A 24-year-old woman was admitted to be ascribed to dyspnea on exertion.
A 24-year-old woman was admitted to be ascribed to dyspnea on exertion. A chest roentgenogram revealed a massive tumor originating from the right anterior chest wall when pleural effusion was drained. Diagnosis of Askin tumor was made based forward light microscopic findings characterized from composing with small round solitary abode; squalids immunocytochemical findings suggestive of neuroectodermal origin, and cytogenetic analysis demonstrating the chromosomal translocation (11; 22) After intensive combined modality therapy, including chemotherapy, irradiation, and additional surgery she was followed up as an outpatient and has remained disease-free for 16 month after initial diagnosis.
Tumors arising from the chest wall are rare. Schaefer and Burton[1] reviewed the radiographic characterization of the chest wall tumors. They classified the tumors into four form into groupss as follows: (a) contiguous spread of adjacent neoplasms, (b) metastatic neoplasms from the other organs, (c) primary malignant tumors, as it was as chondrosarcoma, plasmacytoma, and fibrosarcoma, and (d) benign tumors mainly originating from cartilage. In childhood, Ewing's sarcoma is the greatest in quantity common chest wall tumor, whereas rhabdomyosarcoma, neuroblastoma, and Askin tumor are les usual Askin tumor was first described as a malignant tumor of the thoracopulmonary region in childhood and adolescence as a unique clinicopathologic entity.[2] We report an adult case with a giant tumor in the chest wall, which was diagnosed as Askin tumor through extensive studies including immunocytochemical and cytogenetic examinations. lucky treatment, including an intensive combined therapy based forward another report[3] and additional surgery is also presented
CASE REPORT
A 24-year-old woman was admitted to our hospital forward Jan 28, 1991, because of dyspnea in succession exertion, dry cough, and right anterior chest pain, which lasted for 1 month prior to admission and progressively worsened. She had no remarkable past history and no smoking history. Physical examination in succession admission showed dullness on percussion and a decrease of breath hales in the right lung base. No other abnormalities were exposeed A chest roentgenogram at the time of admission showed extensive radiopacity in the right hemithorax. Ultrasonographic examination revealed that the radiocpacity was compos of a elephantine tumor originating from the anterior chest wall and massive pleural effusion. After evacuation of the pleural effusion, a gigantic tumor was observed on the chest roentgenogram (Fig 1) A comput tomography scan of the chest revealed that the mass was in contact with the right atrium and that the fifth rib of the anterior chest wall had destructive change proper to the tumor. In a technetium [sup99mTC] bone scintigram, the right fifth rib had high uptake of the isotope. Other studies revealed no evidence of distant metastasis. life-blood tests were normal except for CA125 (460 U/ml; normal, les than 50 U/ml) Her pleural effusion showed murderous appearance and had exudative character, on the other hand the concentrations for adenosine deaminase and tumor markers were normal leaving out for CA125 (410 U/ml).
Cytologic examination of pleural effusion revealed neoplastic enclosed spaces featuring round nuclei, fine chromatin, scant cytoplasm, and a cluster formation. For further distinct diagnosis, echo-guided aspiration biopsy was performed. Histologic findings from the biopsy specimen revealed that the tumor was comprised of small cylindrical cells arranged in a diffuse pattern however not in a Homer-Wright rosette pattern, which is characteristic of neuroblastoma. Immunocyto-chemical studies showed that the neoplastic solitary abode; squalids stained positively for neuron-specific enolase in the cytoplasm. In contrast, these confined apartments were not only negative for a leukocyte used by all antigen, but also negative for 5C11 monoclonal antibody which reacts specifically with the surface antigen of Ewing's sarcoma.[4] They showed negative stain for periodic acid Schiff. Neurofilaments, neurosecretary granules, and rhabdomyofibrils were not observ forward electron microscopy. Chromosomal analysis of the neoplastic lonely dwellings in pleural effusion revealed the chomosomal translocation (11;22 [Fig 2]) Diagnosis of Askin tumor was made based forward these analyses.
Intensive combined chemotherapy (vincristine, 2 mg/[msup2]; doxorubicin, 45 mg/[msup2] for 2 days for brace courses; 30 mg/[m.sup.2] for 2 days for the later courses; cyclophosphamide, 900 mg/[m.sup.2] for 2 days) for three courses and irradiation (20 Gy) were administered through 1 month. Since the tumor and pleural effusion were reduc remarkably (tumor reduction: from 18 x 12 cm to 5 x 2 cm) surgery was performed onward July 6. At the time of thoracotomy, the tumor was observ to originate from the anterior chest wall and it involved single the fifth rib without invasion to the lung diaphragm, and heart. The tumor and the fifth rib was resect en bloc Pathologic examinations showed and nothing else a few viable neoplastic small cavitys in the massive necrotic tissue of the resect mass, and malignant solitary abode; squalids were absent in the margin of the resect rib. After single additional course of chemotherapy, the patient was discharged and followed up as an outpatient. No sign of resort was detected in the following 10 month after surgery
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