			Home About us Contact

Small Round Cell Tumor (small + round_cell_tumor)

Distribution by Scientific Domains

Medical Sciences	100%

Kinds of Small Round Cell Tumor

desmoplastic small round cell tumor

Selected Abstracts

Desmoplastic small round cell tumor: Using FISH as an ancillary technique to support cytologic diagnosis in an unusual case

DIAGNOSTIC CYTOPATHOLOGY, Issue 8 2007
Michael 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 round cell tumor of childhood: Can cytology with immunocytochemistry serve as an alternative for tissue diagnosis?

DIAGNOSTIC CYTOPATHOLOGY, Issue 6 2005
Dr 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]

Desmoplastic small round cell tumor in childhood: The St. Jude Children's Research Hospital experience

PEDIATRIC BLOOD & CANCER, Issue 3 2007
Raya 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?

The orphan nuclear receptor DAX1 is up-regulated by the EWS/FLI1 oncoprotein and is highly expressed in Ewing tumors

INTERNATIONAL JOURNAL OF CANCER, Issue 6 2006
Marta Mendiola
Abstract The Ewing family of tumors harbors chromosomal translocations that join the N-terminal region of the EWS gene with the C-terminal region of several transcription factors of the ETS family, mainly FLI1, resulting in chimeric transcription factors that play a pivotal role in the pathogenesis of Ewing tumors. To identify downstream targets of the EWS/FLI1 fusion protein, we established 293 cells expressing constitutively either the chimeric EWS/FLI1 or wild type FLI1 proteins and used cDNA arrays to identify genes differentially regulated by EWS/FLI1. DAX1 (NR0B1), an unusual orphan nuclear receptor involved in gonadal development, sex determination and steroidogenesis, showed a consistent up-regulation by EWS/FLI1 oncoprotein, but not by wild type FLI1. Specific induction of DAX1 by EWS/FLI1 was confirmed in two independent cell systems with inducible expression of EWS/FLI1. We also analyzed the expression of DAX1 in Ewing tumors and derived cell lines, as well as in other nonrelated small round cell tumors. DAX1 was expressed in all Ewing tumor specimens analyzed, and in seven out of eight Ewing tumor cell lines, but not in any neuroblastoma or embryonal rhabdomyosarcoma. Furthermore, silencing of EWS/FLI1 by RNA interference in a Ewing tumor cell line markedly reduced the levels of DAX1 mRNA and protein, confirming that DAX1 up-regulation is dependent upon EWS/FLI1 expression. The high levels of DAX1 found in Ewing tumors and its potent transcriptional repressor activity suggest that the oncogenic effect of EWS/FLI1 may be mediated, at least in part, by the up-regulation of DAX1 expression. © 2005 Wiley-Liss, Inc. [source]

Primary Cutaneous Ewing's Sarcoma

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005
K.S. Draft
A 57-year-old woman presented with a three-week history of an erythematous nodule on her palm. The clinical diagnosis was pyogenic granuloma. Histology revealed a circumscribed dermal nodule of uniform, primitive round cells with numerous mitotic figures and apoptopic cells. The tumor cells showed strong membranous immunoreactivity for CD99 and nuclear immunoreactivity for Fli-1. The tumor cells were negative for S-100 protein, cytokeratin AE1/3, cytokeratin 20, chromogranin, synaptophysin, and actin. The diagnosis of Ewing's sarcoma was made. CT scans showed no evidence of an osseous or deep soft tissue primary tumor or metastatic disease. The tumor was excised with 1 cm margins, and the patient received six courses of adjuvant chemotherapy. One year after diagnosis, the patient is alive without evidence of disease. Extraosseous Ewing's sarcoma (EES) rarely presents as a primary skin tumor and should be considered in the differential diagnosis of small round cell tumors involving the skin. It is important to distinguish primary cutaneous EES from secondary involvement of the skin by ES, as primary cutaneous EES has a more indolent course compared to classic EES or osseous ES. Immunohistochemical stains for CD99 and Fli-1 are useful markers to confirm the diagnosis of cutaneous ES. [source]