Alveolar Soft Part Sarcoma of the Lung -A Report of Six Cases and Clinicopathological Analysis-
김나래, 이미숙, 윤영철, 김대수, 이경수, 서지영, 김진국, 한정호,
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김나래 ( Kim Na-Rae )
가천의대 길병원 병리과
이미숙 ( Lee Mi-Sook )
성균관대학교 의과대학 삼성서울병원 병리과
윤영철 ( Yoon Young-Cheol )
성균관대학교 의과대학 삼성서울병원 영상의학과
김대수 ( Kim Dae-Su )
성균관대학교 의과대학 삼성서울병원 병리과
이경수 ( Lee Kyong-Soo )
성균관대학교 의과대학 삼성서울병원 영상의학과
서지영 ( Suh Gee-Young )
성균관대학교 의과대학 삼성서울병원 내과학교실
김진국 ( Kim Jhin-Gook )
성균관대학교 의과대학 삼성서울병원 흉부외과학교실
한정호 ( Han Joung-Ho )
성균관대학교 의과대학 삼성서울병원 병리과
KMID : 0357920030370020087
Abstract
Background: Alveolar soft part sarcoma (ASPS) accounts for 0.5-1% of soft tissue sarcomas, and often metastasizes to the lung. Cases of pulmonary ASPS of unknown primary site have rarely been reported in literature.
Methods: Here, we report three cases of metastatic pulmonary ASPS and three cases of presumably primary ASPS using immunohistochemistry and clinicoradiologic findings.
Results: All of the cases occurred in young females. Two of the cases had metastasized from soft tissue ASPS of the lower extremities, and one case had metastasized from one of the patient’s femur bones. Immunohistochemical stains were applied to four cases that had available paraffin blocks. The tumor cells of all cases on which immunohistochemical stains were done were positive for vimentin (4/4, 100%). None of the tumors were positive for myoglobin, desmin, smooth muscle actin, progesterone receptor, estrogen receptor, thyroid transcription factor-1, S-100 protein, pancytokeratin, and HMB-45 antibodies.
Conclusion: The present study revealed that the rare pulmonary ASPS has non-specific clinicoradiologic findings. In the immunohistochemical results, no differences existed between the presumably primary ASPS and the metastatic ASPS except for a higher Ki-67 labeling index in the latter (less than 0.1% vs. 30%). The higher index was not dissimilar to those of the extrapulmonary ASPS which showed a tumor with a low proliferation index, signifying a better prognosis and have a low potential to metastasize.
키워드
Sarcoma;Alveolar Soft Part;Lung Neoplasms;Immunohistochemistry
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