Assessment and Diagnosis of

Abdominal Masses in Children

Resident Education Lecture Series

General approach to solid tumors
 What is it?
 Where is it?
 Where can it go?

 The answer to any one of these questions will

help answer the other two
Work up – two components
Staging Evaluate for complications of
 X-ray of primary site the tumor
 CT chest, abdomen, &  CBC with diff
pelvis  TPN panel
 CXR (baseline)  LDH, uric acid – tumor
 bone scan lysis, rapid cell growth
 Specialty tests  Lytes, creatinine – renal

 Gallium, MIBG, PET function

 Transaminases – hepatic
 Bone marrow
involvement
 ESR
 Specialty tests
 Tumor markers
 HCG, AFP
 HVA/VMA ….
 Tissue diagnosis
 Incisional biopsy
 Excisional biopsy
 Special cases…
 Calicified suprarenal mass + bone scan – might

consider getting dx from bone marrow

 FNA vs excisional biopsy
 Bias towards excisional → sufficient sample to be

representative and to send for special research

studies (histology, chromosomes, special studies,
research studies)
 Abdominal Masses
Trends
 Abdominal masses are most common in
children under the age of 5 years
 Most abdominal masses in neonates are
retroperitoneal, of kidney origin and are not
malignant
 The older the child the more likely the mass
represents a malignant process
 Possible Diagnoses of Abdominal
Masses in Infancy and Childhood

Atlas of Pediatric Physical Diagnosis, Fourth Edition

Abdominal Masses in Older Children
 Renal 55%  Gastrointestinal 12%
 Wilms (& other) 25%  Appendiceal Abscess
 Hydronephrosis 20%  Lymphoma
 Cystic disease 5%  Hepatobiliary 6%
 Non Renal  Tumors
Retroperitoneal 23%  Hepatoblastoma

 Neuroblastoma 21%  HCC

 Teratoma 1%  Genital 4%
 Other 1%  Ovarian Cysts and
Teratoma

Kirk et al., 1981 Radiol. Clin.

North Am., 19:527-545
 Neonatal Abdominal Masses
 Renal 55%  Gastrointestinal 15%
 Hydronephrosis 35% Duplication
 Cystic disease 10%  Mesenteric omental cyst
 Multicystic dysplastic  Pseudocyst from
 Polycystic dysplastic complicated obstruction
 Solid Tumors 10%  Meconium ileus
 Mesonephric nephroma  Hepatobiliary 5%
 nephroblastomatosis  Hepatic tumors
 Pelvic / Genital 15%  Hemangioendothelioma
Teratoma  Cystic mesenchymal
 Ovarian Cysts hamartoma
 Hydrometrocolpos
 Hepatoblastoma
 Obstructed bladder
 Neuroblastoma
 non-Renal Retroperitoneal
 Choledochal cyst
10%
 Adrenal
 Hemorrhage
 neuroblastoma Kirk et al., 1981 Radiol. Clin.
North Am., 19:527-545
 Examination of the Pediatric Abdomen
 History – time the mass has been present, rapidity of
growth, symptoms
 Undress patient: evaluate for genetic or inherited
predisposition as well as the belly
 Palpate from the pelvis toward the thorax
 Describe location
 Size
 Consistency
 Ascites
 Venous congestion of surface

Golden and Feusner, 2002, Pediatr Clin N Am, 49:1369-1392

 Neuroblastoma
 Malignancy in neural crest cells in sympathetic
ganglia, adrenal medulla, chest, abdomen;
small round blue tumor cells
 Nonmalignant form is ganglioneuroma
 Clinical effects r/t tumor size and location
 Genetic links/factors involved: N-myc oncogene,
chromosome deletion
 NB Incidence/ Etiology
 4th peds cancer (7-10%)
500-550 new US per year
 Most common cancer in infants – accounts for 50%
of cancer in NBs. M:F ratio: 1.2:1
 Average age is 18 months; 80% < 5;
small #, genetic?
 May be a “Silent” tumor
presenting with widespread disease at dx 50
(younger) – 70 (older) % of time
 Clinical Presentation
 Pain, abd mass, other masses, malaise; skin
 Can occur anywhere in sympathetic NS
 >50% are retroperitoneal; head/neck, pelvis,
posterior mediastinum; +/- spinal cord
compression**
 Metastatic to lymph nodes, bone, BM, liver
 Fever and malaise;
catecholamine secretion: HTN, sweats, irritability;
diarrhea;
opsoclonus-myoclonus; cerebellar ataxia
 Diagnostic Workup
 Hx: catecholamine related sx
(htn, flushing, sweating, irritability);
wt loss, pain, limp
 PE: preorbital ecchymosis, cutaneous nodules; abd
mass; weakness/paralysis
 CT/MRI to locate tumor; bone scan;
MIBG; PET?
 Labs (urinary catecholamines);
 Bilateral BMA and bx; chromosome studies
 Neuroblastoma Staging
1 Localized tumor; complete excision
2A Unilateral, incomplete gross resection;
negative microscopic nodes
2B Unilateral, positive ipsilateral nodes;
negative contralateral
3 Across midline, or contralateral nodes
4 Dissemination: bone marrow, liver, skin, bones

4S <1y: local stage 1-2

with mets to BM, liver, skin
 Treatment and Prognosis
 Surgery: debulk or total removal;
curative in low-stage disease;
2nd-look after other Rx
 Chemotherapy – often platinum based
multi-agent ~ stage
 RT: to primary tumor site;
NB cells very radiosensitive;
before or after surgery;
emergency relief for cord compression, respiratory
compromise, proptosis
 NB Treatment cont’d
 BMT:
 children with poor prognosis initially may be treated
with high dose chemotherapy with autologous stem
cell rescue(s);
 BMT may be used with relapse

 Prognosis: <1 best (75+% survival);

worst for children >2 with stage IV disease
(10-20%);
Stage IV disease – survival trends
NEJM 341:1165-1173, 1999

p = 0.034
NEJM 341:1165-1173, 1999

p = 0.027
NEJM 341:1165-1173, 1999

p = 0.02
 Tumors of the Kidney
 Primary tumors arising from the kidney, usually
Wilms, rapidly growing vascular abdominal tumors;
fragile gelatin capsule
 Others: clear cell sarcoma, renal cell CA,
lymphoma, PNET, rhabdoid, …
 Wilms tumor pathology may be favorable or
unfavorable depending on degree of anaplasia
present;
prognosis and treatment r/t pathology
 Incidence and Etiology
 Renal tumors represent 5-6% of peds cancer; 460
new US cases/yr
 Higher in AA, lower in Asians
 Peak age at 2-3; rare in kids >5;
M:F 0.9:1.0 (unilateral) 0.6:1.0 (bilateral)
males younger age at diagnosis
 1.5% familial in origin; associated with aniridia,
hemihypertrophy, GU malforms
 Genetic factors, deletion or translocations
What is this syndrome?
 Omphalocele
 Macroglossia
 Gigantism
 Exophthalmos
 Hypoglycemia

 Beckwith-Wiedemann
Hemi-hypertrophy
 Clinical Presentation
 Asymptomatic abdominal mass found by
family or on routine PE
 Pain, malaise, hematuria in 20-30%; 25%
with HTN; rare subcapsular hemorrhage,
with rapid increase in size, anemia, HTN
 Mets to lungs, liver, regional nodes
 7% bilateral, at dx or later
 Diagnostic Workup
 H and P
 Labs, renal and hepatic function
 Imaging studies:
US to determine size and shape, vessel
involvement, thrombi in major vessels; chest
film/CT to check for mets
 Liver, brain, and bone mets not routinely assessed
unless indicated by S/S
 Prognosis
 Histology is most important prognostic
factor (favorable histology vs. anaplastic)
 Stage at diagnosis also crucial
 Genetic factors
 Age
Staging of Wilms Tumors
I Limited to kidney; complete resection
II Extent beyond kidney, but complete R
III Residual tumor, confined to abdomen
IV Hematogenous mets (lung, liver, bone, brain)
or lymph nodes outside abdomen
V Bilateral renal involvement at diagnosis

Tumor spill at time of surgery – considered stage III

Treatment and Prognosis
 Surgery initially, with exam of
contralateral kidney;
 Preop chemotherapy if intravascular spread
or very large invasive tumors; if bilateral;
 NA argument: Preop Chemo prevents
adequate assessment of staging
 Considered Stage III if imaged only
Treatment and Prognosis cont’d
 Bilateral: preop Chemo; nephrectomy of worse side,
partial on other
 Chemotherapy: regimens based in national groups
 RT: port extended across midline to prevent
scoliosis; if favorable histology, RT only for Stage
III and IV; post lung RT, adjust Chemo
 Recurrence: worse if <1 year; on chemo

 Prognosis: <50% - 100% (stage/histology)

 Malignant Hepatic Tumors
 Hepatoblastoma; median age of 1 yr;
 Hepatocellular carcinoma,
median age of 12 yrs,
associated with hepatitis B <15 yrs,
prolonged use of metabolic steroids

 Nonmalignant: hemangiomas (50% of all

hepatic tumors)
 Clinical Presentation
 Hepatoblastoma (80%):
asymptomatic abdominal mass; osteopenia;
 Hepatocellular Ca (20%):
abdominal distention, RUQ mass; pain, N &
V; jaundice; splenomegaly;
 Elevated alphafetoprotein level
 Treatment and Prognosis
 Preop CTX followed by complete resection

 Hepatoblastoma: High cure rates, with cure

possible if mets are resected (> 65%)
 Hepatocellular Ca: Difficult to resect and
difficult to cure even with complete resection
(<20%)
 RT of little benefit
Chemo-embolization?
Orthotopic liver transplant?
 Prognosis
 Hepatoblastoma
 Resectable tumors
 At diagnosis (stage I & II ) - 90%
 Following chemo-reduction (III) ~ 80%
 Unresectable tumors - 50%
 Metastases at diagnosis - 10%
 Prognosis
 Hepatocellular Carcinoma
 Children with initially resectable HCC have a
good prognosis and may benefit from
adjuvant chemotherapy.
 The outcome for children with unresectable
or metastatic HCC continues to be dismal
with current therapies.
 Intergroup Study for the Treatment
of Childhood Hepatocellular Carcinoma
Event-Free Survival by Stage

Stage I (N=8)
0.8

0.6
P robability

0.4

0.2
P<0.0001
Stage III (N=25)
Stage IV (N=13)

0
0 1 2 3 4 5 6 7
Time from Study Entry (years)
Differential diagnosis of Thoracic
Masses (malignant)
EXTRA-THORACIC INTRA-THORACIC
 Soft tissue mass  Anterior mediastinum (the 4 “T’s”)
 Soft tissue sarcoma  Teratoma (or germ cell tumor)
 PNET/Ewings  Thymoma
 Lymphoma  Thyroid carcinoma
(much less common)  T-cell leukemia or other lymphoma
 Bony Mass (adenopathy +/- effusion)
 Ewings  Posterior mediastinum
 Neuroblastoma  Neuroblastoma, Ewings, other
 Osteosarcoma soft tissue sarcoma
(much less common)  Pulmonary parenchyma
 Metastatic disease
 Lymphoma
 Primary pulmonary malignancy
(rare, usually embryonal type)
 Hilar
 Lymphoma
 Rare soft tissue sarcoma or
angiosarcoma
Differential diagnosis of extremity
and/or soft tissue masses (malignant)
 Bone
 Osteosarcoma
 Ewings
 Soft tissue
 Rhabdomyosarcoma
 PNET/Ewings
 Fibrosarcoma
 other……
From ABP
Certifying Exam Content Outline
 Formulate a differential diagnosis for an
abdominal mass
 Know that multicystic dysplastic kidneys and
hydronephrosis are the most common causes of
palpable abdominal masses in infants
 Recognize that children with hemihypertrophy and
somatic overgrowth syndromes should be
periodically evaluated for the development of
associated embryonal tumors
From ABP
Certifying Exam Content Outline, continued
 Understand that a neuroblastoma usually presents as a
nontender abdominal mass
 Understand that urinary catecholamine excretion is increased
in most patients with a neuroblastoma and that tests of urine
for VMA and VHA are appropriate screening tests for the
tumor
 Know that Wilms tumor is associated with hemihypertrophy
and aniridia, somatic overgrowth, and/or genitourinary
abnormalities
 Understand that Wilms tumor usually presents as an
abdominal mass and may cause hypertension
 Recognize the tumors that may produce precocious puberty
(eg, in liver, CNS, ovary, testes, adrenal glands)
Credits
 Michael Kelly MD PhD
Anne Warwick MD MPH