Intraabdominal desmoplastic small cell tumor: Report and discussion of five cases

Intraabdominal desmoplastic small cell tumor is a rare malignancy that has only recently been described. In the adult population, it is usually seen in young males. The tumor appears to arise as a dominant mass or masses in the abdominal cavity, particularly in the pelvic region, without a clear visceral origin. Multiple small tumor nodules may be found attached to the peritoneal surface. Visceral metastases occur late in the clinical course, ultimately leading to the patient's death. Five young adults with intraabdominal desmoplastic small cell tumors were treated with aggressive chemotherapy.

[1]  R. Feddersen,et al.  EWS and WT-1 gene fusion in desmoplastic small round cell tumor of the abdomen. , 1995, Human pathology.

[2]  D. Frappaz,et al.  Desmoplastic small round cell tumors of the abdomen , 1994, Cancer.

[3]  R. Motzer,et al.  A recurring translocation, t(11;22)(p13;q11.2), characterizes intra-abdominal desmoplastic small round-cell tumors. , 1993, Cancer genetics and cytogenetics.

[4]  C. Fletcher,et al.  Intra-abdominal desmoplastic small-cell tumours with divergent differentiation. Report of two cases and review of the literature. , 1992, Pathology, research and practice.

[5]  U. Khoo,et al.  Intra‐abdominal desmoplastic small round‐cell tumour , 1992, Histopathology.

[6]  J. Sawyer,et al.  A novel reciprocal chromosome translocation t(11;22)(p13;q12) in an intraabdominal desmoplastic small round-cell tumor. , 1992, The American journal of surgical pathology.

[7]  P. Monaghan,et al.  Intra‐abdominal desmoplastic small cell tumour with divergent differentiation , 1991, Histopathology.

[8]  L. Layfield,et al.  Desmoplastic small cell tumors of the peritoneum coexpressing mesenchymal and epithelial markers. , 1991, American journal of clinical pathology.

[9]  W. Gerald,et al.  Intra‐abdominal Desmoplastic Small Round‐Cell Tumor: Report of 19 Cases of a Distinctive Type of High‐Grade Polyphenotypic Malignancy Affecting Young Individuals , 1991, The American journal of surgical pathology.

[10]  J. Goepel,et al.  Intra‐abdominal neuroectodermal tumour of childhood with divergent differentiation , 1991, Histopathology.

[11]  S. Crawford,et al.  Intraabdominal Desmoplastic Small‐Cell Tumors with Divergent Differentiation: Observations on Three Cases of Childhood , 1990, The American journal of surgical pathology.

[12]  N. Ordóñez,et al.  Malignant small-cell epithelial tumor of the peritoneum coexpressing mesenchymal-type intermediate filaments. , 1989, The American journal of surgical pathology.

[13]  F. Mostofi,et al.  Undifferentiated Malignant Epithelial Tumors Involving Serosal Surfaces of Scrotum and Abdomen in Young Males , 1987 .

[14]  E. Wills,et al.  Intra‐Abdominal Desmoplastic Small Round Cell Tumor , 1993, Pathology.