Oncology Flashcards
What genetic conditions have a predisposition for leukemia?
Trisomy 21 NF 1 Shwachman-Diamond syndrome Li-Fraumeni syndrome Chromosomal fragility – Bloom’s, Fanconi’s anemia, Ataxia Telangiectasia Paroxysmal Nocturnal Hemoglobinuria Diamond-Blackfan Anemia Kostmann syndrome SCID (Severe combined immune deficiency)
What are typical findings on CBC in Leukemia?
WBC may be low, normal or high.
Hemoglobin and platelets may be normal or low.
What % of patients with leukemia will not have circulating blasts?
20%
How do patients with leukemia typically present?
Low Hemoglobin
Pallor, fatigue, irritability
Low White cells
Fever, sepsis
Low Platelets
Bruising/bleeding, petechiae
Leukemic Blasts
Bone pain, limp
How do you distinguish between leukemia and sJIA?
Wbc low (<4),
Plt low normal (150-250),
Night time pain
What x-ray findings do you see in leukemia?
Transverse radiolucent metaphyseal growth arrest lines
Periosteal elevation with reactive Subperiosteal cortical thickening
Osteolytic lesions
Diffuse osteopenia/ osteoporosis
What are some clinical features of leukemia that indicate extramedullary disease?
Lymphadenopathy HSM Gingival hyperplasia Leukemia Cutis Chloroma (subcutaneous collection of AML cells) Lymphomatous mass CNS disease* Testicular disease*
What are indications for BMA?
Unexplained and significant depression ≥ 1 peripheral blood cell elements
Blasts on peripheral smear
Leukoerythroblastic changes on peripheral smear
Association with unexplained lymphadenopathy or hepatosplenomegaly
Association with an anterior mediastinal mass
What is the most common type of ALL?
80-85% are precursor B, remainder precursor T
What is the prognosis of ALL?
90% cure
How long is treatment for ALL?
2.5-3.5 years
How long is treatment for AML?
6 months, intensive
What is the prognosis of AML?
60% cure
What are poor prognostic signs in ALL?
AGE: < 1 and > 10 yrs*
WHITE CELL COUNT: > 50 *
CNS and/or TESTICULAR DISEASE
CYTOGENETICS
Good:t (12;21) = TEL-AML; hyperdiploidy
Poor: MLL gene rearrangement (11q23)
Very Poor: t (9;22) = Ph chromosome, and hypodiploidy
DISEASE RESPONSE
As assessed by minimal residual disease (MRD) testing at the end of induction chemotherapy
What is a differential diagnosis for cervical adenopathy?
Viral URI Bacterial adenitis EBV Cat scratch Atypical mycobacterium Malignancy Kawasaki TB Rare infections-toxo, tularemia, brucellosis, leptospirosis Sarcoidosis
Differential diagnosis of generalized adenopathy?
Syphilis
HIV
CMV
Toxo
Rare: leukemia, TB, serum sickness, SLE, JRA, sarcoidosis, fungal, histoplasmosis, LCH
What are the predictive factors for malignancy with lymphadenopathy?
Age >10
Node >2.5 cm
Supraclavicular (TB in ddx)
What are the indications for excisional biopsy with lymphadenopathy?
>2 cm Increase over 2 weeks No decrease in size after 4 weeks Supraclavicular Hard, matted, rubbery consistency Abnormal CXR Fever Weight loss HSM
What are the types of lymphoma in children?
Hodgkins
- Classical (chemo + rads)
- Lymphocyte predominant (only surgery if localized)
Non-hodgkins
- Mature B cell: burkitt’s and diffuse B cell
- Precursor B/T cell: Lymphoblastic lymphoma: like leukemia, except <25% BM involvement
- Mature T cell: Anaplastic
Rough ddx:
<5 years:
Lymphoblastic lymphoma
> 5 years:
Hodgkin lymphoma
How does Hodgkin’s lymphoma typically present?
Painless cervical lymphadenopathy
Slow growing
1/3 present with B symptoms
What tests do you need to do pre-chemo for Hodgkins?
PFTs (Bleomycin)
Echo (Anthracyclines)
What is the prognosis of Hodgkin’s lymphoma?
> 90% cure
What chemotherapy agents are used in Hodgkin’s treatment and what are their late toxicities?
Akylator/Etoposide and RT- any
BUT risk of breast cancer 12-20% by 30y!
Anthracycline and RT- CV ds, heart failure
Bleomycin and RT- pulmonary fibrosis
What is more aggressive, NHL or HL?
NHL
Diffuse, aggressive, high grade
What is the average at diagnosis for NHL?
8-10yo
What are conditions that predispose to NHL?
Think immunodeficiency, especially in younger child with NHL:
Ataxia telangiectasia Wiskott-Aldrich syndrome Congenital hypogammaglobulinemia Post-solid organ transplant HIV
How does burkitt’s lymphoma typically present?
- 80% have abdominal mass (mimics appendicitis, intussuseption)
- Can be endemic (EBV driven)
- High risk of TLS
Differential diagnosis for splenomegaly?
1) HEMATOLOGIC
HEMOLYTIC:
- Thalassemia
- Sickle cell anemia
- Hereditary spherocytosis
- Autoimmune hemolysis
LYMPHOPROLIFERATIVE:
- Leukemia
- Lymphoma
- ALPS
MYELOPROLIFERATIVE:
- CML
- Polycythemia vera
- Essential thrombocytosis, Myelofibrosis
HISTIOCYTOSES:
- HLH
- LCH
ONCOLOGIC
- Neuroblastoma
- Metastases
- Hemangioma
INFECTIOUS
EBV, CMV, Toxoplasmosis, Viral hepatitis, Typhoid, Tuberculosis, Syphilis, HIV
Malaria, Leishmaniasis, Schistosomiasis
RHEUMATOLOGIC / INFLAMMATORY
Systemic lupus erythematosus
Rheumatoid arthritis
Sarcoidosis
INFILTRATIVE
Gaucher’s disease, Amyloidosis
CONGESTIVE Sequestration Hypersplenism Cirrhosis / Liver failure Hepatic vein thrombosis Cardiac failure
Differential diagnosis of anterior mediastinal mass?
Lymphoma
- Hodgkins
- NHL
Thymus
Teratoma
Thyroid
Name 4 complications of a mediastinal mass
Tracheal/Bronchial compression
SVC compression/obstruction (SVC Syndrome)
RVOT Obstruction
Pericardial effusion/tamponade
What are the two most common tumour to cause diplopia, headache and ataxia?
Posterior fossa-cerebellar astrocytoma, medulloblastoma
Brainstem gliomas are unlikely to cause raised ICP
Name 5 types of pediatric brain tumours
Low Grade/Pilocytic Astrocytoma Medulloblastoma (MOST COMMON PEDIATRIC MALIGNANT BRAIN TUMOUR) Ependymoma Glioblastoma Multiforme Brain Stem Glioma
How do supratentorial tumours typically present?
Localizing signs
SEIZURES
Visual changes
Hemiparesis , hemisensory loss, hyperreflexia
Subtle localizing signs/symptoms:
Behavioral problems: frontal lobe
Neuroendocrine deficits: hypothalamus/pituitary
DI, precocious/delayed puberty, hypothyroidism
Diencephalic syndrome in <3yo: FTT, hunger, euphoria
In what age group are supratentorial tumours more common?
Infants
Teens/adults
What is the differential for supratentorial tumours?
1) Glioma (low or high grade)
- Optic pathway/hypothalamic (eg NF1)
- Cerebral
2) Craniopharyngioma
3) Ependymoma
How do infratentorial tumours typically present?
Raised ICP (cerebellar>brainstem)
Ataxia(clumsineness, nystagmus)
Long tract signs (worsening handwriting, appendicular dysmetria)
Cranial Neuropathy (diplopia, difficulty swallowing)
In what age group are infratentorial tumours more common?
Children
What is the differential for infratentorial tumours?
1) Glioma (low or high grade)
- Cerebellar
- Brainstem
2) Medulloblastoma
3) Ependymoma
How do you distinguish between a brainstem and cerebellar tumour?
CN neuropathy, long tract signs, ataxia=Brainstem tumor
↑ ICP, ataxia in absence of other signs= Cerebellar tumor
What are headache red flags?
Change in type, increasing severity/frequency
Sudden/severe onset
Associated with trauma
Early AM, awakens from sleep, assoc w/ straining
Abnormal neurologic signs/symptoms
Seizures or loss of consciousness
Linear growth regression
School or behavior change
What type of brain tumour does CT head often miss?
Posterior fossa tumours
What are the clinical features of osteoid osteoma?
Dull pain worse at night
Better with NSAIDs
What are x-ray findings in osteoid osteoma?
Well-defined lucency surrounded by sclerotic bone
Proximal femur and tibia are most common sites
What are clinical features of osteochondroma?
Asymptomatic or non-painful bony mass
Lesion enlarges until reach skeletal maturity
What are the x-ray findings in osteochondroma?
Usually at metaphysis of long bones
Stalk/broad-based projection
What is an enchondroma?
Benign lesion of hyaline cartilage centrally in bone, often in hands
Asymptomatic usually
What is an aneurysmal bone cyst?
Reactive lesion of bone seen in persons <20 yr of age
Cavernous spaces filled with blood and solid aggregates of tissue
Where are aneurysmal bone cysts usually found?
Metaphysis of any bone
How do malignant bone tumours present?
Pain
Limp
Swelling
Nighttime awakening
At what age does osteosarcome typically present?
Around puberty (rapid bone growth)
What part of bones is osteosarcoma usually located?
Metaphysis of long bones (distal femur, proximal tibia/fibula)
What is the typical x-ray appearance of osteosarcoma?
Sunburst appearance*** Hair on end Osteoblastic activity Severe periosteal reaction with new bone formation Periosteal elevation Mottling***
Where does osteosarcoma metastasize?
Lungs
Bone
In what bones is Ewing’s sarcoma typically found?
Diaphysis of long bones or flat bones
Axial skeleton more common
What is the appearance of Ewing’s sarcoma on XR?
Onion-skinning on XR
Primary lytic lesion (moth-eaten radiolucency) with multilaminar periosteal reaction
Cortical destruction
Soft tissue mass
Where does Ewing’s metastasize?
Lungs
Bone
Bone marrow
How can you tell if leg pains are growing pains?
Children 4-12y Poorly localized, often lower limbs Occur at night but never persists until morning No disability Normal physical exam Normal x-rays
What is the malignant differential for abdominal mass?
In younger kids (<5yo), think of embryonal tumors (“blastoma”)
Wilms (nephroblastoma)
Neuroblastoma
Hepatoblastoma (< 3yo)
In older, think of:
Lymphoma - Burkitt’s! (older than 3yo)
Germ Cell Tumour (bimodal)
Sarcoma (EWS, Rhabdo)
What investigations do you order for abdominal mass?
Ultrasound
CT
CBC and Diff, lytes, urate, LDH Liver enzymes/function AFP, BHCG (GCTs) Urinanalysis Urine HVA/VMA (NB) Consider BMA