Malignancies Flashcards

1
Q

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

A

Hodgkin lymphoma

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2
Q

MC solid tumor in children

A

brain tumor

2nd MC malignancy after leukemia

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3
Q

The MC astrocytoma in children

A

pilocytic astrocytoma

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4
Q

Classic site of occurrence of astrocytoma

A

cerebellum

optic pathway region

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5
Q

MC posterior fossa tumor

A

cerebellar astrocytoma

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6
Q

Classic but not exclusive neuroradiologic finding in pilocytic astrocytoma

A

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

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7
Q

Condensed masses of glial filaments occurring in compact areas

A

Rosenthal fibers

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8
Q

2nd most common astrocytoma

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

A

diffuse astrocytoma (DA)

predilection to supratentorial locations

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9
Q

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

A

Pilomyxoid astrocytoma

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10
Q

Manifestations of Astrocytoma

A

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

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11
Q

Management of Astrocytoma

A

surgical resection

radiotherapy and chemotherapy

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12
Q

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

A

Embryonal tumors or primitive neuroectodermal tumors (PNETs)

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13
Q

Accounts for 90% of embryonal CNS tumors

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

A

Medulloblastoma

2nd MC posterior fossa tumor of childhood

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14
Q

Manifestations of Medulloblastoma

A

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

cerebellar dysfunction - gait, ataxia, dysmetria, poor balance

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15
Q

Management of Medulloblastoma

A

surgical resection

radiotherapy and chemotherapy

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16
Q

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

A

Craniopharyngioma

adamantinomatous CP
papillary CP

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17
Q

Manifestations of Craniopharyngioma

A

visual deficits that can be secondary to compression of optic chiasm

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18
Q

Management of Craniopharyngioma

A

surgical resection

radiotherapy

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19
Q

The MC primary malignant bone tumor in children

and adolescents

A

Osteosarcoma

METAPHYSIS

lungs and bone - common site of metastases

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20
Q

Familial cancer syndrome associated with germline mutations of the P53 gene

A

Li-Fraumeni syndrome

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21
Q

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

A

Rothmund-Thomson syndrome

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22
Q

4 pathologic subtypes of conventional high-grade osteosarcoma

A

osteoblastic
fibroblastic
chondroblastic
telangiectatic

23
Q

The MC presenting manifestations of osteosarcoma

A

pain, limp, and swelling

24
Q

The MC presenting manifestations of osteosarcoma

A

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

25
Q

Classic radiographic appearance of osteosarcoma

A

sunburst pattern

26
Q

Differential diagnosis of a lytic bone lesion

A

histiocytosis
Ewing sarcoma
lymphoma
bone cyst

27
Q

Treatment of osteosarcoma

A

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

28
Q

2nd MC primary malignant bone tumor

undifferentiated sarcoma of bone, also may arise from soft tissue

A

Ewing Sarcoma

DIAPHYSIS

lungs and bone - common site of metastases

29
Q

Primary tumors arising in the chest wall

A

Askin tumors

30
Q

Manifestations of Ewing sarcoma

A

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

31
Q

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

A

onion-skinning

32
Q

Treatment of Ewing sarcoma

A
Vincristine
Doxorubicin
Cyclophosphamide
Etoposide
Ifosfamide

irradiation or surgery

33
Q

The MC extracranial solid tumor in children

The most commonly diagnosed malignancy in infants

A

Neuroblastoma

34
Q

Median age of children at diagnosis of neuroblastoma

A

22 mo

90% of cases - diagnosed by 5 yr of age

35
Q

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)

A

Neuroblastoma

36
Q

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

A

Pepper syndrome

37
Q

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

A

Horner syndrome

38
Q

Approximately half of neuroblastoma tumors arise

A

ADRENAL GLANDS

most of the remainder originate in the paraspinal sympathetic ganglia

39
Q

Metastatic spread - more common in children >1 yr

old at diagnosis

A

local invasion or distant hematogenous or lymphatic routes

40
Q

Manifestations of neuroblastoma

A

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)

41
Q

MC sites of metastases (neuroblastoma)

A
long bones and skull
BM
liver
lymph nodes
skin
42
Q

Diagnosis of Neuroblastoma

A

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

43
Q

Diagnosis of Neuroblastoma

A

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

44
Q

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

A

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
45
Q
2 y/o male
abdominal swelling
occasional jerky movements
abdominal mass cross the midline
bluish subcutaneous nodules palpated
A

Neuroblastoma

46
Q

Treatment for children with low-risk neuroblastoma

A

SURGERY for stages L1 and L2

observation for asymptomatic stage MS, with cure rates generally >90% without further therapy

47
Q

Treatment of intermediate-risk neuroblastoma

A

Surgery, chemotherapy, and in some cases, radiation therapy

CHEMOTHERAPY
cisplatin or carboplatin
cyclophosphamide
etoposide
doxorubicin
48
Q

High-risk neuroblastoma

A

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)

49
Q

The MC primary malignant RENAL TUMOR of childhood

The 2nd MC malignant abdominal tumor in childhood after neuroblastoma

A

Wilms tumor (nephroblastoma)

2-5 y/o

50
Q

Wilms tumor is associated w/

A

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

51
Q

Manifestations of Wilms tumor

A

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

hematuria
HPN

52
Q

Diagnostics of Wilms tumor

A

abdominal UTZ, CT scan or MRI

electrolytes, renal function tests

chest x-ray or CT scan

53
Q

Treatment of Wilms tumor

A

COG - advocates upfront surgery prior to initiating
treatment

SIOP - recommends preoperative chemotherapy

54
Q

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

A

Wilms tumor