Christiansen S, Semik M, Dockhorn-Dworniczak B, Rötker J, Thomas M, Schmidt C, Jürgens H, Winkelmann W, Scheld H H
Klinik und Poliklinik für Thorax-, Herz- und Gefässchirurgie, Westfälische Wilhelms-Universität Münster, Germany.
Thorac Cardiovasc Surg. 2000 Oct;48(5):311-5. doi: 10.1055/s-2000-7874.
Askin tumors are highly malignant small-round-cell tumors of the thoracopulmonary region, which occur rarely. Therefore, we report on our experiences with eight patients (5 male, 3 females), who were treated in our department between 11'94 and 10'97 (age: 9-40 years, mean age: 20.5 years). All Askin tumors were diagnosed by histological and immunohistochemical examinations as well as molecular genetic proof of characteristic translocations. In all patients, the tumor arose from the chest wall, infiltrating adjacent ribs and parts of the lung. At the time of first diagnosis, five patients did not reveal any metastases. One patient suffered from intrapulmonary metastases and two patients from an infiltration of the diaphragm and of adjacent vertebral bodies. Treatment consisted of a pre- and postoperative (radio-) chemotherapy according to the EVAIA protocol and a radical tumor resection in all patients. The postoperative course was uneventful in seven patients, one patient suffered from pneumonia after multiple wedge resections for intrapulmonary metastases. Four patients, in whom primary tumor resection was complete, are alive 14, 20, 35 and 84 months after first diagnosis - only one patient had to undergo a second operation for a local relapse 17 months after first diagnosis. The other 4 patients, who suffered from a very extensive primary tumor, expired 13, 17, 18 and 39 months after the diagnosis was made. Our data demonstrate that Askin tumors require an aggressive multimodality treatment consisting of pre- and postoperative chemotherapy, radical surgical resection and postoperative irradiation, which may be performed preoperatively in selected cases, too.
Askin肿瘤是发生于胸肺区域的高度恶性小圆细胞瘤，极为罕见。因此，我们报告了1994年11月至1997年10月期间在我科接受治疗的8例患者（5例男性，3例女性）的情况（年龄9 - 40岁，平均年龄20.5岁）。所有Askin肿瘤均通过组织学和免疫组化检查以及特征性易位的分子遗传学证据得以确诊。所有患者的肿瘤均起源于胸壁，侵犯相邻肋骨及部分肺组织。初诊时，5例患者未发现转移。1例患者有肺内转移，2例患者有膈肌及相邻椎体浸润。治疗方案为根据EVAIA方案进行术前和术后（放）化疗，并对所有患者实施根治性肿瘤切除术。7例患者术后恢复顺利，1例患者因肺内转移接受多次楔形切除术后发生肺炎。4例原发肿瘤切除彻底的患者在初诊后14、20、35和84个月仍存活，仅1例患者在初诊后17个月因局部复发接受了二次手术。另外4例原发肿瘤范围极广的患者在确诊后13、17、18和39个月死亡。我们的数据表明，Askin肿瘤需要积极的多模式治疗，包括术前和术后化疗、根治性手术切除以及术后放疗，在某些特定情况下也可在术前进行放疗。
