Cancer V ( Pediatric Tumors)

ectasia

any local dialtion of a structure

telangiectasia

permanent dilation of preexisting small vessel

benign vascular lesion characteristics

non neoplastic malformation of blood vessels that can be either congenital or aquired

nevus flammeus

salmon patch, present at birth and most common form of vascular ectasia

usually regresses spontaneously as child grows

port wine stain

does not regress, tends to grow and thicken, unilateral on face

part of sturge weber syndrome

sturge webber syndrome

port wine stain in trigeminal nerve distribution that can extend into the eye and lead to glaucoma

ipsilateral venous angiomas in leptomeninges

pediatric tumors aew mostly

benign`

hemangioma

from blood vessels or endothelium

• vessel contains blood

lymphangioma

derived from lymphatic vessels and endothelium

vessel has pale milky fluid no blood

teratoma

totipotent germ cell from 3 layers

what are the benign tumors of childhood

hemangioma, teratoma, lymphangioma

most common neoplasm of infncy

hemangioma

clinical features of hemangioma

present at birth and increases in size with childgrowth

flat to elevates, irregular blue masses

most regress by school age

hereditary von hippel lindau disease

hemangioms in internal organs

capillary hemangioma

lobules of capillaries

cavernous hemangioma

large dilated vascular channeld, larger and difficult to resect

hamartoma

exessive growth of tissue nativa to the organ

lymphangioma cn be either

neoplastic or hamartoma

types of lymphangioma

capillary and cavernous

cavernous lymphangioma can occur in

turner, noonan, or downs syndrome

clinical for lymphangioma

in skin, deep neck, axilla

can increase in size after birth

encroach on vital structures

benign mature teratomas are considered

cystic

75 % of teratomas are

clinicl for teratoma

slow growing and can present at birth as cystic lesion

location is sacrcoccygeal mostly

anaplasia

cells with large, hyperchromatic, pleomorphic nuclei and abnormal mitosis (less common in childhood malignant tumors)mal

malignant childhood tumors

acute leukemia (most common), neuroblastoma (most common solid non cns), wilms tumor, retinoblstoma

neuroblstoma tumors arise from

neural crest derived cells if sympathetic ganglia and adrenal medulla

adrenal medulla is the most common site

clinical features of neuroblastoma

most occur under 5 years old, most are sporadic (only alk mutation is famililal for this)

spontaneous regression and therapy induced maturation may occur

for neuroblastoma what are the features for children under 2 years old

protuberant abdomen due to large abdiminal mass, fever and weifht loss

in older children neuroblastoma predsents as

metastatic disease (bone pain, respiratory symptoms, GI complaints, Liver)

peri orbital spread in neuroblastoma

proptosis (bulging)and eccymosis (broke blood vessels and bruising) of eye

skin metastasis in neuroblastoma

blue berry muffin baby (deep blue nodules in abdominal ski)

horner syndrome

due to tumor in sympathetic pathway arising from brai and supplu face amd eye

type of neuroblastoma

clinical features of horner syndrome

ptosis (drooopy eye), facial anhidrosis, miosis (constricted pupils)wh

where is the most common site for horner syndrome (sympathetic tumor)

adrenal tumor on kidney

neuroblastoma (horner syndrome) sizes

microscopic (may be silent or spontaneously regress

pseudo encapsulated to invasive (into blood vessels and kidney)

classical neuroblastomas (microscopic)

small round blye cells with undifferentiation (poor prognosis) g

ganglioeurpblastoma

intermediate prognosis , gangliion cell in various stages of maturation with neuroblasts

ganglioneuroma (a neuroblastoma)

more differentated than ganglioneuroblastoma, favorable prognosis and mostly mature ganglions and schwann cells with few if any neuroblasts

90% of neurblastoma tumors secrete

catecholamines

in a lab test what will be seen for somone with neuroblastoma

increase in chatecholamine levels, increased urinary levels of metabolites (VMA AND HVA)

what are the prognostic factors for neuroblastom

age of patient and stage of the tumor

for neuroblastoma what stages and age have excellent prognosis

stage 1,2a,2b,4s

also if under 18 months, more favorable prognosis

what is associated with poor prognosis for neuroblastoma

amplification of MCYN oncogene (increased copies, worse prognosis)

increased mitosis→ poor prognosis

wilms tumor (nephroblastoma) is

most comon primary renal tumor of childhoodwi

wilms tumor incidence

2-5 years

what does wilms tumor arise from

nephrogenic rests which are precursor lesions

familar wilms tuor associated with

germ line mutation of WT1 tumor supressor gene, increased risj for 2nd malignancy if germline mutations present or secondary to therapy

WAGR syndrome is

WT1 gene mutation, where 30% get wilms tumor

WAGR syndrome characteristics

get Wilms tumor, Aniridia (absent irises), Genital malformation (abigious genetalia, or intellecular disability

Denys Drash Syndrom is a

WT1 gene mutation

Denys Drash syndrome characteristics

gonadal dysgenesis and early onset nephropathy (renal failure)

beckwith wiedemann syndrome is

WT2 gene abnormality where IGF is over expressed

characteristics of Beckwith Wiedemman syndrome

enlargement of tongue, kidney, or liver, or entire body segments

enlargement of adrenal cortical cells

characteristics of wilms tumor

large abdominal mass, gross hematuria pain in abdomen, intestinal obstruction, fever, hypertnesion

wilms tumor histology

triphasic (blastema)- small round blue cells

epithelial (abortive tubules or glomeruli)

stromal (Fibrocytic, myxoid, musle elements)

what is the most common primary intraocular malignancy of children

retinoblastoma

median age to get retinoblastoma

2 years old

whats the cell of origin for retinoblastoma

neuronal progenitor

for familial retinoblastoma whats the mutation

germline RB mutation- bilateral tumors, increased risk of osteosarcoma and othert soft tissue tumors

clinical features of retinoblastoma

cats eye reflex (whitish hue to pupil)

strabismus (eyes dont look at dsame direction at same tume )

morphology for retinoblastoma

undifferentiated round blue cells with flexner wintersteiner rosettes (small rund blue cells)