A Case of Desmoplastic Small Round Cell Tumor Diagnosed in a Young Female Patient

Kim, Jiwon; Park, Jin Hyun; Cho, Hyeon Jin; Kwon, Jihoon; Koh, Youngil; Kim, Su-Jung; Kim, Se Hyung; Lee, Se‐Hoon; Im, Seock‐Ah; Kim, Yong‐Tae; Kim, Woo Ho

doi:10.4143/crt.2009.41.4.233

Cited by 11 publications

(5 citation statements)

References 12 publications

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“…As seen in table 3, 5-year survival rates are the worst among the 3 small round-cell sarcomas in previous reports. In accordance with the previous reports [14], the median survival of DSRCT was only 17.7 months with a range of 4.3–31.1 months in our series. All 3 patients had metastatic disease at the time of diagnosis, and were administered combination chemotherapy without surgical resection.…”

Section: Discussionsupporting

confidence: 93%

Analysis of Prognostic Factors of Pediatric-Type Sarcomas in Adult Patients

Ahn¹,

Uhm²,

Lee³

et al. 2011

Oncology

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Objective: Pediatric-type sarcomas such as Ewing’s sarcoma (EWS)/primitive neuroectodermal tumor family and rhabdomyosarcoma are relatively uncommon in adult patients. Optimal treatment strategies for this population and prognosis in adult patients compared with pediatric patients remain controversial. Methods: We retrospectively reviewed pediatric-type sarcoma patients older than 15 years at a single institution. Results: A total of 84 consecutive patients between 1995 and 2009 were identified at the Samsung Medical Center, Seoul, Korea. Median age was 30 years with a range of 15–74 years. Forty-seven patients (56.0%) were diagnosed with Ewing’s sarcoma/primitive neuroectodermal tumor family, 34 (40.5%) with rhabdomyosarcoma and 3 (3.6%) with desmoplastic round-cell tumor. Median follow-up duration was 5.9 years. Median overall survival for all patients was 33.1 months (95% CI 13.5–52.7) and median event-free survival for all patients was 14.4 months (95% CI 5.9–22.9 months). Multivariate analysis revealed that localized disease was a significant independent prognostic factor for longer overall survival (hazard ratio 0.30, 95% CI 0.14–0.66, p = 0.003), and favorable primary tumor sites were associated with longer event-free survival (hazard ratio 0.33, 95% CI 0.11–0.98, p = 0.045). Conclusion: We identified the prognostic variables which may facilitate risk-adapted therapies for this rare adult sarcoma group, which should be further investigated.

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Section: Discussionsupporting

confidence: 93%

Analysis of Prognostic Factors of Pediatric-Type Sarcomas in Adult Patients

Ahn¹,

Uhm²,

Lee³

et al. 2011

Oncology

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“…They were arranged in variable histologic patterns and formed tubules or nests encircled by dense desmoplastic fibro-myxoid stroma. The diagnosis of this tumor is challenging in children, even with known polyphenotypic immunohistochemistry markers, because it does not have a specific radiological image and it may show various types of differentiation such as epithelial, mesenchymal, or neuronal [ 11 ]. Characteristically, DSRCT expresses desmin in a dot-like pattern, as in our case, and nuclear positivity for WT-1 along with immunoreactivity for an epithelial component favors DSRCT over other small round blue cell tumors.…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor of Pancreatic Origin in a Young Child: A Case Report and Review of Literature

Saleh

Al-Maghrabi

et al. 2020

Am J Case Rep

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“…The gene fusion between Ewing sarcoma (EWS) and WT1 genes resulting in the characteristic translocation t(11;22)(p13;q12), is the unique molecular hallmark and no other genetic factor has been linked to this aggressive tumor [15]. Horseshoe kidney is the most common fusion anomaly of the kidneys accounting for 0.25% of the population [16]. One of the major concerns that affected the treatment decision in the present case is the encountered technical difficulty of retroperitoneal lymph node dissection.…”

Section: Discussionmentioning

confidence: 99%

Primary desmoplastic small-round-cell tumor of the ovary

Atef

Gaballa

Zuhdy

et al. 2019

J Egypt Natl Canc Inst

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Background: Desmoplastic small-round-cell tumor (DSRCT) is an extremely rare and highly aggressive malignancy. It is of yet unclear origin, but it is assumed to be of a mesothelial origin based on its tendency for widespread metastasis in serosal linings. Case presentation: In this report, we describe a young female who presented with bilateral ovarian masses that mimicked the classic clinical picture of ovarian cancer. The patient had a cytoreductive surgery done in the form of total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic peritonectomy, low paraaortic and bilateral iliac lymphadenectomy. Postoperative course was smooth with no adverse events. The final pathology report revealed desmoplastic small-round-cell tumor. Afterwards, the patient was referred to medical oncologist to receive her adjuvant therapy. Conclusions: DSRCT is still an unknown disease to us given the limited number of cases and poor survival. Given the lack of clear guidelines, treatment is offered based on the best available evidence and the collaborative effort of a multidisciplinary team.

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A Case of Desmoplastic Small Round Cell Tumor Diagnosed in a Young Female Patient

Cited by 11 publications

References 12 publications

Analysis of Prognostic Factors of Pediatric-Type Sarcomas in Adult Patients

Analysis of Prognostic Factors of Pediatric-Type Sarcomas in Adult Patients

Desmoplastic Small Round Cell Tumor of Pancreatic Origin in a Young Child: A Case Report and Review of Literature

Primary desmoplastic small-round-cell tumor of the ovary

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