PEDIATRIC SOLID TUMORS Flashcards
(36 cards)
RENAL TUMORS
______________ ( ______ Tumor )
___________ Nephroma
Renal _______________
____________________ of the kidney
__________ Tumor of the kidney
Nephroblastoma ( Wilm’s Tumor ) Mesoblastic
Renal Cell Carcinoma
Clear cell sarcoma
Rhabdoid Tumor
Most common primary renal tumour of childhood is?
Nephroblastoma (Wilm’s Tumour)
____________ is the commonest malignant abdominal tumour in our practice but the 2nd most common worldwide
Nephroblastoma (Wilm’s Tumour)
Nephroblastoma (Wilm’s Tumour)
It accounts for approximately _____% of all cancers in childhood
It is commoner amongst (blacks or whites?) and slightly commoner in (girls or boys?)
Rare in _______ race
Occurs in the age range ___-___years (Median age at diagnosis is ____ years) Most are ____lateral with bilateral cases occurring in 7%
blacks ; girls
Asians ; 2 – 5 years
3.5 ; unilateral
Wilm’s Tumor: Pathology
Alterations of genes responsible for normal genitourinary development
Mutations of the _____ gene occurs in approximately 40% of tumours
Other mutations are ____, _____ , _____ & deletions of chromosomes 16q, 1p, 7p
Associated with syndromes like _____, Denys-Drash, __________ , _________
WT1
WT2, WTX, TP53
WAGR ; Beckwith Wiedemann
LI- Fraumeni
Wilm’s tumor
They are typically ___________ lesions within the kidneys.
Approximately 10% are _______
Extra-renal sites like ______ , ____________ , _______ occur but are uncommon
Always demarcated from the surrounding renal tissue by a _______________________
solitary ; multi focal
pelvis ; retro-peritoneum
thorax
fibrous pseudo capsule
SYMPTOMS of Wilm’s tumor
Pain(ful or less?) (symptomatic or asymptomatic?) abdominal mass - >80%
Abdominal _____ in 40%
___________ (Gross) in 18%
(Microscopic) in 24%
___________
Fever
NB: 7% of children with Wilm’s have a known syndromic association
Painless ; asymptomatic
pain ; Hematuria
Hypertension
DIFFERENTIAL DIAGNOSIS of Wilm’s tumor
__________
Renal ________
___________ disease
________ _______
Rhabdomyosarcoma
Neuroblastoma
Other primary renal malignant conditions
Hydronephrosis
Renal abscess
Polycystic kidney disease
Burkitt’s lymphoma
INVESTIGATIONS for Wilm’s tumor
____________
______________ TEST
URINALYSIS
_______________ TEST
_______________ STUDIES
COMPLETE BLOOD COUNT
RENAL FUNCTION TEST
LIVER FUNCTION TEST
COAGULATION STUDIES
STAGING of Wilm’s tumor
Stage 1
The tumor is limited to the kidney, is ________ and can be completely removed __________
Tumor is not _________ or _________ prior to removal
The vessels of the _________ are not involved
No evidence of tumor is present at or beyond margins of resection
encapsulated ; surgically
biopsied ; ruptured
renal sinus
STAGING of Wilm’s tumor
Stage 2
The tumor extends beyond the kidney (penetration of __________ , involvement of ___________) but is __________________
renal capsule
renal sinus
completely removed
STAGING of Wilm’s tumor
Stage 3
•The tumor is _____________ surgically, but disease is still ___________________.
• Positive lymph nodes in the abdomen or pelvis are noted
• Penetration through the _____________ is observed and ______________ are present
• Gross / microscopic tumor
resection remains post operatively including _________________________
• Tumor is treated with _________________________
not completely removed
still limited to the abdomen.
peritoneal surface ; peritoneal implants
margins of resection
post operative chemotherapy
STAGING of Wilm’s tumor
Stage 4
The disease has spread
to the ______ , ______, ______, ______, or to lymph nodes outside the
______ region.
lung, liver, bone, brain
pelvic region.
STAGING of Wilm’s tumor
Stage 5
Both kidneys contain ___________ at diagnosis.
tumor
TREATMENT: LOW RISK of NEUROBLASTOMA
_______ is the preferred Rx for low risk tumor
__________ is used for tumors that have 50% resection rates
Infants with 4S tumors and no symptoms, adopt ______________ attitude
Surgery; Chemotherapy
watch and wait
TREATMENT: INTERMEDIATE RISK of NEUROBLASTOMA
Neoadjuvant _____________ or _______________ before __________
Second look, surgery advocated
chemotherapy or radiotherapy
surgery
TREATMENT: HIGH RISK of NEUROBLASTOMA
________,___________, and _________
Myeloablative ChemoRx & HSCT
13 Cis-retinoic acid Dinutuximab (anti GD2)
ChemoRx, Surgery & Radiation
HEPATOBLASTOMA
They are commonly _____focal and affect the ________ lobe more often
The median age at diagnosis is _______
The tumour is surrounded by a ______________
It occurs more in (males or females?)
unifocal ; right
1 year ; pseudo capsule
males
HEPATOBLASTOMA
It is not usually associated with _________
Associated conditions include hemi-hypertrophy, _______________ syndrome, _________
cirrhosis
Beckwitt-Wiedemann syndrome
Prematurity
HEPATOBLASTOMA: Clinical features
Asymptomatic abdominal mass
Advanced disease in 40% of patients with 20% having ________ metastasis
Weight ______
___________ in some patients
________ pain
Progressive ________ and pain in advanced disease
Rarely tumour _______ which leads to ________ and __________
Assess the _______ length & ________ volume
lung ; loss ; Osteopenia
Abdominal ; anorexia
rupture ; haemorrhage ; anemia
penile ; testicular
HEPATOBLASTOMA: INVESTIGATIONS
__________ is contra-indicated
_________ biopsy is advocated
Needle biopsy
Open biopsy
HEPATOBLASTOMA: TREATMENT
Surgery: __________ or complete resection
Chemotherapy: SIOPEL 6 with cisplatin or cisplatin/doxorubicin depending on stage
Radiotherapy for local control of micro metastasis & pulmonary metastases
Liver transplantation: For patients that complete resection is not possible
Lobectomy
