PEDIATRIC SOLID TUMORS Flashcards
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
TREATMENT
What stages of Wilm’s tumor can you use chemotherapy for?
All stages
TREATMENT
What stages of Wilm’s tumor can you do surgery for?
Stage 1-5
TREATMENT
What stages of Wilm’s tumor can you use radiotherapy for?
3 and 4
__________ is the most common extra-cranial malignancy diagnosed in infancy
NEUROBLASTOMA
NEUROBLASTOMA
It arises from _________ tissue of the ___________ or ___________
Accounts for 8% to 10% of childhood tumours
neural crest tissue
sympathetic ganglia
adrenal medulla
NEUROBLASTOMA
is slightly more common in (boys or girls?)
Median age at diagnosis is 19 months
Spontaneous regression of the tumour occurs in infancy
Most patients (70%-80%) older than 18 months present with _____________disease
boys
metastatic disease
NEUROBLASTOMA: PATHOLOGY
It is composed of (small or large?) fairly uniform cells with little cytoplasm and hyper- chromatic nuclei that may form _________
________ is an over expressed oncogene of prognostic importance present in 25% of cases and is commonly found in advanced diseases
small ; rosettes
MYCN
NEUROBLASTOMA: Pathology
Three classical histo-pathologic patterns are known; ____________ , ____________ & ____________.
neuroblastoma
ganglioneuroblastoma
ganglioneuroma
NEUROBLASTOMA: pathology
The _______________ rosette is a classic finding in neuroblastoma
The Homer-Wright pseudo
NEUROBLASTOMA: pathology
_________ tumours are commoner in children than infants
_________ & _________ tumours are commoner in infants
Adrenal tumours
Thoracic & Cervical tumours
SYMPTOMS: Neuroblastoma
Symptoms will depend on site of primary tumour
__________ mass 65%
_______ pains 60%
Fever
Weight _____
_________ mass
____________ tumour
20%-25% present with localized disease
50% of patients present with metastatic disease
Abdominal mass
Bone
Weight loss
Cervical neck mass
Dumb-bell tumour
