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J Thorac Cardiovasc Surg 2002;123:391-392
© 2002 The American Association for Thoracic Surgery

Brief Communications

The treatment of Askin tumor: Results of two cases

Iwao Takanami, MDa, Tetsuo Imamura, MDb Tokyo, Japan

From the Departments of Surgerya and Pathology,b Teikyo School of Medicine, Tokyo, Japan.

Received for publication July 20, 2001. Accepted for publication Sept 28, 2001. Address for reprints: Iwao Takanami, MD, Department of Surgery, Teikyo School of Medicine, 2-11 Kaga 2-Chome, Itabashi-Ku, Tokyo, 173 Japan (E-mail: takanami{at}med.teikyo-u.ac.jp).



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  		Dr Takanami

 
Primitive neuroectodermal tumor of the thoracopulmonary region is a rare tumor found in children and young adults and has been referred to as Askin tumor.Go 1 Askin tumors typically involve the periosteum, soft tissues, and extrapulmonary tissue of the thoracic wall, and the prognosis is very poor.Go 2 This article describes long-term survival in 2 patients whom we treated for Askin tumor.

Patients and methods
Two patients, 1 woman and 1 man, 32 and 16 years of age, with Askin tumor were treated in our institute between 1990 and 2001(Table 1). The clinical and morphologic findings with immunohistochemical stainingGo 3 involving HBA-71 support the diagnosis of Askin tumor in these patients. At the time of the initial diagnosis, neither of the patients had metastases. We performed chest wall resections involving the ribs in both patients, and both subsequently received chemotherapy. Their chemotherapeutic regimens consisted of cyclophosphamide, doxorubicin (Adriamycin), vincristine, and dactinomycin (actinomycin D).


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  		Table 1. Patients with Askin tumor: Primary site and treatment
 
Results
Patient 1 remains well with no evidence of disease at 7 years. Patient 2 subsequently required 6 further excisions of local recurrences of Askin tumor with postoperative chemotherapy and radiotherapy over a 7-year period. He finally died of the disease 7.5 years after the initial diagnosis(Table 2).


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  		Table 2. Results with Askin tumor
 
Discussion
Askin tumor is not easy to diagnose because its appearance under a light microscope is similar to Ewing sarcoma, rhabdomyosarcoma, and neuroblastoma.Go 4 In our patients, most of the clinical and morphologic findings supported an Askin tumor diagnosis. Askin tumor is more commonly seen in girls in older childhood or adolescence,Go 1 but one of our patients was a 32-year-old woman.

Askin tumor may present as a chest wall mass with thoracopulmonary symptoms such as chest pain, cough, or dyspnea. In our patient 1 a posterior pleural mass was discovered during routine radiographic screening without symptoms, whereas patient 2 had an expanding mass in the chest wall accompanied by pain. Removal was possible in both patients. In patient 1, complete removal of the tumor with chest wall resection involving 4 ribs, followed by chemotherapy, was possible. In patient 2, complete removal may not have been achieved during the first operation. Askin tumor has a predilection to reappear in approximately the same area as the primary neoplasm.Go 1 Metastases to the pleura, rib, pericardium, diaphragm, and vertebra/spinal cord were easily detected by computed tomographic scanning and magnetic resonance imaging in patient 2. Even though we performed relatively wide excisions of the local Askin tumor recurrences followed by chemotherapy and radiotherapy, tumors continued to appear at sites adjacent to the excised tumor, and Askin tumor recurred locally 6 times in 7 years.

The prognosis in Askin tumor is very poor despite the combination of chemotherapy, radiotherapy, and surgical removal, with a 2-year survival of 38% and a 6-year survival of only 14%.Go 2 Very few cases have been reported in which the patient is still alive. Our patients received aggressive combined therapy, and the outcome was more favorable than that reported in the literature. From our preliminary data, we suggest that Askin tumor should be treated with complete surgical excision followed by an aggressive chemotherapy regimen. For local recurrences, postoperative chemotherapy and radiotherapy after complete excision help to prolong survival. A multimodal therapy consisting of surgical resection, appropriate chemotherapy, and radiotherapy seems to be of some benefit in treating Askin tumor, and even in treating recurrences.

References

  1. Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer. 1979;43:2438-51.[Medline]
  2. Contesso G, Llombert-Bosch A, Terrier P, Peydro-Olaya A, Henry-Amar M, Oberlin J, et al. Does malignant small round cell tumor of the thoracopulmonary region (Askin tumor) constitute a clinicopathologic entity? An analysis of 30 cases with immunohistochemical and electron-microscopic support treated at the Institute Gustave Roussy. Cancer. 1992;69:1012-9.[Medline]
  3. Fellinger EJ, Garin-Chesa P, Su SL, DeAngelis P, Lane JM, Retting WJ. Biochemical and genetic characterization of HBA71 Ewing's sarcoma cell surface antigen. Cancer Res. 1991;51:336-40.[Abstract/Free Full Text]
  4. Triche TJ, Askin FB, Kissane JM. Neuroblastoma, Ewing's sarcoma and the differential diagnosis of small-, round-, blue-cell tumors. Philadelphia: WB Saunders; 1986. p. 145-95.Dr Takanami



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