Gu Path - 14 - Pediatric Tumors

Front 1

Common benign masses in kids...........................

Back 1

HAMARTOMAS
HETEROTOPIAS (CHORISTOMAS)
PERSISTENT EMBRYONIC REMNANTS
Thyroglossal cysts
Branchial cleft cysts
Urachal cysts- around bladders

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Hamartoma

Back 2

Well differentiated
Disorganized
Wrong Place

Front 3

Heterotopias

Back 3

Well differentiated, Organized and in the wrong place.

Front 4

Most common tumor in infancy and second most

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Hemangioma,

Lymphangioma

Both are benign

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Embryonic tumor

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Embryonic tumors are tumors which
arise during embryonic, fetal, or early post-natal development from a particular organ rudiment or tissue while this is still immature”

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How embryonic tumors differ from other adult tumors ?

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1. They are intrinsic disturbances and not malformations

2. They can be identified as fetal or embryonic tissues in different stages of maturity

3. Are NEOPLASTIC from the outset

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This tumor is derived from all three germ cell layers, and you know its not a malformation because it is actively growing

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Teratoma -Composed of multiple tissues FOREIGN to the part in which it arises

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Major malformations occur in what percentage of child teratomas and what are they ?

Back 8

25% and they are defects of the posterior mesoderm resulting in

Meningomyelocele
Imperforate anus
Complex lower urogenital anomalies.
Hemihypertrophy (one side of the body)

Front 9

Anatomically, where do you find most teratomas

Back 9

Along the midline of the boddy with the sacrococcygeal being the most common place in infants

In young adults, its usually the gonads. moreso for girls

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Which cancers have the highest incidence in kids upto the age of 15 ?

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Leukemia + CNS

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Age of incidence for most malignant childhood tumors

Cancer likelihood - boys or girls ? Whites or blacks ?

Back 11

0-4

Boys

Whites

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Cancers
0-4 years

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Leukemia
Retinoblastoma
Neuroblastoma
Wilms Tumor

hepatoblastoma

Rhabdomyosarcoma

Teratomas
CNS tumors

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5-9 years

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Leukemia
Retinoblastoma

Hepato CA

CNS tumors
Ewings Sarcoma

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10-14

Back 14

Hodgkins
Non Hodgkins

Hepato CA

Osteosarcoma

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Small round blue cell tumor - pathologists interpretation ? 4 types

Back 15

A pathologist just knows that this is of embryonic origin and that it can be resected out.

The 4 types are
Alveolar rhabdomyosarcoma
Ewings Sarcoma
Burkitts Lymphoma
Poorly differentiated neuroblastoma

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Neprhoblastoma - (Wilms Tumor)

What are some less common clinical presentations other than a mass

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Most common renal malignancy
Boys and girls equally affected

Half occur under 3
More than 80% are cured

Less common :
less common:
- pain
- painless hematuria
- fever, weight loss, malaise
- mass effect (displacement of viscera)
- hypertension

Front 17

Clinical presentation of a nephroblastoma ?

Other congenital abnormalities associated ?

Back 17

Abdominal mass
Lab studies - NORMAL

Beckwith-Wiedemann syndrome (omphalocele, macroglossia, visceromegaly, and gigantism)

WAGR syndrome (Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation)

Drash syndrome (ambiguous genitalia, nephritis, and Wilms tumor)

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Histology of Wilms tumor ?

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It contains 3 elements
Blastema - primitive embryonic cells
--- In Wilms tumor, these go awry and proliferate autonomously
Epithelium - glomerular, tubular
Mesenchymal - muscle, cartilage

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Characteristic feature of persistent renal blastema ?

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Nephrogenic rest !

New nephrons develop at
the periphery (NEPHROGENIC ZONE) of the developing kidney lobules, not at
the center. The peripheral superficial tissue is called METANEPHRIC BLASTEMA

Front 20

Wilms is anaplastic. T or F

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True

Front 21

What are the three embryonic tumors of the sympathetic nervous system ?

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Neuroblastoma
Ganglioneuroblastoma
Ganglioneuroma

Front 22

What are the characteristic featuers of neuroblastomas ?

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Neurofilaments and ganglion cells

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Can neuroblastomas and ganglioneuroblastomas differentiate further ?

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yes, either by treatment or spontaneously, they can become ganglioneuromas

Front 24

Age range of NB and GNB ?

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Most found in children(M=F) under 10 yrs old

Front 25

Location of most neuroblastic tumors ?

Back 25

While most of these occur along the sympathetic chain, most of them occur in the adrenal glands

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A 5 year old comes in with an abdominal mass, and you are debating if its Wilms or neuroblastoma. What is an easy test to figure this out and why ?

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The easiest way is to measure VMA and HMA, since 90% of neuroblastic tumors produce excess cateocholamines in both BLOOD and URINE

Front 27

What is the clinical presentation of a neuroblastic tumor ?

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Incidental mass

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Several unique syndromes are associated with neuroblastomas
Hutchinson syndrome ?

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Hutchinson syndrome: malaise, low grade fever, and limp due to metastasis to bone and bone marrow

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Raccoon eyes

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proptosis and periorbital ecchymoses due to metastasis to retrobulbar and periorbital regions

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Blueberry muffin

Opsoclonus Myoclonus

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appearance: cutaneous metastasis

paraneoplastic syndrome – Immune sytem attacking the cells, but since the tumor is similar to own, then there is cross reactivity leading to paraneoplastic or immune destruction

Front 31

Characteristic histologic features of neuroblastomas

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Homer-Wright Pseudo Rossettes

and Neuropils

Front 32

What are the most differentiated neuroblastic tumors ?

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Ganglioneuroma : Has the best prognosis

Front 33

What should you know about the Shimada classification

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- Younger better
- High MKI worse
- Undifferentiated worse

Front 34

Which staging for neurblastomas have the better prognosis

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1,2A,2B and 4S

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What percent of NB have already metastasized ?

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50%

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Favorable vs Unfavorable

Stage

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1,2a,2b,4s vs 3,4

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Age

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<1 vs >1

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Histology

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Stroma is present and adequate (Schwannian stroma rich) vs Absent (Schwannian stroma poor)

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MKI

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<= 200/5000 cells vs >200/5000 cells

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DNA ploidy

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Hyperdiploid or near triploid

vs

Diploid or near tetraploid

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N -myc

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Not amplified vs amplified

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Chromosome 17q gain

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Absent vs present

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Chromosome 1p loss

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Absent vs Present

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What has happened to the 5 year survival rates of all pediatric tumors since the 70s

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GONE UP !

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Back 45

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Front 46

3 most common tumors in infants and children

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Leukemias
Brain tumors
Lymphomas

Front 47

Leukemias which is more common ALL or AML ?

Brain tumors location ?

Hodgkins vs Non Hodgkins

Sarcomas vs soft tissue

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75% vs 25%

Mostly infratentorial

Hodgkins - nodular sclerosing

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Examples of heterortopias

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adrenal tissue in spleen

Meckel’s diverticulum w/ pancr. tissue

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Histological arrangement of a Teratoma

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Neural tissue (N) derived from ectoderm
lies adjacent to mesodermal (M) derivatives
(smooth muscle and fibroblasts) which in
turn abuts endodermal derivative (E) mucin
secreting gut epithelium.

Front 50

Characteristic histological features of a teratoma

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Teratoma - Neural tissue (Neu) lies immediately adjacent to
epithelium (Epi) typical of the gut in the center of a mass.

Front 51

Where do you find teratoms in infants vs young adults ?

Back 51

Presacral & coccygeal
Retroperitoneal
Pineal (other CNS)

vs

Neck
Ovary / testes
Ant. mediastinum

Front 52

What kind of tissue is found in 75% of Wilms Tumor ?

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Nervous tissue

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Most common tumors found in malignant teratomas

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embryonal carcinomas, adenocarcinomas, sarcomas

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Three major components in classic Wilms tumor

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- primitive glomeruli

- primitive tubules

- malignant stroma

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Other variant of Wilms tumor

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Epithelial and blastemal variants

glomeruloid in upper half vs epithelial andblastemal in lower

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Neuroblastoma

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most malignant of neuroblastic tumors
- tumor cells resemble embyronic neural crest cells

CAN SPONTANEOUSLY REGRESS

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Ganglioneuroblastoma

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- same malignant potential as neuroblastoma
- immature neuroblasts: undifferentiated embyronic
neural crest cells & differentiated cells (ganglion
cells & neural fibers)

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Ganglioneuroma

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- benign
- ganglion cells & neural fibers in no specific patterns

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Other neural crest abnormalities associated with NB

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* neurofibromatoses carcinoid tumors
pheochromocytoma medullary thyroid carcinoma
(MEN 2A and 2B syndromes)

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Most common site of a neuroblastoma

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Retroperitoneum and adrenals

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Gross usually a large tumor at dx,
friable, hemorrhagic w/ foci of
necrosis and multiple small
calcifications

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Neuroblastoma

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Histology of a neuroblastoma

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cells lie in
sheets w/ intervening neurofibrillary stroma (neurofibrils)
small round blue cells (round to oval
dark nuclei, narrow rim of cytoplasm
cell margins often indistinct vs lymphocytes & other tumors)

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The typical pseudorosette has a tangle of
neurofibrillary fibrils within a ring of small ovoid nuclei

Back 63

Neuroblastoma - PagF