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askin tumor in Egyptian Patients;5 Years Experience at the National Cancer Institute, Cairo University

Journal of Cancer Therapy 2016年第3期7卷 216-222页

作者： Ahmed El Sayed Fathalla Bahaa El Din Ahmed National Cancer Institute Cairo University Giza Egypt

Background: askin tumor is a primitive neuroectodermal tumor of the Ewing sarcoma family arising from soft tissues of the chest wall. It is the commonest chest wall malignancy in children and adolescents. Its diagnosis is complex and management requires a multidisciplinary work including chemotherapy for systemic disease and radiation therapy to assist local control which is achieved through surgery with or without reconstruction. Objective: To analyze report and understand the clinicopathological features, results and outcome of this tumor with assessment of early and late postoperative complications following resection and chest wall reconstruction. Materials and Methods: This is a retrospective analysis of 30 cases with chest wall ES/PNET presented to the National Cancer Institute;Cairo University between January 2011 and December 2015. All patients’ records were revised for age, sex, clinical presentation, imaging, pathology, operative notes, different treatment modalities given, early and late postoperative complications following surgical resection or reconstruction, and outcome including overall survival (OS) and disease free survival (DFS). Results: Our cohort included 30 patients with chest wall ES. The median follow up period (n = 30) was 33.7 months (ranging from 3.7 to 69.3 months). The median OS was 54.2 months with cumulative OS at 60 months which was 45.6%. The median DFS was 27.9 months with cumulative DFS at 60 months which was 40.6%. All cases were below 18 years with a male predominance (n = 19). The commonest affected sites were ribs (n = 22, 73.3%), the scapula (n = 5, 16.6%), the clavicle (n = 2, 6.66%) and the sternum (n = 1, 3.33%). 23 patients (76.6%) were presented with localized disease, and 7 patients (23.4%) were metastatic from the start. All patients received neoadjuvant chemotherapy (4 cycles of VAC/IE) followed by local control: either surgery (26 cases, 86.6%) or radical radiotherapy (3 cases, 10%). A single case of a rib ES with in

关键词： Chest Wall askin tumor Management & Outcome

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The Origination and Reality of askin tumor

引用

Journal of Nanjing Medical University 2000年第1期14卷 7-10页

作者： Fan Qinhe(范钦和) Philip W Allen\+1\ Xu Tianrong(徐天蓉) Zhang Zhihong(张智弘)\ Zheng Zhaoxun(郑肇巽) Department of Pathology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, P. R. China \+1Flinders Medical Center, SA 504 Department of Pathology the First Affiliated Hospital of Nanjing Medical UniversityNanjing 210029P. R. China Flinders Medical Center SA 5042 Australia

Objective\ To study the origination and reality of askin tumor. Methods\ Twenty two cases of these lesions have been studied by clinical pathologic observation, immunohistochemistry, electron microscopy, and cytogenics in this paper. Results\ The results revealed the positiveness of MIC2(CD99), NSE, Chromogranin A(CgA) by immunohistochemistry, neurosecretory granules by electron microscopy, and the specific genotypic translocation of t (11∶22) (q24∶q12) by cytogenetic analysis, which were identical to the malignant primitive neuroectodermal tumor (PNET). Conclusion\ We conclude that this lesion is actually the one member of the PNET/Ewing′s sarcoma family, except the special location. Differential diagnoses between this tumor and other small round cell sarcomas such as lymphoma, embryonal rhabdomyosarcoma, rhabdoid tumor, round cell liposarcoma and desmoplastic small round cell tumor were also discussed in this article.

关键词： askin tumor PNET/Ewing′s sarcoma immunohistochemistry electron microscopy cytogenetic analysis

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