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)