PEDIATRIC TUMORS Page 1 of 6

Disease Age Most Clinical Symptoms Radiographic Pathologic Features Prognosis Genetics / Other
Common Features Information
Sites
Retinoblastoma Commonly RETINA Decreased visual N/A Primitive small blue Untreated, fatal. Loss of tumor
around age 2 acuity, strabismus, cells (PSBCs from But, with early suppressor gene (Rb)
(familial = whitish hue to pupil, here on) making intervention, survival on long arm of
earlier, multiple pain and tenderness rosettes around is common. Chromosome 13.
tumors) in the eye lumens (Flexner- Treatment = Can be sporadic
Wintersteiner enucleation, (more common) or
Rosettes) radiation, chemo familial (in which
case, more likely
multiple tumors and
bilateral)
Neuroblastoma (also 85% <5 yo, 50% Anywhere Secrete Because areas Cells range from Degree of The most
ganglioneuroblastoma, <2 yo, but can along catecholamines in of widespread PSBCs to more differentiation differentiated (mature
ganglioneuroma) arise in older sympathetic >90%, therefore their necrosis are differentiated cells correlates with ganglion cells) =
children, rarely chain, but 40% breakdown products common, these with elongated prognosis (more = ganglio-neuroma. It
in adults. in adrenal (VMA, HVA) can be areas can cytoplasmic better). is benign.
medulla. detected in the urine. calcify processes (primitive Intermediate =
Other hormones may (dystrophic neurites). More The prognosis is ganglioneuro-
be produced. calcification), differentiated tumors highly variable blastoma.
and these can may contain (possibly infants with tumors
be seen on entirely) mature limited to primary Genetics:
radiographs. ganglion cells or sites or Chr 1p abnormality,
intermediate cells metastasized to N-myc amplification
(ganglioneuroblasts). skin, liver, marrow: = poor prognosis
Rosettes may be excellent, tumor
presentprocesses may even
oriented inward, no spontaneously
lumen (Homer- regress.
Wright rosettes).
Older children,
especially if met to
other sites, 90%
fatal.

Familial cases
uncommon, poor
prognosis (1p abnl)
PEDIATRIC TUMORS Page 2 of 6

Disease Age Most Clinical Symptoms Radiographic Pathologic Features Prognosis Genetics / Other
Common Features Information
Sites
Wilms Tumor Usually KIDNEY Most frequently Calcification not Soft, encapsulated If spread beyong The two WT tumor
diagnosed detected by parents as prominent as tumor. Histologically, renal capsule, or if suppressor genes =
between the as an abdominal neuroblastoma, 3 components: histologic anaplasia chromosome 11.
ages of 2 and 5. mass (asymptomatic so not as well epithelium (tubules), (tumor cells >3X Familial and sporadic
at detection). seen on stroma (usu fibroblast size of neighboring forms.
radiographs. like, maybe skel cells, atypical
muscle, cartilage), mitoses) = poor Associated with
and blastema prognosis. several syndromes:
(PSBCs).
Prior to 1960s, 5 yr - Beckwith-
survival 10-40%, Wiedemann
now 90%). (organomegaly,
macroglossia,
hemihypertrophy, inc.
risk of
hepatoblastoma,
rhabdomyosarcoma,
adrenocortical
tumors, brain tumors)

- WAGR complex
(Wilms, aniridia (no
iris), GU malform.,
mental retardation)

- Denys-Drash
(gonadal dysgenesis,
nephropathy)
Rhabdomyosarcoma 2/3 <10 yo, also Head and neck Palpable mass or N/A Primitive cells w/ Good: embryonal, Alveolar subtype =
teens, rarely region, GU symptoms due to a evidence of skeletal botyroid/spindle cell t(2;13) or t(1;13).
adults tract, mass muscle differentiation. subtypes. (95, 88%
extremities, Actin/myosin Z-bands 5yr surv)
trunk. on EM, cross-
striations possible on Intermediate:
light micro. Other embryonal, not
cases, PSBCs. subtyped (MOST
COMMON) 66%
5yr surv)

Poor: alveolar
(common (31%),
53% 5 yr surv)
PEDIATRIC TUMORS Page 3 of 6

Disease Age Most Clinical Symptoms Radiographic Pathologic Features Prognosis Genetics / Other
Common Features Information
Sites
Rare, poor:
anaplastic, diffuse;
undifferentiated
sarcoma (<50% 5 yr
surv)

In general, extent of
disease is the most
important prog
indicator (site and
subtype also play a
role). Localized =
70-85% 5 yr surv;
mets = 30%)
Ewings Sarcoma Second decade Pelvis, femur, Pain, fever, signs Bone lesion PSBC tumor, usu 1960s = 10% 5yr t(11;22)
(peripheral humerus resembling infection often arranged in sheets, surv. Today, 50%, characteristic, less
neuroepithelioma/PNET diaphyseal, nests, organoid but metastatic = commonly t(7;22) or
(primitive neuroectodermal lytic, patterns. much worse. t(21;22).
tumor) permeative;
prominent PNET vs. Ewings?
periosteal - PNET = greater
reaction, soft neural differentiation
tissue mass (rosettes), Ewings =
common. sheets, etc
- Same translocation,
so probably just
variable histology of
same tumor.
Hemangioma Most common Skin, usu head Some irregularly N/A Blood vessels of Benign. N/A
tumor of infancy. and neck raised (strawberry varying sizes,
Histologically region hemangiomas); capillaries to larger
similar to adult others flat and vessels. Mostly
hemangiomas, widespread (port cutaneous.
but many will wine stains).
spontaneously
regress.
Teratoma Congenital. Most in the Can be N/A Tissues from all three Normally benign, but Rather common
Depends on age sacrococcygeal extraordinarily large embryonal layers, all 10-15% contain tumor 1/20,000 to
of discovery, not area, others (largest tumor on of which are generally areas of immaturity/ 1/40,000 live births.
age of onset mediastinal, record 300 lb mature/benign. malignancy.
other midline teratoma)
locations. Older children =
more likely
PEDIATRIC TUMORS Page 4 of 6

Disease Age Most Clinical Symptoms Radiographic Pathologic Features Prognosis Genetics / Other
Common Features Information
Sites
malignant.
Langerhans Cell Any age may be Isolated, lytic Bone lesions, may Lytic bone Clonal proliferation of Excellent. Adverse Immunohistochemical
Histiocytosis (LCH) affected, most bone lesions, be associated with lesions. If in Langerhans cell outcome correlated markers: S-100 and
(Histiocytosis X, are <5 yo at usually diabetes insipidus long bones, usu histiocytes with: CD1a.
Eosinophilic granuloma, diagnosis. craniofacial. and exophthalmos in diaphysis. (macrophages), >3 bones involved
Langerhans cell (Hand-Schuller- with variable amounts hepatosplenomegaly
granulomatosis Christian of inflammatory cells thrombocytopenia
Syndrome) or be (principally eos, less young age at
disseminated often PMN/lymphs). diagnosis (<3 yo)
(Leterrer-Siwe Histiocytes =
Syndrome). folded/grooved nuclei
(both of these with abundant
syndromes are rare cytoplasm.
HSC <5%, L-S even EM = Birbeck
less) granules (tennis-
racquet shaped)
within cytoplasm.
PEDIATRIC TUMORS Page 5 of 6

Disease Age Most Clinical Symptoms Radiographic Pathologic Features Prognosis Genetics / Other
Common Features Information
Sites
Osteosarcoma Predominantly Distal femur Pain, swelling or Permeative, Strict definition: Traditionally treated
second decade; and proximal mass, frequent destructive, malignant tumor in by amputation or
secondary forms tibia (knee pathologic fracture. maybe which the tumor cells disarticulation of
older (5th decade bones), but lytic/blastic, produce malignant affected limb; but 5
+) virtually any Codmans osteoid/bone. yr surv <=20%.
bone possible. triangle Chondroid and Now, chemo + limb-
(periosteal hyaline cartilage may sparing surgery 5
reaction also be seen. yr surv <= 60%.
elevated s.t.
triangular area Histologic subtypes: Prognosis is
between cortex correlated with post-
& periosteum), Intramedullary chemotherapy
frequent soft (within bone): necrosis. If >= 90%
tissue - conventional of tumor has
extension osteoblastic, necrosed, good
chondroblastic, prognosis. <90%,
fibroblastic. MOST bad. (Necrosis =
COMMON. tumor cell dropout
- well-differentiated bone with holes
resembles fibrous where tumor used to
dysplasia w/o typical be)
clinical/radiologic
features.
- small cell
resembles Ewings
but assoc with
malignant bone
production
- telangiectatic
may resemble an
aneurysmal bone
cyst, but obvious
anaplastic malignant
cells.

Juxtacortical (arises
from bone surface):
- parosteal well-
differentiated
involving cortical
bone surface.
- periosteal
chondroblastic
PEDIATRIC TUMORS Page 6 of 6

Disease Age Most Clinical Symptoms Radiographic Pathologic Features Prognosis Genetics / Other
Common Features Information
Sites
involving cortical
bone surface
- high grade surface
conventional
osteosarcoma
involving cortical
bone surface.

Secondary:
- irradiation, Pagets
disease, fibrous
dysplasia all usually
high grade
conventional OS.