Solid tumours Flashcards
What are some familial syndrome associated with brain tumours?
NF-1 (optic glioma, astrocytoma, neurofibromas)
NF-2 (acoustic neuroma, meningioma, spinal cord ependymoma/astrocytoma)
Tuberous sclerosis (SEGA, cortical tubers)
Von Hippel-Lindau - hemangioblastoma
Li-Fraumeni - astrocytoma, PNET
Cowden 0 dysplastic ganlgiocytoma of cerebellum
Turcot 0 medulloblastoma, glioblastoma
Nevoid basal cell carcinoma (autosomal dominant)
What is the clinical course of low grade astrocytoma?
Indolent. Low metastatic potentitla. Small proportion have leptomeningeal spread (especially in optic nerve lesion.
What’s the most common site for pilocytic astrocytoma?
cerebellum
What are Rosenthal fibers
condensed masses of glial filaments - diagnostic for PA.
What is the second most common astrocytoma?
fibrillary infiltrating astrocytomma (diffuse infiltration of tumour cells among normal neural tissue adn potential for anaplastic progression.
Where are most ependymal tumours found?
posterior fossa (60%)
What is the most common CNS tumour under <1 year of age?
Choroid plexus tumours.
Where are germ cell tumours usually found?
midline.
What biochemical markers can help you diagnose germ cell tumour?
AFPBCG elevated.
Which CNS tumour is a small round blue tumour?
Medulloblastoma
What are high risks for medulloblastoma?
Significant residual tumour
Leptomeningeal spread
Brainstem invasion.
What are the bad CNS tumours to have?
Brainstem gliomas, high grade gliomas (multiforme)
What are the poor prognostic factors of neuroblastoma?
- N-myc amplification
- 1p deletion in tumour cells
- Additional 17q
- Expression of MRP gene
- High ferritin
- High LDH
- Age - older children with stage IV disease.
What are some associations with Wilm’s tumour?
WAGR syndrome (Wilm's, Aniridia, genitourinary, mental retardation) Hemihypertrophy Cryptorchidism Hypospadius Renal anomalies Familial Wilms' tumour - Denys-Drash (gonadal dysgenesis, nephrotic syndrome, WT) Beckwith-Wiedemann. Sampson Golabi-Behmel Syndrome.
What are some causes of osteosarcoma?
Radiation
Retinoblastoma families
Li-Fraumeni syndrome
What are the most common sites for osteosarcoma?
Distal femur
Proximal tibia
Proximal humerus
What is the most common site for rhabdomyosarcoma?
Genitourinary
What are the small round blue tumours?
Medulloblastoma Rhabdomyosarcoma Neuroblastoma Ewing's sarcoma NHL Retinoblastoma
What is t(2;13) associated with?
alveolar rhabdomyosarcoma
What is t(11:22) associated with?
Ewing’s
What is t (2;5) associated with?
Large cell anaplastic lymphoma
What is t(8;14) associated with?
Burkitt’s lymphoma
What is the prognosis of hodgkin’s lymphoma?
Favourable prognosis - 95% survival at 5 years.
Unfavourable prognosis - 90% at 5 years.
What are B symptoms for HL?
Fever >39
Weight loss >10% over 3 months
Drenching night sweats
What are poor prognostic factors for HL?
B symptoms
Tmour bulk
Stage at diagnosis.
Who are candidates for HSCT in HL?
Never achieve remission or relapse <12mnth after therapy.
What is 5 year prognosis for relapse HL (>12mn)
60-65%
What is rasburicase?
Recombinant urate oxidase
What is the prognosis for NHL?
90-100% for localised disease
60-95% for advanced disease
What are prognostic factors for NHL?
Pathologic subtype
Tumour burden at diagnosis (LDH level)
CNS disease
Metastatic spread
How many brain cancer survivors have long term neurological problems?
50%
What is the survival rate for high risk neuroblastoma?
25-35%
What is the cure rate for Stage 1/2/4S neuroblastoma?
> 90% usually
What is the 5 year survival for bilateral wilm’s tumour with favourable histology?
80%
What are the types for rhabdomyosarcoma?
- Embryonal type (60%) - intermediate prognosis
- Botryoid type
- Alveolar type (25-40%)
- Pleomorphic type (<1%)
What are some predisposing factors for osteosarcoma?
Retinoblastoma Li Fraumeni Pagets Radiotherapy Rothmund-Thomson syndrome
What is the most common site for osteosarcoma?
Metaphysis of long bones (distal femur, prox tibia, humerus)
What is the inheritance of retinoblastoma?
Hereditary or sporadic
Hereditary - multifocal adn bilateral.
Loss of function of RB1 gene via mutation/deletion
RB 1 on 13q14 encodes for retinoblastoma protein, tumour suppressor protein that controls cell-cycle phase transition.
2 hit hypothesis - 1stly inherited via germline cells but then 2nd mutation in somatic retinal cells.
Sporadic - unifocal
Which cancers secrete AFP?
Yolk sac tumours
Teratomas (small amount)
Hepatoblastoma
Hepatomas
Which cancers secrete BHCG?
Choriocarcinoma
Germinoma, seminoma, dysgerminomas
