SYSTEMIC%20HEMANGIOMATOSIS%20WITH%20ATYPICAL%20LIVER%20HEMANGIOMAS%20AND%20DIAPHRAGM%20INVOLVEMENT - PowerPoint PPT Presentation

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SYSTEMIC%20HEMANGIOMATOSIS%20WITH%20ATYPICAL%20LIVER%20HEMANGIOMAS%20AND%20DIAPHRAGM%20INVOLVEMENT

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Title: SYSTEMIC%20HEMANGIOMATOSIS%20WITH%20ATYPICAL%20LIVER%20HEMANGIOMAS%20AND%20DIAPHRAGM%20INVOLVEMENT


1
SYSTEMIC 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
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Grodno 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.

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Grodno Regional Clinical Hospital, Grodno, Belarus
INTRODUCTION
  • CT scan without contrast enhancement shows
    typical haemangioma in the 4th liver segment.

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Grodno 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.

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Grodno 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.

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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.

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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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Grodno Regional Clinical Hospital, Grodno, Belarus
CASE REPORT diagnosis?
Based on imaging findings and history of resent
operation on abdomen first diagnostic choice was
  • Multiple liver abscesses

Second choice was
  • Cystic metastases

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Grodno 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.

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Grodno 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.

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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.
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Grodno 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.

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Grodno 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).

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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.
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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.
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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
  • ovarian carcinoma,
  • colorectal carcinoma,
  • melanoma,
  • cervical carcinoma,
  • leiomyosarcoma,
  • lung cancer.

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Grodno Regional Clinical Hospital, Grodno, Belarus
DIFFERENTIAL DIAGNOSIS cystic metastases
CT scan shows multiple metastases from colorectal
carcinoma. Some of the lesions demonstrates
central hypodensity.
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Grodno 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.
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Grodno 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.

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Grodno 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.
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