title: Different subtypes of epithelioid sarcoma and their clinical implication: long-term multi-institutional experience with a rare sarcoma.
abstract: Epithelioid sarcoma (ES) is a very rare soft-tissue sarcoma with a high tendency of recurrence and metastasis. We analyzed clinical features of ES and aimed to identify the potential role of radio- and chemotherapy in ES. Fifty-five patients diagnosed with ES between 1997 and 2014 were enrolled from seven tertiary hospitals in Korean Cancer Research Group. The clinical variables were retrospectively reviewed and analyzed. Forty-six (84%) patients underwent surgical resection of ES, and among them, 27 experienced recurrence. In these patients, resection margin status and adjuvant radiotherapy were independent prognostic factors for longer recurrence-free survival (RFS), while adjuvant chemotherapy did not influence RFS. Twenty-two (40%) patients received palliative chemotherapy for metastatic or recurrent ES, and in these patients, palliative chemotherapy was the only independent prognostic factor for longer overall survival. Intriguingly, the clinical benefit of radio- and chemotherapy was observable only in proximal ES, but not in extremity ES, indicating that subtypes of ES might respond to radio- or chemotherapy differently. Proximal ES seems to benefits more from active anticancer treatment than conventional extremity ES. The aggressive characteristics of proximal ES could be overcome with an optimal multimodal treatment.
authors.affiliation: Medical Oncology, CHA Bundang Medical Center, CHA University, Seongnam-si, Korea.
authors.forename: Chan
authors.initials: C
authors.lastname: Kim
authors.affiliation: Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Kwai Han
authors.initials: KH
authors.lastname: Yoo
authors.affiliation: Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Min Hwan
authors.initials: MH
authors.lastname: Kim
authors.affiliation: Medical Oncology, CHA Bundang Medical Center, CHA University, Seongnam-si, Korea.
authors.forename: Hong Jae
authors.initials: HJ
authors.lastname: Chon
authors.affiliation: Division of Hematology-Oncology, Department of Medicine, Dankook University College of Medicine, Cheonan, Korea.
authors.forename: Soon Il
authors.initials: SI
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, School of Medicine Chungnam National University, Daejeon, Korea.
authors.forename: Hyo Jin
authors.initials: HJ
authors.lastname: Lee
authors.affiliation: Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.
authors.forename: Sujin
authors.initials: S
authors.lastname: Koh
authors.affiliation: Division of Hematology-Oncology, Department of Internal Medicine, Kyunghee University Hospital at Gangdong, Seoul, Korea.
authors.forename: Ha Yeon
authors.initials: HY
authors.lastname: Lee
authors.affiliation: Division of Hematology/Oncology, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea.
authors.forename: Hye Ran
authors.initials: HR
authors.lastname: Lee
authors.affiliation: Department of Surgery, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Kyung Sik
authors.initials: KS
authors.lastname: Kim
authors.affiliation: Department of Urology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Young Deuk
authors.initials: YD
authors.lastname: Choi
authors.affiliation: Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Sun Young
authors.initials: SY
authors.lastname: Rha
authors.affiliation: Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Su Jin
authors.initials: SJ
authors.lastname: Lee
authors.affiliation: Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Hyo Song
authors.initials: HS
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012008:Recurrence; D012189:Retrospective Studies; D012509:Sarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D055815:Young Adult
keywords: Epithelioid sarcoma; chemotherapy; clinical outcome; radiotherapy; subtype