Approach to solid

abdominal masses in
children

Samrawit Degu GSR III

Moderator : Dr Belachew Degene
Objectives

1. Review an organized approach to a child presenting with an

abdominal mass
2. Formulate a differential diagnosis for the common causes of
abdominal masses in children
3. To Pick those who need further work up, early treatment or referral
4. To have a brief idea about the surgical management of the
commonest childhood solid tumors
Outline

• Introduction
• Evaluation of abdominal mass in pediatric age group
• Brief presentation on management on of common abdominal mass
• Neuroblastoma
• Wilms tumor
• References
Introduction

• Abdominal masses are most common in children < 5 years

• Most abdominal masses in neonates are retroperitoneal,
of renal origin
• The older the child the more likely the mass represents a malignant
process
• Has extensive differential and can be categorized based on Anatomy/
location, Etiology and Age
 Etiology

Congenital Aquired

Cystic Solid Inflamatory Traumatic

Ovarian cyst Neuroblastoma Pseudocyst Hematoma

Mesentric cyst Teratoma Hydatid Pseudocyst
Omental Renal tumors Absess Abscess
Duplication Tubercular bscess
Choledocal Appendicular
Lymphangeoma abscess/ mass
Hydronephrosis
RU
Q:
He Duplication cysts, pyloric
e p h ro sis ,
He man stenosis, pancreatic Q : H y dron s ea se.
LU ney d i
HC p
C , ato giom pseudocyst, mesenteric and sti c k id
HEO
polycy , N B , P
, w B, P blas as,
N omental cysts
ilm s ’ t u m or s s u ch as
illm HE tom W f t um o r
s O
Gastric bezores. Lymphoma p e s o a,
a teratoma rare ty ll ca rci n o m
renal ce

Impacted stool, Full

bladder, Ovarian
cysts ovarian /
testicular GCT, SCT,
MMC, teratoma,
Sarcoms
Neonatal Abdominal Masses
 FLANK  Intraperitoneal
 Renal 55% Gastrointestinal 15%
 Hydronephrosis 35%  Duplication
 Cystic disease 10%  Mesenteric omental cyst
 Multicystic dysplastic  Pseudocyst from complicated
 Polycystic dysplastic obstruction
 Solid Tumors 10%  Meconium ileus
 Mesonephric nephroma Hepatobiliary 5%
 nephroblastomatosis  Hepatic tumors
 non-Renal Retroperitoneal 10%  Hemangioendothelioma
 Adrenal  Hepatoblastoma
 Hemorrhage  Neuroblastoma
 Neuroblastoma  Choledochal cyst
 GB cyst
Pelvic / Genitalia 15%
Teratoma
Ovarian Cysts
Hydrometrocolpos
Obstructed bladder
Abdominal Masses in Older Children
 Flank  Intraperitoneal
Renal 55% Gastrointestinal 12%
 Wilms (& other) 25%  Appendiceal Abscess
 Hydronephrosis 20%  Lymphoma
 Cystic disease 5%  Congenital
abnormalities
Non Renal Hepatobiliary
Retroperitoneal 23%
Hepatoblastoma
 Neuroblastoma 21%  HCC
 Teratoma 1%
 Other
 PELVIC : Ovarian Cysts and
1%
Teratoma,Hydrometrolops
Approach continued
• HISTORY: Age Where , Duration,
 Characterize Progress,
Associated symptoms Pain, weight loss, Anemia, Fever
Urinary symptoms, obstruction
History of trauma symptoms, distension, edema,
Metastatic symptoms like bone
Birth history, Antenatal ultrasoundspain, jaundice, bleeding
Family hx, Genetic sxx, other malignancies
Polyhydroaminos, difficult birth,
prematurity
Physical examination
Emaciated, Malnutrited, in CPD
• GA: Pale,
LAP icteric, skull
distress
: >2.5cm/ mass,
matted, Horner
fixed, har,sxx,
non
Exophthalmos,
tender / Associatedraccoons eye,
with constitutional
• HEENT:
symptoms, >10 weeks, >4wks with
• LGS: antibiotic, enalging
Symmetry, Bulge, visible veins
• Chest : Origin, intra or retroperitoneal; Size,
Consistency, Mobility, Mildline or
• CVS not, Relation with adjacent structure
Ambigious genitalia, hyposphadia,
• Abdomen : undecended testis
Bone tenderness, swelling,
• GUS: Hemihyperthrophy,
Pallor, Jaundice, Petechial, Blue Berry
• MSS:
Paraplegia,muffins
Monoparesis, Ataxia,
• CNS tremors
Initial workup

• CBC/D & peripheral blood smear which may show abnormalities in

the case of malignancy.
• LFT: are important if the mass is thought to have arisen from a
hepatobilliary cause.
• RFT, U/A, Serum ELE : If a renal mass is suspected, dehydrated or is
vomiting, or if there are urinary signs such as hematuria
Specific
• Urine VMA & HMA
• AFP : Hepatoma, HCC / GCT
• B- HCG: Ovarian/testicular germ cells or the liver.
• LDH, Ferittin
• LN Biopsy
• Bone marrow Biopsy
Imaging
• Plain abdominal x- ray :
Site of the mass, calcifications, bowel gas, airfluid levels
• Abdominal US
Should be the initial modality
Origin of the mass, Solid or Cystic
Vascularity, LAP, Peritoneal fluid
Relation with vessels and other organs
Ct scan and MRI
• Better characterization of the mass
• Anatomy and vascularity, LAP, Metastasis
• Presence or absence of other abnormalities
• Best for diagnosis and staging
• MRI : more detailed information for vascular and soft tissues, brain
and Spinal cord, bile duct and pancreatic anatomy
• Even Best if ordered in tertiary center
Most solid abdominal masses are Malignant, thorough workup
 Early referral is the key

Most present late.

Timely surgery is safe and curative.
Even for most advanced malignancies
Surgery has curative intent

The most common solid tumors are

Neuroblastomas
Wilms tumors
Neuroblastoma
 Introduction
Common sites
• Neuroblastoma is a highly aggressive tumor that arise from primitive
neural crest cells
• Heterogeneous disease with a broad range of clinical presentations.
• About 37% of patients are diagnosed as infants
• Stage IV diseases is seen in 40 % of infants unlike in
50-60% of older children
• Prognosis
Its mainly depend
sporadic, on the age, the histologic and biologic
1-2 % familial
characteristics
Multifocal or bilateral of the tumor
disease is seen in 20% of familial NB
Earlier age of onset in these patients
Has been observed in infants BWS, HSD
 Clinical features
• Primary tumor site • Metastatic / PNP
Abdominal mass +/- pain Racoon’s Eye
Constitutional symptoms SC Compresion
Hypertension Blue Berry muffin Sx
Respiratory distress+/ dysphagia Cytopenia
Horner’s SXX Jaundice
Paraplegia • Intractable diarrhea
Bowel, Bladder dysfunction • Dancing eye Sxx
Diagnosis
Baseline:

• CBC, OFT, ELE

• Increased levels of urinary metabolites of catecholamines ,
 VMA & HMA
Seen in 75% of patients
• LDH
• Ferritin
• Diagnostic criteria - by an international group of conference
one of the two

1. An unequivocal pathologic diagnosis made from tumor

tissue with or without immunohistochemistry or increased
catecholamine

2. Metastasis to bone marrow on an aspirate or biopsy with

elevation of catecholamine
Management
Staging
INSS/ INRGSS
Options

• Surgery
• Chemotherapy
• Radiotherapy
• Myeloablative chemo + stem cell transplant ( ASCT )
• Imunotherapy / Retinoid therapy
 Amplification of N-myc gene
loss of chromosome 1p, 11q
DNA diploid
Age
INSS/INRGS stage: stage3, 4
• For other low-risk tumors, surgery is the mainstay of treatment.
• Chemotherapy is added for tumors that cannot be resected or for SC
compression or respiratory compromise.
• Patients at intermediate risk usually receive chemotherapy in
combination with surgical resection.
• High risk, an aggressive multimodality approach: neoadjuvant
chemotherapy, surgical resection, adjuvant high-dose
chemotherapy with ASCT
Outcomes for patients at low risk (event-free survival, >95%) and
intermediate risk (event-free survival, 80%–95%) are excellent
Surgery

Goals:
• Establish diagnosis
• Accurate staging,
• Complete resection as safely as possible-initial or after induction
chemotherapy.
Incision

• Transverse cervical
• Trap door
• Posterolateral thoracotomy
• Upper abdominal transverse transperitoneal
Surgical principles

• Accurate and safe display of vital vascular anatomy

• Optimal exposure of the tumor to achieve gross resection.
• Tumor encasing major NVS that are not easily dissectible should be
left in place and biopsied
• Aggressive resection should never be tried
• Routine LN samples should be taken from 6-9 LNs
 Continued…

• Dissection should start at the lower limit of the tumor

• plane should be created between media and adventitia of the vessel,
and carried proximally 1-2cm steps
• Iliac, aorta, IVC, and RVs should be exposed and controlled early to
prevent contralateral RVs damage
• Protect lumbosacral plexus during pelvic dissection
Recurrence

• Over 50% of high‐risk patients continue to relapse.

• Have poor outcomes with 5‐year OS < 10%
• Locoregional recurrence, in primary low and intermediate risk,
additional surgery, radiation, and chemotherapy
• Recurrence in high risk, treat with chemotherapy with
immunotherapy +/- Surgery
Complications

• Hemorrhage
• Neurovascular damage
• Infection

Prognosis
• Intestinal obstruction
Wilms tumor
Introduction
• Named after the German Surgeon Dr Max Wilms
• It is a highly malignant Embryonal neoplasm arising from kidney
• It’s the most common renal malignancy
• Affecting children 2-5ys Median 36ms
• Incidence is 1 in 10000/yr
affecting 450-500 children annually
BWS
Clinical features

• Constitutional symptoms
• Painless abdominal mass
• Haematuria
• Hpertenstion
• Lt Varicocele sometimes Rt
• Abdominal pain if….
• +/- Associated abnormalities
Diagnosis
baseline

• CBC : Anemia, Thrombocytopenia

• OFT, Chemistry
• Coagulation
• 24 Hr Urine VMA, HMA
• LDH, Ferritin
CT scan
• Differentiating between WT and Neuroblastoma
• Relation with adjacent structures.
• To r/o rupture, abdominal dissemination and pulmonary metastasis.
• Assessment of cystic areas.
• Evaluation of bilateral disease (5%) or coexisting urinary
malformations (e.g., single or horseshoe kidney).
• Evaluation of intravascular extension (10–15%).
• Evaluation of response to chemotherapy.
Biopsy

• The typical age of diagnosis for WT is more than 6 months and less
than 7 years.
• Tissue diagnosis (nephrectomy, if feasible; or biopsy) is required to
plan therapy for atypical patients
• Atypical : Age < 6ms, or > 10ys
: Atypical imaging features
Staging
1.Limited to kidney and completely excised (not ruptured/biopsied,
The
no renal sinus Children’s
vessel Oncology Group (COG) :
involvement)
recommends surgery before chemotherapy
COG = no capsule involvement, SIOP = capsule infiltration
except: Inoperable WT; Solitary kidney;
2.Beyond capsule but
Bilateral completely
WT; excised,
tumor thrombus renal
in IVC; sinus vessel
tumor
COG = noinvolving
ruptureadjacent
or biopsy,and SIOP = biopsied
extensive pulmonary
metastases.
3.Residual SIOP
tumor confined
suggests to abdomen
preoperative (LN in hillus,
chemotherapy for allperiaortic,
 Decreases
spilage beyond flank,risk of ruptureimplants,
peritoneal or hemorrhage
unresectable tumor,
transectedIncreases resectablitytumor
tumor thrombus, and downstaging
rupture or biopsied)
4.Hematogenous mets or LN outside abdomen
5.Bilateral renal tumors
Management

• Requires a multidisciplinary approach

• Surgical resection and biopsy is the mainstay of treatment
• In combination with chemotherapy and radiotherapy
• The specific treatment plan varies by stage, patient age, tumor
weight, and histology.
Stage I-II FH, Stage I F/D Anaplasia= Surgery + CT
Stage II – IV F Anaplasia + Stage IV FH = Surgery + Rada + Triple drug CT
Stage II – IV D-anaplasia = Surgery + TA Radn + 4 drug CT
• Preoperative considerations
 Preoperative multidisciplinary planning should include
Assessment of comorbidities,
 Cross matched blood preparation
Capacity of the anesthesia team, intraoperative monitoring
Postoperative level of care and monitoring, postop pain control.
If a neoadjuvant chemotherapy protocol is used, surgery should
follow blood count recovery
Surgery
• Goals of surgery
 Transabdominal Nephrectomy with
Thorough surgical staging
 Achieve R0 resection, and/or take biopsy
 Prevent tumor spillage, and
 Mitigate complications and resection of other organs.
 Incision

• Adequate exposure is the key

• Supine position flank raised
• Transverse, supraumblical,
transperitonial( flank to flank) incision
• Thoracoabdominal incision might be
needed
Surgical Principles
• Take out small bowel, Opening fascia of tolt and reflect the colon
• Complete exploration and palpation
• Opening of Gerota fascia and careful exploration of the whole contra
lateral kidney is recommended, based on preoperative imaging.
• Renal vessels and IVC should be exposed and palpated
• All parts of kidney mobilized and examined
• Renal Vessels ligated, ?Artery > Vein
Continued…

• The adrenal gland may be left in place unless abutting the tumor
• The ureter should be ligated and divided as low as possible.
• The tumor and the kidney are mobilized and removed intact.
• Radical en bloc resection of adjacent organs not needed
• If incomplete resection, a biopsy should be done, and the site
identified with metallic clips.
Continued…

• Routine LN sample from the hilar, paracaval,

para-aortic at least 7 LNS
• Formal lymph node dissection is not recommended.
• The peritoneum is considered "soiled" if biopsy
was taken, tumor spill or ruptured.
• Tumor bed should be evaluated and suspicious
area sampled
Challenges

• IVC & RV tumor thrombus extension 10-15%

Can be floating or adhering
Cavectomy or Cavotomy should be done
Suction or fogarthy cathether removal
Thoracotomy with CP bypass maybe needed
Preoperative chemotherapy increases operative
success
Challenges….

• Bilateral Wilms tumor

Seen in 5-10 %
ESRD is common in these patients
Goal of surgery is to achieve a cure by removing all tumoral renal
tissue while preserving the maximum functioning kidney.
Preoperative chemo for 6-12wks then Bilateral NSS
If no response Radiotherapy
Continued…

• WT in Horseshoe kidney
 Willms tumor is 1.96* common in HS kidney
Collecting system, vascular and ureteric anatomy poses difficulty
 Resection : if only one side is involved by resectable tumor
 In bilateral cases, accurate surgical staging should be performed
 Stage-appropriate adjuvant therapy should be given,
Continued…

Prognosis
• Recurrent disease Histology
 10–15% of WT, usually theStage2ys
1 st

Age lack of LN sample increases risk of RR

 UFH, Stage III, tumor spillage,
Tumor weight
 The leading locations areResponse
the lung,toabdomen/flank
therapy and liver.
 Surgery has limited role Loss of heterozygosity at
 prognosis depends on many1p and 16q
factors
IPSO Practice Guidelines on Wilms Tumor

References
Handbook_Neuroblastoma_
Spring2018.pdf

Current treatment for Wilms tumor: COG and SIOP standards

1.Jinhu Wang1,
2.http://orcid.org/0000-0002-3672-4479Minju Li1,
3.Daxing Tang1,
4.Weizhong Gu2,
5.Junqing Mao1 and
6.Qiang Shu1
7.Correspondence to Dr Minju Li; wjpch2@zju.edu.cn