Abdominal Tumors

WILM’S TUMOR

Clinical Presentation
•Asymptomatic mass in the flank or upper abdomen
•Discovered by parent while bathing or dressing the child; following a blunt abdominal
trauma
•Associated symptoms: hypertension, hematuria, obstipation or weight loss

Syndromes Associated with Wilm’s Tumor

•WAGR
•Aniridia, genitourinary abnormalities, mental retardation
•Denys-Drash
•Early onset Renal Failure with renal mesangial necrosis, ale pseudohermaphrodism,
inc. risk of wilm’s tumor
•Beckwith-Wiedenhamm
•Organomegaly (liver, kidney, adrenal, pancreas), macroglossia, omphalocoele,
hemihypertrophy

Epidemiology
•Most common primary malignant tumor of the kidney in children
nd
•2 most common malignant abdominal tumor in childhood
•Mixed embryonal neoplasm
(blastema, epithelia, stroma)
•Diagnosed b/w: 1-5 yrs old
•Peak incidence: 3 yrs old
•Overall cure rate: 90%

Causes
•Sporadic
•Familial incidence: 1-2%, autosomal dominant, 19q13, 17q
•WT mutation: chr. 11 (chr 11p13)
o WT1: 20% - critical to the development of the genitals and urinary system
o WTX: 30%

Diagnostic Procedures
•Ultrasonography
o Initial procedure
o Useful not only in confirming that the mass originates from the kidney but also
whether the mass is cystic or solid.
o Typical ultrasound findings are a large, well-defined mass originating from the
kidney, usually increased in echogenecity but often heterogenous secondary to
accompanying hemorrhage or calcification.
o Doppler ultrasound is excellent in evaluating the presence and extent of renal
vein and IVC tumor thrombus.
•CT
o Large, spherical intrarenal mass that enhances to a lesser degree than the
remaining normal renal parenchyma.
o Areas of heterogenous attenuation can be present secondary to hemorrhage,
calcification, or fat.
•MRI
o isointense to renal parenchyma on T1-weighted images
o hyperintense on T2-weighted images
o enhancement but to a lesser degree than that of normal renal parenchyma after
intravenous administration of gadolinium contrast material

Lab studies

•Complete blood count

•Basic metabolic panel
•Coagulation abnormalities (to rule out acquired von Willebrand disease)
•Chest x-ray - As a baseline for pulmonary metastases
•Bone scan - Necessary for children with clear cell sarcoma of the kidney

Staging
 Stage 1 – cancer is found only in one kidney and can be completely removed by
surgery
 Stage 2 – cancer is still in only one kidney, but has spread to the areas near the
kidney. It can be completely removed by surgery.
 Stage 3 – Cancer is still only in one kidney but has spread to areas near the kidney,
and cannot be completely removed by surgery. The cancer may have spread to
important blood vessels or organs near the kidney or throughout the abdomen, so that
the doctor cannot remove all the cancer during surgery. The cancer may also have
spread to the lymph nodes (small bean-shaped structures found throughout the body
that produce and store infection-fighting cells) near the kidney.
 Stage 4 – Cancer has spread to organs further away from the kidney, such as the
lungs, liver, bone, and brain.
 Stage 5 – cancer cells are found in both kidneys when the disease is first diagnosis.
Each kidney will be staged separately as I, II, III, or IV.

Treatment Generalities
•Surgery
o If possible, the surgeon will try to surgically remove the tumor and leave the rest
of the kidney in place. If the surgeon is unable to remove the entire tumor, a
biopsy (a small piece of the tumor) is taken to be able to determine the stage of
the tumor.
•Chemotherapy
o Given to all kids diagnosed with Wilm's Tumor
•Radiation therapy
o may be given along with the chemotherapy if the disease has spread outside of the
kidney.
 o The cure rate for the lower stages of Wilm's Tumor is 90 percent. This is among
the highest cure rate in all pediatric cancers.
o The other good news is that the therapy used does not usually cause long term
side effects. However, survivors of this disease usually have only one of their two
kidneys.

Treatment by Stage
•Stage 1
o Nephrectomy with lymph node removal followed by combination chemotherapy.
•Stage 2
o Nephrectomy with lymph node removal followed by combination chemotherapy.
•Stage 3
o Nephrectomy with removal of lymph nodes, followed by radiation therapy to the
abdomen and combination chemotherapy.
•Stage 4
o Nephrectomy with removal of lymph nodes, followed by radiation therapy to the
abdomen and combination chemotherapy. If cancer has spread to the lungs,
patients will also receive radiation therapy to the lungs.
•Stage 5
o Chemotherapy to shrink the tumor, followed by surgery to remove as much of the
cancer as possible. This may be followed by more chemotherapy and/or radiation
therapy if cancer remains after surgery.

NEUROBLASTOMA

Clinical Presentation

 Varies with site of presentation

 Generally, symptoms include abdominal pain, emesis, weight loss, anorexia, fatigue,
bone pain, and chronic diarrhea.
 Hypertension is an uncommon sign of the disease and generally is caused by renal
artery compression, not catecholamine excess.
 Because more than 50% of patients present with advanced-stage disease, usually to
the bone and bone marrow, the most common presentation includes bone pain and a
limp.
 The presence of bone metastases can lead to pathologic fractures.
 Approximately two thirds of patients with neuroblastoma have abdominal primaries.
In these circumstances, patients can present with an asymptomatic abdominal mass
that usually is discovered by the parents or a caregiver.
 In a small proportion of infants younger than 6 months, neuroblastoma presents with
a small primary tumor and metastatic disease confined to the liver, skin, and bone
marrow (Stage 4S). If this type of tumor develops in neonates, skin lesions may be
confused with congenital rubella, and, if the patient has severe skin involvement, the
term "blueberry muffin baby" may be used.
Epidemiology

 Embryonal cancer of the peripheral sympathetic nervous system

 3rd most common pediatric cancer esp. in infants < 12 months
 one in 7,000-10,000 live births
 most common solid malignant tumor in infancy, and children outside the CNS

Causes

 Genetic trigger: unknown

 Neuroblastic tumors arise from primitive sympathetic ganglion cells which are
derived from neural crest cells during fetal development.
 Neural crest cells are normally destined to become adrenal medulla and sympathetic
nervous system.
 Amplified MYCN, hyperdiplody of tumor cell DNA content

Diagnostic Procedures

• Ultrasound
o cystic, solid, or complex mass in the region of the adrenal gland, directly above
the level of the kidney and under the diaphragm. Occasionally, calcifications are
apparent within the tumor.
o Polyhydramnios and fetal hydrops may accompany the lesion. The tumor can
metastasize in utero (placenta, liver, blood vessels).
• CXR / SFA
o evaluate for the presence of a posterior mediastinal mass or calcifications.
• CT scan of the primary site
o determine tumor extent
o main body of the tumor usually is indistinguishable from nodal masses.
• MRI
o presence of intraspinal tumor and cord compression.
• I123/131-methyliodobenzylguanadine (MIBG)
o accumulates in catecholaminergic cells and provides a specific way of identifying
primary and metastatic disease if present
• Tc 99 bone scan
o evaluate bone metastases

Lab Studies

 Serum lactate dehydrogenase (LDH, useful as biologic marker)

 Ferritin (useful as biologic marker)
 CBC and differential (Anemia or other cytopenias suggest bone marrow
involvement.)
 Urine collection for catecholamines (VMA/HVA)
o The spot test for VMA/HVA is highly inaccurate.
 o elevated if it is 3 SDs higher than the age-related reference range levels.
 Serum creatinine
 Liver function tests
 Electrolytes
 Calcium
 Magnesium
 Uric acid

Staging (International Neuroblastoma Staging System)

 Stage 1
o Localized tumor with complete gross excision and/or microscopic residual disease
o Ipsilateral lymph nodes negative for tumor (Nodes attached to the primary tumor
may be positive for tumor.)
 Stage 2A
o Localized tumor with incomplete gross resection
o Representative ipsilateral nonadherent lymph nodes negative for tumor
microscopically
 Stage 2B
o Localized tumor and/or complete gross excision, with ipsilateral nonadherent
lymph nodes positive for tumor
o Enlarged contralateral lymph nodes, which are negative for tumor microscopically
 Stage 3
o Unresectable unilateral tumor infiltrating across the midline and/or regional
lymph node involvement
o Alternately, localized unilateral tumor with contralateral regional lymph node
involvement
 Stage 4
o Any primary tumor with dissemination to distant lymph nodes, bone, bone
marrow, liver, skin, and/or other organs (except as defined for stage 4S)
 Stage 4S
o Localized primary tumor (as defined for stages 1, 2A, or 2B) with dissemination
limited to skin, liver, and/or bone marrow (<10% involvement)
o Limited to infants

Treatment
 Surgery, irradiation and chemotherapy depend on the stage
 Early disease without MYCN amplification or chromosome 1p deletion- surgery
alone
 Advanced disease= surgery + chemo
 Cisplatin, etoposide, vincristine, cyclophosphamide
HYDRONEPHROSIS

Clinical Presentation
 Newborn: Often silent except for a palpable abdominal mass
 When severe: infection, pain and bleeding
 If there is infection:
o Dysuyria
o cloudy urine
o back pain
o fever
o difficulty passing urine, either by being irregular or uncontrolled.

Epidemiology
 The incidence of fetal hydronephrosis caused by UPJ obstruction detected in routine
antenatal ultrasound screening is nearly 1 in 500 live births
 Boys are affected with UPJ obstruction more commonly than are girls.
 Lesions are found more commonly on the left than on the right side, and 10 to 40
percent of cases are bilateral
 Usually diagnosed antenatally

Causes
 Dilatation of the upper urinary tract
 2° to urinary tract obstruction
 Congenital (Anatomic), Trauma, Neoplasia. Calculi. Inflammation, Surgical procedures

Common congenital etiologies

 Ureteropelvic junction (UPJ) Obstruction
o Most common in neonates
o Intrinsic stenosis, abnormal development of smooth muscle at the UPJ, extrinsic
compression by an aberrant lower pole vessel crossing the UPJ
 Vesicoureteral Reflux
o 2nd most common
o Primary reflux: congenital anomaly caused by a deficiency of the longitudinal
bladder muscle surrounding the intramural portion of the ureter
o Secondary reflux: bladder outlet obstruction and an increase in intravesical
pressure.
 Ureterocele
o Cystic dilatation of distal ureter due to stenotic ureteral opening
o 80% assoc with upper pole- moiety of a duplicated ureter.
o duplicated ureter with associated reflux (65%)
 Posterior urethral valve
o Most common cause of bilateral hydronephrosis
 o Obstruction due to thin membranous folds (valves) in the prostatic urethra
o Common cause of bilateral hydronephrosis

Diagnostic Tests
 Prenatal US
o renal pelvic diameter is used to evaluate the significance of dilation,
o evaluate renal architecture, renal size, and echogenicity of the renal parenchyma
 Renal US
o Rapid, inexpensive, and reasonably accurate method of detecting hydronephrosis
o serves as the preferred screening test to establish the diagnosis of hydronephrosis
 VCUG
o Ureteral reflux and urethral obstruction
o x-ray examination of a child's bladder and lower urinary tract
o uses fluoroscopy and a water-soluble contrast material injected through a catheter
o view and assess the anatomy and function of the bladder and lower urinary tract
 Excretory Urogram / intravenous pyelogram – Renal pelvis, ureteral filling defects
o Easily identify intraluminal causes
o Functional study (determine the functional status of a chronically obstructed
kidney)
 Tc 99 DTPA
o Diethylenetriaminepentaacetic acid injected intravenously, followed by
furosemide administered after 10 min
o <15 minutes = no obstruction
o >20 minutes = obstruction

Laboratory Studies
 Urinalysis: Assess for signs of infection
 Complete blood cell count: Leukocytosis may indicate acute infection
 Serum chemistry: Bilateral hydronephrosis can result in an elevation of BUN and
creatinine levels. In addition, hyperkalemia can be a life-threatening condition

Management
 Medical
o UPJ Obstruction
 Watchful waiting up to 2 years with antibiotic prophylaxis
o Vesicoureteral Reflux
 Determine the grade of the reflux:
 Spontaneous resolution:
 Grade I-II: (85%)
 Grade III-IV (30-40%)
 Grade V (5%)
 Conservative Mngt regardless of grade: w/ antibiotic prophylaxis
 Surgical
o UPJ Obstruction
 Severe hydronephrosis
breakthrough UTI
dismembered pyeloplasty: renal pelvis and ureter anastomosed (the
dyskinetic segment resected)
 Nephrectomy: w/ minimal function
o Vesicoureteral Reflux
 breakthrough infection
 Before puberty in girls
 Gold standard: open surgical reimplant of the ureter into the bladder
 Bulking agent injection- to increase resistance in the ureteral orifice with
cystoscopy
o Ureterocele
 Urosepsis: emergent endoscopic incision of the ureterocele and
establishing ureteral drainage.
o Posterior urethral valve
 Cathetherization
 Endoscopic ablation of the valve
 Monitor renal function (high risk for renal failure)

HEPATOBLASTOMA

Clinical Manifestation
 large, asymptomatic abdominal mass.
 three times in the right lobe compared to the left lobe.
 Uusually unifocal.
 As the disease progresses, weight loss, anorexia, vomiting and abdominal pain may
ensue.
 Hepatoblastoma also undergoes metastatic spread which may usually involve regional
lymph nodes and lungs.

Epidemiology
 predominantly in children younger than 3 years of age
 79% of all liver tumors in children and almost two thirds of primary malignant liver
tumors in the pediatric age group.
 Annual incidence of hepatoblastoma in infants younger than 1 year is 11.2 cases per
million

Causes
 Etiology is unknown but are associated familial adenomatous polyposis; alterations in
the antigen-presenting cell (APC)/ beta-catenin pathways
 For epithelial type, contains fetal or embryonal malignant cell (either mixture or pure
elements). For mixed type is usually contains mesenchymal and epithelial elements.
Lastly for pure fetal histology, it predicts a more favorable outcome

Diagnosis
Imaging Studies:
o Radiographs
 Flat plate abdominal radiographs will demonstrate a RUQ abdominal mass
 Chest radiographs can be used to determine the presence of lung metastasis
o Ultrasound
 Hepatoblastomas usually appear hyperechoic
 Will help in determining vascularity of tumor because vessels are hypoechoic
compared to liver parenchyma
 For assessment of anatomical involvement and tumor size
o CT scan
 Contrast enhanced abdominal CT
o demonstrate involvement of adjacent structures and lymph nodes
 Chest CT can also be done to determine lung metastasis

Laboratory Studies
o - fetoprotein
 Increased in 85%-90% of patients with hepatoblastoma
 Used to monitor therapeutic response
o Interpretation can be difficult because - fetoprotein is relatively
elevated in infants < 1 y/o
o reaches adult levels at ~1st year of life
o Usually falls to normal levels ~4-6 weeks after resectioning of tumor
o Complete blood count
 Mild anemia and thrombocytosis have been found in some patients
o Glomerular filtration rate and/or creatinine clearance
 Baseline GFR and creatinine clearance should be determined before
administration of some chemotherapeutic agents (particularly cisplatin)

Staging
 Stage I
o Tumor is completely resectable via wedge resection or lobectomy.
o Tumor has PFH results.
o AFP level is within reference range within 4 weeks of surgery.
 Stage IIA
o Tumor is completely resectable.
o Tumor has histologic results other than pure fetal histologic results (PFH).
 Stage IIB
o Tumor is completely resectable.
o AFP findings are negative at time of diagnosis (ie, no marker to follow).
 Stage IIC
o Tumor is completely resected or rendered completely resectable by initial
radiotherapy or chemotherapy, or microscopic residual disease is present.
o AFP level is elevated 4 weeks after resection.
 Stage III (any of the following)
o Tumor is initially unresectable but is confined to 1 lobe of liver.
o Gross residual disease is present after surgery.
o Tumor ruptures or spills preoperatively or intraoperatively.
o Regional lymph nodes are involved.
 Stage IV
o Distant bone or lung metastasis is present.

Treatment

 Chemotherapy
o vincristine (VCR), cyclophosphamide (CPM), and doxorubicin with 5-
fluorouracil (5-FU)
o Cisplatin is the most active single agent used to treat hepatoblastoma.
Doxorubicin is active as well.
o Cisplatin/5-FU/VCR combination was regarded as standard in hepatoblastoma.
 Radiotherapy
o 1200-2000 cGy. These dose limits are based on the liver's limited ability to
regenerate after radiation
o May be used when microscopic disease is seen at the resection margins
 Surgery
o Initial resection of operable primary tumors by lobectomy is the standard of care.
o Second-look laparotomy is warranted if AFP levels remain elevated following
resection.
o The most frequent complication of surgery is intraoperative hemorrhage
o In cases involving a substantial portion of the liver, particularly when
diaphragmatic extension precludes complete surgical resection, liver transplant
has been advocated.
o Increasing evidence exists that arterial chemoembolization is feasible in
unresectable hepatoblastoma and/or patients who are not candidates for liver
transplant.

RHABDOMYOSARCOMA

Clinical Presentation

 Symptoms vary depending on the location of the tumor

 Abdominal mass
 most common sites are the head and neck (28%), extremities (24%), and
genitourinary (GU) tract (18%). Other notable sites include the trunk (11%), orbit
(7%), and retroperitoneum (6%)

Epidemiology

 6 cases per 1,000,000 population per year

 Bimodal age predilection: 2-5y/o and 15-19y/o

Causes

 cause of RMS is unknown

 believed to arise from primitive muscle cells, tumors can occur anywhere in the body
except bone

Diagnostic Procedure

Imaging Studies

 Plain radiography: helpful to determine the presence of calcifications and bone

involvement of the primary tumor and to search for metastatic lung lesions.
 CT scanning
o evaluate for metastases to the lungs
o Obtain a CT scan of the liver in patients with abdominal or pelvic primary tumors
to assess for metastatic spread. Ultrasonography is an alternative.
o Findings :Axial and coronal contrast enhanced CT demonstrates a heterogenous
strongly enhancing mass originating from the right masticator space, with skull
base invasion and intracranial extent. The skull base foramina are destroyed in
this region, but the tumor may have extended through the foramen ovale initially.
Coronal and axial enhanced T1 images show the enhancing mass with infiltration
of the right pterygoid muscles.
 MRI: MRI improves definition of the mass and its invasion of adjacent organs,
especially in orbital, paraspinal, or parameningeal regions. Obtain an MRI of the head
if the patient is symptomatic at diagnosis.
 Bone scanning: Search for metastases to the bones.
 Ultrasonography: Obtain sonograms of the liver in patients with abdominal or pelvic
tumors. CT scanning is an alternative.
 Echocardiography: Assess cardiac function before chemotherapy.

Lab Studies

• CBC: Anemia may be present because of inflammation, or pancytopenia may be

present from bone marrow involvement.
• Liver function tests, including measurement of lactic acid dehydrogenase (LDH),
aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline
 phosphatase, and bilirubin levels: Metastatic disease of the liver may affect values of
these proteins. Liver function must be assessed before chemotherapy.
• Renal function tests, including measurements of BUN and creatinine levels: Renal
function must be assessed before chemotherapy.
• Urinalysis (UA): Hematuria may indicate involvement of the GU tract.
• Blood electrolyte and chemistry, including evaluation of sodium, potassium, chlorine,
carbon dioxide, calcium, phosphorous, and albumin values: Assess for abnormalities
before chemotherapy.

Treatment
 If resectable, surgical excision with or without chemotherapy and radiotherapy
 If unresectable, neoadjuvant chemo/XRT then surgical excision

TERATOMAS
Clinical Presentation
 tumors can arise in several different places within the body: testes, ovaries, abdomen
and pelvis, mediastinum (part of chest between breastplate and the spinal column),
brain
 Testicular germ cell tumors
o painless mass in the scrotum (In small boys, tumors here may be noticed by the
parents)
 Ovarian and abdominal tumors
o abdominal pain or constipation; caused by torsion of the ovary or irritation of its
ligaments
 Mature cystic teratomas of the ovary
o often discovered as incidental findings on physical examination, during
radiographic studies, or during abdominal surgery performed for other indications
• Embryonic teratomas
o occur on the body midline: in the brain, elsewhere inside the skull, in the nose, in
the tongue, under the tongue, neck (cervical teratoma), mediastinum,
retroperitoneum, attached to the coccyx

Epidemiology
 Germ cell tumors
o 2.4 children out of 1 million develop a germ cell tumor each year
o 4% of all cancers in children and adolescents <20 y/o
o most commonly appear in the gonads (sex organs)
 Sacrocococygeal teratoma
•Presents in infants in utero or at birth
•0.25-0.28:10,000 live births
•M:F 1:4 ratio
•<10% younger than 2 months and >50% older than 4 months
Causes

 Teratomas are made up of a variety of parenchymal cell types representative of more

than a single germ layer, usually all 3.
 Because they arise from totipotential cells, they can be of germ cell or embryonal
origin

Diagnosis
 Imaging Studies
o Chest radiography may be used at diagnosis to detect metastasis.
o CT scanning of the abdomen and pelvis is essential for the staging
o MRI of the abdomen and pelvis may be substituted for CT scanning. If so, it
should be used throughout therapy to maintain consistency in imaging studies.
o At diagnosis, chest CT scanning is necessary to evaluate the presence and extent
of metastatic disease that originates in the abdomen or pelvis
o Bone scanning is a nuclear medicine test that is used to detect metastatic disease
Lab Studies

 Alpha-fetoprotein (AFP) is present in tumors with the following histologic features:

o Fetal liver or endodermal sinus tumor elements
o Embryonic carcinoma
o Endodermal sinus tumor
o Teratoma
 Beta–human chorionic gonadotropin (b-HCG) is present in tumors with the following
histologic features:
o Embryonal carcinoma
o Choriocarcinoma
o Teratoma

Treatment
 Sacrococcygeal tumor
o posterior trans-sacral route
o coccyx must be resected en bloc with the tumor to minimize the risk of
recurrence.
o If the sacrum or rectum is invaded by the tumor, complete resection may not be
advisable at the initial operation.
o Treating these tumors with chemotherapy is reasonable, with resection after the
maximum response is obtained.
 Ovarian Tumor
o Open resection
o Ipsilateral oophorectomy or salpingo-oophorectomy should be performed
 o Uninvolved fallopian tubes should be preserved if possible. In cases of mature
teratoma, the contralateral ovary should be inspected. If it appears normal, it
should be left alone
o Hysterectomy is unnecessary
 Testicular tumor
o Local resection in prepubertal patients, removed with a small rim of normal
testicle
o In all malignant cases, radical inguinal orchiectomy should be performed with
high ligation of the spermatic cord