peds35 Flashcards
Reed-sternberg cell
hodgkins lymphoma; a large multinucleated cell with abundant cytoplasm
symptom onset for hodgkins vs nonhodgkins
hodgkins has a slow onset, whereas NH is rapid
common location of H and NH lymphoma
H is cervical and supraclavicular nodes; NHL is abdominal, mediastinal, and supraclavic nodes
systemic symptoms in H and NH lymphoma
H common, in NH uncommon
SVC syndrome
obstruction of the SVC by malignancies; dyspnea, headache, edema, other sx
SVC in H or NH lymphoma?
rare in hodgkins; common in nonhodgkins
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
treatment for hodgkns
chemo and XRT; male sterility is common side effect of therapy
prognosis for hodgkins
stage 1 and 2 is excellent; more advanced disease less
non-hodgkins lymphoma
more aggressive and more common than hodgkins
non-hodgkins lymphoma gender pref?
male predominance; incr incidence after 5 years of age
three major categories of NHL
lymphoblastic lymphoma; burkitt’s lymphoma; large cell lymphoma
lymphoblastic lymphoma
type of NHL; T cell in origin; similar to the lymphoblast in ALL
Burkitt’s lymphoma
a type of small noncleaved cell lymphoma; most common lymphoma in childhood;b cell origin; type of NHL
large cell lympoma
b cell in origin; type of NHL
most common presenting sign in NHL
painless lymphadenopathy
lymphoblastic lymphoma- how doess it present?
anterior mediastinal mass; patient may develop SVC syndrome or airway obstruction as a result
Burkitt’s lymphoma clinicl features
intussusception (any kid older than 3 w intussus think BL); abdominal pain or mass
jaw mass
burkitt’s
large cell lymphoma prsents how?
enlargment of lymphoid tissue in the tonsils, adenoids, or peyer’s patches
adenoids
lymphatic tissue in the roof of the mouth
peyers patches
lymphoid nodules in the small intestine
treatment for NHL
must be rapid because it is aggressive; surgery, chemo, prophylaxis for CNS spread
prognosis for NHL
outlook good for localized lymphoma but poor for disseminated
second most common childhood cancer
brain tumors
most common solid tumors in kids
brain tumors
most common type of brain tumor
glial cell tumors
astrocyte
type of glial cell
glial cell
non-neuronal cells that make myelin and provide support
primitive neuroectodermal tumors quintessential example
medullablastomas arising from the cerebellum
ependymomas
tumor that arises from the epithelial-like lining of the ventricles
craniopharyngioma
tumor derived from the pituitary gland
infratentorial
cerebellum and below
infrantentorial tumors vs tentorial tumors
infratentorial are more common
most common infratentorial tumor
medullablastoma
most common supratentorial tumor
astrocytoma
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
sixth nerve palsy
sign of ICP; can’t contract LR to abduct the eye; so you get convergent strabismus
CT versus MRI for brain imaging
MRI generally better
principle treatment for brain tumor
surgery; radiation therapy reserved for kids over 5; chemo sometmes
astrocytoma prognosis
low grade fully-resectable have a good prognosis; high grade bad prognosis
PNETs prognosis
worse in kids less than 4; good if tumor is fully resectable
prognosis of brainstem gliomas
poorest prognosis; not possible to resect and chemo is ineffective
neuroblastoma
malignant tumor of neural crest cells; can arise anywhere in the symp ganglion chain or adrenal medulla
peak incidence for neuroblastomas
first 5 years of life; median age at dx is 2 yo
most neuroblastomas occur where?
abdomen or pelvis; some can occur in posterior mediastinum and few in the neck
diagnosis of neuroblastoma
urine excretion of excess catecholamines; definitive dx by bone marrow biopsy plus elevated urine catecholamines
acute cerebellar atrophy
occurs in 2% of neuroblastoma; ataxis, random eye jerks, and myoclonus (“dancing eyes and dancing feet”)
management of neuroblastoma
surgery for stages 1 and 2; chemo for metastatic disease; radiation therapy for advanced dz
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;
wilms tumor cell origing
nephroblastoma;
most common childhood renal tumor
wilms tumor
median age at dx for wilms tumor
3 years
how does htn develop in wilms tumor kdis?
compression of renal artery or increased renin secretion by tumor
wilms tumor associated congenital anomalies
GU malformations, hemihypertrophy, sporadic aniridia
aniridia
absence of the iris
wilms tumor bilateral?
5% of cases
management of wilms tumor
surgery for staging and debulking; chemo used for alll stages; radiation therapy for advanced disease
prognosis for wilms tumor
outcome is excellent
rhabdomyosarcoma
cancer cells arise from skeletal muscle precursors
most common soft tissue sarcoma in childhood
rhabdomyosarcoma
most common sites of involvement for soft tissue tumors
head and neck(orbital, nasopharyngeal, laryngeal)
second most common site of soft tissue tumor
GU tract
prognosis for soft tissue tumors
good for head, neck, and GU tumors; poor for other sites
osteogenic sarcoma
malignant tumor that forms new bone
most common malignant bone tumor
osteogenic sarcoma
when does incidence of osteogenic sarcoma peak?
rapid growth spurt during adolescence
clinical features of osteogenic sarcoma
about 50% occur near the knee; periosteal racton with “sunburst” appearance; occur at metaphysis; mets in 15%
management of osteogenic sarcoma
surgery to remove the primary tumor; chemo improves survival; pulm mets removed surgically
prognosis of osteogenic sarcoma
survival greater than 60%
ewing’s sarcoma
sarcoma characterized by small, round, blue cell tumor
ewing’s sarcoma what population?
most commonly during adolescence and more common in males; rare in asians and african americans
ewing’s sarcoma genetic cause
translocation between chroms 11 and 21 (similar to PNET brain tumors)
treatment of ewing’s tumor
multiagent chemo due to high risk of mets and also surgery
prognosis for ewing’s sarcoma
good for local disease; poor if mets
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;
two types of liver tumors
hepatoblastoma and HCC
most common type of liver tumor in kids
hepatoblastoma
hepatoblastoma seen in what age kids? Associated with what syndrome
less than 3 yo; beckwith weidemann syndrome
HCC seen in what age? Associated with what?
kids and adolescents; associated with chronic active Hep B
jaundice in liver tumors?
not usually
treatment of liver tumors
surgical resection and chemot
prognosis for liver tumros?
very poor; most unresectable and will met to the lungs; HCC less curable than hepatoblastoma
germ cell tumors
derived from cellular precursors of sperm and eggs
most common teratoma during first years of life
sacrococcygeal teratoma
sacrococcygeal tumor
75% females and most are benign; surgical resection of the tumor and the coccyx
how do anterior mediastinal teratomas present?
airway obstruction; usually being
ovarian teratomas
generally benign; most common ovarian tumor
most common germinomas of the testicle
yolk sac tumor, followed by teratomas
peak age for testicular cancer
5 yo
testicular tumors in kids versus adults
in adults, testicular tumors are always malignant; in kids, one third are benign
serum alpha fetoprotein is elevated in what type of testicular cancer?
yolk sac tumors; also in ovarian yolk sac tumors
treatment of yolk sac tumors
radical orchiectomy and, if necessary, retroperitoneal lymph node dissection
ovarian tumors- are they malignant?
1/3 are malignant and the younger the child, the more likely the tumor is malignant
ovarian tumor freq in puberty
ovarian tumors incr in frequency in puberty
Langerhans cell histiocytosis
uncontrolled growth of langerhans cell (activated dendritic cells and macrophages); not a true malignancy but immune dysregulation
examples of langerhans cell histiocytosis
eosinophilic granuloma, hand-schuller-christian disease, and letterer-siwe disease
clinical features of langerhans cell histiocytosis
skeletal involvemnt, skin involvement, pituitay or hypothal involvemtn, systemic features
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
anaphylaxis
IgE mediated; release of potent mediators that affect vascular tone and bronchial reactivity
when do symptoms of anaphylaxis appear?
within 30 mins after exposure
