desmoplastic small round-cell tumour

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desmoplastic small round-cell tumour

An aggressive, poorly-differentiated malignancy that often arises on serosal surfaces—peritoneum, tunica vaginalis, pleura—as well as in the CNS and lymph nodes of younger, more commonly male, patients.
 
Clinical findings
Abdominal distention, mass, and pain; back pain; gastrointestinal obstruction; anorexia; ascites; anaemia; cachexia.
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References in periodicals archive ?
Study population included any patient who received treatment at CHAM with histologic diagnosis of DSRCT. Data were collected on patients' treatment regimens, disease-free survival (DFS) rate, and OS rate.
Inclusion criteria consisted of those patients with histologically confirmed diagnosis of DSRCT who received [greater than or equal to] 1 treatment at CHAM between the years 1996 and 2013.
Published reports of 1-2 cases were felt to represent a positive publication bias; thus, a study was only included if it included [greater than or equal to] 3 cases of histologically confirmed DSRCT. Studies that contained general descriptions of patient populations but did not individually identify patients and their respective disease course (namely, last known event and time to last known event) were excluded.
In advanced cases, abdominopelvic DSRCT can develop into bulky and multiple masses that displace the neighboring organs.
Proper consensus about treatment has not yet been established, and the treatment of DSRCT remains a clinical challenge and lacks standard treatment modalities.
Aggressiveness of DSRCT may add a surgical burden and the impact of surgical resection upon survival remains unclear; in our series, patients who underwent complete surgical excision seem to provide a better survival.
This is the first SEER database analysis evaluating both incidence and survival data among patients with DSRCT. The majority of published data on DSRCT are single institution studies analyzing small numbers of cases given heterogeneous treatment.
Whether such differences influence outcome in DSRCT or whether there are differences in biology of this tumor between various ethnic groups remains unknown.
In a study of 65 patients, FDG uptake was seen in all primary intra-abdominal and pelvic tumors [22] and accurately detected 97% of all DSRCT lesions with sensitivity, specificity, positive, and negative predictive values of 96%, 99%, 98%, and 97%, respectively.
Fine-needle aspiration specimens, although commonly employed, are not adequate during the workup of DSRCT due to issues with low cellularity of the sample, necrosis, and predominantly a desmoplastic reaction.
Of the aforementioned diagnostic considerations for the current case, alveolar rhabdomyosarcoma, extraskeletal myxoid chondrosarcoma, epithelioid sarcoma, myoepithelial tumors, and DSRCT may all demonstrate polyphenotypic immunohistochemical profiles.
Originally detected through cytogenetic analyses, the reciprocal translocation t(11;22) (p13;q12) that results in the fusion of EWS and WT1 genes has been a consistently reported feature of DSRCT. (3-5,12) Although the function of the EWS-WT1 gene fusion and its role in tumorigenesis remains unclear, detection of the chimeric transcripts by reverse transcriptase/polymerase chain reaction provides a reliable means to accurately differentiate DSRCT from its numerous diagnostic mimics.