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Liver mesenchymal tumor mimicking Klatskin tumor

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Presentation on theme: "Liver mesenchymal tumor mimicking Klatskin tumor"— Presentation transcript:

1 Liver mesenchymal tumor mimicking Klatskin tumor
Hong Joo Kim, Department of Internal Medicine, Sungkyunkwan University Kangbuk Samsung Hospital, Seoul, Korea

2 Age : 72 years Gender : Male C/C General Weakness Abdominal discomfort Dyspepsia Cough

3 Present illness 기침, 소화불량, 전신 쇄약감 등으로 Local 내원하여 시행한 흉부 방사선 및 객담 검사
상, Pul Tbc진단되어 Anti-Tb Medication 중, 복부 불편감 지속되어 시행한 복부 CT상 Cholangiocarcinoma 의심되는 소견보여 본 원으로 전원된 환자임

4 Past Illness Social History Family history
DM/HTN/Tbc/Hepatitis (-/-/+/-) 현재 Tbc medication 중 Social History 무직 (과거에 상업) Alcohol : None drinker (10년전 stop) Smoking : 30 pack year (10년전 stop) Family history N/S

5 Systemic Review General Weakness (+) Fever (-) Cough/Sputum (+/+)
Chest pain/Respiratory discomfort (-/-) Poor oral intake (+) Nausea/Vomiting (-/-) Abdominal pain (+) Diarrhea/Constipation (-/-) Hematochezia/Hematetmesis (-/-)

6 Physical Exam Vital Sign (Initial)
BP - 130/90, HR : 79/min, RR : 9/min Normal heart beat Normocephaly Neurological Exam : W.N.L Abdomen: DT/RT (-/-) Normal bowel sound Limitation of movement: (-)

7 Initial Lab Finding CBC : 7390 - 11.0/32.6 – 471K AST/ALT : 130/93
Bilirubin total : 0.37 Na-K-Cl : Total protein/Albumin : 6.9/4.0 Tumor marker AFP : 2.41 ng/Ml CA 19-9 : U/mL

8 Sputum AFB (+)

9 CT Finding

10 PTBD Klatskin tumor IIIa

11 Sono-guided liver biopsy

12 Pathology H&E stain x 200 Vimentin stain x 200

13 H&E stain x 400

14 Final diagnosis: Undifferentiated Mesenchymal tumor
Obilteration of liver parenchyme Myxoid cellular components Rhabdoid feature Spindle feature Atypical feature Vimentin (+) : mesenchymal marker Epithelial marker (-) Hematolymphoid marker (-) Final diagnosis: Undifferentiated Mesenchymal tumor

15 Biliary stent insertion

16 Malignant Mesenchymal Tumor of Liver
Reviews Malignant Mesenchymal Tumor of Liver

17 The Korean Journal of Hepatolog y : Vol. 4. No. 3. 1998
one of the rare primary malignant tumor of the liver typically occurring in later childhood Very rare in adult Incidence rate : 0.14 to 0.25 per million 8-13 % of malignant neoplasm in childhood (Western) Male : Female = 2:1 Pathologic distribution was defined in 1978 year (Stocker et al) The Korean Journal of Hepatolog y : Vol. 4. No

18 4 types of mesenchymal tumor
Angiosarcoma Epitheloid Hemangioendothelioma Undifferentiated Embryonal Sarcoma Undifferentiated Malignant Mesenchymal Tumor

19 The Korean Journal of Hepatolog y : Vol. 4. No. 3. 1998
Location : Rt lobe > Lt lobe (Western) Hilum invasion : rare ( some in childhood) No report in adult The Korean Journal of Hepatolog y : Vol. 4. No

20 Pathogenesis Cytogenetic abnormalities in chromosome 19q Variation of Li-fraumani syndrome Symptom Abdominl pain Abdominal mass General symptom (Weakness, Fatigue) Jaundice (rare)

21 Obliteration of hepatic structure Vimentin (+)
Pathology Rhabdoid cell Spindle cell Amorphous cell Obliteration of hepatic structure Vimentin (+) Hematolymphoid marker (-) Epithelial marker (-) Mixoid pattern

22 Treatment Surgical operation (1 st choice if possible) Only 30 % were resectable Combined systemic chemotherapy doxorubicin, cis-diaminodichloroplatinum, cyclophosphamide, dacarbazine, 5-FU Prognosis Extremely poor Frequently metastasis and early recurrence Leuschner et al : 47.5 % patients < 1 year survial (Average 11.9 months)


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