حسین سلطان زاده
استاد دانشگاه علوم پزشکی شهید بهشتی
متخصص کودکان ونوزادان
طی دوره بالینی عفونی از میوکلینیک آمریکا
دبیر برگزاری کنفرانس های ماهیانه گروه اطفال
دانشگاه علوم پزشکی شهید بهشتی
معرفی : دکترفریده
به اتفاق اعضای هیئت علمی گروه کودکان
Liver & spleen scan by
with high blood flow
a hugely enlarged liver
occupied by a loculated
no uptake of
Hepatomegaly with a huge space-occupying lesion
Tc 99m-RBC scan:
Liver is occupied by a very
huge mass which
revealed gradual accumulation
of RBC-labeled radiotracer.
Impression: Scan is in favor
very huge and loculated
probable AV malformation
inside the lesion.
Hemangiomas are benign tumors
of the endothelial cells which normally line the blood vessels.
Approximately 60% hemangiomas
-- head and neck
about 25% -- trunk, 15% arms
Most (about 80%) hemangiomas
grow as a single tumor, about 20% multiple areas.
is most common benign tumor of the liver (0.4-7.3% incidence at
range from 2 mm to more than 20 cm.
measure less than 5 cm; larger than 4-5 cm are called giant hemangiomas
(Cappellani, 2000; Yang, 2001).
Natural history of liver
not completely understood.
Probably congenital in origin.
Several pharmacologic agents
have been postulated to promote tumor growth.
Steroid therapy, estrogen
therapy, and pregnancy increase the size
of already existing hemangioma
be multiple in as many as 50% of patients (Mergo, 1998).
are uncommon in cirrhotic livers; the fibrotic process in cirrhotic liver
prohibit their development . (Dodd, 1999).
female-to-male ratio of 5:1 to 6:1.
: can occur in
individuals of any age.
in middle-aged women.
: Hepatic hemangiomas are
rare in infancy. (report of 2 infants, Kullendorff 2002,)
: Have been detected
prenatally in a growing fetus. (Gembruch, 2002. Pott Bartsch, 2003)
an arterial bruit over the
right upper quadrant.
Lab Studies :
and reduced hematocrit levels in patients with ruptured hemangiomas.
-- from sequestration and destruction of platelets in large lesions.
has been attributed to intratumoral fibrinolysis.
patients with giant hemangiomas associated with Kasabach-Merritt
syndrome, bleeding and clotting parameters may be abnormal.
alpha-fetoprotein and carcinogenic embryonic antigen (CEA) levels.
on the size / location of tumor.
on the stomach and duodenum may cause vague abdominal pain, early
satiety, nausea, and vomiting. (Tran-Minh,
hemangiomas may twist and cause acute abdominal
of the inferior vena cava -- Budd-Chiari
syndrome. (Hanazaki, 2001)
Acute thrombosis --- acute
inflammatory changes --- consumption coagulopathy, fever,
abdominal pain, abnormal liver function.
In one review, 32 case
reports of spontaneous rupture of hepatic hemangioma in patients
greater than 14 yo without trauma.
post-traumatic rupture is a catastrophic complication that occurs in about
1-4% of hemangiomas; with mortality rate, as high as 60%. (Cappellani,
Clinical Syndromes :
Hepatic hemangiomas plus
congenital hemiatrophy and nevus flammeus, with / without hemimeganencephaly.
Giant hepatic hemangiomas,
thrombocytopenia & intravascular coagulation.
Numerous small hemangiomas
of face, nares, lips, tongue, oral mucosa, GIT, and liver.
Von Hippel-Lindau disease:
Cerebellar & retinal
angiomas, with lesions in the liver and pancreas.
hemangiomas reported in SLE.
Differential Diagnoses :
Other hypervascular benign
and malignant space-occupying liver lesions.
cysts, adenomas, focal
nodular hyperplasia, and regenerating nodules.
and metastasis hemangioendothelioma.
Ultrasonography is a
cost-effective imaging modality for diagnosis of a hemangioma.
CT and/or MRI may be
required to specifically diagnose hemangioma
Most commonly initial
masses (though few can appear relatively hypoechoic
when imaged within a fatty liver)
Giant lesions can appear
heterogeneous secondary to internal complex composition
Gray-scale and Doppler
sonograms show a well-defined, uniformly hyperechoic liver mass
with peripheral feeder vessels. These features are characteristic of a
CT with sequential scans
Focal, well-circumscribed, low
on pre-contrast images
contrast enhanced imaging
magnetic resonance imaging (MRI),
and specificity >90%
hemangiomas from other liver lesions
hemangiomas are homogeneously hypointense relative to the liver on
T1-weighted and markedly hyperintense (lightbulb sign) on T2-weighted images
relative to the liver
dynamic, contrast-enhanced MR imaging, Peripheral, nodular centripetal
enhancement pattern progressing to homogeneity (lesions 1.5-5cm)
nodular centripetal enhancement with persistent central hypointense region
of the liver:
Axial T1-weighted pre-contrast
shows a hypointense mass within the right hepatic lobe.
Sequential enhanced delayed
peripheral nodular centripetal enhancement with persistent central hypointensity
RBC pool studies
used for many years.
For lesions that are greater
than 2 cm in diameter,
Sensitivity reported at 82%.
is up to 100%
(SPECT) with colloid
Single-photon emission computerized tomography (SPECT)
with colloid 99m-labeled RBCs appears to be as sensitive and specific as MRI.
present, SPECT scan is most likely the investigation of choice to confirm
the diagnosis of hepatic hemangioma.
as small as 0.5 cm may be detected with SPECT.
SPECT examination: Axial scans
of blood-pool scintigraphy with 99mTc-labeled erythrocytes: A well-circumscribed
area (arrow) of increased activity is present in the left lobe of the liver,
which indicates pathology with a high blood content.
Cavernous hemangioma are atypical or irregular in arrangement and size.
Mesenchymal in origin.
composed of cavernous
vascular channels lined by single layers of flattened endothelium
and separated by fibrous septa.
These vascular spaces may
contain thrombin, calcifications, or prominent scarring with
hyalinization (sclerosed hemangioma).
Malignant transformation has
not been reported.
embolization, (polyvinyl alcohol particles)
Surgical ligation of feeding
Percutaneous & laporoscopic
to improve abdominal pain.
With a dose of 30 Gy in 15
fractions for3 weeks
reported to produce complete
regression of hemangiomas.