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168 Cards in this Set

  • Front
  • Back
Medulloblastoma

In children 2-5 and 12-15, where do most intracranial neoplasms arise?

What tumors comprise this group?
Posterior fossa

2/3 are medulloblastomas and cerebellar astrocytomas

37% Cerebellar astrocytoma
35% Medulloblastoma
11% Brain stem astrocytoma
7% Ependymoma
10% Other
Medulloblastoma characteristics
Originate from the vermis
Posterior to 4th ventricle
Grow rapidly and lead to hydrocephalus
Spread via subarachnoid spaces
Up to 50% with CSF mets at dx
Describe medulloblastoma on imaging.
Usually midline, hyperdense masses due to tightly packed nuclei with DNA and little cytoplasm
CSF seeding in 30%
Small cystic changes in up to 50%
Calcification in up to 20%
Hydrocephalus in 90%
Hypointense to gray matter on T1
Enhances intensely with contrast on both CT and MR
Describe medulloblastoma mets on imaging.
Brightly enhancing foci studding the meninges
Medulloblastoma
Where do systemic mets occur most frequently?
Skeleton with desmoplastic or sclerotic appearance.
Childhood CNS tumors
Supratentorial?
Infratentorial?
Childhood CNS tumors
Supratentorial: 47%
Infratentorial: 53%
Childhood CNS tumors

Overall incidence
- Medulloblastoma
- Astrocytoma
- Craniopharyngioma
Childhood CNS tumors

Overall incidence
- Medulloblastoma: 27%
- Astrocytoma: 26%
- Craniopharyngioma: 14%
Childhood CNS tumors

Posterior fossa only
- Medulloblastoma (PNET)
- Astrocytoma
- Ependymoma
Childhood CNS tumors

Posterior fossa only
- Medulloblastoma (PNET): 54%
- Astrocytoma: 23%
- Ependymoma: 17%
Incidence of calcification in brain tumors (adult)
Old Elephants Age Gracefully
O: Oligodendroglioma
E: Ependymoma
A: Astrocytoma, pilocytic
G: GBM
Cerebello-pontine angle cistern tumors
Vestibular schwannoma
Meningioma
Epidermoid
- Doesn't enhance
- Undulating margin
Glomus Tumor
Mets
Endolymphatic sac tumor
Extension from 4th or primary ependymoma
4th ventricle/Vermian mass
PNET
- Medulloblastoma
- Usually hyperdense on NCT
Ependymoma
- Usually heterogeneous
- Usually Ca++ and small cysts
Choroid plexus papilloma (adults)
Hemispheric cerebellar mass
Pilocytic astrocytoma (5-15 yrs)
Hemangioblastoma (35-45 yrs)
Mets
Infarction
Abscess (intense on DWI)
Sella or parasellar / Suprasellar
Pituitary adenoma (adults)
Craniopharyngioma
Hypothalamic glioma (kids)
Chordoma
Meningioma
Mets
Langerhans histiocytosis
Germinoma
Lymphoma
Sarcoid
Aneurysms
3rd ventricle
Colloid cyst
CPP
Ependymoma
Vertical extension of suprasellar mass
Anterior extension of pineal mass
Cysticercosis
Basal ganglia
Thalamus glioma (astrocytoma)
Hypertensive hematoma
Lateral ventricle
Foramen of Monro
- Subependymal giant cell astrocytoma
- Subependymoma
Body: Subependymoma
Trigone
- Choroid plexus papilloma (kids)
- Meningioma
Pineal region

(Quadrigeminal cistern)
Germ cell tumor
- Seminoma
= Germinoma
= Dysgerminoma
= Atypical teratoma
Pineal parenchyma
- Mature pineocytoma
- Immature pineoblastoma
Pineal cyst
Extension of glioma (astrocytoma)
Meningioma
Lipoma
Vein of Galen malformation
Epidermoid inclusion cyst
Corpus callosum
Enlarged, expanded, or enhancing
- Astrocytoma
- Lymphoma
Destructive, geographic
- Leukoencephalopathy
Patchy periventricular
- Vascular (HT change)
- Multifocal rings: Toxo in AIDS
Uniformly periventricular
- Hydrocephalic edema
Corpus callosum-splenium
Marchiafava-Bignami Disease
High altitude cerebral edema (HACE)
ADL (Adrenoleukodystrophy)
Subdural space
Subdural hematoma
Subdural empyema
Dural/subdural mets
- Prostate
- Breast
- Lymphoma
Granulomatous disease
- Sarcoid
- TB
- Fungal
Epidural space
Epidural hematoma
Epidural empyema
Dural/epidural mets
- Prostate
- Breast
- Lymphoma
Granulomatous disease
- Sarcoid
- Tuberculosis
- Fungal
Peds

What is the most common secondary malignant neoplasm inside the radiation field for patients with retinoblastoma?

A) Squamous cell carcinoma
B) Non-Hodgkin lymphoma
C) Fibrosarcoma
D) Osteosarcoma
D) Osteosarcoma
Peds

to the right femur. Biopsy results are consistent with neuroblastoma. What cancer stage would be most appropriate for this patient's condition?

A) Stage 2
B) Stage 3
C) Stage 4
D) Stage 4S
C) Stage 4
Peds

What percent of patients with neuroblastoma have metastases at diagnosis?

A) 10%
B) 25%
C) 60%
D) 80%
C) 60%
Peds

Which of the following types of abdominal tumor most commonly occurs in a 2-year-old child?

A) Neuroblastoma
B) Rhabdomyosarcoma
C) Non-Hodgkin lymphoma
D) Wilms tumor
D) Wilms tumor
Peds

Which of the following histologic subtypes of rhabdomyosarcoma has the worst prognosis?

A) Alveolar
B) Embryonal
C) Spindle cell
D) Botryoid
A) Alveolar
Peds

What percent of neuroblastomas exhibit N-myc amplification?

A) 5%
B) 25%
C) 50%
D) 75%
B) 25%
Peds

Which of the following body sites is most commonly involved in patients with rhabdomyosarcoma?

A) Thorax
B) Extremities
C) Head and neck
D) Genitourinary tract
C) Head and neck
Peds

What is the most common group classification of rhabdomyosarcoma at initial diagnosis?

A) Group I
B) Group II
C) Group III
D) Group IV
C) Group III
Peds

What is the most appropriate therapy for a 6-year-old child with a completely resected medulloblastoma with no evidence of metastasis?

A) Craniospinal irradiation to a dose of 23.4 Gy and 55.8-Gy boost dose to the posterior fossa
B) Concurrent chemotherapy with craniospinal irradiation to 23.4 Gy and 55.8-Gy boost dose to the posterior fossa, followed by additional chemotherapy
C) Concurrent chemotherapy with craniospinal irradiation to 36 Gy and 55.8-Gy boost dose to the posterior fossa, followed by additional chemotherapy
D) Induction chemotherapy, craniospinal irradiation to a dose of 36 Gy and 55.8-Gy boost dose to the posterior fossa, followed by additional chemotherapy
B) Concurrent chemotherapy with craniospinal irradiation to 23.4 Gy and 55.8-Gy boost dose to the posterior fossa, followed by additional chemotherapy
Peds

Which of the following tumors is LEAST likely to be treated with craniospinal irradiation?

A) Pineoblastoma
B) Ependymoblastoma
C) Nongerminomatous germ cell tumor
D) Pure germinoma
D) Pure germinoma
Peds

Which of the following eye tumors should be diagnosed clinically without a biopsy?

A) Retinoblastoma
B) Orbital rhabdomyosarcoma
C) Conjunctival lymphoma
D) Pseudolymphoma
A) Retinoblastoma due to concern for choroidal seeding with biopsy
Peds

Which of the following primary sites is most commonly involved in patients who are diagnosed with Ewing sarcoma?

A) Pelvis
B) Humerus
C) Fibula
D) Tibia
A) Pelvis
Peds

Which of the following radiation doses is standard definitive radiation therapy for an unresectable Ewing sarcoma?

A) 45.0 Gy
B) 55.8 Gy
C) 64.8 Gy
D) 70.2 Gy
B) 55.8 Gy
Peds

Which of the following genetic abnormalities is most commonly associated with Ewing sarcoma?

A) t(8;14)
B) t(11;22)
C) 1p/19q gene deletion
D) Loss of heterozygosity (LOH) of the retinoblastoma gene
B) t(11;22)
Peds

What is the most common site of metastatic disease in patients with Ewing sarcoma?

A) Lung
B) Bone
C) Bone marrow
D) Lymph nodes
A) Lung
Peds

A 3-year-old girl has Wilms tumor with a favorable histology and a positive surgical margin. CT scan of the chest does not reveal any pulmonary nodules. What cancer stage is most appropriate for the patient's condition?

A) Stage I
B) Stage II
C) Stage III
D) Stage IV
C) Stage III
Peds

Which of the following radiation dose ranges administered to the cranium would be most appropriate for providing CNS prophylaxis to patients with acute lymphoblastic

A) 5 Gy to 10 Gy
B) 12 Gy to 18 Gy
C) 24 Gy to 30 Gy
D) 36 Gy to 40 Gy
B) 12 Gy to 18 Gy
Peds

Which one of the following is an indication for radiotherapy in patients with Wilms' tumor?

A) A 5 mm pulmonary nodule identified on CT scan
B) Microscopic tumor involvement of a paraaortic lymph node
C) Bilateral kidney involvement with tumor
D) Histologic identification of nephrogenic rests in the tumor specimen
E) Tumor thrombus in the inferior vena cava
B) Microscopic tumor involvement of a paraaortic lymph node
Peds

All of the following have prognostic value in neuroblastoma, EXCEPT:

A) Presence of t(2;11) translocation
B) Number of copies of n-myc
C) Age of patient
D) Chromosomal ploidy
E) Serum ferritin level
A) Presence of t(2;11) translocation
Peds

Which one of the following disease entities has the LEAST in common with the others in the list?

A) Letterer-Siwe disease
B) Langerhans cell histiocytosis
C) Han-Schüller-Christian disease
D) Askin tumor
E) Eosinophilic granuloma
D) Askin tumor
Peds: Retinoblastoma

Are LP and BM biopsy indicated routinely in workup of retinoblastoma?
Not unless there's concern for more extensive disease
- Optic nerve involvement
- Extraocular extension
Peds: Retinoblastoma

Origin

Characteristics similar to neuroblastoma and medulloblastoma
Origin - nuclear layers of the retina

Characteristics similar to neuroblastoma and medulloblastoma
- Aggregation around blood vessels
- Necrosis
- Calcification
- Flexner-Wintersteiner rosettes
Peds: Retinoblastoma

Four growth patterns
Endophytic
- Grows from retina toward vitreous
- Appears as mass protruding into vitreous chamber
- Often friable and necrotic, detaching a forming satellite nodules = snowstorm effect from vitreous seeding

Exophytic
- Grows from retinal layers beneath detached retina toward choroid
- Erode through Bruch's membrane into choroid

Diffuse plaque-like
- Diffuse growth without mass formation
- Presents confusing clinical picture
Peds: Retinoblastoma

Trilateral retinbolastoma
Bilateral disease accompanied by pineal or suprasellar PNET
Peds: Retinoblastoma

Survival in children with tumors confined to the retina
> 95%
Peds: Retinoblastoma

Treatment of more advanced unilateral tumors

What if there's is vitreous seeding?
Enucleation is standard.

Tumors with vitreous seeding respond poorly to chemo so RT is usually indicated.
Peds: Retinoblastoma

What situations after enucleation usually required radiation and chemo?
Positive margin after enucleation or positive lymph nodes
Peds: Retinoblastoma

Typical RT doses
35-45 Gy depending on use of chemotherapy, response to treatment, and bulk of disease
- Reese-Ellsworth Group I/II: 36 Gy
- Reese-Ellsworth Group III/IV: 44-46 Gy

Goal is to cover entire retina and vitreous while maintaining ttolerance of normal tissues
Peds: Retinoblastoma

Chromosome on which Rb gene resides?
Chromosome 13
Peds: Retinoblastoma

Other treatment options
Cryotherapy
- Indicated for small tumors anterior to the equator that can be reached with the cryo probe
- Contraindicated if vitreous seeding

Photocoagulation
- 1-mm white reetinal burn made around tumor
- Regression based on interruption of blood supply
- Tumor not directly photocoagulated due to risk of vitreous seeding
- Contraindicated if pre-existing vitreous seeding

Radioactive plaque
- Solitary 2- to 16-mm diameter unilateral lesions
- Located > 3 mm from optic disc
Peds: Retinoblastoma

Risk of second cancer at 50 years after diagnosis of Rb
Hereditary 36% vs 6% for nonherditary

RT increases risk
- Nonirradiated: 26%
- Irradiated: 58%
- Rate of about 1% per year
Peds

For the following clinical description, select the one most appropriate 5-year survival that is MOST closely associated with it:

Stage 4 rhabdomyosarcoma
A) 0%
B) 20%
C) 40%
D) 60%
E) 80%
B) 20%
Peds

For the following clinical description, select the one most appropriate 5-year survival that is MOST closely associated with it:

Stage 4 Wilms' tumor
A) 0%
B) 20%
C) 40%
D) 60%
E) 80%
E) 80%
Peds

For the following clinical description, select the one most appropriate 5-year survival that is MOST closely associated with it:

Stage 4 Hodgkin's disease
A) 0%
B) 20%
C) 40%
D) 60%
E) 80%
E) 80%
Peds

For the following clinical description of rhabdomyosarcoma, select the one most appropriate radiotherapy dose that is MOST closely associated with it:

Unresectable embryonal rhabdomyosarcoma of the infratemporal fossa
A) 0 Gy
B) 30 Gy
C) 40 Gy
D) 50 Gy
E) 60 Gy
D) 50 Gy
Peds

For the following clinical description of rhabdomyosarcoma, select the one most appropriate radiotherapy dose that is MOST closely associated with it:

Completely-resected embryonal rhabdomyosarcoma of the neck with negative margins and histologically negative nodes
A) 0 Gy
B) 30 Gy
C) 40 Gy
D) 50 Gy
E) 60 Gy
A) 0 Gy
Peds

For the following clinical description of rhabdomyosarcoma, select the one most appropriate radiotherapy dose that is MOST closely associated with it:

Completely-resected embryonal rhabdomyosarcoma of the neck with negative margins and histologically positive nodes that are also completely resected
A) 0 Gy
B) 30 Gy
C) 40 Gy
D) 50 Gy
E) 60 Gy
C) 40 Gy
Peds

Regarding group III embryonal rhabdomyosarcoma: Actinomycin D is an active chemotherapy agent.

True
False
True
Peds

Regarding group III embryonal rhabdomyosarcoma: The local failure rate is approximately 50%.

True
False
False
Peds

Regarding group III embryonal rhabdomyosarcoma: A t(2;13) translocation is seen in approximately 60% of patients.

True
False
False
Peds

Regarding group III embryonal rhabdomyosarcoma: Patient age >10 years is a negative prognostic factor.

True
False
True
Peds

A 9-year-old child presents with headach, nausea and ataxia. A diagnosis of medulloblastoma is made and the tumor is completely resected. There is no evidence of dissemination beyond the primary site. Which one of the following is the MOST appropriate treatment?

A) High dose chemotherapy with stem cell rescue followed by 1800 cGy craniospinal irradiation (CSI) and a boost to the primary site
B) 3600 cGy CSI and a boost without chemotherapy
C) 1800 cGy CSI and a boost followed by standard dose chemotherapy
D) 2340 cGy CSI and a boost followed by standard dose chemotherapy
E) 3600 cGy CSI and a boost followed by standard dose chemotherapy
D) 2340 cGy CSI and a boost followed by standard dose chemotherapy
Peds

For the following presentation of neuroblastoma, select the one INSS stage that is MOST closely associated with it:

1-month-old with massive liver and skin metastases
A) 1
B) 2
C) 3
D) 4
E) 4S
E) 4S
Peds

For the following presentation of neuroblastoma, select the one INSS stage that is MOST closely associated with it:

3-year-old with localized, incompletely-resected tumor without lymph node or distant metastases
A) 1
B) 2
C) 3
D) 4
E) 4S
B) 2
Peds

For the following presentation of neuroblastoma, select the one INSS stage that is MOST closely associated with it:

1-year-old with resected midline tumor and bilateral lymph node involvement but no distant metastases
A) 1
B) 2
C) 3
D) 4
E) 4S
C) 3
Peds

For the following pediatric malignancy, select the one radiation dose that is generally recommended:

Neuroblastoma
A) 1080 cGy
B) 2100 cGy
C) 3000 cGy
D) 3600 cGy
E) 4500 cGy
B) 2100 cGy
Peds

For the following pediatric malignancy, select the one radiation dose that is generally recommended:

Wilms' tumor
A) 1080 cGy
B) 2100 cGy
C) 3000 cGy
D) 3600 cGy
E) 4500 cGy
A) 1080 cGy
Peds

For the following pediatric malignancy, select the one radiation dose that is generally recommended:

Hodgkin's disease
A) 1080 cGy
B) 2100 cGy
C) 3000 cGy
D) 3600 cGy
E) 4500 cGy
B) 2100 cGy
Peds

Favorable prognostic factors in patients with rhabdomyosarcoma include: Alveolar histology

True
False
False
Peds

Favorable prognostic factors in patients with rhabdomyosarcoma include: Vaginal primary site

True
False
True
Peds

Favorable prognostic factors in patients with rhabdomyosarcoma include: Age 2-10

True
False
True
Peds: Ewing's Sarcoma

Frequency

Most common site of presentation
Second most common childhood bone tumor

Long bones of extremities and pelvis
Peds: Ewing's Sarcoma

Negative prognostic factors
Presence of mets
Axial location, esp pelvis
Large tumor size
Elevated LDH
Poor histologic response to induction chemo at surgery
Older age
Peds: Ewing's Sarcoma

Characteristic translocation

Marker expression versus PNETs
90%: t(11;22)
10%: t(21;22)

Ewing's typically neuron-specific enolase negative while PNETs are positive
Peds: Ewing's Sarcoma

Treatment
Multiagency induction chemo: Alternating cycles of VAC/EI
- Vincristine
- Adriamycin
- Cyclophosphamide
- Ifosfamide
- Etoposide
Then local treatment at week 12
- Surgery
- RT
-- 45 Gy to all pre-chemo bony abnormalities and soft tissues masses
-- 10.4 Gy boost to pre-chemo bony tumor volume and post-chemo soft tissue volume
-- Total 55.8 Gy to gross residual disease
-- Total 50.4 Gy to microscopic disease after resection
- Both
Peds: Ewing's Sarcoma

RT for pulmonary mets
15-18 Gy whole lung radiation
Peds: Ewing's Sarcoma

5-year DFS in localized disease

5-year DFS in metastatic disease
5-year DFS in localized disease: 50-75%

5-year DFS in metastatic disease: 20-25%

Lung-only mets affords better prognosis than multiple sites of metastases.
Peds

Favorable prognostic factors in patients with rhabdomyosarcoma include: t(2;13) translocation

True
False
False
Peds

Favorable prognostic factors in patients with rhabdomyosarcoma include: Extremity primary site

True
False
False
Peds

For patients with metastatic soft tissue sarcomas, which one of the following chemotherapy agents is MOST likely to result in tumor response?

A) Doxorubicin
B) Ifosfamide
C) Decarbazine
D) Cyclophosphamide
E) Dactinomycin
A) Doxorubicin
Peds

Following radiation therapy, which included the elbow or knee, for patients with soft tissue sarcoma, which one of the following was the MOST frequent sequela?

A) Joint stiffness
B) Nerve damage
C) Bone fracture
D) Need for wound re-operation
E) Skin ulceration
A) Joint stiffness
Peds

Regarding leiomyosarcomas in children: These tumors account for about 20% of soft tissue sarcomas in children.

True
False
False
Peds

Regarding leiomyosarcomas in children: The most frequent primary tumor sites are the gastrointestinal tract and the head and neck.

True
False
True
Peds

Regarding leiomyosarcomas in children: Surgery alone rarely is adequate treatment.

True
False
False
Peds

Regarding leiomyosarcomas in children: Leiomyosarcomas "behave better" in children than in adults, in part related to anatomic sites frequently involved.

True
False
True
Peds

Regarding leiomyosarcomas in children: Late recurrences have been described.

True
False
True
Peds

Which one of the following is the MOST common sarcoma in children younger than 10 years?

A) Ewing's sarcoma
B) Osteosarcoma
C) Rhabdomyosarcoma
D) Synovial sarcoma
C) Rhabdomyosarcoma
Peds

Which one of the following pediatric sarcomas frequently arises in bone as well as soft tissue?

A) Ewing's sarcoma
B) Osteosarcoma
C) Rhabdomyosarcoma
D) Synovial sarcoma
A) Ewing's sarcoma
Peds

Children with which one of the following diseases are at highest risk of developing a radiation-induced second cancer?

A) Hodgkin's disease
B) Acute lymphoblastic leukemia
C) Ewing's sarcoma
D) Retinoblastoma
D) Retinoblastoma
Peds

A 3-month-old child is diagnosed with Reese-Ellsworth stage 3 retinoblastoma of the right eye. Family history is significant for 2 uncles with retinoblastoma. Which one of the following is CORRECT?

A) The child has a greater than 50% chance of developing retinoblastoma in the left eye
B) The child is likely male
C) Eunucleation should result in a normal life expectancy
D) Cure rate is approximately 50%
A) The child has a greater than 50% chance of developing retinoblastoma in the left eye
Peds

A 5-year-old girl is diagnosed with a Wilms' tumor of the left kidney without evidence of local spread. Staging evaluation shows a .5-cm metastasis to a single site. Which one of the following single metastatic sites would be associated with the BEST prognosis?

A) Bone marrow
B) Brain
C) Femur
D) Lung
D) Lung
Peds

For patients with nonmetastatic Ewing's sarcoma, radiotherapy is most likely to be used for local control for which one of the following sites?

A) Fibula
B) Lumbar spine
C) Calvarium
D) Clavicle
B) Lumbar spine
Peds

For the following primary site of rhabdomyosarcoma, select the one lettered statement that is MOST closely associated with it:

Prostate
A) 3-year-old with botryoid histology
B) 13-year-old with alveolar histology
C) 4-year-old with embryonal histology
D) 15-year-old with botryoid histology
E) 16-year-old with embryonal histology
C) 4-year-old with embryonal histology
Peds

For the following primary site of rhabdomyosarcoma, select the one lettered statement that is MOST closely associated with it:

Paratestes
A) 3-year-old with botryoid histology
B) 13-year-old with alveolar histology
C) 4-year-old with embryonal histology
D) 15-year-old with botryoid histology
E) 16-year-old with embryonal histology
E) 16-year-old with embryonal histology
Peds

For the following primary site of rhabdomyosarcoma, select the one lettered statement that is MOST closely associated with it:

Proximal thigh
A) 3-year-old with botryoid histology
B) 13-year-old with alveolar histology
C) 4-year-old with embryonal histology
D) 15-year-old with botryoid histology
E) 16-year-old with embryonal histology
B) 13-year-old with alveolar histology
Peds

For the following chemotherapy agent used for Ewing's sarcoma, select the one lettered late complication that is MOST closely associated with it:

Vincristine
A) Cystitis
B) Peripheral neuropathy
C) Interstitial pneumonitis
D) Secondary acute myelogenous leukemia
E) Cardiomyopathy
B) Peripheral neuropathy
Peds

In a recent study, the use of MAID (mesna, adriamycin, ifosfamide, and decarbazine) for patients with soft tissue sarcoma = 8.0 cm in largest dimension improved the disease-free survival compared to historical controls to what new level?

A) From 40% to 70%
B) From 40% to 50%
C) From 25% to 70%
D) From 25% to 50%
E) From 25% to 40%
A) From 40% to 70%
Peds: Wilms' Tumor

Most common presentation

Associated syndromes
Most common presentation - painless abdominal mass

Associated syndromes
- WAGR
-- Wilms' tumor
-- Aniridia
-- GU anomalies
-- Mental retardation
- Beckwith-Wiedemann (overgrowth syn)
-- Visceromegaly
-- Macroglossia
-- Hypoglycemia
Peds: Wilms' Tumor

Pathology
- Favorable
- Anaplastic

Most common met site

Most important prognostic factor
Pathology
- Favorable: 90%
- Anaplastic: 4%

Most common met site: lung

Most important prognostic factor: Histology
Peds: Wilms' Tumor

Staging
National Wilms' Tumor Study Staging

I: Confined to kidney and completely resected
II: Extends beyond kidney but completely resected
- a: Penetration of renal capsule
- b: Invasion of renal sinus vessels
- c: Biopsy of tumor before removal
III: Gross or micro residuum postop
- Inop tumor, positive margins, spillage
- Regional nodes, transected tumor thrombus
IV: Mets outside abdomen
V: Bilateral at onset
Peds: Wilms' Tumor

Risk stratification
Very low risk
- < 2 yoa, stage I, kidney < 550g
- 85% cured with surgery alone
Low risk
- Stage I and II without LOH 1p or 16q
- Tx: Nephrectomy then vincristine and actinomycin-D
Standard risk
- Stage I and II with LOH at 1p and 16q
- Tx: Nephrectomy then vincristine, actinomycin-D, and doxorubicin
Higher risk
- Stage III FH with LOH 1p and 16q
- Tx: Nephrectomy VAD+cyclophosphamide and etoposide and PORT
High risk
- Anaplastic, clear cell
- Tx: Nephrectomy --> chemo
Peds: Wilms' Tumor

Adj RT?
None for stage I and II FH

Stage III: 10 Gy with 10-Gy boost to gross disease

Diffuse peritoneal implants or spillage: WAI 10 Gy due to higher risk of relapse despite stage II classification for spillage.

Rhabdoid: 20 Gy > 1 yr and 10 Gy < 1 yr
Peds: Rhabdomyosarcoma

Freq among soft tissue sarcoma in children

5-year OS with localized disease

Most common locations of primary disease
Most common soft tissue sarcoma in children:

5-year OS with localized disease: >70%

Most common locations of primary disease
1 Head and neck
2 Genitourinary tract
3 Extremities
Peds: Rhabdomyosarcoma

Two major histologic subtypes

Characteristic translocations
Two major histologic subtypes
1 Alveolar
- Less common, worse prognosis
- Extremity tumors
2 Embryonal
- More common, better prognosis
- Head & neck and GU tumors
- Embryonal variants
A Botryoid
B Spindle cell

Characteristic translocations: t(2;13) and t(1;13)
Peds: Rhabdomyosarcoma

IRS Grouping System
I: Localized disease, completely resected
A: Confined to muscle or organ of origin
B: Infiltration outside the muscle of origin
II: Total gross resection with
A: Microscopic residuum
B: Regional lymphatic spread, resected
C: Both
III: Incomplete resection with gross residuum
A: After biopsy only
B: After major resection (>50%)
IV: Distant mets present at dx
Peds: Rhabdomyosarcoma

Risk stratification
Low risk
- Complete resection or microscopic residuum
- Localized embryonal disease at favorable or unfavorable site
Intermediate risk
- Embryonal with gross residuum at unfavorable site
- Alveolar histology
High risk - Mets at dx
Peds: Rhabdomyosarcoma

Off-protocol RT dose
At least 50.4 Gy to bulky or gross residuum

36-41.1 Gy for microscopic disease
Peds: ALL

Freq among childhood cancers

Freq among leukemias

Current 5-year OS
Freq among childhood cancers: Most common malignancy of childhood (30%)

Freq among leukemias: Most common leukemia

Current 5-year OS: >80%
Peds: ALL

Role of RT


RT Dose
limited to high-risk patients
- WBC > 50K
- B-precursor disease
- Age > 10 years
- T-cell ALL
- t(9;22) Philadelphia chromosome
- CNS involvement

Reduced from 24 Gy to 12-18 Gy
Peds: Lymphoma

Freq among children
Third most common malignancy in children
- 50% NHL
- 50% HL

NHL RT reserved for palliation, emergent intervention for mediastinal or cord compression, adj therapy after incomplete remission, and consolidation after transplant for recurrent disease
Peds: HL

Curability

10-year OS

Unfavorable factors
1
2
3
4
5
Curability: Highly!

10-year OS > 90%

Unfavorable factors
1 B symptoms
2 Bulky mediastinal or peripheral nodes
3 Extranodal extension
4 Hilar nodal involvement
5 Advanced stage
Peds: HL

RT role

RT dose
RT role: Involved field RT

RT dose: 15-25 Gy depending on bulkiness and response to treatment
Peds: Neuroblastoma

Origin

Freq

Age at presentation
Arise from embryonic neural crest cells
- In parasympathetic nervous system
- In the adrenal gland
- In paraspinal sympathetic ganglia

Most common extracranial solid tumor of childhood

90% present < age 10 (median 2 years)
Peds: Neuroblastoma

Three main subtypes

IHC
Three main subtypes (by diff)
1 Neuroblastoma
2 Ganglioneuroblastoma
3 Ganglioneuroma

IHC
+ NSE
+ Synaptophysin
+ Chromogranin A
+ Neuronal filaments
Peds: Neuroblastoma

Shimada classification criteria

LOH that confers worse prognosis
Patient age
Presence of stroma and nodularity
Mitosis-karyorrhexis index
Degree of differentiation

LOH 1p and 11q confers worse prognosis
Peds: Neuroblastoma

Typical presentations

Classic imaging finding
Typical presentations
1 Abdominal mass
2 Dumbbell configuration with tumor extension through neuroforamina causing cord compression

Classic imaging finding: soft tissue suprarenal mass with calicifications
Peds: Neuroblastoma

Prognosis by age

Prognosis children vs adults
Prognosis by age: Children < one year at dx have higher OS and DFS

Children have better prognosis than adults
Peds; Neuroblastoma

Low risk criteria

Tx

Px
Low risk
- Stage I, II with favorable histology
- Favorable DNA ploidy, no myc amplication

Tx: surgery alone

Px: 5yr OS > 95% EFS 90%
Peds: Neuroblastoma

Intermediate risk criteria

Tx

Px
Intermediate risk
- Stage III < 1 year
- Older than 1 year with no adverse features
- Infants with stage IV disease

Tx
- Multi-drug chemo for regional and metastatic disease
- Resection of primary
- RT if progression after chemo and surgery: 24 Gy

Px: 5yr OS >90% EFS >87%
Peds; Neuroblastoma

High risk criteria

Tx

Px
High risk criteria
- Older than one year with disseminated disease
- Localized disease with myc amplification

Tx-Aggressive multimodality
- Multi-agent chemo
- Resection then RT
- Consolidation chemo

Px: 5yr OS 30-60%
Peds: Neuroblastoma

Cord compression tx

Peri-orbital mets causing vision compromise
Cord compression tx
1 Chemo - quickly responsive
2 Surgery
3 RT: Limit to 4.5-6 Gy / 3-4 Fx
Peds: Neuroblastoma

Staging
INSS
I: Localized with complete gross and microscopic excision and nonadherent neg nodes
IIA: Localized tumor with incomplete gross excision and non-adherent negative nodes
IIB: Localized tumor completely or not excised with ipsi adherent positive nodes
III
- Unresectable unilaterl tumor infiltrating across midline +/- nodes
- Localized unilateral tumor with positive contralateral nodes
- Midline tumor with bilateral extension by infiltration (unresectable) or nodes
IV: Spread to distant nodes, bone, bone marrow, liver, skin
IVS: < one year with stage I, IIA, or IIB with dissemination to skin, liver, bone, +/- bone marrow
Peds

For the following chemotherapy agent used for Ewing's sarcoma, select the one lettered late complication that is MOST closely associated with it:

Doxorubicin
A) Cystitis
B) Peripheral neuropathy
C) Interstitial pneumonitis
D) Secondary acute myelogenous leukemia
E) Cardiomyopathy
E) Cardiomyopathy
Peds

For the following chemotherapy agent used for Ewing's sarcoma, select the one lettered late complication that is MOST closely associated with it:

Etoposide
A) Cystitis
B) Peripheral neuropathy
C) Interstitial pneumonitis
D) Secondary acute myelogenous leukemia
E) Cardiomyopathy
D) Secondary acute myelogenous leukemia
Peds

Concerning prognostic factors for rhabdomyosarcoma: Alveolar histology is unfavorable

True
False
True
Peds

Concerning prognostic factors for rhabdomyosarcoma: Extremity site is favorable

True
False
False
Peds

Concerning prognostic factors for rhabdomyosarcoma: Age 2-10 years is favorable

True
False
\
True
Peds

Concerning prognostic factors for rhabdomyosarcoma: Botryoid histology is unfavorable

True
False
False
Peds

Concerning prognostic factors for rhabdomyosarcoma: Orbit primary site is unfavorable

True
False
False
Peds

Concerning germ cell tumors of the CNS: High levels of beta human ehorionic gonadotropin (B-hCG) are associated with yolk sac tumors (endodermal sinus tumors)

True
False
False
Peds

Concerning germ cell tumors of the CNS: The most common sites are suprasellar and pineal

True
False
True
Peds

Concerning germ cell tumors of the CNS: Nongerminoma germ cell tumors rarely spread through the CSF

True
False
False
Peds

Concerning germ cell tumors of the CNS: Germinomas produce alpha fetoprotein (AFP)

True
False
False
Peds

Concerning germ cell tumors of the CNS: A cure rate of approximately 90% can be achieved with radiotherapy alone for germinomas

True
False
True
Peds

Cognitive difficulties after CNS irradiation in children correlate with: Increasing radiation dose

True
False
True
Peds

Cognitive difficulties after CNS irradiation in children correlate with: Age younger than 8 years at the time of radiotherapy

True
False
True
Peds

Cognitive difficulties after CNS irradiation in children correlate with: Prior exposure to topoisomerase-inhibiting chemotherapy agents

True
False
False
Peds

Cognitive difficulties after CNS irradiation in children correlate with: Asian ethnicity

True
False
False
Peds

Involvement of the following site by neuroblastoma is NOT compatible with a designation of stage IV-S:

Liver

True
False
False
Peds

Involvement of the following site by neuroblastoma is NOT compatible with a designation of stage IV-S:

Bone

True
False
False
Peds

Involvement of the following site by neuroblastoma is NOT compatible with a designation of stage IV-S:

Lung

True
False
True
Peds

Involvement of the following site by neuroblastoma is NOT compatible with a designation of stage IV-S:

Skin

True
False
False
Peds

Involvement of the following site by neuroblastoma is NOT compatible with a designation of stage IV-S:

Brain

True
False
True
Peds

What is the most common cancer in children?

A) Medulloblastoma
B) Wilms tumor
C) Acute lymphoblastic leukemia
D) Neuroblastoma
C) Acute lymphoblastic leukemia
Peds

A child with leukemia is to undergo prophylactic irradiation of the CNS. Which of the following fields should be treated?

A) Entire craniospinal subarachnoid space
B) Entire cranial subarachnoid space extending to base of skull
C) Entire cranial subarachnoid space extending to second cervical vertebra
D) Entire cranial subarachnoid space extending to second cervical vertebra and posterior orbits
D) Entire cranial subarachnoid space extending to second cervical vertebra and posterior orbits
Peds

Which of the following radiation doses is currently used in Children's Oncology Group (COG) studies for CNS prophylaxis of patients with acute lymphoblastic leukemia?

A) 12.6 Gy
B) 18.0 Gy
C) 24.0 Gy
D) 36.0 Gy
A) 12.6 Gy
Peds

An asymptomatic 5-year-old child with neurofibromatosis type 1 (NF1) has a low-grade glioma of the optic chiasm. Recommended management should consist of which of the following?

A) Biopsy, followed by chemotherapy and low-dose irradiation to 40 Gy
B) Intensity-modulated radiation therapy (IMRT) to 50.4 Gy
C) Gross total resection, followed by six cycles of chemotherapy with vincristine and carboplatin
D) Close monitoring with imaging, visual, and neurologic testing
D) Close monitoring with imaging, visual, and neurologic testing
Peds

Which of the following tumors is most likely to be associated with Parinaud syndrome?

A) Cerebellar astrocytoma
B) Posterior fossa ependymoma
C) Craniopharyngioma
D) Pineal germinoma
D) Pineal germinoma
Peds

Which of the following tumors does NOT require craniospinal irradiation?

A) Nongerminomatous germ cell tumor
B) Ependymoblastoma
C) Astroblastoma
D) Pineoblastoma
C) Astroblastoma
Peds

Which of the following statements about radiation therapy for Ewing sarcoma is FALSE?

A) MRI of the primary site should be used to determine the local extent of the tumor.
B) The whole bone should be irradiated to 39.6 Gy, followed by a boost to 16.2 Gy.
C) Before initiation of chemotherapy, the bone and soft tissue tumor need to be included in the original field that receives 45 Gy.
D) All children receive multiagent chemotherapy during radiation therapy.
B) The whole bone should be irradiated to 39.6 Gy, followed by a boost to 16.2 Gy.
Peds

Which of the following statements about osteosarcoma is true?

A) Osteosarcoma is the second most common primary malignant bone tumor in children.
B) The majority of patients are in the first decade of life.
C) Osteosarcoma typically occurs in the diaphysis of long bones.
D) When the bony cortex is penetrated at the edge of the tumor, there may be a periosteal elevation and the clinical sign of a Codman triangle.
D) When the bony cortex is penetrated at the edge of the tumor, there may be a periosteal elevation and the clinical sign of a Codman triangle.
Peds

Which of the following statements about the National Wilms Tumor Study (NWTS) staging system is true?

A) Positive margins of resection is classified as stage II.
B) Tumor extending into the renal sinus, but completely excised is classified as stage I.
C) Tumor involving the regional lymph nodes is classified as stage III.
D) Bilateral renal involvement is classified as stage IV.
C) Tumor involving the regional lymph nodes is classified as stage III.
Peds

Which of the following radiation doses was used for stage III Wilms tumor with favorable histology in the National Wilms Tumor Study-5?
A) 10 Gy
B) 20 Gy
C) 30 Gy
D) 40 Gy
A) 10 Gy
Peds

Which of the following findings does NOT require irradiation of the flank or abdomen after nephrectomy?

A) Stage II diffuse anaplastic tumor
B) Stage II clear cell sarcoma of the kidney
C) Stage II Wilms tumor with favorable histology
D) Wilms tumor with diffuse periotoneal tumor spillage and favorable histology
C) Stage II Wilms tumor with favorable histology
Peds

Which of the following National Wilms Tumor Studies (NWTX) demonstrated that in patients who have Wilms tumor with favorable histology the flank irradiation dose could be reduced to 10 Gy when therapy combines use of vincristine, dactinomycin, and doxorubicin chemotherapy or to 20 Gy when used in conjunction with vincristine and dactinomycin?

A) NWTS-1
B) NWTS-2
C) NWTS-3
D) NWTS-4
C) NWTS-3
Peds

The Reese-Ellsworth staging system for retinoblastoma is predictive of:

A) overall survival.
B) disease-free survival.
C) preservation of vision.
D) extent of metastatic disease.
C) preservation of vision.
Peds

The retinoblastoma gene is an exampole of a:
A) tyrosinase kinase-receptor gene.
B) tumor-suppressor gene.
C) checkpoint gene mutation.
D) DNA repair enzyme.
B) tumor-suppressor gene.
Peds

What is the most common abdominal malignancy in 15-year-old children?

A) Wilms tumor
B) Neuroblastoma
C) Lymphoma
D) Ewing sarcoma
C) Lymphoma
Peds

What is the most important prognostic factor for patients with Wilms tumor?

A) Age at diagnosis
B) Tumor size
C) Lymph node involvement
D) Histology
D) Histology
Peds

A 2-year-old child has Wilms tumor with favorable histology. On review of pathologic and surgical findings, the radiologist determines that the tumor penetrated the renal capsule with no invasion of the renal hilium. The tumor is completely resected without an intraoperative spill. Which of the followintg statements about management of this patient's disease is true? A) The patient has stage I disease that should be managed with radiation therapy to a dose of 21 Gy delivered to the flank.
B) The patient has stage II disease that should be managed with radiation therapy to a dose of 21 Gy delivered to the flank.
C) The patient has stage II disease that should be managed with radiation therapy to a dose of 10.8 Gy delivered to the flank.
D) The patient has stage II disease and does not receive radiation therapy.
D) The patient has stage II disease and does not receive radiation therapy.
Peds

Prognostic factors for patients with neuroblastoma do NOT include which of the following?

A) Shimada classification
B) Primary site of disease
C) N-myc amplification status
D) Age
B) Primary site of disease
Peds

A 2-year-old child with an abdominal mass and elevated homovanillic acid (HVA) and vanillylmandelic acid (VMA) levels should NOT have which of the following studies?

A) Bone scan
B) Bone survey
C) Metaiodobenzylguanidine (MIBG) scan
D) MRI of the brain
D) MRI of the brain
Peds

What is the most common histology of rhabdomyosarcoma in the head and neck region?

A) Embryonal
B) Botryoidal
C) Alveolar
D) Plemorphic
A) Embryonal
Peds

What is the most common clinical group of rhabdomyosarcoma at initial presentation?

A) Group I
B) Group II
C) Group III
D) Group IV
C) Group III
Peds

A 7-year-old patient presents with a 6-cm alveolar rhabdomyosarcoma of an extremity and undergoes biopsy. The metastatic work-up is otherwise negative. How should this patient's disease be classified?

A) Stage 2, clinical group II
B) Stage 2, clinical group III
C) Stage 3, clinical group II
D) Stage 3, clinical group III
D) Stage 3, clinical group III
Peds

For which of the following pediatric malignancies is age less than one year a favorable prognostic factor?

A) Medulloblastoma
B) Neuroblastoma
C) Wilms tumor
D) Rhabdomyosarcoma
B) Neuroblastoma
Peds

Which of the following is the most common non-rhabdomyosarcoma soft tissue sarcoma in children?

A) Fibrosarcoma
B) Synovial sarcoma
C) Malignant fibrous histiocytoma
D) Liposarcoma
B) Synovial sarcoma