Malignancy Flashcards
malignant childhood renal tumours
nephroblastoma /Wilms renal cell cancer clear cell sarcoma rhabdoid renal tumour neuroblastoma
benign childhood renal
mesoblastic nephroma
angiomyolipoma
haemangioma
cystic nephroma
DDx of chidlhood abdominal mass
massive HN renal tumour renal cysts bowel duplication neuroblastoma hepatic tumour soft tissue sarcoma
conditions predisposing to Wilms
Denys Drash
WAGR
Beckwith Wiedemann
WAGR
wilms
aniridia
GU malformation
mental retardation
bilateral disease Wilms
5%
peak age presentation
when do wilms present
male vs female wilms
bilateral disease
3-4 years
majority wilms present under age 5 75%
M and F equally affected
5%
neuroblastoma urine test
urine VMA vanillylmandelic acid
staging wilms tumour
1 = completely confined to kidney excised
2 =out of capsule completely excised
3= local invasion, incomplete resection, positive LN, tumour spill not confined to flank
4= metastatic disease, usually lung
5= bilateral
prognosis wilms low risk histology 5 year survival
1/2 = >90 III = 70-80 IV= 60-80% V= 70-80%
poor prognosis histology types
nephroblastoma blastemal type
diffuse anaplasia type
clear cell sarcoma kidney
rhabdoid tumour kidney
survival 5 year bad histology
clear cell sarcoma = 70-80%
rhabdoi 20%
anaplastic wilms 60%
triphasic histology of Wilms
stromal
blastemal
epithelial / tubules
preop chemo regimen
vincristine
actinomycin D
for 4 weeks then repeat imaging preop us doppler and MRI
post op histology risk stratification
low risk stage 1 no further treatment
most other VA for 27 weeks
stage III need RT
High risk add doxorubicin
mesoblastic nephroma
benign tumour under 6 months
homogenous non enhacing solid mass needs just primary nephrectomy
most common tumour neanatal period
metanephric blastema
embronal or primitive cells give rise to renal parenchyma, if persis beyond 36/40 week called nephrogenic rests
nephroblastomatosis
multifocal diffuse nephrogenic rests
appear as oval shapes in periphery
no psuedocapsle
homogenous hypoechoic
types of RMS
embryonal >50% - botryoid, spindle cell paratesticular
alveolar 20%
investigations RMS
pelvic and urinary tract US
histological components Wilms
metanephric blastema
primitive renal tubular epithelium
connective tissue components
mutation wilms
chromosome 11 mutation or deletion WT1 tumour supressor gene
Denys Drash syndrome
Wilms
ambiguous genitalia
Beckwith Wiedemann syndrome
Macroglossia
associations wilms tumour
Denys Drash
Beckwith Wiedemann
Horseshoe kidney
Rhabdomyosarcoma
organs involved
bladder base prostate paratesticular uterus vagina
Rhabdomyosarcoma association
Li Fraumeni syndrome p53 mutation
Rhabdomyosarcoma better vs worse prognosis form
embryonal forms have better prognosis, of which bladder mostly embryonal
Neuroblastoma organ
arising in neuro ectoderm
50% arise adrenal
rest from sympathetic chain
Neuroblastoma median age diagnosis
2 years
Neuroblastoma poor prognosis
deletion short arm chromosome 1
Neuroblastoma scan
MIBG scan highly sensitivie
differential abdominal mass / renal mass
WIlms tumours
XGP usually with anaemia and listlessness
Benign XGP Large HN Renal cysts Splenomegaly Bowel duplication
Malignant
Nephroblastoma
Neuroblastoma
Hepatic tumour
Soft tissue sarcoma
wilms 5 year survival
85% five year survival
if unilateral localised then prognosis in high 90s
pain in Wilms tumour
incidence haematuria
Pain is uncommon and usually results from bleeding into the tumour
Haematuria occurs in 10–15% of cases
imaging vs biopsy
if presents in typical age 6 months to 6 years of age imaging diagnosis is sufficient
what to look for on imaging with wilms
neuroblastoma encase vessels
Wilms grow into vessels
imaging look for tumour extension into vessels
manage with nephrectomy or need vascular surgery
staging Wilms
can met into lungs so need CT check
bilateral Wilms tumour
stage 5
need to think of nephron
syndromes associated with Wilms
hemihypertrophy
WAGR
Denys Drash
Beckwith Wiedemann
tend to be multifocal and bilateral
chemotherapy Wilms
Always give neoadjuvant chemo
vincristine actinomycin doxorubicin added selected cases give 6 weeks shrinks tumours reduced rate of spillage at surgery
Wilms surgery
aim to sample LN at least 7
