Malignancies

Question 1

16 y/o male
weight loss over the past 3 mos
body malaise, (+) night sweats and intermittent febrile episodes
dry cough, no dyspnea, occasional episodes of epistaxis
(+) episode of flu 2 wks ago
pale palpebral conjunctiva, pale buccal mucosa
(+) multiple nontender cervical and supraclavicular lymphadenopathy

Answer 1

Hodgkin lymphoma

Question 2

MC solid tumor in children

Answer 2

brain tumor

2nd MC malignancy after leukemia

Question 3

The MC astrocytoma in children

Answer 3

pilocytic astrocytoma

Question 4

Classic site of occurrence of astrocytoma

Answer 4

cerebellum

optic pathway region

Question 5

MC posterior fossa tumor

Answer 5

cerebellar astrocytoma

Question 6

Classic but not exclusive neuroradiologic finding in pilocytic astrocytoma

Answer 6

the presence of a contrast-enhancing nodule within the wall of a cystic mass

Question 7

Condensed masses of glial filaments occurring in compact areas

Answer 7

Rosenthal fibers

Question 8

2nd most common astrocytoma

consists of a group of tumors characterized by a pattern of diffuse infiltration of tumor cells amidst normal neural tissue

Answer 8

diffuse astrocytoma (DA)

predilection to supratentorial locations

Question 9

Occurs most commonly in the HYPOTHALAMIC/OPTIC CHIASMIC REGION and carries a high risk of local as well as cerebrospinal spread

affects young children and infants

WHO grade II tumor

Answer 9

Pilomyxoid astrocytoma

Question 10

Manifestations of Astrocytoma

Answer 10

headache, seizure, weakness, memory loss, motor weakness, visual symptoms, personality changes

Question 11

Management of Astrocytoma

Answer 11

surgical resection

radiotherapy and chemotherapy

Question 12

The MC group of MALIGNANT CNS tumors of childhood, accounting for approximately 20% of pediatric CNS tumors

Answer 12

Embryonal tumors or primitive neuroectodermal tumors (PNETs)

Question 13

Accounts for 90% of embryonal CNS tumors

Cerebellar tumor occurring predominantly in MALES and at a median age of 5-7 yr

Answer 13

Medulloblastoma

2nd MC posterior fossa tumor of childhood

Question 14

Manifestations of Medulloblastoma

Answer 14

obstruction of the 4th ventricle –> increased ICP - headache, vomiting, nausea, altered mental status, HPN

cerebellar dysfunction - gait, ataxia, dysmetria, poor balance

Question 15

Management of Medulloblastoma

Answer 15

surgical resection

radiotherapy and chemotherapy

Question 16

Common tumor of childhood, accounting for 7–10% of all childhood tumors

Solid or solid-cystic tumors from remnants of Rathke pouch of the pituitary stalk in the SUPRASELLAR region

Answer 16

Craniopharyngioma

adamantinomatous CP
papillary CP

Question 17

Manifestations of Craniopharyngioma

Answer 17

visual deficits that can be secondary to compression of optic chiasm

Question 18

Management of Craniopharyngioma

Answer 18

surgical resection

radiotherapy

Question 19

The MC primary malignant bone tumor in children

and adolescents

Answer 19

Osteosarcoma

METAPHYSIS

lungs and bone - common site of metastases

Question 20

Familial cancer syndrome associated with germline mutations of the P53 gene

Answer 20

Li-Fraumeni syndrome

Question 21

Rare condition associated with short stature, skin telangiectasia, small hands and feet, hypoplasticity or absence of the thumbs, and a high risk of osteosarcoma

Answer 21

Rothmund-Thomson syndrome

Question 22

4 pathologic subtypes of conventional high-grade osteosarcoma

Answer 22

osteoblastic
fibroblastic
chondroblastic
telangiectatic

Question 23

The MC presenting manifestations of osteosarcoma

Answer 23

pain, limp, and swelling

Question 24

The MC presenting manifestations of osteosarcoma

Answer 24

pain, limp, and swelling

initial complaints may be attributed to a sports injury or sprain
any bone or joint pain not responding to conservative therapy within a reasonable time should be investigated thoroughly

Question 25

Classic radiographic appearance of osteosarcoma

Answer 25

sunburst pattern

Question 26

Differential diagnosis of a lytic bone lesion

Answer 26

histiocytosis Ewing sarcoma lymphoma bone cyst

Question 27

Treatment of osteosarcoma

Answer 27

MAP - the standard chemotherapy regimen for osteosarcoma METHOTREXATE DOXORUBICIN CISPLATIN treat patients with preoperative chemotherapy in an attempt to facilitate limb salvage operations and to treat micrometastatic disease immediately chemotherapy and surgery, 5 yr disease-free survival of patients with nonmetastatic extremity osteosarcoma is 65–75% long-term follow-up of patients with osteosarcoma is important to monitor for late effects of chemotherapy, such as cardiotoxicity from anthracycline and hearing loss from cisplatin

Question 28

2nd MC primary malignant bone tumor undifferentiated sarcoma of bone, also may arise from soft tissue

Answer 28

Ewing Sarcoma DIAPHYSIS lungs and bone - common site of metastases

Question 29

Primary tumors arising in the chest wall

Answer 29

Askin tumors

Question 30

Manifestations of Ewing sarcoma

Answer 30

pain, swelling, limitation of motion, and tenderness over the involved bone or soft tissue often associated with systemic manifestations, such as fever and weight loss, and may be accompanied by elevated inflammatory markers

Question 31

Radiographic appearance of a primarily lytic bone lesion with periosteal reaction in Ewing sarcoma

Answer 31

onion-skinning

Question 32

Treatment of Ewing sarcoma

Answer 32

``` Vincristine Doxorubicin Cyclophosphamide Etoposide Ifosfamide ``` irradiation or surgery

Question 33

The MC extracranial solid tumor in children The most commonly diagnosed malignancy in infants

Answer 33

Neuroblastoma

Question 34

Median age of children at diagnosis of neuroblastoma

Answer 34

22 mo 90% of cases - diagnosed by 5 yr of age

Question 35

Derived from primordial neural crest cells, form a spectrum with variable degrees of neural differentiation, ranging from tumors with primarily undifferentiated small round cells (neuroblastoma) to tumors consisting of mature and maturing schwannian stroma with ganglion cells (ganglioneuroblastoma or ganglioneuroma)

Answer 35

Neuroblastoma

Question 36

Massive involvement of the liver with metastatic disease, with or without respiratory distress

Answer 36

Pepper syndrome

Question 37

Unilateral ptosis, myosis, and anhidrosis associated with a thoracic or cervical primary tumor. Symptoms do not resolve with tumor resection

Answer 37

Horner syndrome

Question 38

Approximately half of neuroblastoma tumors arise

Answer 38

ADRENAL GLANDS most of the remainder originate in the paraspinal sympathetic ganglia

Question 39

Metastatic spread - more common in children >1 yr | old at diagnosis

Answer 39

local invasion or distant hematogenous or lymphatic routes

Question 40

Manifestations of neuroblastoma

Answer 40

abdominal mass that crosses the midline abdominal pain proptosis periorbital ecchymosis - raccoon eyes Horner syndrome - unilateral ptosis, myosis and anhidrosis subcutaneous tumor nodules opsoclonus/myoclonus (dancing eyes, dancing feet)

Question 41

MC sites of metastases (neuroblastoma)

Answer 41

``` long bones and skull BM liver lymph nodes skin ```

Question 42

Diagnosis of Neuroblastoma

Answer 42

abdominal CT scan - hemorrhage and calcification 24 hr urine catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) - elevated in ~95% of patients and help to confirm the diagnosis

Question 43

Diagnosis of Neuroblastoma

Answer 43

abdominal CT scan - hemorrhage and calcification 24 hr urine catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) - elevated in ~95% of patients and help to confirm the diagnosis bone marrow - myelophthisic marrow biopsy

Question 44

International Neuroblastoma Risk Group (INRG) Staging System (INSS)

Answer 44

L1 tumors (previously classified as INSS stage 1) - localized and confined to 1 body compartment without any IDRFs L2 tumors (previously classified as INSS stages 2 and 3) - localized tumors with the presence of IDRFs. M (previously classified as INSS stage 4) - Disseminated tumors with metastases to bones, bone marrow, liver, distant lymph nodes, and other organs ``` Stage MS (previously stage 4S) - refers to neuroblastoma in children <18 mo old with dissemination to liver, skin, or bone marrow without bone involvement and with a primary tumor that would otherwise be staged as L1 or L2 ```

Question 45

``` 2 y/o male abdominal swelling occasional jerky movements abdominal mass cross the midline bluish subcutaneous nodules palpated ```

Answer 45

Neuroblastoma

Question 46

Treatment for children with low-risk neuroblastoma

Answer 46

SURGERY for stages L1 and L2 observation for asymptomatic stage MS, with cure rates generally >90% without further therapy

Question 47

Treatment of intermediate-risk neuroblastoma

Answer 47

Surgery, chemotherapy, and in some cases, radiation therapy ``` CHEMOTHERAPY cisplatin or carboplatin cyclophosphamide etoposide doxorubicin ```

Question 48

High-risk neuroblastoma

Answer 48

poor long-term survival rates between 25% and 35% with treatment that consisted of intensive chemotherapy, high-dose chemotherapy with autologous stem cell rescue, surgery, radiation, and 13-cis-retinoic acid (isotretinoin, Accutane)

Question 49

The MC primary malignant RENAL TUMOR of childhood The 2nd MC malignant abdominal tumor in childhood after neuroblastoma

Answer 49

Wilms tumor (nephroblastoma) 2-5 y/o

Question 50

Wilms tumor is associated w/

Answer 50

neurofibromatosis Beckwith-Wiedemann syndrome - hemihypertrophy, visceromegaly, macroglossia) WAGR syndrome - Wilms tumor, aniridia, GU abnormalities, mental retardation)

Question 51

Manifestations of Wilms tumor

Answer 51

painless abdominal enlargement w/ flank mass that does NOT cross the midline hematuria HPN

Question 52

Diagnostics of Wilms tumor

Answer 52

abdominal UTZ, CT scan or MRI electrolytes, renal function tests chest x-ray or CT scan

Question 53

Treatment of Wilms tumor

Answer 53

COG - advocates upfront surgery prior to initiating treatment SIOP - recommends preoperative chemotherapy

Question 54

asymptomatic 2 y/o abdominal mass on the L flank IVP - enlarged kidney w/ distorted pelvis and calyces

Answer 54

Wilms tumor