Diagnosis of desmoplastic small‐round‐cell tumor by cytogenetic analysis: a case report

Wakahashi, Senn; Sudo, Tamotsu; Ichida, Kotaro; Sugita, Shintaro; Hasegawa, Tadashi; Nagao, Shoji; Yamaguchi, Satoshi; Sakuma, Takashi; Yamada, Hideto

doi:10.1002/ccr3.558

Cited by 3 publications

(2 citation statements)

References 16 publications

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“…No specific tumor markers have been identified for the diagnosis of IDSRCT, except elevated serum CA125 and NSE levels in some patients (3,57,71). For imaging information, ultrasound usually shows lobulated peritoneal masses with variable echogenicity, and dystrophic intratumoral calcification is discovered in 20% of cases (75).…”

Section: Diagnosismentioning

confidence: 99%

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

et al. 2021

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Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor prognosis. The mechanism underlying the development of this neoplasm remains elusive, but all cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 gene fusion. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens or laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. FISH or reverse transcription polymerase chain reaction detecting EWS-WT1 fusion can be performed to assist in molecular confirmation. There is no standard of care for patients with IDSRCT currently, and majority of newly diagnosed patients received the aggressive therapy, which includes >90% resection of surgical debulking, high-dose alkylator-based chemotherapy, and radiotherapy. More recently, targeted therapy has been increasingly administered to recurrent IDSRCT patients and has been associated with improved survival in clinical conditions. Immunotherapy as a possible therapeutic strategy is being explored in patients with IDSRCT. In this review, we summarize currently available knowledge regarding the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT to assist oncologists in comprehensively recognizing and accurately treating this malignancy.

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Section: Diagnosismentioning

confidence: 99%

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

et al. 2021

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“…Desmoplastic smallround-cell tumor arises from mesenchymal cells of the abdominopelvic peritoneum. 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].…”

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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Desmoplastic Small Round Cell Tumors: A review with focus on clinical management and therapeutic options

Hendricks¹,

Boerner

Germer

et al. 2021

Cancer Treatment Reviews

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Diagnosis of desmoplastic small‐round‐cell tumor by cytogenetic analysis: a case report

Cited by 3 publications

References 16 publications

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

Primary desmoplastic small-round-cell tumor of the ovary

Desmoplastic Small Round Cell Tumors: A review with focus on clinical management and therapeutic options

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