Malignant Disease Flashcards
(37 cards)
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
