Malignant Disease Flashcards
What is the most common CNS tumour in childhood?
Pilocytic Astrocytoma (GRADE-1)
common in Neurofibrimatosis 1
What is the histopathology of Policytic Astrocytoma?
With what gene mutation is it usually associated with?
- Slow growing and low mitotic activity
- Piloid cell (hair cell)
- Rosenthal Fibers
70% is associated with BRAF mutation
What are the presenting Symptoms of Policytic Astrocytoma?
- Headaches
- Balance issues, co-ordination problems
- Vomiting when walking
- Visual changes
- Failure to thrive-irritability- behavioral changes
What are the signs found with Policytic Astrocytoma?
What is the appropriate modality for investigation?
Papilldema: due to an increased ICP
* disc oedema, obscuration of margins, venous congestions, haemmorrhages
MRI> CT/PET (too much radiation)
What is the appropriate management of Policytic Astrocytoma?
There needs to be an MDT approach which will include: Surgery, Councelor, padiatrician, neurologists and , CLIC Sargent (Cancer and Leukemia in children social worker)
1st line: Surgery (depends on the type and location of tumor)
2nd line: Radiotherapy for low and high grade; gliomas and metastases
3rd line: Chemotherapy used for high grade gliomas
What are the most common Leukemias in children?
What is the most common age for incidence of Leukemia?
- 80% ALL (85% are B-lineage, 15% T-cell linaege)
- 20% AMM
2-5yrs old, more common in Males
What are the symptoms of Leukemia in children?
- BM failure (thrombocytopenia, aneamia, Neutropenia)
- Hepatosplenomegaly
- Lymphadenopathy
- Leukaemia cutis: petechial rash: face and trunk
- Tumor lysis syndrome: high K+, LDH, PO2-4, Uric acid
What are the appropriate management for Tumor Lysis Syndrome?
Allopurinol and hyperhydration
What are the appropriate investigations for Leukemias?
FBC and clotting studies: anaemia, thrombocytopenia and neutropenia (may show Tumor Lysis Syndrome)
Blood film: lymphoblasts
CXR: enlarged thymous
BM biopsy
What is the appropriate management of ALL ?
MDT: hollistic approach to treating the patient (paeds, haemoligists, councelor)
- Systemic Chemotherapy: 2-3 years of chemo, males might need a longer course as lymphoblasts accumulate in the testes
- CNS directed therapy: even if there are no cells in the CNS treatment is administered (6-8 session), where intrathecal chemotherapy in given
- Mollecular treatment: Imatinib, Rituximab
- Transplantation
- Supportive care: blood products, Antibiotics
What is the prognosis of ALL for children and adults?
What are some poor prognostic factors?
for children: 80% 5-year survival
for adults 30-40% 5-year survival
Poor prognostic factors:
1. <2yrs old
2. >10yrs old
3. Non-caucasian
4. Males
5. T or B cell surface markers
What are the different types of Lymphoma in children?
- Hodgkin’s Lymphoma(HL): more common in children, more localised and spreads contiguously (more common in children)
- Non-Hodgkin’s Lymphoma (NHL): involves multipe sides and spreads sporadically (more common in adolescents)
If a specific type of cell called a Reed-Sternberg cell is seen, the lymphoma is classified as Hodgkin’s.
What are the signs and symptoms of Hodgkin’s Lymphoma?
- Painless lymphadenopathy
- B symptoms (fever, night sweats, weight loss)
- Painful on drinking alcohol
What are the appropriate investigations for Hodgkin’s Lympoma and Non-hodgkin’s lymphoma?
LN biopsy
Bloods: FBC, ESR, Alb, LFT’s
PDG-PET
What is the appropriate Staging for Hodgkin’s and non-hodgkin’s lymphoma?
Ann-Arbor staging
- I: one group of lymph nodes
- II: more than one group of lymph nodes but on the same side of the diaphragm
- III: nodes are on more than one side of the diaphragm
- IV: extranodular metastases
What is the prognosis for Hodgkin’s Lympoma?
80% cured (in disseminated disease, this falls to 60%)
What is the management of Hodgkin’s lymphoma?
Combination Radiotherapy (ABVD)+ Chemotherapy
1. Adriamycin
2. Bleomycin
3. Vincristine
4. DTIC (Dacarbazine)
Pet-scan to monitor for progress
What is the presentation of Non-hodkin’s lympoma?
Can be low or high grade which describes the speed and aggresivness of onset
Painless lympadenopathy
B-cell symptoms: fever, nigh sweats, weight loss
What is the management of Non-hodgkin’s lymphoma?
(1) Urgent chemotherapy
(2) Monitor only
(3) Antibiotic eradication (H. pylori gastric MALToma)
What type of malignancy is Burkitt’s lymphoma and what are the different variants?
It is a subtype of B-cell Non-hodgkin’s Lymphoma
There are 3 variants:
1. Endemic – EBV infection (chronic malaria may reduce EBV resistance):
Most commonly in children living in malaria endemic regions
Most common childhood cancer in Africa
Involves JAW or facial bones
2. Sporadic – EBV infection: Western world, associated with EBV infection
3.Immunodeficiency – HIV infection
What is the histopathology of Burkitt’s Lymphoma?
- Arises from germinal center cells
- Starry-sky appearances
What is the most common primary bone malignancy of chilhood?
Osteosarcoma: more common in Males, long bones (60-75% in knee)
What are the symptoms of Osteosarcoma, where does it commonly metastasize?
- Relatively painless, mass swelling, restricted movments
- commonly metastasized to lungs
What are the appropriate investigations for Osteosarcoma?
(1) X-ray: bone destruction and formation
There are soft tissue calcification (sunburst appearance)
Elevated periosteum: Codman’s Triangle
(2) Biopsy
(3) CT/PET/MRI
What is the appropriate management of Osteosarcoma?
- Specialist Sarcoma team
- Surgery (limb sparing surgery/ amputation)
- Chemotherapy
- Post-treatment: OT, PT dietician
What is the difference between Osteosarcoma and Ewing’s Sarcoma?
Osteosarcoma: forms bone
Ewing’s Sarcoma forms mesenchymal tissue
What type of tumor is Ewing’s sarcoma?
What’s the commonest age and which bones are usually affected?
It is a bone tissue malignancy, Primitive Neuroendocrine Tumour (PNET)
- <25y, median 15y
- Long bones of arms, legs, chest, skull and trunk
What are the main Symptoms of Ewing’s Sarcoma?
Mass or swelling and bone pain
Malaise, fever, paralysis (may precipitate osteomyelitis)
What are the appropriate investigations for Ewing’s Sarcoma?
XR (bone destruction with overlying onion-skin layers of periosteal bone formation)
Biopsy (small round blue cells)
CT/PET/MRI
What is the appropriate management for Ewing’s sarcoma?
o Specialised sarcoma team (London) management
o Surgery (limb-sparing surgery ± amputation) + chemotherapy (VIDE) + radiotherapy
o Post-treatment OT, PT, dietician, orthotics/prosthetics, support
o Prognosis survival 5-year at 75% (20-40% for metastasis
What is the aetiology of Retinoblastoma?
What are the different types?
What is the most common age ?
RARE but accounts for 5% of severe visual impairment in children
- Unilateral (80% spontaneous, 20% hereditary) or
- billateral (100% hereditary)
Autosomal dominant, chromosome 13–> encodes pRB (protein retinoblastoma)
Average age of diagnosis = 18 months
What are the signs of Retinoblastoma and what are the appropriate investigations?
Signs & symptoms:
- Red reflex absent (i.e. a white pupillary reflex instead of normal red one)
Squint
Investigations:
- MRI and EUA
What is the appropriate management of Retinoblastoma and what is the prognosis?
Management:
-
Enucleation (removal of eye, leaving eye muscles intact)
2.** Chemotherapy (bilateral)** + laser treatment to retina ± chemotherapy (advanced disease)
Prognosis:
- Most cured, some may be visually impaired (>90% survive to childhood)
- Risk of secondary malignancy (sarcoma) in survivors of hereditary retinoblastoma
What type of tumor is a neuroblastoma?
Which is the commonest age?
Neuroblastoma arises from neural crest tissue in adrenal medulla and SNS
Most common extra-cranial tumour in children
Most common in bellow 5 years of age
What are the signs and symptoms with Neuroblastoma?
- **Abdominal Mass **(tumour can be anywhere in the sympathetic chain)
- Systemic symptoms: WL pallor, hepatomegaly, bone pain, limp
- Symptoms of spinal cord compression
- Over 2yo–> symptoms of metastatic disease (bone pain, BM supression, WL, malaise)
What are the appropriate investigations for Neuroblastoma?
o Radiological findings
o Raised urinary catecholamine metabolites (VMA/HVA)
o Confirmatory biopsy from BM and MIBG sampling
What is the appropriate management for Neuroblastoma?
What is the prognosis ?
Spontaneous regression can occur in very young infants
Localised primaries without metastatic disease–> surgery alone
Metastatic disease–> chemotherapy + radiotherapy (with autologous stem cell rescue) + surgery (High risk of relapse )
Prognosis–> cure rates for children with metastatic disease is around 40%
