• 대한세포병리학회지
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• 제18권1호
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• pp.81-86
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• 2007

Desmoplastic small round cell tumor (DSRCT) is a rare malignant mesenchymal neoplasm. It mainly involves the abdominal or pelvic peritoneum of male adolescents. We report here the imprint cytologic features of a case of DSRCT occurring in the intraabdominal cavity of a 21-year-old man. A microscopic examination showed moderate cellularity. The tumor cells were singly arranged and arranged in clusters. The cells had round to oval nuclei with finely granular chromatin, inconspicuous nucleoli and scanty cytoplasm. Some tumor cells showed nuclear molding, and some cells had an epitheloid appearance with a large amount of lightly eosinophilic cytoplasm. A rosette-like pattern was present. Spindle-shaped, fibroblastic stromal cells were occasionally found. The tumor cells were immunoreactive for the markers cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), desmin, vimentin and neuron specific enolase (NSE).

• Investigative Magnetic Resonance Imaging
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• 제23권4호
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• pp.361-366
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• 2019

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy common in young male patient. Typical imaging features of DSRCT include multiple soft tissue masses in the peritoneal cavity, omentum, or mesentery without an organ of origin. This report presents a rare manifestation of DSRCT revealing a solitary large retroperitoneal mass with hepatic metastasis and malignant portal vein thrombosis in 70-year-old women together with the review of literature. The tumor showed a hemorrhagic and necrotic mass with peripheral portion of T2 hypo-intensity and delayed enhancement that indicated desmoplastic stroma with dense cellularity.

• Journal of Yeungnam Medical Science
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• 제23권1호
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• pp.90-95
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• 2006

결합조직형성소원형세포종양은 매우 드문 악성 연부조직 종양으로 소아와 청소년기에 복강과 골반강 내에 주로 발생한다. 저자들은 복강에 발생한 결합조직형성소원형세포종양 1예를 경험하였기에 문헌 고찰과 함께 보고한다. 50세 남자가 2개월간의 복부 불쾌를 주소로 내원하였다. 방사선 소견에서 오른쪽 복강 내에 종괴가 관찰되었다. 절제된 종괴는 크기가 $14.0{\times}12.5{\times}8.5cm$ 이며, 회백색을 띠었으며 가로 잘록창자의 장막에 붙어 있었다. 조직학적으로 종괴는 결합조직을 형성하는 기질 내에 작고 둥근 핵을 가진 종양세포들의 증식이 보였다.

• Journal of Chest Surgery
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• 제40권2호
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• pp.147-150
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• 2007

결합조직형성 소원형세포 종양(desmoplastic small round cell tumor)은 최근에 발생 빈도가 증가하는 추세인 희귀한 종양으로 주로 복강 내에서 발생하는 것으로 알려져 있다. 저자들은 15세 여자 화자의 종격동에 발생한 결합조직형성 소원형세포 종양에 대해 적극적인 항암화학요법, 수술, 방사선치료 및 자가 말초혈 조혈모세포 이식 등의 복합요법으로 만족스러운 결과를 얻었기에 이를 국내 최초로 보고하고자 한다.

• Advances in pediatric surgery
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• 제15권2호
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• pp.103-112
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• 2009

Catheter related and perianal problems are common surgical complications encountered during the treatment of pediatric malignancies. However acute surgical abdominal emergencies are rare. The aim of this study is to review acute surgical abdominal complications that occur during the treatment of childhood malignancies. Out of a total of 1,222 patients who were newly diagnosed with malignant disease, between January 2003 and May 2008, there were 10 patients who required surgery because of acute abdominal emergencies. Their medical records were reviewed retrospectively. Hematologic malignancies were present in 7 patients (4 leukemia, 2 lymphoma, 1 Langerhans cell histiocytosis) and solid tumors in 3 patients (1 adrenocortical carcinoma, 1 desmoplastic small round cell tumor, 1 rhabdomyosarcoma). Seven patients had intestinal obstruction, two had gastrointestinal perforation and one, typhlitis. Intestinal obstructions were treated with resection of the involved segment with (N=2) or without (N=3) enterostomy. Two patients had enterostomy alone when resection could not be performed. Intestinal perforation was treated with primary repair. Typhlitis of the ascending colon was treated with ileostomy. Right hemicolectomy was necessary the next day because of the rapidly progressing sepsis. Three patients are now alive on chemotherapy and one patient was lost to followed-up. Among six patients who died, five died of their original disease progression and one of uncontrolled sepsis after intestinal perforation. Although rare, acute surgical abdominal complications can occur in childhood malignancies. Rapid and accurate diagnosis and appropriate operation are required for effective treatment of the complications.