1. KCP 760 (C )
중앙대학교 병원
전공의 이 정훈

2. 병력 M/75 주소 가슴통증 (6개월전) 과거력: 결핵성 가슴막염 (35년전) CT: 가슴막삼출 및 광범위한 가슴막벽 비후소견
Debridement 시행
=> 혈성괴사, 섬유화 및 만성 염증소견
객담 및 가슴막삼출액
=> 염증세포, AFB(-)
결핵치료에도 호전 없어 세포검사 재시행

8. Differential diagnosis
Adenocarcinoma
Large cell carcinoma
Mesothelioma
Epithelioid hemangioendothelioma
Epithelioid Angiosarcoma 8

9. Differential diagnosis
Adenocarcinoma
acini, mucin vacuoles
eccentrically placed, round or irregular nuclei
translucent, foamy cytoplasm
finely textured chromatin
pleomorphism, increased mitosis 9

10. Differential diagnosis
Large cell carcinoma
syncytial clusters and dispersed cells
irregular nuclei
striking chromatin clearing
prominent, often multiple nucleoli
ill-defined, feathery cytoplasm 10

11. Differential diagnosis
Mesothelioma
large clusters with scalloped (“knobby”) edges
“noncohesive” (isolated cells)
round, centrally placed nucleus
prominent nucleolus
can have vacuolated cytoplasm
dense cytoplasm with peripheral “halo”
windows 11

12. Differential diagnosis
Epithelioid hemangioendothelioma
round to polygonal plasmacytoid cells
only slight nuclear pleomorphism and frequent
binucleation
abundant cytoplasm with occasional intracytoplasmic lumina
few mitoses 12

13. Differential diagnosis
Epithelioid Angiosarcoma
large, dyshesive epithelioid cells
moderate to marked nuclear pleomorphism
prominent nucleolus
occasional angioformative structures: pseudoacini with central erythrocytes or intracytoplasmic lumina containing erythrocytes
numerous mitoses 13

14. Metastatic non-small cell carcinoma
Diagnosis
Metastatic non-small cell carcinoma 14

17. CK
Vimentin
Calretinin
CD31

18. Diagnosis Pleura, excision : Epithelioid angiosarcoma
[Immunohistochemistry]: CK(-), Vimentin(+), Calretinin(-), CD31(+) 18

19. Angiosarcoma 1-2% of soft tissue tumors
From endothelial cells of small vessels

20. Primary Pleural Angiosarcoma
1. Epidemiology
Very rare
Only 31 cases in English literature until 2010
Age : yr (mean 57 yr)
Male: Female = 9: 1
Dainese E, , et al. Primary pleural epithelioid angiosarcoma. A case report and review of the literature. Pathol Res Pract Jun 15;206(6):415-9.
Roh MS, Seo JY, Hong SH. Epithelioid angiosarcoma of the pleura: a case report. J Korean Med Sci Dec;16(6):792-5.

21. Primary Pleural Angiosarcoma
2. Etiology: unclear
Radiation
Asbestos
Tuberculous pyothorax:
only in Japanese
first described in 1973
m/c causative factor in Japan
3600-fold higher frequency in Tb pyothorax
Kimura M, et al. Pyothorax-associated angiosarcoma of the pleura with metastasis to the brain. Pathol Int Aug;53(8):
Aozasa K. , et al. Angiosarcoma developing from chronic pyothorax. Mod Pathol Dec;7(9): 21

22. Primary Pleural Angiosarcoma
3. Clinical features
Pleural effusion
Diffuse pleural thickening
Chest pain
=> DDx: diffuse malignant mesothelioma
Dainese E, , et al. Primary pleural epithelioid angiosarcoma. A case report and review of the literature. Pathol Res Pract Jun 15;206(6):415-9. 22

23. Primary Pleural Angiosarcoma
4. Prognosis
Unfavorable
most died within 6 months after Dx
Zhang PJ, et al. Malignant epithelioid vascular tumors of the pleura: report of a series and literature review. Hum Pathol Jan;31(1):29-34. 23

24. 감사합니다 24