Primary desmoplastic small round cell tumor of the kidney in a 7-year-old girl
Department of Urology, Children's Hospital Boston, 300 Longwood Avenue, HU-390, Boston, MA 02115, USA.Journal of pediatric urology (Impact Factor: 0.9). 03/2006; 2(1):52-4. DOI: 10.1016/j.jpurol.2005.05.008
Desmoplastic small round cell tumor (DSRCT) is a rare pathologic entity that most frequently affects the peritoneal cavity and presents in adolescent boys. It often presents at an advanced stage and has a generally poor prognosis. We present an unusual case of DSRCT of the kidney presenting in a young girl. This is the first report of DSRCT of the kidney in the pediatric population, and it suggests that this condition should be a diagnostic consideration when assessing a child with a kidney mass.
Article: Unusual intra-abdominal tumor[Show abstract] [Hide abstract]
ABSTRACT: Desmoplastic small round cell tumor is a rare poorly differentiated, aggressive neoplasm that most frequently affects the abdominal cavity. The diagnosis is often difficult requiring morphological and immunohistochemical study. The purpose of our study is to clarify the anatomoclinical aspects of this rare tumor and to discuss the approach of the diagnosis and the prognosis. A 18-year old teenager had presented an enormous intra-abdominal mass. The radiologic findings showed a mass for 20 cm in diameter repulsing the liver and the right kidney. A surgical biopsy was performed. Histopathologic examination demonstrated a proliferation of small round cells surrounded by a desmoplastic stroma. Immunohistochemical staining was positive for pancytokeratin (KL1), epithelial membrane antigen (EMA), vimentin, neuron specific enolase (NSE) and desmine; thus, the diagnosis of a desmoplastic small round cell tumor was made. The patient received a first chemotherapy followed by a maximal surgical reduction. A local recurrence and bone metastasis occurred and the patient eventually died 18 months after the diagnosis from hepatic failure. The diagnosis of desmoplastic small round cell tumor may be suspected in young patient presenting an intra-abdominal small round cell tumor. The treatment is multidisciplinary and its prognosis is poor.
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ABSTRACT: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm usually present with the widespread abdominal serosal involvement and affects mainly adolescents and young adults. When presenting within visceral organs, as kidney, the diagnosis of DSRCT imposes significant difficulties. We present a case of primary DSRCT of the kidney in a 10-year-old boy mimicking clinically and pathologically Wilms tumor. The tumor showed morphologic and immunohistochemical features of DSRCT and the presence of the Ewing sarcoma and Wilm tumor 1 fusion transcripts resulting from the t(11;22) (p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of Wilm tumor and other small blue-round cell tumors of the kidney.
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ABSTRACT: A desmoplastic small round cell tumor (DSRCT) is a rare, aggressive mesenchymal neoplasm. Although a DSRCT can develop at various sites, the intra-abdominal site is the most common location. These tumors are found most commonly among young adolescents and the prognosis is extremely poor. Multimodal treatment with surgery, chemotherapy and radiotherapy is very important for these rare cases, and this treatment can improve patient survival. In this report, we describe the case of an 8-year-old boy diagnosed with DSRCT located in the retroperitoneal space. The patient has undergone surgical resection and adjuvant chemoradiation therapy, and is currently alive without disease recurrence.
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