Diagnosis, treatment and outcome of patients with Askin-tumors

Askin tumors are highly malignant small-round-cell tumors of the thoracopul monary 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 moleculargenetic 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 no t reveal any metastases. One patient suffered from intrapulmonary metastase s and two patients from an infiltration of the diaphragm and of adjacent ve rtebral bodies. Treatment consisted of a pre- and postoperative (radio-) ch emotherapy according to the EVAIA protocol and a radical tumor resection in all patients. The postoperative course was uneventful in seven patients, o ne patient suffered from pneumonia after multiple wedge resections for intr apulmonary metastases. Four patients, in whom primary tumor resection was c omplete, are alive 14, 20, 35 and 84 months after first diagnosis - only on e patient had to undergo a second operation for a local relapse 17 months a fter first diagnosis. The other 4 patients, who suffered from a very extens ive primary tumor, expired 13, 17, 18 and 39 months after the diagnosis was made. Our data demonstrate that Askin tumors require an aggressive multimo dality treatment consisting of pre- and postoperative chemotherapy, radical surgical resection and postoperative irradiation, which may be performed p reoperatively in selected cases, too.