臨床上較少見之肝臟腫瘤20130906 management

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Page 1: 臨床上較少見之肝臟腫瘤20130906 management

臨床上較少見肝臟腫瘤之處理

蘇建維1,2,3 侯明志2,4 吳肇卿3,5  

1台北榮民總醫院內科部胃腸科2陽明大學醫學院醫學系

3陽明大學醫學院臨床醫學研究所4台北榮民總醫院內視鏡診斷暨治療中心

5台北榮民總醫院教學研究部

Page 2: 臨床上較少見之肝臟腫瘤20130906 management

肝臟結節之分類來源 良性 惡性

肝細胞 (hepatocellular)

腺瘤 (adenoma) 肝細胞癌 (hepatocellular carcinoma)

  再生結節 (regeneration nodules) 纖維板層肝細胞癌(fibrolamellar carcinoma)

  結節性再生性增生 (nodular regenerative hyperplasia)

肝母細胞瘤(hepatoblastoma)

  局部結節性增生 (focal nodular hyperplasia)

 

膽管細胞(cholangiocellular)

膽管腺瘤 (bile duct adenoma) 膽管癌(cholangiocarcinoma)

  膽管囊腺瘤 (biliary cystadenoma) 囊腺癌(cystadenocarcinoma)

  單純囊腫 (simple hepatic cyst)  

  多囊性肝疾病 (polycystic liver disease)  

間質細胞(mesenchymal)

血管瘤 (hemangioma) 血管肉瘤 (angiosarcoma)

  血管脂肪瘤 (angiolipoma) 淋巴瘤 (lymphoma)

異位性 (heterotopic) 腎上腺 / 胰 / 脾 (adrenal/pancreatic/splenic)

轉移癌 (metastases)

感染性 (infectious) 膿瘍 (abscess)  

  結核 (tuberculosis)  

  血吸蟲 (schistosomiasis)  

Page 3: 臨床上較少見之肝臟腫瘤20130906 management

常見肝臟良性結節臨床表徵之比較  血管瘤

(hemangioma)局部結節性增生

(FNH)腺瘤 (adenoma)

發生率 (%) 0.4-20 0.3-3 1x10-6 至 1.2 x 10-4

好發年齡 30-50 歲 30-50 歲 各種年齡層性別 ( 男 : 女 ) 1:2-6 1:5-17 1: 8-15

超音波所見回音性 高回音 (hyerechoic)

不一定 不一定

電腦斷層 靜脈性影像增強 中央疤 (central scar)

動脈相廣泛性影像增強

磁振造影 腦脊髓液質地 肝臟質地 肝臟質地T1 時相影像為高訊號(hyperintense) 病灶

血管攝影 高血管性 高血管性 高血管性核醫檢查 (Tc99m sulfur colloid scan)

紅血球吸收 吸收 因缺乏 Kupffer 細胞,減少吸收

鈣化 會 不會 不會結節破裂機會 極少 無 可能

Page 4: 臨床上較少見之肝臟腫瘤20130906 management

Hemangioma: management • Most hepatic hemangiomas remain stable over time and require no

treatment• Treatment or follow-up is not indicated for asymptomatic lesions

that are < 5cm in diameter• Rupture

– Large, peripheral-located– Trauma– Liver biopsy or fine needle aspiration

• Resection surgery:– Symptomatic – Rapidly enlarging – > 15 cm in diameter at initial presentation

• Other treatment: radiofrequency ablation; cryoablation; liver transplantation (for Kasabach-Merritt syndrome)

Page 5: 臨床上較少見之肝臟腫瘤20130906 management

Adenoma: management (I)• Depends on– Symptoms– Size– Number of lesions– Risk of bleeding, rupture, and malignant

transformation• Risk of malignant transformation– Around 10%– Increase in size– Rise in serum AFP levels– β-catenin gene mutations

Page 6: 臨床上較少見之肝臟腫瘤20130906 management

Adenoma: management (I)• Small lesions (< 5cm): – Periodic imaging– OCP and steroid should be discontinued– Therapy when symptomatic or increase in size despite

discontinuation of estrogen• Resection surgery or liver transplantation

(glycogen storage disease or multiple adenomas)• Mortality rate: < 1% in selection; 5-8% in

emergency resection of bleeding or ruptured lesions

• Avoid pregnancy prior to resection

Page 7: 臨床上較少見之肝臟腫瘤20130906 management

Liver adenomatosis

• Presence of 10 or more adenomas not associated with steroid use, but with underlying glycogen storage diseases

• Pathogenesis: thought to be secondary to congenital or acquired abnormalities of hepatic vasculature

• Hemorrhage appears to be common, particularly in lesions > 4 cm

• Liver transplantation should be considered due to high risk of bleeding, rupture and malignant transformation

Page 8: 臨床上較少見之肝臟腫瘤20130906 management

Management of suspected liver benign lesions

Follow-up OCP use Pregnancy Treatment

Hemangioma Classic features: no follow-up

Not absolutely contraindicated

Not contraindicated

Frequent follow-up; resect if symptomatic (rarely needed)

FNH Classic features: no follow-up

Not absolutely contraindicated

Not contraindicated

Frequent follow-up; resect if symptomatic (rarely needed)

Adenoma Variable Stop No Stop OCP; resect if solitary and large (> 5cm)

Brhirwani R, Reddy KR. Aliment Pharmacol Ther 2008; 28:953-65

Page 9: 臨床上較少見之肝臟腫瘤20130906 management

Algorithm for the diagnosis and treatment of FNH and adenoma

Nault JC, et al. Gastroenterology 2013; 144:888-902

Page 10: 臨床上較少見之肝臟腫瘤20130906 management

奧地利 多瑙河

Page 11: 臨床上較少見之肝臟腫瘤20130906 management

Hepatic angiomyolipoma (AML): management

• Hepatic AML was considered as a benign disease in the past.

• Nevertheless, tumor rupture, metastasis, recurrence after resection surgery of primary tumor, as well as concurrent hepatic AML and HCC were reported recently.

• Conservative management with close follow-up is reserved in asymptomatic patients with good compliance, no chronic hepatitis, as well as small AML which is less than 5 cm in size and is proved through fine needle aspiration biopsy.

• Surgical intervention might be considered if the progression of the tumor or the emergence of symptoms occurs during the follow-up, especially for those with a high risk for HCC.

Page 12: 臨床上較少見之肝臟腫瘤20130906 management

Hepatic epithelioid hemangioendothelioma: treatment

HEHE

Hemi-liver involvement

Extra-hepatic involvement

(+)

Liver resection ± chemotherapy

TACE, chemotherapy, radiotherapy

Extra-hepatic involvement

(-)

Liver resection

Diffuse involvement

Extra-hepatic involvement

(+)

Liver transplantation ± chemotherapy

TACE, chemotherapy, radiotherapy

Extra-hepatic involvement

(-)

Liver transplantation

Mehrabi A, et al. Cancer 2006; 107:2108-21

Page 13: 臨床上較少見之肝臟腫瘤20130906 management

Prognosis of HEHE

Mehrabi A, et al. Cancer 2006; 107:2108-21

Page 14: 臨床上較少見之肝臟腫瘤20130906 management

Long –term prognosis of HEHE and angiosarcoma after liver transplantation

Orlando G, et al. Transplantation 2013; 95:872-7

Page 15: 臨床上較少見之肝臟腫瘤20130906 management

Management of hepatic angiosarcoma

Kim HR, et al. Ann Oncol 20:780-7

Page 16: 臨床上較少見之肝臟腫瘤20130906 management

Hepatic TB management and prognosis

• Hepatic TB is treated like any other extra-pulmonary tuberculosis lesion.

• Chemotherapy with standard anti-TB drugs remains the corner stone of treatment.

• Most authors have used four drugs (INH, Rifampicin, Streptomycin and Pyrazinamide) during the initial two months, followed by INH and Rifampicin for the next seven months.

• Cumulative mortality for hepatic tuberculosis ranges between 15% and 42%. The factors associated with adverse prognosis are: age < 20 years, miliary tuberculosis, concurrent steroid therapy, AIDS, cachexia, associated cirrhosis and liver failure.

Page 17: 臨床上較少見之肝臟腫瘤20130906 management

Cystadenoma: treatment

• Resection surgery is preferred due to malignant transformation occurring in 15% of patients

• Aspiration and partial resection are associated with worse prognosis compared with complete resection and an increased risk of recurrence

Page 18: 臨床上較少見之肝臟腫瘤20130906 management

Polycystic liver disease: treatment• Asymptomatic: require no treatment• Large cysts with symptom: hepatic resection• Obstructive jaundice caused by extrinic compression

of the biliary tree: unroofing of large cysts• Portocaval shunting may be attempted for patients

with portal hypertension• Percutaneous TAE: decrease intrahepatic cyst volume• Mammalian target of rapamycin (mTOR) inhibitors

decrease polycystic liver volume by inhibiting the proliferation of biliary epithelium

Page 19: 臨床上較少見之肝臟腫瘤20130906 management

捷克 布拉格