Oncology 2

Question 0

Brain tumors by incidence?

Answer 0

1. Glial cell tumors (astrocytomas)
2. primitive neuroectodermal tumors (medulloblastoma)
3. ependymomas
4. craniopharyngiomas

Question 1

Most common type of brain tumor? High-grade arise from? Low-grade arise from?

Answer 1

Glial cell tumors; supratentorial region, infratentorial region

Question 2

Features of optic glioma?

Answer 2

Diminished vision, visual field deficits, strabismus

Question 3

Features of craniopharyngioma?

Answer 3

1. Growth Retardation, delayed puberty
2. Visual changes
3. Diabetes insipidus and other hormonal problems

Question 4

Tumor markers:

1. Germs cell tumors?
2. Medulloblastoma?

Answer 4

1. AFP or B-HCG

2. Homovanillic acid, vanillylmandelic acid, Polyamines

Question 5

Prognosis for

1. astrocytoma?
2. Medulloblastoma?
3. Brainstem gliomas?

Answer 5

1. > 75% if low-grade; 35% if high-grade
2. Greater than 75% if resectable and child is over four years
3. Poor prognosis – not resectable and Chemotherapy is ineffective

Question 6

Second most common solid tumor? Peak incidence?

Answer 6

Neuroblastoma (tumor of neural crest cells); within first five years of life;

Pelvis >mediastinum > neck

Question 7

Clinical features of neuroblastoma – Based on location? All tumors may have?

Answer 7

Pelvic – abdominal mass crossing midline, abdominal pain, anorexia

Mediastinal – respiratory distress, incidental finding

Cervical location – tracheal compression, Horners

1. Catecholamine effects – flashing, hypertension, headache, sweating,
2. Non-Specific – fever, weight loss
3. Acute cerebellar atrophy

Question 8

Acute cerebellar atrophy?

Answer 8

Ataxia, monoclonals, opsoclonus (random eye jerks)

Question 9

Neuroblastoma – prognosis? (Serum markers of poor prognosis?)

Answer 9

Good prognosis in children under 1

Poor prognosis associated with N-myc, ferritin, lactic dehydrogenase, neuron specific Enolase

Question 10

Wilm’s tumor – age of onset? Associated genetic syndromes? Clinical features?

Answer 10

<5 years; Beckwith-Weigman, chromosome 11 deletion, WAGR

1. Abdominal mass that does not cross the midline
2. abdominal pain
3. Hematuria
4. Hypertension
5. GU abnormalities
6. Hemihypertrophy, aniridia

Question 11

Treatment for Wilms tumor? Prognosis?

Answer 11

Chemotherapy; add radiation for advanced disease

Cure rare >90%

Question 12

Rhabdomyosarcoma – age of onset? Associated with? Initial presentation? Most common sites of involvement? Diagnosis? Treatment?

Answer 12

<10 years; neurofibromatosis

Painless soft tissue mass

1. Head and neck
2. GU tract
3. Extremities

Biopsy

Complete surgical resection with adjunct chemo/radiation

Question 13

Specific location of head/neck rhabdomyosarcomas (and Symptoms)?

Answer 13

1. Orbit – proptosis, eyelid swelling, nerve palsies
2. Nasal pharyngeal – epistaxis, chronic sinusitis, airway obstruction
3. Laryngeal – hoarseness

Question 14

Most common malignant bone tumor? Incidence when? Associated with? Diagnosis? Management?

Answer 14

Osteogenic sarcoma; growth spurt

Retinoblastoma, paget’s disease, previous radiation, fibrous dysplasia

Biopsy;

Surgery, chemo

Question 15

Ewings sarcoma – characterization? Genetic association? Diagnosis? Management?

Answer 15

Small, round, blue cell tumor

chromosomes 11 and 22

Radiographic findings, MRI. Definitive diagnosis via biopsy

Multiagent chemotherapy followed by surgical excision (add radiation if complete excision not possible)

Question 16

Osteogenic sarcoma versus Ewings sarcoma:

1. Site
2. Local findings
3. systemic findings
4. Radiographic findings
5. Metastasis

Answer 16

1. Metaphysis of tubular bones (femur, tibia, humorous) versus flat bones and diaphysis of tubular bones (pelvis, humorous, femur)
2. Pain, swelling, soft tissue mass in both
3. None versus fever, malaise, leukocytosis, ESR
4. Sunburst appearance versus onionskin appearance
5. Less (lungs > bone) versus more (Lungs >bone/BM)

Question 17

Most common type of liver tumor childhood? Age? Associated with?

Answer 17

Hepatoblastoma; younger than 3; Beckwith-Wiedemann

Question 18

Hepatocellular carcinoma – age? Associated with?

Answer 18

Both children and adolescents

1. Chronic hepatitis B infection
2. Biliary atresia
3. Glycogen storage disease
4. Alphaz1-antitrypsin deficiency
5. Hereditary tyrosinemia

Question 19

Liver tumors – clinical features? Absent feature? Marker? Diagnosis? Treatment? Prognosis?

Answer 19

1. Upper right abdominal mass
2. Anorexia/weight-loss

NO jaundice

AFP

CT/MRI with increased AFP

Surgical resection and chemo

Poor

Question 20

Teratoma – definition? Types?

Answer 20

Containing 2+ primary germ so players

1. Sacrococcygeal – 1st year of life in females. Benign. Resection
2. Anterior mediastinal – airway obstruction. Benign
3. Ovarian – most common. Benign. Calcium in tumor on radiograph.

Question 21

Most common testicular tumors in order of occurrence? Ages? Association with? Unlike adult testicular tumors?

Answer 21

Yolk sac tumors > teratomas >seminomas/embryonal

Younger than 5 or in adolescence

Cryptorchid testes

One third are benign (all malignant in adults)

Question 22

Clinical features of yolk sac tumor? Treatment?

Answer 22

1. Solid, firm, painless testicular mass
2. Elevated AFP

Radical orchiectomy and retroperitoneal node dissection