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Desmoplastic Small Round Cell Tumor (desmoplastic + small_round_cell_tumor)
Selected AbstractsDesmoplastic small round cell tumor: Using FISH as an ancillary technique to support cytologic diagnosis in an unusual caseDIAGNOSTIC CYTOPATHOLOGY, Issue 8 2007Michael S. Waugh M.D. Abstract Desmoplastic small round cell tumor is a rare and aggressive neoplasm that predominantly affects young males. In almost all cases, a reciprocal translocation is present resulting in the fusion of the Ewing sarcoma gene with the Wilms' tumor gene. Here we describe an unusual case occurring in a 59-year-old male, in which fluorescence in situ hybridization (FISH) was used in conjunction with immunohistochemical studies to confirm the diagnosis. To our knowledge, this is the first reported case of using FISH as an ancillary technique to confirm the cytologic diagnosis of this tumor. Diagn. Cytopathol. 2007;35:516,520. © 2007 Wiley-Liss, Inc. [source] Desmoplastic small round cell tumor in childhood: The St. Jude Children's Research Hospital experiencePEDIATRIC BLOOD & CANCER, Issue 3 2007Raya Saab MD Abstract Background Desmoplastic small round cell tumor (DSRCT) is a rare, primarily intra-abdominal tumor that has a poor outcome with current therapies. Procedure We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of 11 pediatric patients with DSRCT at our institution. Results The cohort included 1 female and 10 male patients. Median age at diagnosis was 14 years (range 5,21 years). In eight (73%) patients, the primary tumor was abdominal or pelvic, and in one patient each, it was submental, mediastinal, and paratesticular. Nine (82%) patients had metastatic disease. All tumors showed polyphenotypic differentiation by immunohistochemistry. The EWS-WT1 transcript was detected in six of seven tumors tested. One tumor showed rhabdomyoblastic differentiation after therapy. All patients received chemotherapy; eight underwent surgical resection, seven received primary site radiation, and four received myeloablative chemotherapy with stem-cell support. Three (27%) patients are alive 23 months, 8 years, and 10 years from diagnosis. Two died of treatment-related toxicity, six died of disease. None of the patients in whom surgery and initial chemotherapy failed to induce complete remission survived. Conclusions DSRCT is an aggressive malignancy that does not respond well to contemporary treatments, and patients who do not enter complete remission after initial chemotherapy and surgery appear to have a particularly dismal outcome. Patients with localized extra-abdominal disease have a better prognosis, most likely due to increased feasibility of resection. Better understanding of molecular and genetic mechanisms of tumorigenesis and treatment-related changes may contribute to development of more effective therapy for DSRCT. Pediatr Blood Cancer 2007;49:274,279. © 2006 Wiley-Liss, Inc. [source] Desmoplastic round cell tumor of childhood: Can cytology with immunocytochemistry serve as an alternative for tissue diagnosis?DIAGNOSTIC CYTOPATHOLOGY, Issue 6 2005Dr Brijal Dave M.D. Abstract There are limited reports on the cytology of desmoplastic small round cell tumors (DSRCT). Fine needle aspiration biopsy (FNAB) findings in seven aspirates from four cases of histologically and immunohistochemically confirmed cases were analyzed with the main intention of ascertaining if cytological diagnosis of DSRCT is possible. Also assessed were the immunocytochemistry(ICC) findings in these cases. The basic cytological impression was that of a cohesive small round cell tumor. Nuclei showed granular chromatin with grooves, nuclear molding and inconspicuous nucleoli. Stromal fragments were noted in all four cases. In two cases, awareness of cytological features in the appropriate clinical context led to a suggestion of the diagnosis of DSRCT on cytology itself. ICC on destained smears showed positivity for cytokeratin, epithelial membrane antigen (EMA), desmin and WT-1 in two cases. In conclusion, given the right clinical setting, a cytological diagnosis of DSRCT is plausible and in conjunction with ICC may help in documenting the polyphenotypic nature and thereby confirming the diagnosis. Diagn. Cytopathol. 2005;32:330,335. © 2005 Wiley-Liss, Inc. [source] | ||||||||||