Ped Tumors Flashcards
Most common malignancy in childhood
ALL –> most are also B cell
adolescent males, mediastinal mass
T cell leukemia (lymphoblastic)
Trisomy 21
higher incidence of M4 AML
Presents in jaw in Africa
Burkitts lymphoma –> B cell in origin
t(8;14)
(present in the abdomen in the US)
Langerhans Cell Histiocytosis
Eosinophilic Granuloma
Letterer-Siwe disease
contain Birbeck granules (tennis racket handles)
Ped brain tumors
below the tentorium
Medulloblastoma
occur in the cerebellum
neuroendocrine tumor
Astrocytoma
benign in children
Ependymoma
tumor of cells lining ventricles
Craniopharyngiomas
dx/dy from Rathke’s pouch near pituitary
difficult to remove
most common solid tumors in children
brain tumors
Pinealoblastoma
pineal gland tumor
poor prig
RARE in children
Metastatic lesions
rare in the brain in children
usually neoplasms are primary
Two tumors with abdominal masses in children
Neuroblastoma
Wilms Tumor
second most common solid tumor in children
Neuroblastoma
Neuroblastoma
adrenal medulla
also seen in sympathetic gangl –> paraganglioma –> posterior mediastinum
secrete HVA (catecholamine)
can mature to benign tumor
small round blue cell tumor –> forms rosettes
Neuroblastoma cont’
prognosis worse in older children (4-5yrs)
can be congenital –> two-hit hypothesis
deletion short arm of chromosome 1
amplification of N-myc gene
most common intra-abdominal tumor in children
Wilms’ Tumor
11p13 deletion/mutation
Wilms’ Tumor
tries to make a kidney primitive looking tissue nodular renal blastema also seen THE YOUNGER THE BETTER two-hit hypothesis
Denys-Drash Syndrome
kidney disease at birth
Wilms’ tumor
malformation of the sexual organs
Beckwith Wiedemann Syndrome
hemihypertrophy
omphalocele
large tongue
–equivalent of first hit
WAGR complex
deletion of gene on p arm of chromosome 11 Wilms' tumor aniridia genitourinary anomalies mental retardation syndrome
Most common soft tissue neoplasm in children
rhabdomyosarcoma
Rhabdomyosarcoma
eosinophilic cyto
striations in cyto
muscle specific actin +
desmin +
Alveolar rhabdomyosarcoma
t(2;13)
Sarcoma Botryoides –> embryonal form
protrudes from vagina
Hemangioma
benign tumors of blood vessels
can spontaneously regress
malignant : angiosarcoma
Osteogenic sarcoma
most common in adolescents
distal femur and prox tibia
Ewing’s Sarcoma
small round blue cell tumor
t(11;22) –> MIC2
Teratoma
ovaries mediastinum sacro-coccygeal tertoma benign is CYSTIC form immature teratomas of ovaries usually seen in children
most common neoplasm in infant testes
endodermal sinus tumor (yolk sac tumor)
secretes AFP
Embryonal Ca
germ cell neoplasm in children
Choriocarcinoma
germ cell neoplasm in children
secretes b-HCG
Retinoblastoma
two-hit hypothesis
sporadic; not hereditary
first hit –> deletion of RB1 gene on chromosome 13
see leukocoria
