Plis Andrzej, Zygulska Aneta, Püsküllüoğlu Mirosława, Szczepański Wojciech, Białas Magdalena, Krupiński Maciej, Krzemieniecki Krzysztof
Department of Clinical Oncology, Krakow University Hospital, Krakow, Poland; current employment: Clinic of Hematology and Oncology, Transplant and Palliative Medicine, University Hospital in Greifswald, Greifswald, Germany.
Department of Clinical Oncology, Krakow University Hospital, Krakow, Poland.
Contemp Oncol (Pozn). 2016;20(6):496-500. doi: 10.5114/wo.2016.65612. Epub 2017 Jan 12.
Ewing's sarcoma (ES) and primitive neuroectodermal tumour (PNET) are now considered to be the same tumour and usually occur in long bones. Extraskeletal Ewing's sarcoma is an extremely rare neoplasm, accounting for 1% of soft tissue sarcomas, with most common location in the thorax. Gallbladder cancer (GBC) represents the most common type among the biliary tract cancers with a poor prognosis even among patients undergoing aggressive therapy. We present study of extraskeletal ES/PNET found in the hilus of the liver of an elderly, diagnosed one month prior with GBC woman. The patient underwent two cycles of chemotherapy SAIME/SAVAC for ES and thereafter was operated. During three-year follow-up no recurrence of ES/PNET has been reported. However, two years after chemotherapy the patient suffered a relapse of adenocarcinoma of the gallbladder and thus received palliative chemotherapy of gemcitabine and cisplatin. After 16 months of recurrence she died. To the best of our knowledge, this is the first case of ES/PNET located in the hilus of the liver and as a synchronous neoplasm.
尤因肉瘤（ES）和原始神经外胚层肿瘤（PNET）现在被认为是同一种肿瘤，通常发生在长骨中。骨外尤因肉瘤是一种极其罕见的肿瘤，占软组织肉瘤的1%，最常见的部位是胸部。胆囊癌（GBC）是胆道癌中最常见的类型，即使在接受积极治疗的患者中预后也很差。我们报告了一例在一位老年女性肝脏门部发现的骨外ES/PNET病例，该女性一个月前被诊断为GBC。患者接受了两个周期的SAIME/SAVAC化疗治疗ES，之后接受了手术。在三年的随访中，未报告ES/PNET复发。然而，化疗两年后，患者胆囊腺癌复发，因此接受了吉西他滨和顺铂的姑息化疗。复发16个月后患者死亡。据我们所知，这是首例位于肝脏门部且为同步肿瘤的ES/PNET病例。
