4. In this lecture we’ll discuss:-
1. Pediatric hepatic masses.
represent 5% of pediatric intra abdominal masses and the 3rd
most common
pediatric abdominal masses after Wilm’s tumor and neuroblastoma.
2. Gastrointestinal tract duplication cysts
(GTDC's).
3. Lymphangioma.
4. GIT lymphoma.
5. Imaging Investigations
1. PXR:
provides clue for location of the mass
and calcifications.
1. US:
–The most valuable procedure for the
initial evaluation.
–Differentiates cystic from solid
masses,
–Indicates the organ of origin, and
suggests the diagnosis.
1. MSCT
2. MRI
6.
7. Pediatric Liver Masses
Benign
1. Infantile
Hemangioendothelioma.
2. Mesenchymal Hamartoma
of the Liver (MHL)
3. Focal Nodular Hyperplasia
(FNH)
4. Hepatic adenoma
Malignant
1. Hepatoblastoma.
2. HCC.
3. Undifferentiated Embryonal
Sarcoma (UES).
4. Embryonal
Rhabdomyosarcoma.
5. Epithelioid
Hemangioendothelioma (EHE).
As in adults, the most common neoplasm involving the liver in children is
metastatic disease –Not Primary Tumor-, usually from neuroblastoma, Wilms
tumor, or lymphoma.
8. Hepatoblastoma
• Hepatoblastoma occurs almost exclusively in
patients younger than 5 years on background
of healthy liver.
• Like HCC, hepatoblastoma can invade the
portal or hepatic veins.
• Serum AFP levels serve as a marker not only to
diagnose, but also monitor therapy and detect
recurrence.
• Lung is a common site for metastasis.
10. Hepatoblastoma
• US: hyperechoic with
hypoechoic septae.
• CT: hypodense to liver in
pre contrast and all phases
of postcontrast study.
11. Hepatoblastoma
• MR:
– T1: hypointense
– T2: hyperintense
– T1& T2 hypointense
septae.
• DDX:
– IHE: with fine calcifications and
peripheral nodular enhancement.
12. Question for clinician, regarding
clinical practice of
hepatoblastoma
Are imaging features and raised s.AFP sufficient
for diagnosis of hepatoblastoma and no need
for biopsy as in case of HCC?
13. HCC
• In children 10–14 years of age.
• The three main growth patterns of HCC are
solitary, multinodular, and diffuse infiltrative,
the last is the least common.
• Raised AFP in 80 % and normal in 20 % of
cases.
14. HCC
• Well or ill defined.
• Occurs on background of LC
• US: Variable.
• CT: hyo or iso on non contrast
study with early arterial
enhancement and rapid washout
in portal or venous phase.
• MRI:
• T1: variable
• T2: Hyperintense
15. Undifferentiated Embryonal sarcoma (UES)
• Well defined.
• No raising of AFP.
• US: Iso to Hyperechoic.
• CT: has cystic appearance (due to myxoid
content) with foci of soft tissue and internal
septations with peripheral enhancement.
• MRI: CSF parallel signal intensity
• T1: hypointense
• T2: Hyperintense
• DDx: MHL diagnosed mostly by age of 2 y while
UES after 5y
16. Embryonal Rhabdomyosarcoma
• Arising from CBD, so it occurs at
porta hepatis.
• US: Hypoechoic.
• CT: hypodense intraductal (inside CBD) soft tissue
mass with IHB channel dilatation.
• MRI:
• T1: hypointense
• T2: Hyperintense
18. Epithelioid Hemangioendothelioma
(EHE)
• Hepatic EHE is a tumor of intermediate malignant
potential, between that of benign IHE and the
highly aggressive angiosarcoma.
• Two patterns are demonstrated at imaging.
1. The multifocal form consists of multiple, predominantly
peripheral nodules (early stage)
2. As nodules enlarge, they tend to coalesce into confluent
masses, producing the diffuse pattern (advanced stage).
20. Infantile hemangioendothelioma =
Hemangioma
• The most common infantile benign hepatic masses.
• Presented in first 6 month of life.
• 50% solitary mass & 50 % multifocal.
• Its diagnosis depends on typical imaging features in
typical age and the biopsy is contraindicated due to risk
of hemorrhage.
21. Infantile hemangioendothelioma =
Hemangioma
• Infantile hemangioendotheliomas
may be focal, multifocal, or diffuse.
• Well defined.
• 50% ca.
• Hypo echoic/dense.
• Small multifocal tumors enhance
intensely and uniformly.
23. Infantile hemangioendothelioma =
Hemangioma
• The enhancement pattern of large
infantile hemangioendothelioma is
similar to that of adult
hemangioma i.e. peripheral
nodular enhancement with
centripetal pattern of filling and
vascular pool sign.
• Incomplete fill-in is due to central
necrosis and hemorrhage.
• diffuse disease demonstrate near
total replacement of the liver by
innumerable lesions with
centripetal enhancement.
24. Infantile hemangioendothelioma =
Hemangioma
• DDX:
1. Metastasis.
2. Angiosarcoma
• Most infantile hemangioendotheliomas are
asymptomatic and spontaneously involute, so lack of
response to treatment should prompt further
evaluation with biopsy.
25. Infantile hemangioendothelioma =
Hemangioma
Complications:
1. High-output congestive heart failure (CHF) due to
associated large arteriovenous shunts.
2. Kasabach-Merritt syndrome of coagulopathy due
to intertumoral platelet sequestration.
3. Hypothyroidism due to iodothyronine deiodinase
produced by the tumor
4. hemoperitoneum due to tumor rupture
27. Mesenchymal Hamartoma of liver
(MHL)
• Mesenchymal hamartoma of the
liver is the second most common
benign liver mass in children after
infantile hemangioendothelioma.
• MHL is discovered under age of 2
years.
• It’s predominately cystic with
multiple internal thin or thick
septations or soft tissue
component, but occasionally may
be solid.
• DDx:
– UES: occurs at older age (6-10y)
28. Mesenchymal Hamartoma of liver
(MHL)
• After administration of intravenous contrast material,
enhancement of the septa and solid elements occur.
• Solid portions may appear hypointense to adjacent liver on both
T1- and T2-weighted images owing to fibrosis.
29. Focal Nodular Hyperplasia (FNH)
• Uncommon, but can be
seen in 2-5 years with
marked female
predominance.
• US:
– homogeneous, well-defined mass
that may be iso-, hypo-, or
hyperechoic.
– The central scar appears
hyperechoic relative to the
remainder of the mass.
– Pulse wave doppler: arterial flow
to differentiate it from hepatic
adenoma that shows venous flow.
– Color doppler: Spoke wheel
pattern.
30. Focal Nodular Hyperplasia (FNH)
• Oral contraceptive use and
pregnancy, once considered risk
factors for the development of
FNH, are no longer considered
etiologic factors.
• CT:
– Occurs on background of normal liver
(i.e. not cirrhotic)
– well circumscribed and isodense in
precontrast.
• DDx:
– Fibrolamellar carcinoma a variant of
HCC that occurs in normal liver and
shows central scar that is T1 & T2 low
signal and shows no enhancement
event in late phases.
31. Focal Nodular Hyperplasia (FNH)
• MRI.
– T1: iso
– T2: iso
– Arterial phase:
homogenous
enhancement more
than the liver.
– Portal phase:
become iso to liver.
– Central scar is T1
hypo, T2 hyper
with late
enhancement
T1 T2
Arterial Portal
32. Hepatic adenoma
• Rare pediatric liver tumor occurs mainly in girls over 10 years old, most of
whom have a history of oral contraceptive use or Androgenic steroid therapy
used for ttt of Fanconi anemia.
• US.
– Hypo or hyperechoic.
– Pulse wave doppler shows venous flow.
• CT.
– Hypo in precontrast phase.
• MRI.
– T1: Hyper due to fat or hemorrhage.
– T2: Hyper
– Arterial phase: homogenous enhancement more than the liver.
– Portal phase: become iso to liver.
• DDx:
– Actually FNH, FLC and hepatic adenoma are differential for each other.
33. Gastrointestinal Tract Duplication
Cysts (GTDC's)
• The most common site of
gastrointestinal tract
duplication cysts (GTDC's) are
the ileum, followed by
esophagus, large bowel and
jejunum.
• Endo/exo luminal.
• C/O:
• IO.
• Abdominal pain.
• Palpable mass.
• US: Gut signature i.e. echogenic inner
mucosal layer and hypoechoic outer
muscular layer.
34. Gastrointestinal Tract Duplication
Cysts (GTDC's)
• CT: show a fluid-attenuation
cystic mass.
• MRI:
• T1: low signal
• T2: high signal
• T1 C+ (Gd): the cyst wall can
show slight enhancement
• DDx:
1. Pancreatic pseudocyst
2. Mesenteric cyst.
35. Lymphangioma
• Lymphangiomas are benign lesions
of vascular origin that may be
cystic, cavernous or capillary.
• 95% occur in the neck and axillary
regions; the remaining 5% are
located in the abdomen and chest.
• Has insinuating nature that make
its complete surgical excision
difficult.
• US: anaechoic with septataion, +
Ca.
• CT: hypodense.
• MRI: T1 low signal, T2 high signal,
may shows debris, septataions
37. GIT lymphoma
• Site: Stomach > small intestine (ileum > jejunum > duodenum)>
colorectal, however the small intestine (especially terminal
ileum) in children is the most commonly affected site.
• Pathological type:
– B-cell lymphoma NHL.
• Modality of choice.
Comparing MR and CT, they show a similar diagnostic capability but due to
high costs, long time required for examination, and possible artifacts, MR is
used just when the patient cannot be submitted to CT.
38. GIT lymphoma
• Imaging features:
– Focal /diffuse thickening.
– Polypoidal mass.
– Stenosing/stricture thickening.
– Exophytic mass.
• Intussusceptions is a complication
of GIT lymphoma.
39.
40. A periportal infiltrating mass, which is hypodense on CT and hypoechoic
on ultrasound, with or without distal biliary duct dilatation
41. • As in adults, the most common neoplasm
involving the liver in children is metastatic
disease, usually from neuroblastoma, Wilms
tumor, or lymphoma.
• Two third of primary liver tumors are benign and
one third is malignant.
• S.AFP is only raised in hepatoblastoma and HCC.
• Intestinal wall thickening in children diagnose
lymphoma till proved otherwise.
Take Home Message