Diagnosis, Treatment and Outcome of Patients with Askin-Tumors

Abstract

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's tumor: 11 cases and a review of the literature

“…In addition, the characteristics of the disease phenotype were identified using immunohistochemical analysis, which demonstrated positive expression of CD99, NSE, S-100 and vimentin protein, while tests for CK, CgA, Syn, LCA, TTF-1 provided negative results. These results are in accordance with previous reports (3,16,17).…”

“…For immunohistochemical analysis, the tumor was previously reported to be positive for several neural markers including NSE, CD99 and vimentin (13,15). Cellular and molecular genetics studies have suggested that PNET has a characteristic chromosomal translocation t (11:22) (q24; q12), which provides an additional firm diagnostic criterion for Askin's tumor (16,17). In addition, the characteristics of the disease phenotype were identified using immunohistochemical analysis, which demonstrated positive expression of CD99, NSE, S-100 and vimentin protein, while tests for CK, CgA, Syn, LCA, TTF-1 provided negative results.…”

“…However, no previous studies have reported which treatment solution is the most effective strategy for Askin's tumor. Christiansen et al (17) recommended that the most appropriate treatment plan should include preoperative and postoperative chemotherapy combined with complete surgical resection. Takanami et al (18) reported one case of a 16-year-old male patient, who suffered local recurrence and underwent six surgeries and postoperative chemotherapy; this patient was still alive at 7 years post surgery, therefore showing the importance of integrated treatment.…”

Peripheral Primitive Neuroectodermal Tumour of the Pleura in a Young Adult- A Case Report

“…(7) Distant metastasis has been known to spread to lungs, mediastina and retroperitoneal nodes, bones, diaphragm, liver, adrenals, sympathetic nerve chain and brain. (8) (9) DISCUSSION OF MANAGEMENT Peripheral Primitive Neuroectodermal Tumours (PNETs) are a group of small round cell malignancies with neural crest origin that arise outside central and sympathetic nervous system. (10) It is a rare disease in children as well as in adults.…”

Peripheral Primitive Neuroectodermal Tumor of the Chest Wall of a 69-year-old Man

“…Operation, combination chemotherapy or radiotherapy has been selected, but not satisfactory (15,18). This tumor is thought to require an aggressive multimodality treatment consisting of preoperative and postoperative chemotherapy, radical surgical resection and postoperative irradiation, which may be performed preoperatively in selected cases (18). …”