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Case Reports
- Malignant Small Cell Tumor of the Thoracopulmonary Region (Askin Tumor): Report of a case.
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Young Im Han, Hye Jin Lee, Kang Suek Suh, Sun Kyung Lee
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Korean J Pathol. 1995;29(5):687-690.
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Abstract
- Malignant small cell tumor(MSCT) of the thoracopulmonary region(Askin tumor) is extremely rare and is seen predominantly in children and adolescents. This tumor represents a distinct clinicopathologic entity of neuroectodertnal origin, arising from the soft tissues of the chest wall or peripheral lung. This tumor tends to recur locally, but does not seem to disseminate widely. The overall survival is poor. Recently, we experienced a case of the MSCT of the thoracopulmonary region of a 12-year-old female. She was admitted because of a chest wall mass on radiographic examination, and a complaint of intermittent chest pain. Grossly, the mass was lobulated, round and had a solid appearance with focal necrosis and hemorrhage on the cut surface. Histologically, small round to oval cells were arranged in compact sheets, nests and lobular patterns with intervening fibrovascular stroma. Ultrastructurally, the presence of loose-fitting membrane-bound neurosecretory granules was noted.
- Intraabdominal Desmoplastic Small Cell Tumors with Divergent Differentiation: Report of two cases with immunohistochemical and ultrastructural studies.
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Young Ha Oh, Nam Hoon Kim, Joo Seob Keum, Moon Hyang Park
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Korean J Pathol. 1996;30(1):40-49.
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Abstract
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- We studied two intraabdominal desmoplastic small cell tumors. The patients were two men, 37 and 23 years old, with jaundice and palpable abdominal masses. On exploratory laparotomy, each patient revealed a huge mass in the greater omentum with disseminated peritoneal seeding, measuring 32 cm and 11 cm in its greatest dimension, respectively. The tumor involved the diaphragm, rectal shelf, and cul de sac in case 1, and it involved the porta hepatis, retroperitoneum, and serosal surface of the ascending and transverse colon in case 2. Omentectomy of the huge mass and satellite masses was performed in each patient. Both tumors showed nearly the same histopathologic features. The histologic pattern was suggestive of a metastatic small cell carcinoma, but there was no specific, single primary site.
The tumors consisted of variably sized, discrete islands of epithelial-like small cells in dense desmoplastic stroma.
The tumor cells revealed divergent epithelial, mesenchymal, and neural differentiation by histologic, immunohistochemical, and electron microscopic observations.
Only one cycle of chemotherapy including cisplatin and VP-16 was given in case 1 because of a subsequent hepatic problem, who, thereafter, showed massive intraabdominal recurrent tumors 6 months after diagnosis. In case 2, the poor condition of the patient had made chemotherapy and radiotherapy impossible. Case 2 died of disseminated intravascular coagulation following progressive cachexia 7 months after diagnosis.
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