Pediatric tumors

Although childhood cancer account for only 2%of all reported

.cancer,they account for 10% of all death in children

.The incidence varies by age,gender,and race

The exact diagnosis is often more difficult to establish due to

common histological appearance given that chemotherapy
regimens must be carefully tailored to each specific tumor
.type

Tumor biology; genetic alteration within a single cell such as

activation of an oncogene or the loss of tumor suppressor gene
can lead to the accumulation of cells lacking the ability to
respond to growth –regulating signals and the subsequent
.development of cancer

Wilms tumor
Renal tumors are the second most common abdominal tumor
seen in infant and children behind neuroblastoma.and it is the
most frequent tumor of kidney .the mean age at diagnosis is 36
months,it is often noted during bath or by the pediatrician or as
an abdominal pain post truma

.US is the diagnostic tool with Doppler and abdominal CT scan

Treatment including operation ,chemotherapy and radiation

.therapy
Neuroblastoma
Neuroblastoma is the most common solid extracranial
malignancy of childhood and infants,it is an embryonal tumor
of sympathetic nervous system so it can originate from the
adrenal medulla,paraspinal sympathetic ganglia and
sympathetic paraganglia.so it can arise in adrenal
.gland,posterior mediastinum,cervical region and pelvis

Neuroblastoma is characterized by several unique clinical

behavior including the secreation of catecholamine products
.and the potential to regress or mature

The patient usually presents with signs and symptoms that

reflect the primary site and the extent of disease, abdominal
mass,respiratory distress,urinary problem ,horner syndrome
.,acute cerebellar ataxia ,anemia and bone marrow infiltration

Diagnostic imaging;
US,CT,MRI,metaiodobenzylguaidine(MIBG),bone marrow
.examination in addition to laboratory finding

Complete resection of tumor offers definitive therapy with

.generally excellent outcome
Teratomas
Teratoma from the Greek teratos(of the monster) are
composed of multiple tissues foreign to the organ or site from
which they arise, they are generally divided into gonadal and
.extragonadal types

Teratomas arise from totipotent primordial germ cells or from

the remnants of the primitive streak or primitive node so it can
composed of all three layers(endoderm,mesoderm and
.ectoderm) and even malignant cells

It is premalignant condition if not resected completely in early

life

The tumor site sacrococcygeal ,thoracic,abdominal and head

and neck teratomas

Diagnosis by :antepartum US,neoborn US,CT scan ,alfa-feto

.proten,B HCG

.Early complete surgical excision is the treatment of choice

Rhabdomyosarcoma
Rhabdomyosarcoma is a soft tissue tumor originating from
immature mesenchymal cells that form any tissue except bone

Diagnosis done by taking biobsy

.Treatment with surgical resection with or without radiotherapy

Bladder and cloacal exstrophy
Exstrophic anomalies referred to as exstrophy-epispadias
:complex including

Epispadias urethra open on the dorsal surface of the -1

phallus

classic bladder exstrophy-2

cloacal exstrophy known as the -3

omphalocele\exstrophy\imperforate anus\spinal defect (OEIS)
.complex

.exstrophy variants-4
Imperforate anus and cloacal malformations
Imperforate anus is a condition known since antiquity when the
baby born with no anal orifice or abnormal position of it ,it can
be divided into high type where the rectum end above the
levator ani muscle or low type where the rectum inter through
.the levator muscle and end in different levels

The incidence is 1:4000-5000 birth and associated with DOWN

.syndrome

Male anorctal defects

Rectoperineal fistula

Rectourethral fistula lower bulbar

High prostatic

Rectobladderneck fistula

Imperforate anus without fistula

Rectal atresia\rectal stenosis

Female anorectal defects

Rectoperineal fistula

Rectovestibular fistula

Imperforate anus without fistula

Persistent cloaca

Associated defects including missing of one or several sacral

vertebrae which effect the prognostic implications

It can be associated with VACTREL anomalies

(vertebrae,anal,cardiac,trachea,renal,esophagus,and limb )

Management of newborn baby

Resustation of the baby

Proper examination with careful inspection

X-Ray in upside down baby (the head is down and lower limbs
up) in lateral view after putting a land mark on the dimple of
expected anal site ,so that we use the gas as contrast media to
.determine the rectum after 12-24 h

In low type fistula primary anoplasty can be done

In high type proximal colostomy should be done followed by

PSARP(posterior sagittal anorectoplasty ) done at few months
old then closure colostomy done

The prognosis depends on the development of pelvic floor

. muscles
Biliary atresia
Biliary atresia is a relatively rare obstructive condition of the
.biliary ducts causing neonatal jaundice

Reovirus type 3 infection,rotavirus,

cytomegalovirus,papillomavirus and Epstein-barr virus all
proposed as possible etiologic agents.even genetic mutation

Classification
Macroscopic appearance and cholangiography finding classify
:biliary atresia into

Atresia of common bile duct (type 1)

Atresia of common hepatic duct (type 2)

Atresia of CBD and the common hepatic duct (type 2b)

Atresia of all extrahepatic bile duct up to the porta hepatis(type

.3) which is most common

Clinical presentation and diagnosis

Cardinal signs are jaundice,clay-color stool,
.hepatomegaly,growth retardation,anemia

Jaundice more than 2 weeks should be investigated by US,liver

.function test and liver biopsy to confirm the diagnosis

Open surgical technique for portoenterostomy(kasai operation)

.Liver transplantation has an indication too

Choledochal cyst
.Chledochal cyst is a congenital dilatation of the biliary tract

Classification

Type 1
1a cystic dilatation of the CBD

1b fusiform dilatation of the CBD

Type 2 diverticulum of the CBD

Type 3 choledochocele (dilatation of the terminal CBD within
the duodenal wall)

Type 4
4a multiple cysts of the extrahepatic and intrahepatic ducts

4b multiple extrahepatic duct cysts

Type 5 intrahepatic duct cyst(single or multiple)

The etiology unclear ,it can be congenital or acquired,female

more than male

US ,ERCP ,MRCP ,are the diagnostic tools

Cyst excision and a bilio-enteric anastomosis is the preferred

.approach for most of patients
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