• Title/Summary/Keyword: Desmoplastic small-round-cell tumor

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Imprint Cytology of a Desmoplastic Small Round Cell Tumor -A Case Report- (결합조직형성소원형세포종양의 압착도말 세포학적 소견 -1예 보고-)

  • Kim, Yong-Jin;Kim, Jae-Hwang;Choi, Joon-Hyuk
    • The Korean Journal of Cytopathology
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    • v.18 no.1
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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).

MRI Finding of Retroperitoneal Desmoplastic Small Round Cell Tumor with Hepatic Metastasis and Portal Vein Thrombosis: a Case Report

  • Kim, Youe Ree;Lee, Young Hwan;Choi, Keum-Ha
    • Investigative Magnetic Resonance Imaging
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    • v.23 no.4
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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.

Desmoplastic Small Round Cell Tumor : A Case Report

  • Choi, Joon-Hyuk;Yun, Sung-Soo;Chang, Jay-Chun
    • Journal of Yeungnam Medical Science
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    • v.23 no.1
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    • pp.90-95
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    • 2006
  • Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant mesenchymal tumor found in the abdominal cavity. It mainly affects young male patients. We report a case of DSRCT that occurred in the abdominal cavity of a 50-year-old man. The tumor was characterized by small round tumor cells with irregular nests in the prominent desmoplastic stroma. The tumor cells showed immunoreactivity for epithelial membrane antigen, desmin, vimentin, and neuron specific enolase.

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Desmoplastic Small Round Cell Tumor of the Mediastinum -A case report - (종격동에 발생한 결합조직형성 소원형세포 종양 - 1예 보고-)

  • Kim, Jeong-Won;Cha, Hee-Jeong;Park, Sang-Kyu;Jung, Jong-Pil;Shin, Je-Kyoun;Park, Chang-Ryul
    • Journal of Chest Surgery
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    • v.40 no.2 s.271
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    • pp.147-150
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    • 2007
  • Desmoplastic small round cell tumor (DSRCT) is rare and a recently described, poorly differentiated malignant tumor and it usually presents with widespread intra-abdominal involvement. We report a case of DSRCT arising form the mediastinum which was treated with multimodality anticancer therapy in 15 year-old girl.

Acute Surgical Abdomen in Childhood Malignancies (소아암환자에서 발생하는 급성 복증)

  • Park, Byung-Kwan;Moon, Suk-Bae;Jung, Sung-Eun;Jung, Kyu-Whan;Park, Kwi-Won
    • Advances in pediatric surgery
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    • v.15 no.2
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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.

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