SYSTEMIC HEMANGIOMATOSIS WITH ATYPICAL LIVER HEMANGIOMAS AND DIAPHRAGM INVOLVEMENT Serguey A....

SSYSTEMIC HEMANGIOMATOSIS WITH YSTEMIC HEMANGIOMATOSIS WITH ATYPICAL LIVER HEMANGIOMAS AND ATYPICAL LIVER HEMANGIOMAS AND

DIAPHRAGM INVOLVEMENTDIAPHRAGM INVOLVEMENT

SergueySerguey A. A. Khoruzhik, MDKhoruzhik, MDComputed TomographyComputed Tomography, ,

Grodno Regional Clinical HospitalGrodno Regional Clinical Hospital,,BLK 52, BLK 52, 230023001717 Grodno, Belarus Grodno, BelarusTel.: + (375 152) 331320Tel.: + (375 152) [email protected]@grsmi.unibelunibel..bybyhttphttp://://nldnld.hut..hut.ruru/e//e/cvcv..htmhtm

The 60th Jubilee Congress of The Association of Polish Surgeons,

Warsaw 12-15.IX.2001

Khoruzhik S. A.Khoruzhik S. A.11, Maslakova N. D., Maslakova N. D.22, Siljaeva N. F., Siljaeva N. F.33

From The From The 11Departments of Radiology, Departments of Radiology, 22Abdominal Surgery, and Abdominal Surgery, and 33Pathology,Pathology,Grodno Regional Clinical Hospital, Grodno, BelarusGrodno Regional Clinical Hospital, Grodno, Belarus

Contact:Contact:

Haemangioma is the most common benign hepatic Haemangioma is the most common benign hepatic tumor, being present in about 5 % of livers autopsy. tumor, being present in about 5 % of livers autopsy.

In systemic haemangiomatosis multiple organs In systemic haemangiomatosis multiple organs involved including liver, spleen, muscles, bowel, involved including liver, spleen, muscles, bowel, lungs, brain. lungs, brain.

On unenhanced CT haemangioma usually appears as On unenhanced CT haemangioma usually appears as well-marginated solid mass of the same or decreased well-marginated solid mass of the same or decreased relative to liver parenchyma density what corresponds relative to liver parenchyma density what corresponds to hyperechoic lesion on US.to hyperechoic lesion on US.

Grodno Regional Clinical Hospital, Grodno, Belarus

INTRODUCTIONINTRODUCTION

CT scan without contrast enhancement shows typical CT scan without contrast enhancement shows typical haemangioma in the 4haemangioma in the 4thth liver segment. liver segment.


INTRODUCTIONINTRODUCTION

A 68-year-old female presented with abdominal pain, A 68-year-old female presented with abdominal pain, nausea, weakness, intermittent temperature of 38° C nausea, weakness, intermittent temperature of 38° C and chilling. She was underwent spleenectomy one and chilling. She was underwent spleenectomy one month earlier in other hospital because of spontaneous month earlier in other hospital because of spontaneous spleen rupture followed by subfebrile temperature up spleen rupture followed by subfebrile temperature up to the time of present admission.to the time of present admission.


CASE REPORT: historyCASE REPORT: history

Post-contrast liver CT scan shows high contrast Post-contrast liver CT scan shows high contrast enhancement in the capsules of multiple nodular lesions enhancement in the capsules of multiple nodular lesions measuring 2 to 5 cm in diameter. Central parts of the measuring 2 to 5 cm in diameter. Central parts of the lesions remain unenhanced.lesions remain unenhanced.


CASE REPORT: imaging findingsCASE REPORT: imaging findings

Post-contrast liver CT scan. Free fluid in the both spleen Post-contrast liver CT scan. Free fluid in the both spleen bed and left pleural cavity present. Left hemidiaphragm bed and left pleural cavity present. Left hemidiaphragm appeared irregularly thickened.appeared irregularly thickened.



This corresponded to hyperechoic ring-shaped structures This corresponded to hyperechoic ring-shaped structures with anechoic centre on US examwith anechoic centre on US exam..




CASE REPORT: diagnosis?CASE REPORT: diagnosis?

Multiple liver abscessesMultiple liver abscesses

Based on imaging findings and history of resent operation Based on imaging findings and history of resent operation on abdomen first diagnostic choice was:on abdomen first diagnostic choice was:

SecondSecond choice was: choice was:

Cystic metastasesCystic metastases


CASE REPORT: diagnosis?CASE REPORT: diagnosis?

Hemorrhagic fluid was aspirated from the liver lesion under Hemorrhagic fluid was aspirated from the liver lesion under

CT guidance what raised CT guidance what raised suspicion of haemangiomas.suspicion of haemangiomas.

1,5 litres of hemorrhagic fluid was aspirated from the left 1,5 litres of hemorrhagic fluid was aspirated from the left pleural cavity. Explorative thoracotomy revealed diffuse pleural cavity. Explorative thoracotomy revealed diffuse bleeding from the left hemidiaphragm into pleural cavity. bleeding from the left hemidiaphragm into pleural cavity.

Spleen microphotographs were revised and proliferation of Spleen microphotographs were revised and proliferation of

vascular channels characteristic for vascular channels characteristic for haemangiomashaemangiomas was was found.found.


CASE REPORT: outcomeCASE REPORT: outcome

During next few weeks patient’s condition During next few weeks patient’s condition deteriorated rapidly with clinic of sepsis and deteriorated rapidly with clinic of sepsis and disseminated intravascular coagulation. In one month disseminated intravascular coagulation. In one month after admission to the hospital patient died.after admission to the hospital patient died.

On post-mortem study On post-mortem study multiple cavernous multiple cavernous haemangiomashaemangiomas were found in liver, epiploon, were found in liver, epiploon, abdominal ligaments, and left diaphragmatic muscle. abdominal ligaments, and left diaphragmatic muscle. All liver lesions contained large central areas of All liver lesions contained large central areas of intratumoral hemorrhage. intratumoral hemorrhage.


CASE REPORT: final diagnosisCASE REPORT: final diagnosis

Systemic haemangiomatosis with multiple liver Systemic haemangiomatosis with multiple liver cavernous haemangiomas and left cavernous haemangiomas and left diaphragmatic musclediaphragmatic muscle haemangiomas haemangiomas with with clinical presentation of Kasabach-Merrit clinical presentation of Kasabach-Merrit syndromesyndrome..


DIFFERENTIAL DIAGNOSIS OF DIFFERENTIAL DIAGNOSIS OF LIVER CYSTIC MASSESLIVER CYSTIC MASSES

Abscesses.Abscesses. Cystic metastases.Cystic metastases. Atypical liver haemangiomas.Atypical liver haemangiomas. Hepatocellular carcinoma with cystic change.Hepatocellular carcinoma with cystic change. Hepatic tumor after treatment (embolisation, radio-Hepatic tumor after treatment (embolisation, radio-

frequency ablation).frequency ablation). Polycystic liver disease.Polycystic liver disease.


DIFFERENTIAL DIAGNOSIS: DIFFERENTIAL DIAGNOSIS: atypical liver haemangioma atypical liver haemangioma

On US: solid tumor with an echogenic rim and On US: solid tumor with an echogenic rim and hypoechoic internal echo pattern.hypoechoic internal echo pattern.

On CT: ring-like structure with hypodense On CT: ring-like structure with hypodense unenhancing centre.unenhancing centre.

The reasons for central hypoechoity/hypodensity are The reasons for central hypoechoity/hypodensity are internal hemorrhage with necrosis, thrombosis, internal hemorrhage with necrosis, thrombosis, scaring, and myxomatous change. scaring, and myxomatous change.

In one study this kind of lesions were present in 15 of In one study this kind of lesions were present in 15 of 5000 abdominal US exams (0.3 % prevalence of 5000 abdominal US exams (0.3 % prevalence of atypical haemangiomas in the general population).atypical haemangiomas in the general population).


DIFFERENTIAL DIAGNOSIS: abscessDIFFERENTIAL DIAGNOSIS: abscess

CT scans of the liver in two different patients. Abscess in CT scans of the liver in two different patients. Abscess in the right liver lobe (left) and atypical liver haemangiomas the right liver lobe (left) and atypical liver haemangiomas (right) for comparison.(right) for comparison.


DIFFERENTIAL DIAGNOSIS: abscessDIFFERENTIAL DIAGNOSIS: abscess

Liver CT scan shows hepatic abscesses in the right lobe with Liver CT scan shows hepatic abscesses in the right lobe with blood products precipitated along the posterior wall. In such blood products precipitated along the posterior wall. In such instance blood might be aspirated from the abscess.instance blood might be aspirated from the abscess.


DIFFERENTIAL DIAGNOSIS: cystic metastasesDIFFERENTIAL DIAGNOSIS: cystic metastases

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

Most often liver metastases are solid. Cystic metastases are Most often liver metastases are solid. Cystic metastases are uncommon and may arise from mucin-producing uncommon and may arise from mucin-producing primaries or be due to necrosis within the tumor in:primaries or be due to necrosis within the tumor in:


DIFFERENTIAL DIAGNOSIS: cystic metastasesDIFFERENTIAL DIAGNOSIS: cystic metastases

CT scan shows multiple metastases from colorectal CT scan shows multiple metastases from colorectal carcinoma. Some of the lesions demonstrates central carcinoma. Some of the lesions demonstrates central hypodensity.hypodensity.


DIFFERENTIAL DIAGNOSIS: DIFFERENTIAL DIAGNOSIS: polycystic liver diseasepolycystic liver disease

CT scan shows multiple liver cysts. Some of the cysts have CT scan shows multiple liver cysts. Some of the cysts have calcified walls as consequence of chronic inflammation.calcified walls as consequence of chronic inflammation.


DISTINCTIVE FIATURES OF THE DISTINCTIVE FIATURES OF THE PRESENTED CASEPRESENTED CASE

Atypical liver haemangiomas simulating liver abscesses Atypical liver haemangiomas simulating liver abscesses or cystic metastases both radiologically and clinically.or cystic metastases both radiologically and clinically.

PPresence of diaphragmatic haemangiomas. resence of diaphragmatic haemangiomas. Intramuscular haemangiomas are rare benign tumors, Intramuscular haemangiomas are rare benign tumors, making up 0.8% of all haemangiomas. There were just making up 0.8% of all haemangiomas. There were just two reports of diaphragmatic haemangiomas in English two reports of diaphragmatic haemangiomas in English medical literature.medical literature.


CONCLUSIONCONCLUSION

Atypical hepatic cavernous haemangiomas may Atypical hepatic cavernous haemangiomas may present on imaging as multiple ring-like non-present on imaging as multiple ring-like non-specific lesions mimicking abscesses and specific lesions mimicking abscesses and metastases. This diagnosis should be considered in metastases. This diagnosis should be considered in patients with cystic hepatic lesions without patients with cystic hepatic lesions without primary malignancy. Possibility of diaphragm primary malignancy. Possibility of diaphragm involvement in systemic haemangiomatosis has to involvement in systemic haemangiomatosis has to be taken into account.be taken into account.

SYSTEMIC HEMANGIOMATOSIS WITH ATYPICAL LIVER HEMANGIOMAS AND DIAPHRAGM INVOLVEMENT Serguey A....

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