Transcript SYSTEMIC HEMANGIOMATOSIS WITH ATYPICAL LIVER …

The 60th Jubilee Congress of The
Association of Polish Surgeons,
Warsaw 12-15.IX.2001
SYSTEMIC HEMANGIOMATOSIS WITH
ATYPICAL LIVER HEMANGIOMAS AND
DIAPHRAGM INVOLVEMENT
Khoruzhik S. A.1, Maslakova N. D.2, Siljaeva N. F.3
From The 1Departments of Radiology, 2Abdominal Surgery, and 3Pathology,
Grodno Regional Clinical Hospital, Grodno, Belarus
Contact:
Serguey A. Khoruzhik, MD
Computed Tomography,
Grodno Regional Clinical Hospital,
BLK 52, 230017 Grodno, Belarus
Tel.: + (375 152) 331320
[email protected]
http://nld.hut.ru/e/cv.htm
Grodno Regional Clinical Hospital, Grodno, Belarus
INTRODUCTION
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Haemangioma is the most common benign hepatic
tumor, being present in about 5 % of livers autopsy.
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In systemic haemangiomatosis multiple organs
involved including liver, spleen, muscles, bowel,
lungs, brain.
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On unenhanced CT haemangioma usually appears as
well-marginated solid mass of the same or decreased
relative to liver parenchyma density what corresponds
to hyperechoic lesion on US.
Grodno Regional Clinical Hospital, Grodno, Belarus
INTRODUCTION
CT scan without contrast enhancement shows typical
haemangioma in the 4th liver segment.
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CASE REPORT: history
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A 68-year-old female presented with abdominal pain,
nausea, weakness, intermittent temperature of 38° C
and chilling. She was underwent spleenectomy one
month earlier in other hospital because of spontaneous
spleen rupture followed by subfebrile temperature up
to the time of present admission.
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CASE REPORT: imaging findings
Post-contrast liver CT scan shows high contrast
enhancement in the capsules of multiple nodular lesions
measuring 2 to 5 cm in diameter. Central parts of the
lesions remain unenhanced.
Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT: imaging findings
Post-contrast liver CT scan. Free fluid in the both spleen
bed and left pleural cavity present. Left hemidiaphragm
appeared irregularly thickened.
Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT: imaging findings
This corresponded to hyperechoic ring-shaped structures
with anechoic centre on US exam.
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CASE REPORT: diagnosis?
Based on imaging findings and history of resent operation
on abdomen first diagnostic choice was:
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Multiple liver abscesses
Second choice was:
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Cystic metastases
Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT: diagnosis?
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Hemorrhagic fluid was aspirated from the liver lesion under
CT guidance what raised suspicion of haemangiomas.
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1,5 litres of hemorrhagic fluid was aspirated from the left
pleural cavity. Explorative thoracotomy revealed diffuse
bleeding from the left hemidiaphragm into pleural cavity.
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Spleen microphotographs were revised and proliferation of
vascular channels characteristic for haemangiomas was
found.
Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT: outcome
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During next few weeks patient’s condition
deteriorated rapidly with clinic of sepsis and
disseminated intravascular coagulation. In one month
after admission to the hospital patient died.
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On post-mortem study multiple cavernous
haemangiomas were found in liver, epiploon,
abdominal ligaments, and left diaphragmatic muscle.
All liver lesions contained large central areas of
intratumoral hemorrhage.
Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT: final diagnosis
Systemic haemangiomatosis with multiple liver
cavernous haemangiomas and left
diaphragmatic muscle haemangiomas with
clinical presentation of Kasabach-Merrit
syndrome.
Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS OF
LIVER CYSTIC MASSES
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Abscesses.
Cystic metastases.
Atypical liver haemangiomas.
Hepatocellular carcinoma with cystic change.
Hepatic tumor after treatment (embolisation, radiofrequency ablation).
Polycystic liver disease.
Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS:
atypical liver haemangioma
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On US: solid tumor with an echogenic rim and
hypoechoic internal echo pattern.
On CT: ring-like structure with hypodense
unenhancing centre.
The reasons for central hypoechoity/hypodensity are
internal hemorrhage with necrosis, thrombosis,
scaring, and myxomatous change.
In one study this kind of lesions were present in 15 of
5000 abdominal US exams (0.3 % prevalence of
atypical haemangiomas in the general population).
Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS: abscess
CT scans of the liver in two different patients. Abscess in
the right liver lobe (left) and atypical liver haemangiomas
(right) for comparison.
Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS: abscess
Liver CT scan shows hepatic abscesses in the right lobe with
blood products precipitated along the posterior wall. In such
instance blood might be aspirated from the abscess.
Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS: cystic metastases
Most often liver metastases are solid. Cystic metastases
are uncommon and may arise from mucin-producing
primaries or be due to necrosis within the tumor in:
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ovarian carcinoma,
colorectal carcinoma,
melanoma,
cervical carcinoma,
leiomyosarcoma,
lung cancer.
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DIFFERENTIAL DIAGNOSIS: cystic metastases
CT scan shows multiple metastases from colorectal
carcinoma. Some of the lesions demonstrates central
hypodensity.
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DIFFERENTIAL DIAGNOSIS:
polycystic liver disease
CT scan shows multiple liver cysts. Some of the cysts have
calcified walls as consequence of chronic inflammation.
Grodno Regional Clinical Hospital, Grodno, Belarus
DISTINCTIVE FIATURES OF THE
PRESENTED CASE
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Atypical liver haemangiomas simulating liver abscesses
or cystic metastases both radiologically and clinically.
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Presence of diaphragmatic haemangiomas.
Intramuscular haemangiomas are rare benign tumors,
making up 0.8% of all haemangiomas. There were just
two reports of diaphragmatic haemangiomas in English
medical literature.
Grodno Regional Clinical Hospital, Grodno, Belarus
CONCLUSION
Atypical hepatic cavernous haemangiomas may
present on imaging as multiple ring-like nonspecific lesions mimicking abscesses and
metastases. This diagnosis should be considered in
patients with cystic hepatic lesions without
primary malignancy. Possibility of diaphragm
involvement in systemic haemangiomatosis has to
be taken into account.