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Flashcards in peds35 Deck (101):
1

Reed-sternberg cell

hodgkins lymphoma; a large multinucleated cell with abundant cytoplasm

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symptom onset for hodgkins vs nonhodgkins

hodgkins has a slow onset, whereas NH is rapid

3

common location of H and NH lymphoma

H is cervical and supraclavicular nodes; NHL is abdominal, mediastinal, and supraclavic nodes

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systemic symptoms in H and NH lymphoma

H common, in NH uncommon

5

SVC syndrome

obstruction of the SVC by malignancies; dyspnea, headache, edema, other sx

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SVC in H or NH lymphoma?

rare in hodgkins; common in nonhodgkins

7

staging system for hodgkins lymphoma

ann arbor system; subclassified into A or B (systemic sx); stage 1- single lymph node; stage 2- 2 or nodes on same side of diaphragm; stage 3- nodes on both sides of diaphragm; stage 4- dissem inovlvement of extralymph tissues

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treatment for hodgkns

chemo and XRT; male sterility is common side effect of therapy

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prognosis for hodgkins

stage 1 and 2 is excellent; more advanced disease less

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non-hodgkins lymphoma

more aggressive and more common than hodgkins

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non-hodgkins lymphoma gender pref?

male predominance; incr incidence after 5 years of age

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three major categories of NHL

lymphoblastic lymphoma; burkitt's lymphoma; large cell lymphoma

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lymphoblastic lymphoma

type of NHL; T cell in origin; similar to the lymphoblast in ALL

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Burkitt's lymphoma

a type of small noncleaved cell lymphoma; most common lymphoma in childhood;b cell origin; type of NHL

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large cell lympoma

b cell in origin; type of NHL

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most common presenting sign in NHL

painless lymphadenopathy

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lymphoblastic lymphoma- how doess it present?

anterior mediastinal mass; patient may develop SVC syndrome or airway obstruction as a result

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Burkitt's lymphoma clinicl features

intussusception (any kid older than 3 w intussus think BL); abdominal pain or mass

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jaw mass

burkitt's

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large cell lymphoma prsents how?

enlargment of lymphoid tissue in the tonsils, adenoids, or peyer's patches

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adenoids

lymphatic tissue in the roof of the mouth

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peyers patches

lymphoid nodules in the small intestine

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treatment for NHL

must be rapid because it is aggressive; surgery, chemo, prophylaxis for CNS spread

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prognosis for NHL

outlook good for localized lymphoma but poor for disseminated

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second most common childhood cancer

brain tumors

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most common solid tumors in kids

brain tumors

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most common type of brain tumor

glial cell tumors

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astrocyte

type of glial cell

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glial cell

non-neuronal cells that make myelin and provide support

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primitive neuroectodermal tumors quintessential example

medullablastomas arising from the cerebellum

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ependymomas

tumor that arises from the epithelial-like lining of the ventricles

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craniopharyngioma

tumor derived from the pituitary gland

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infratentorial

cerebellum and below

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infrantentorial tumors vs tentorial tumors

infratentorial are more common

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most common infratentorial tumor

medullablastoma

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most common supratentorial tumor

astrocytoma

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ICP symptoms seen in brain tumors are worse when?

worse during sleep or on awakening; better during the day as venous return from the head improves with upright posture

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sixth nerve palsy

sign of ICP; can't contract LR to abduct the eye; so you get convergent strabismus

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CT versus MRI for brain imaging

MRI generally better

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principle treatment for brain tumor

surgery; radiation therapy reserved for kids over 5; chemo sometmes

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astrocytoma prognosis

low grade fully-resectable have a good prognosis; high grade bad prognosis

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PNETs prognosis

worse in kids less than 4; good if tumor is fully resectable

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prognosis of brainstem gliomas

poorest prognosis; not possible to resect and chemo is ineffective

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neuroblastoma

malignant tumor of neural crest cells; can arise anywhere in the symp ganglion chain or adrenal medulla

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peak incidence for neuroblastomas

first 5 years of life; median age at dx is 2 yo

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most neuroblastomas occur where?

abdomen or pelvis; some can occur in posterior mediastinum and few in the neck

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diagnosis of neuroblastoma

urine excretion of excess catecholamines; definitive dx by bone marrow biopsy plus elevated urine catecholamines

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acute cerebellar atrophy

occurs in 2% of neuroblastoma; ataxis, random eye jerks, and myoclonus ("dancing eyes and dancing feet")

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management of neuroblastoma

surgery for stages 1 and 2; chemo for metastatic disease; radiation therapy for advanced dz

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prognosis for neuroblastoma

good in stage 1 and 2; you can actually get spontaneous regression without tx in infants with stage IVS disease' poor prognosis in stage III and IV;

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wilms tumor cell origing

nephroblastoma;

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most common childhood renal tumor

wilms tumor

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median age at dx for wilms tumor

3 years

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how does htn develop in wilms tumor kdis?

compression of renal artery or increased renin secretion by tumor

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wilms tumor associated congenital anomalies

GU malformations, hemihypertrophy, sporadic aniridia

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aniridia

absence of the iris

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wilms tumor bilateral?

5% of cases

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management of wilms tumor

surgery for staging and debulking; chemo used for alll stages; radiation therapy for advanced disease

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prognosis for wilms tumor

outcome is excellent

60

rhabdomyosarcoma

cancer cells arise from skeletal muscle precursors

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most common soft tissue sarcoma in childhood

rhabdomyosarcoma

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most common sites of involvement for soft tissue tumors

head and neck(orbital, nasopharyngeal, laryngeal)

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second most common site of soft tissue tumor

GU tract

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prognosis for soft tissue tumors

good for head, neck, and GU tumors; poor for other sites

65

osteogenic sarcoma

malignant tumor that forms new bone

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most common malignant bone tumor

osteogenic sarcoma

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when does incidence of osteogenic sarcoma peak?

rapid growth spurt during adolescence

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clinical features of osteogenic sarcoma

about 50% occur near the knee; periosteal racton with "sunburst" appearance; occur at metaphysis; mets in 15%

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management of osteogenic sarcoma

surgery to remove the primary tumor; chemo improves survival; pulm mets removed surgically

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prognosis of osteogenic sarcoma

survival greater than 60%

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ewing's sarcoma

sarcoma characterized by small, round, blue cell tumor

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ewing's sarcoma what population?

most commonly during adolescence and more common in males; rare in asians and african americans

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ewing's sarcoma genetic cause

translocation between chroms 11 and 21 (similar to PNET brain tumors)

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treatment of ewing's tumor

multiagent chemo due to high risk of mets and also surgery

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prognosis for ewing's sarcoma

good for local disease; poor if mets

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ewing's sarcoma affects what part of the bone?

flat bones and diaphysis (as opposed to metaphysis in osteogenic); on radiograph see periosteal reaction with onion skin appearance;

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two types of liver tumors

hepatoblastoma and HCC

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most common type of liver tumor in kids

hepatoblastoma

79

hepatoblastoma seen in what age kids? Associated with what syndrome

less than 3 yo; beckwith weidemann syndrome

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HCC seen in what age? Associated with what?

kids and adolescents; associated with chronic active Hep B

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jaundice in liver tumors?

not usually

82

treatment of liver tumors

surgical resection and chemot

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prognosis for liver tumros?

very poor; most unresectable and will met to the lungs; HCC less curable than hepatoblastoma

84

germ cell tumors

derived from cellular precursors of sperm and eggs

85

most common teratoma during first years of life

sacrococcygeal teratoma

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sacrococcygeal tumor

75% females and most are benign; surgical resection of the tumor and the coccyx

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how do anterior mediastinal teratomas present?

airway obstruction; usually being

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ovarian teratomas

generally benign; most common ovarian tumor

89

most common germinomas of the testicle

yolk sac tumor, followed by teratomas

90

peak age for testicular cancer

5 yo

91

testicular tumors in kids versus adults

in adults, testicular tumors are always malignant; in kids, one third are benign

92

serum alpha fetoprotein is elevated in what type of testicular cancer?

yolk sac tumors; also in ovarian yolk sac tumors

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treatment of yolk sac tumors

radical orchiectomy and, if necessary, retroperitoneal lymph node dissection

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ovarian tumors- are they malignant?

1/3 are malignant and the younger the child, the more likely the tumor is malignant

95

ovarian tumor freq in puberty

ovarian tumors incr in frequency in puberty

96

Langerhans cell histiocytosis

uncontrolled growth of langerhans cell (activated dendritic cells and macrophages); not a true malignancy but immune dysregulation

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examples of langerhans cell histiocytosis

eosinophilic granuloma, hand-schuller-christian disease, and letterer-siwe disease

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clinical features of langerhans cell histiocytosis

skeletal involvemnt, skin involvement, pituitay or hypothal involvemtn, systemic features

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management of langerhans cell histiocytosis

if single lesion is involved, local curretage or low dose radiation; steroids or single agent chemo; if multiple sites, multiagent chemo is used

100

anaphylaxis

IgE mediated; release of potent mediators that affect vascular tone and bronchial reactivity

101

when do symptoms of anaphylaxis appear?

within 30 mins after exposure