KCP 760 (C-12-455) 중앙대학교 병원 전공의 이 정훈

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

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

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

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

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

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

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

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

CK Vimentin Calretinin CD31

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

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

Primary Pleural Angiosarcoma 1. Epidemiology Very rare Only 31 cases in English literature until 2010 Age : 22-79 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. 2010 Jun 15;206(6):415-9. Roh MS, Seo JY, Hong SH. Epithelioid angiosarcoma of the pleura: a case report. J Korean Med Sci. 2001 Dec;16(6):792-5.

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. 2003 Aug;53(8):547-51. Aozasa K. , et al. Angiosarcoma developing from chronic pyothorax. Mod Pathol. 1994 Dec;7(9):906-11. 21

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. 2010 Jun 15;206(6):415-9. 22

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. 2000 Jan;31(1):29-34. 23

감사합니다 24