0000">Rhabdoid tumour'''
*# '''<span style="color:#ff0000">Hydronephrosis'''
# '''<span style="color:#ff0000">Other rare tumours'''
*#'''<span style="color:#ff0000">Renal cystic disease'''
# '''<span style="color:#ff0000">Hydronephrosis'''
#'''<span style="color:#ff0000">Renal cystic disease'''


== Neuroblastoma ==
== Neuroblastoma ==
Line 20: Line 21:
=== Background ===
=== Background ===
* '''Arises from cells of the neural crest''' that form the adrenal medulla and sympathetic ganglia
* '''Arises from cells of the neural crest''' that form the adrenal medulla and sympathetic ganglia
* '''Tumors may occur anywhere along the sympathetic chain''' within the neck, thorax, retroperitoneum, pelvis '''or adrenal gland'''
* '''Tumors may occur anywhere along the sympathetic chain''' '''within the'''
** '''75% originate in the retroperitoneum of which 50% originate in the adrenal and 25% in the paravertebral ganglia'''
*#'''Neck'''
* '''These tumors can undergo spontaneous regression, differentiate to benign neoplasms, or exhibit extremely malignant behavior'''
*#'''Thorax'''
*#'''Retroperitoneum (including adrenal gland)'''
*#*75% originate in the retroperitoneum of which 50% originate in the adrenal and 25% in the paravertebral ganglia
*#'''Pelvis'''


=== Epidemiology ===
=== Epidemiology ===
Line 34: Line 38:
* PHOX2B and ALK as hereditary predisposition genes
* PHOX2B and ALK as hereditary predisposition genes
* '''≥20% of patients with familial neuroblastoma have bilateral adrenal or multifocal primary tumors; bilateral adrenal or multifocal primary tumors are rare in spontaneous cases'''
* '''≥20% of patients with familial neuroblastoma have bilateral adrenal or multifocal primary tumors; bilateral adrenal or multifocal primary tumors are rare in spontaneous cases'''
=== Natural History ===
* '''Tumors can either (3)'''
** '''Undergo spontaneous regression'''
** '''Differentiate to benign neoplasms'''
** '''Exhibit extremely malignant behavior'''


=== Pathology ===
=== Pathology ===
Line 63: Line 74:
* '''Clinical manifestations vary widely'''
* '''Clinical manifestations vary widely'''
** '''<span style="color:#ff0000">Most children have abdominal pain or a palpable mass'''
** '''<span style="color:#ff0000">Most children have abdominal pain or a palpable mass'''
*** '''Most primary tumors arise within the abdomen (65%);'''
*** '''Most primary tumors arise within the abdomen (65%)'''
**** Physical examination often reveals a fixed, hard abdominal mass
**** Physical examination often reveals a fixed, hard abdominal mass
*** '''Pelvic neuroblastoma arising from the organ of Zuckerkandl account for 4% of tumors.'''
*** '''Pelvic neuroblastoma arising from the organ of Zuckerkandl account for 4% of tumors.'''
Line 74: Line 85:


==== Labs ====
==== Labs ====
* '''<span style="color:#ff0000">Increased levels of urinary metabolites of catecholamines, vanillylmandelic acid (VMA) and homovanillic acid (HVA)''', are found in 90% to 95% of patients
* '''<span style="color:#ff0000">Urinary metabolites of catecholamines, vanillylmandelic acid (VMA) and homovanillic acid (HVA)'''
** These metabolites can be monitored to detect tumor relapse and response to therapy
**'''Increased levels are found in 90-95% of patients'''
* '''Anemia''' '''is found with widespread bone marrow involvement.'''
*** These metabolites can be monitored to detect tumor relapse and response to therapy
** '''Marrow biopsies add substantially to the detection of marrow involvement by tumor,''' compared with marrow aspirates alone.
* '''CBC'''
*** '''2 marrow aspirates and 2 biopsies are recommended'''
**'''Anemia''' '''is found with widespread bone marrow involvement'''
[[File:Neuroblastoma 103.jpg|alt=Axial CT scan of Neuroblastoma|thumb|500x500px|Axial CT scan of Neuroblastoma. Source: [[commons:File:Neuroblastoma_103.jpg|Wikipedia]]]]


==== Imaging ====
==== Imaging ====
Line 96: Line 108:
* '''Radionuclide bone scan and metaiodobenzylguanidine (MIBG) scans for can be used staging'''
* '''Radionuclide bone scan and metaiodobenzylguanidine (MIBG) scans for can be used staging'''


* Insert figure
==== Other ====
* '''Bone marrow biopsies'''
**'''Marrow biopsies add substantially to the detection of marrow involvement by tumor,''' compared with marrow aspirates alone.
*** '''2 marrow aspirates and 2 biopsies are recommended'''


=== Staging ===
=== Staging ===
Line 119: Line 134:
* Localized tumor
* Localized tumor
* Complete or incomplete gross resection
* Complete or incomplete gross resection
* '''Ipsilateral ymph nodes involvement positive under microscopic examination'''
* '''Ipsilateral lymph nodes involvement positive under microscopic examination'''
* Enlarged contralateral lymph nodes but with negative involvement under microscopic examination
* Enlarged contralateral lymph nodes but with negative involvement under microscopic examination
|-
|-
Line 128: Line 143:
<center>'''or'''</center>
<center>'''or'''</center>


* '''Unresectable unilateral tumor infiltrating across the midline''' with positive or negative regional lymph node involvement
* '''<span style="color:#ff0000">Unresectable unilateral tumor</span> infiltrating across the midline''' with positive or negative regional lymph node involvement


<center>'''or'''</center>
<center>'''or'''</center>
Line 136: Line 151:
|'''4'''
|'''4'''
|
|
* '''Metastasis of the tumor to distant lymph nodes'''
* '''<span style="color:#ff0000">Metastasis of the tumor to distant lymph nodes'''
* '''Metastasis of the tumor to liver, skin, organs (except as defined by Stage 4S)'''
* '''<span style="color:#ff0000">Metastasis of the tumor to liver, skin, organs (except as defined by Stage 4S)'''
|-
|-
|'''4S'''
|'''4S'''
Line 167: Line 182:
*# Radiation therapy
*# Radiation therapy
** The role of each depends on tumor stage, age, and biologic prognostic factors.
** The role of each depends on tumor stage, age, and biologic prognostic factors.
* '''Infants < 6 months with localized small adrenal masses can be managed with serial observation.'''
* '''<span style="color:#ff0000">Infants < 6 months with localized small adrenal masses can be managed with serial observation.'''
** '''Frequently associated with spontaneous regression'''
** '''Frequently associated with spontaneous regression'''
** '''Surgical resection can be avoided in 80% of such patients'''
** '''Surgical resection can be avoided in 80% of such patients'''
* '''Low-Risk Disease (Stages 1 and 2): surgery (adrenalectomy)'''  
* '''<span style="color:#ff0000">Low-Risk Disease (Stages 1 and 2): surgery (adrenalectomy)'''  
** Disease-free survival with surgical excision (adrenalectomy) alone in children with stage 1 neuroblastoma: > 90%
** Disease-free survival with surgical excision (adrenalectomy) alone in children with stage 1 neuroblastoma: > 90%
** '''Radical resection resulting in removal of normal organs, particularly the kidney, is not justified'''
** '''<span style="color:#ff0000">Radical resection resulting in removal of normal organs, particularly the kidney, is not justified'''
***'''In stage 3 disease, or in stage 2 with extensive tumor around the kidney and renal vessels, preoperative treatment with chemotherapy significantly decreases the risk of nephrectomy as a result of resection of the tumor.'''
***'''In stage 3 disease, or in stage 2 with extensive tumor around the kidney and renal vessels, preoperative treatment with chemotherapy significantly decreases the risk of nephrectomy as a result of resection of the tumor.'''
** Radiation of the local tumor bed has been advocated for treatment of residual disease in stage 2
** Radiation of the local tumor bed has been advocated for treatment of residual disease in stage 2
* '''Intermediate- and High-Risk Disease (Stages 3 and 4): neoadjuvant chemotherapy'''
* '''<span style="color:#ff0000">Intermediate- and High-Risk Disease (Stages 3 and 4): neoadjuvant chemotherapy followed by surgery'''
** '''Usually the safest approach for advanced tumors is to defer resection until after initial chemotherapy'''
** '''Usually the safest approach for advanced tumors is to defer resection until after initial chemotherapy'''
** Neoadjuvant chemotherapy, given the efficacy of modern agents, is very successful in reducing the size of primary tumors
** Neoadjuvant chemotherapy, given the efficacy of modern agents, is very successful in reducing the size of primary tumors
** There is debate regarding the extent of surgical resection that is required for stage 3 lesions
** There is debate regarding the extent of surgical resection that is required for stage 3 lesions
** Conflicting evidence regarding the benefit of extensive resection in children with stage 4 disease  
** Conflicting evidence regarding the benefit of extensive resection in children with stage 4 disease  
** '''Surgery usually is performed 13-18 weeks after initiation of chemotherapy, allowing 3-4 courses of treatment'''
** '''<span style="color:#ff0000">Surgery usually is performed 13-18 weeks after initiation of chemotherapy, allowing 3-4 courses of treatment'''
** Infants age <1 year with extensive local disease or stage 4 disease comprise a special subset of patients. They have historically fared much better than children older than 1 year of age with comparable disease, but not as well as infants with stage 4S disease. It is now recognized that the biologic markers can be used to identify which infants have high-risk disease and require intensive therapy and which have intermediate-risk disease requiring less intensive therapy.
** Infants age <1 year with extensive local disease or stage 4 disease comprise a special subset of patients. They have historically fared much better than children older than 1 year of age with comparable disease, but not as well as infants with stage 4S disease. It is now recognized that the biologic markers can be used to identify which infants have high-risk disease and require intensive therapy and which have intermediate-risk disease requiring less intensive therapy.
* '''Stage 4S'''
* '''Stage 4S'''
Line 219: Line 234:
****'''Found in Denys Drash, Frasier, and WAGR syndromes'''
****'''Found in Denys Drash, Frasier, and WAGR syndromes'''
****'''Predispose patients to renal insufficiency'''
****'''Predispose patients to renal insufficiency'''
** '''WT1 mutations'''
* '''<span style="color:#ff0000">Syndromes associated with Wilms tumours (4):</span><span style="color:#0000ff"> Bruce Willis' Dish Feels Warm'''
* '''<span style="color:#ff0000">Syndromes associated with Wilms tumours (4):</span><span style="color:#0000ff"> B.W.’s Dick Feels Warm'''
*#'''<span style="color:#0000ff">B</span><span style="color:#ff0000">eckwith-</span><span style="color:#0000ff">W</span><span style="color:#ff0000">eideman'''
*#'''<span style="color:#ff0000">Beckwith-Weideman'''
*#'''<span style="color:#0000ff">D</span><span style="color:#ff0000">enys Drash'''
*#'''<span style="color:#ff0000">Denys Drash'''
*#'''<span style="color:#0000ff">F</span><span style="color:#ff0000">rasier'''
*#'''<span style="color:#ff0000">Frasier'''
*#'''<span style="color:#0000ff">W</span><span style="color:#ff0000">AGR'''
*#'''<span style="color:#ff0000">WAGR'''


{| class="wikitable"
{| class="wikitable"
Line 232: Line 246:
|'''Risk of WT (%)'''
|'''Risk of WT (%)'''
|-
|-
|'''Beckwith-Weideman'''
|'''<span style="color:#ff0000">Beckwith-Weideman'''
|11p15.5
|11p15.5


WT2
WT2
|Wilm's Tumour
|'''Wilm's Tumour'''
 
'''<span style="color:#ff0000">Hemihypertrophy (growth asymmetry)'''


Macroglossia
'''Macroglossia'''


Nephromegaly
'''Nephromegaly'''


Hepatomegaly
'''Hepatomegaly'''


Pre- and post-natal overgrowth
Pre- and post-natal overgrowth
Hemihypertrophy (growth asymmetry)


Anterior abdominal wall defects
Anterior abdominal wall defects
Line 253: Line 267:
|7
|7
|-
|-
|'''Denys Drash'''
|'''<span style="color:#ff0000">Denys Drash'''
|'''WT1'''
|'''WT1'''
|Wilm's Tumour
|'''Wilm's Tumour'''


'''Genital abnormalities''' (under-masculanized male manifested by cryptorchidism and proximal hypospadias)
'''Genital abnormalities''' (under-masculinized male manifested by cryptorchidism and proximal hypospadias)


'''Nephropathy''' (mesangial sclerosis, membranoproliferative glomerulonephritis) with early onset proteinuria (common denominator of syndrome)
'''Nephropathy''' (mesangial sclerosis, membranoproliferative glomerulonephritis) with early onset proteinuria (common denominator of syndrome)
|74
|74
|-
|-
|'''Frasier'''
|'''<span style="color:#ff0000">Frasier'''
|'''WT1'''
|'''WT1'''
|Wilm's Tumour
|'''Wilm's Tumour'''


'''Genital abnormalities'''
'''Genital abnormalities'''
Line 273: Line 287:
|6
|6
|-
|-
|'''WAGR'''
|'''<span style="color:#ff0000">WAGR'''
|11p13
|11p13


'''WT1,''' PAX6
'''WT1,''' PAX6
|'''W'''ilm's Tumour
|'''Wilm's Tumour'''


'''A'''niridia
'''<span style="color:#ff0000">Aniridia (absence of the iris)'''


'''G'''enital abnormalities
'''G'''enital abnormalities
Line 287: Line 301:
|}
|}


* '''The development of a renal tumor in a child known to have aniridia, hemihypertrophy, or other syndromes associated with an increased incidence of nephroblastoma is most likely to be a Wilms tumor.'''
* '''Beckwith-Wiedemann syndrome (BWS)'''
* '''Beckwith-Wiedemann syndrome (BWS)'''
** '''Excess growth''' at the cellular, '''organ''' ('''macroglossia, nephromegaly, hepatomegaly'''), '''or body segment (hemihypertrophy) levels'''
** '''Results in excess growth at the cellular,''' '''organ (macroglossia, nephromegaly, hepatomegaly),''' '''or body segment (hemihypertrophy) levels'''
** '''Children with nephromegaly are at the greatest risk for the development of Wilms tumor.'''
** '''Children with nephromegaly are at the greatest risk for the development of Wilms tumor.'''
* '''Denys-Drash syndrome'''
* '''Denys-Drash syndrome'''
Line 295: Line 308:
** Although XY individuals have been reported most often, the syndrome has been reported in genotypic/phenotypic females.
** Although XY individuals have been reported most often, the syndrome has been reported in genotypic/phenotypic females.
** One should have a high index of suspicion for the development of renal failure and Wilms tumor in patients with under-masculanized male disorder of sexual differentiation
** One should have a high index of suspicion for the development of renal failure and Wilms tumor in patients with under-masculanized male disorder of sexual differentiation
* Aniridia
** Absence of the iris
** Found in 1% of Wilms tumor patients
** Caused by an abnormality of the PAX6 gene located adjacent to the WT1 gene
*** A deletion of chromosome 11 has been found most frequently in Wilms tumor patients with aniridia
* '''Other syndrome associated with Wilm’s tumour:'''  
* '''Other syndrome associated with Wilm’s tumour:'''  
**'''Perlman'''
**'''Perlman'''
Line 347: Line 355:
*** Hypertension can be caused by elevated plasma renin levels
*** Hypertension can be caused by elevated plasma renin levels
* Compression or invasion of adjacent structures may result in an atypical presentation. A persistent varicocele in the supine position or hepatomegaly may be reflective of inferior vena cava obstruction from tumor thrombus
* Compression or invasion of adjacent structures may result in an atypical presentation. A persistent varicocele in the supine position or hepatomegaly may be reflective of inferior vena cava obstruction from tumor thrombus
*Aniridia
**
** Found in 1% of Wilms tumor patients
** Caused by an abnormality of the PAX6 gene located adjacent to the WT1 gene
*** A deletion of chromosome 11 has been found most frequently in Wilms tumor patients with aniridia
*'''The development of a renal tumor in a child known to have aniridia, hemihypertrophy, or other syndromes associated with an increased incidence of nephroblastoma is most likely to be a Wilms tumor.'''
*'''Genitourinary anomalies (renal fusion anomalies, cryptorchidism, hypospadias) are present in 4.5% of patients with Wilms tumor'''
*'''Genitourinary anomalies (renal fusion anomalies, cryptorchidism, hypospadias) are present in 4.5% of patients with Wilms tumor'''
** '''May be associated with horseshoe kidney'''
** '''May be associated with horseshoe kidney'''
Line 361: Line 375:


==== Imaging ====
==== Imaging ====
* '''Regional'''
* '''<span style="color:#ff0000">Regional'''
** '''<span style="color:#ff0000">Abdominal ultrasound'''  
** '''<span style="color:#ff0000">Abdominal ultrasound'''  
***'''<span style="color:#ff0000">For all patients with a suspected renal mass, evaluation should begin with an abdominal ultrasound to confirm a solid renal mass and preliminarily evaluate the contralateral kidney.'''  
***'''<span style="color:#ff0000">For all patients with a suspected renal mass, evaluation should begin with an abdominal ultrasound to confirm a solid renal mass and preliminarily evaluate the contralateral kidney.'''  
Line 378: Line 392:
*** CT was able to detect all clinically significant IVC tumor extension when compared with ultrasonography
*** CT was able to detect all clinically significant IVC tumor extension when compared with ultrasonography
** '''<span style="color:#ff0000">Determination of inoperability must be made at surgical exploration, not based on imaging.'''
** '''<span style="color:#ff0000">Determination of inoperability must be made at surgical exploration, not based on imaging.'''
* '''Distant metastases'''
* '''<span style="color:#ff0000">Distant metastases'''
** '''<span style="color:#ff0000">Chest CT'''
** '''<span style="color:#ff0000">Chest CT'''
*** '''Lung is the most common site of distant metastasis'''
*** '''Lung is the most common site of distant metastasis'''
Line 391: Line 405:


=== Staging ===
=== Staging ===
* '''<span style="color:#ff0000">The current staging system used by the Children’s Oncology Group (COG) is based primarily on the surgical and histopathologic findings (not imaging)'''
* '''<span style="color:#ff0000">Current staging system used by the Children’s Oncology Group (COG) is based primarily on the surgical and histopathologic findings (not imaging)'''


{| class="wikitable"
{| class="wikitable"
Line 436: Line 450:


==== Initial Management ====
==== Initial Management ====
*'''<span style="color:#ff0000">Upfront open transperitoneal radical, adrenal-sparing, nephrectomy with lymph node sampling and without tumour spillage is the mainstay of initial treatment of unilateral, non-syndromic tumors, even in patients with metastatic disease'''
*'''<span style="color:#ff0000">Principles of initial treatment of unilateral, non-syndromic tumors, even in patients with metastatic disease:'''
** '''In general, Children's Oncology Group advocates for upfront nephrectomy, confirmation of diagnosis and then chemotherapy'''
*#'''<span style="color:#ff0000">Upfront open transperitoneal radical, adrenal-sparing, nephrectomy with'''
***International Society of Pediatric Oncology (SIOP) advocates for an assumed diagnosis of WT, followed by pre-operative chemotherapy and then surgery for all patients.
*#'''<span style="color:#ff0000">Lymph node sampling and'''
**** Outcomes are similar, regardless of which protocol is used but there are differences in cumulative doses of therapies and the number of patients exposed to various therapies.
*#'''<span style="color:#ff0000">Without tumour spillage'''
**** In North America children, and adolescents are generally treated per COG guidelines
*#* '''In general, Children's Oncology Group advocates for upfront nephrectomy, confirmation of diagnosis and then chemotherapy'''
** '''<span style="color:#ff0000">Nephron-sparing surgery'''
*#**International Society of Pediatric Oncology (SIOP) advocates for an assumed diagnosis of WT, followed by pre-operative chemotherapy and then surgery for all patients.
***'''<span style="color:#ff0000">Indications (4):'''
*#*** Outcomes are similar, regardless of which protocol is used but there are differences in cumulative doses of therapies and the number of patients exposed to various therapies.
***# '''<span style="color:#ff0000">Bilateral tumors'''
*#*** In North America children, and adolescents are generally treated per COG guidelines
***# '''<span style="color:#ff0000">Tumor in a solitary kidney'''
* '''<span style="color:#ff0000">Nephron-sparing surgery'''
***# '''<span style="color:#ff0000">Pre-disposition syndrome'''
**'''<span style="color:#ff0000">Indications (4):'''
***# '''<span style="color:#ff0000">Clinical trials'''
**# '''<span style="color:#ff0000">Bilateral tumors'''
**** '''Typically, patients undergoing nephron-sparing surgery will undergo preoperative chemotherapy''' with an assumed diagnosis of WT (COG does not recommend routine biopsy in this setting) '''to allow tumor shrinkage to preserve as much normal renal tissue as possible'''
**# '''<span style="color:#ff0000">Tumor in a solitary kidney'''
** '''<span style="color:#ff0000">Approach'''
**# '''<span style="color:#ff0000">Pre-disposition syndrome'''
***'''<span style="color:#ff0000">Open radical nephrectomy is standard'''
**# '''<span style="color:#ff0000">Clinical trials'''
****'''Extreme caution must be used applying minimally-invasive surgery to nephron-sparing surgery in children,''' and neither is advocated nor been studied by COG or SIOP
*** '''Typically, patients undergoing nephron-sparing surgery will undergo preoperative chemotherapy''' with an assumed diagnosis of WT (COG does not recommend routine biopsy in this setting) '''to allow tumor shrinkage to preserve as much normal renal tissue as possible'''
** '''<span style="color:#ff0000">Principles in Wilm's Tumor Surgery (6)'''
* '''<span style="color:#ff0000">Approach'''
**#'''<span style="color:#ff0000">Thorough exploration of the abdominal cavity is necessary to exclude local tumor extension, liver and nodal metastases, and peritoneal seeding</span>'''
**'''<span style="color:#ff0000">Open radical nephrectomy is standard'''
**#* Accurate staging is essential for the subsequent determination of the need for radiation therapy and the appropriate chemotherapy regimen.
***'''Extreme caution must be used applying minimally-invasive surgery to nephron-sparing surgery in children,''' and neither is advocated nor been studied by COG or SIOP
**# '''<span style="color:#ff0000">Perform surgery without tumor spillage</span>'''
* '''<span style="color:#ff0000">Principles in Wilm's Tumor Surgery (6)'''
**#* Local recurrence is increased in patients with local tumor spillage, and is classified as stage III disease. 2-year survival rate after local recurrence is 43%
*#'''<span style="color:#ff0000">Thorough exploration of the abdominal cavity is necessary to exclude local tumor extension, liver and nodal metastases, and peritoneal seeding</span>'''
**#'''<span style="color:#ff0000">Palpate the renal vein and IVC</span>''' to exclude intravascular tumor extension before vessel ligation.
*#* Accurate staging is essential for the subsequent determination of the need for radiation therapy and the appropriate chemotherapy regimen.
**#'''<span style="color:#ff0000">Lymph node sampling must be included at the same time as resection even with nephron-sparing.'''
*# '''<span style="color:#ff0000">Perform surgery without tumor spillage</span>'''
**#* '''Selective sampling of suspicious nodes is an essential component of local tumor staging'''.
*#* Local recurrence is increased in patients with local tumor spillage, and is classified as stage III disease. 2-year survival rate after local recurrence is 43%
**#** '''Formal retroperitoneal lymph node dissection is not recommended'''
*#'''<span style="color:#ff0000">Palpate the renal vein and IVC</span>''' to exclude intravascular tumor extension before vessel ligation.
**#*The extent of LN dissection and location of nodal sampling need to be better defined to allow further study.
*#'''<span style="color:#ff0000">Lymph node sampling must be included at the same time as resection even with nephron-sparing.'''
**#'''<span style="color:#ff0000">Adrenal gland can be spared</span> without increasing the risk for tumor spill or recurrence if it is not in close proximity to the tumor'''
*#* '''Selective sampling of suspicious nodes is an essential component of local tumor staging'''.
**#'''<span style="color:#ff0000">Routine exploration of the contralateral kidney at the time of nephrectomy is not necessary when preoperative imaging with CT or MRI demonstrates a normal contralateral kidney</span>'''
*#** '''Formal retroperitoneal lymph node dissection is not recommended'''
*#*The extent of LN dissection and location of nodal sampling need to be better defined to allow further study.
*#'''<span style="color:#ff0000">Adrenal gland can be spared</span> without increasing the risk for tumor spill or recurrence if it is not in close proximity to the tumor'''
*#'''<span style="color:#ff0000">Routine exploration of the contralateral kidney at the time of nephrectomy is not necessary when preoperative imaging with CT or MRI demonstrates a normal contralateral kidney</span>'''


==== Adjuvant Treatment[https://wjps.bmj.com/content/2/3/e000038 §] ====
==== Adjuvant Treatment[https://wjps.bmj.com/content/2/3/e000038 §] ====
Line 536: Line 553:


=== 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'''
Line 597: Line 618:


=== 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 ==
Retrieved from "https://urologyschool.com/wikiuro/index.php/Pediatrics:_Renal_and_Adrenal_Oncology"