EXAM #3: CHILDHOOD CANCER II Flashcards
What is Acute Lymphoblastic Leukemia?
Hyperproliferation of lymphoid progenitor
What gender is ALL more common in?
Males
What are the signs and symptoms of Luekemia?
Constellation of symptoms associated with:
1) Anemia
2) Thrombocytopenia
3) Neutropenia
AND bone pain that causes a limp/decreased walking
What luekemia is gingival hypertrophy associated with?
Monoblastic leukemia
What are the CBC findings that are diagnostic for Leukemia?
1) Increased WBC
2) Lymphoblasts on PBS
3) 2+ cytopenias
What should be included in the differential for Leukemia?
- EBV (Mono)
- Acute anemia
- Parvovirus B19
- ITP
Kids with what genetic abnormality are at an increased risk for ALL?
Down’s Syndrome
What are the nongenetic risk factors for ALL?
X-rays/ radiation
What is the most common type of ALL?
B-ALL
What is the most common involvement of B-ALL?
Extramedullary
What group of children is T ALL more common in?
Male adolescents
What is the presentation of T-ALL?
- Mediastinal mass
- High WBC mass
- LAD that resembles Mumps
What is the greatest risk factor in B-ALL?
Response to induction therapy in the first 28 days
What is the most common solid tumor of childhood?
Brain tumors
What is the most common brain tumor of childhood?
Astrocytomas
Note that this is specifically a low grade JPA
Where is the most common location for an astrocytoma?
Posterior fossa
What is the standard of care for an astrocytoma?
Total resection i.e. neurosurgery
What are the signs and symptoms of a brain tumor?
- Neuro/ increased ICP signs/sx
- Regression of milestones
Why is chemotherapy relatively ineffective in treating astrocytomas?
- Low grade means less proliferation
- Slow growing tumors are hard to target with chemotherapy
Radiation has a potential for use in all tumors but which one?
Choroid plexus tumors
What are the long-term effects of radiation therapy in children for brain tumors?
1) Neuro-cognitive impairment
2) Hearing changes
3) Secondary cancer
4) Endocrine abnormalities
5) Skeletal growth abnormalities
What disease are astrocytomas most commonly associated with? What is second?
1) NF-1
2) Li-Fraumeni Syndrome
What is a Wilm’s tumor?
Renal tumor that is the 2nd most common type of abdominal malignancy in children
What gene is mutated in Wilm’s tumors?
WT1 tumor suppressor mutation
What race are Wilm’s tumors more common in?
African Americans
What are the symptoms associated with Wilm’s Tumor?
- HTN
- Hematuria
- Fever
Describe the etiology of Retinoblastoma.
Loss of both Rb alleles
- 60% are spontaneous
- 40% are hereditary
Where are neuroblastomas located? Where are the common metastases of neruoblastomas located at diagnosis?
Any neural crest tissue for primary tumor i.e.
- Adrenal tissue
- Parasympathetic tissue (cerivical, thoracic, pelvic)
Mets: bone, lymph nodes, skin, liver
What are the signs and symptoms of a neuroblastoma?
- Proptosis
- Bone lesions
- Periorbital ecchymoses i.e. “Racoon eyes”
How do neuroblastomas commonly present?
1) Large abdominal mass that crosses the midline
2) LE weakness
3) Cervical or high thoracic mass causing Horner’s Syndrome
What is the average age of onset for a neuroblastoma?
18 months
What is the most common extracranial solid tumor in children?
Neuroblastoma
What is the most common type of soft tissue sarcoma?
Rhabdomyosarcoma
What are the signs and symptoms of a Rhabdomyosarcoma involving the head and neck?
Proptosis
Periorbital swelling
What are the signs and symptoms of a parameningeal rhabdomyosarcoma?
1) CN palsy
2) Hearing loss
3) Chronic aural or sinus drainage
What are the signs and symptoms of a male GU rhabdomyosarcoma?
- Hematuria
- Urinary obstruction
What are the signs and symptoms of a paratesticular rhabdomyosarcoma?
Pain testicular mass
What are the signs and symptoms of a vaginal/uteral rhabdomyosarcoma?
1) Abdominal mass
2) Vaginal mass
3) Vaginal bleeding or discharge
What is a Rhabdomyosarcoma?
Undifferentiated mesenchymal cells that differentiate into muscle
At what age do Ewing’s Sarcomas peak?
11-15 years old
How do Ewing’s Sarcomas present?
Limb pain and swelling, frequently after a sports related injury
More common in boys*
Where do osteosarcomas typically present?
Long bones metaphyses (wide part near growth plate):
- Distal femur
- Proximal tibia
- Proximal humerus
- Pelvis
Where do Ewing Sarcoma’s typically present?
Long bone diaphyses (narrow part)
Flat bones
