Title: SYSTEMIC%20HEMANGIOMATOSIS%20WITH%20ATYPICAL%20LIVER%20HEMANGIOMAS%20AND%20DIAPHRAGM%20INVOLVEMENT
1SYSTEMIC HEMANGIOMATOSIS WITH ATYPICAL LIVER
HEMANGIOMAS AND DIAPHRAGM INVOLVEMENT
The 60th Jubilee Congress of The Association of
Polish Surgeons, Warsaw 12-15.IX.2001
Khoruzhik S. A.1, Maslakova N. D.2, Siljaeva N.
F.3From The 1Departments of Radiology,
2Abdominal Surgery, and 3Pathology, Grodno
Regional Clinical Hospital, Grodno, Belarus
- Serguey A. Khoruzhik, MDComputed Tomography,
- Grodno Regional Clinical Hospital,BLK 52, 230017
Grodno, BelarusTel. (375 152)
331320khoruzhik_at_grsmi.unibel.byhttp//nld.hut.ru
/e/cv.htm
Contact
2Grodno Regional Clinical Hospital, Grodno, Belarus
INTRODUCTION
- Haemangioma is the most common benign hepatic
tumor, being present in about 5 of livers
autopsy. - In systemic haemangiomatosis multiple organs
involved including liver, spleen, muscles, bowel,
lungs, brain. - 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.
3Grodno Regional Clinical Hospital, Grodno, Belarus
INTRODUCTION
- CT scan without contrast enhancement shows
typical haemangioma in the 4th liver segment.
4Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT history
- 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.
5Grodno Regional Clinical Hospital, Grodno, Belarus
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.
6Grodno 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.
7Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT imaging findings
- This corresponded to hyperechoic ring-shaped
structures with anechoic centre on US exam.
8Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT diagnosis?
Based on imaging findings and history of resent
operation on abdomen first diagnostic choice was
Second choice was
9Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT diagnosis?
- Hemorrhagic fluid was aspirated from the liver
lesion under CT guidance what raised suspicion of
haemangiomas. - 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. - Spleen microphotographs were revised and
proliferation of vascular channels characteristic
for haemangiomas was found.
10Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT outcome
- During next few weeks patients condition
deteriorated rapidly with clinic of sepsis and
disseminated intravascular coagulation. In one
month after admission to the hospital patient
died. - 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.
11Grodno 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.
12Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS OF LIVER CYSTIC MASSES
- Abscesses.
- Cystic metastases.
- Atypical liver haemangiomas.
- Hepatocellular carcinoma with cystic change.
- Hepatic tumor after treatment (embolisation,
radio-frequency ablation). - Polycystic liver disease.
13Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS atypical liver
haemangioma
- 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).
14Grodno 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.
15Grodno 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.
16Grodno 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
- ovarian carcinoma,
- colorectal carcinoma,
- melanoma,
- cervical carcinoma,
- leiomyosarcoma,
- lung cancer.
17Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS cystic metastases
CT scan shows multiple metastases from colorectal
carcinoma. Some of the lesions demonstrates
central hypodensity.
18Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS polycystic liver disease
CT scan shows multiple liver cysts. Some of the
cysts have calcified walls as consequence of
chronic inflammation.
19Grodno Regional Clinical Hospital, Grodno, Belarus
DISTINCTIVE FIATURES OF THE PRESENTED CASE
- Atypical liver haemangiomas simulating liver
abscesses or cystic metastases both
radiologically and clinically. - 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.
20Grodno Regional Clinical Hospital, Grodno, Belarus
CONCLUSION
Atypical hepatic cavernous haemangiomas may
present on imaging as multiple ring-like
non-specific 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.