Desmoplastic Small Round Cell Tumors in a Young Man

Hirano, Genryu; Irie, Makoto; Nakashima, Yuta; Shakado, Satoshi; Sohda, Tetsuro; Tanaka, Toshihiro; Takamatsu, Yasushi; Aoki, Mikiko; Tamura, Kazuo; Nabeshima, Kazuki; Sakisaka, Shotaro

doi:10.2169/internalmedicine.52.0431

Cited by 5 publications

(6 citation statements)

References 13 publications

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“…Aggressive behavior with local and distal spread has frequently been reported in the literature. Currently, the immunohistochemical diagnosis of this tumor is well established, as well as the specific chromosomal translocation t(11;22)(p13;q12) EWS gene juxtaposed with the tumor suppressor gene WT1 [1, 8]. Yet, despite the advances and new isolated therapies with complex treatment plans, the prognosis of patients with desmoplastic tumors remains poor, especially in cases with distant metastases, and new therapeutic options need to be studied [10].…”

Section: Discussionmentioning

confidence: 99%

Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

et al. 2019

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Introduction Desmoplastic small round cell tumor is an extremely rare and aggressive cancer that affects mainly adolescents and young adults. Despite multiple therapeutic strategies, most patients have resistant disease with very poor survival rates. Case presentation We present a case of a 10-year-old Caucasian boy with a desmoplastic small round cell tumor refractory to conventional treatment who exhibited a good response to alternative treatment. With use of irinotecan and vincristine in association with radiation therapy, a reduction of 96.9% of the dimensions of the target lesions compared with the initial image was observed. Conclusion This chemotherapy regimen, in association with radiation therapy, demonstrated efficacy for refractory desmoplastic small round cell tumor in our patient, and it is cost-effective.

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Section: Discussionmentioning

confidence: 99%

Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

et al. 2019

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“…These include tumors such as Hodgkin's lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma, Wilms tumor and peripheral primitive neuroectodermal tumor [2]. The origin of these cells is still unknown, but their predilection for involvement of serosa indicates the possibility of mesothelial origin [7,8]. Several other cases were subsequently described in the international literature.…”

Section: Discussionmentioning

confidence: 99%

“…Several other cases were subsequently described in the international literature. It affects mainly adolescents and young adults, with a 4:1 ratio of men to women [6,7]. In most cases, patients with desmoplastic tumors present with advanced disease.…”

Section: Discussionmentioning

confidence: 99%

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Metastasized Intraabdominal Desmoplastic Small Round Cell Tumor

Costa¹,

Lourenço²,

Guimarães³

et al. 2019

JCR

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Background:The desmoplastic small round cell tumor is a rare disease with poor prognosis. It affects predominantly young males with preferential involvement of the abdominal cavity. Case Report: A 33 year old male presented with right hypochondrium pain, associated with anorexia and weight loss for last 2 months. The imaging studies identified an abdominal mass, without hollow organ association, and multiple liver metastases. The anatomopathological study of the tissue obtained with the percutaneous abdominal mass biopsy confirmed the presence of desmoplastic small round and blue tumor cells. The immunohistochemical and cytogenetic study confirmed the diagnosis. It was proposed for systemic chemotherapy as primary treatment. Conclusion: We intend to emphasize that although this tumor is rare, it should be included in the differential diagnosis of intra-abdominal solid lesions. It also includes a review of the epidemiology, clinical manifestations, as well molecular and pathologic features.

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“…A further published case was located in the perihepatic space (Chouli et al 2005). In many situations of the liver involved, metastatic disease from primary tumor outside the liver is present (Arra et al 2013;Hirano et al 2013). Among 62 patients with primary intraabdominal disease, 40 % presented liver metastases (Arra et al 2013).…”

Section: Dsrct Of the Livermentioning

confidence: 99%

Undifferentiated Small Cell Tumors of the Hepatobiliary Tract

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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A small group of primary hepatobiliary tumors is characterized by the presence of morphologically undifferentiated small cells. These small cell tumors (SCTs), which histologically appear as "small cell blue tumors" due to the dense crowding of nuclei together with poorly developed cytoplasm, have several cell lineage courses that can only be identified by means of immunohistochemistry and molecular methods. One type of hepatic SCT is a member of the complex family of neuroectodermal tumors that predominantly occur in the skeleton and soft tissues of adolescents and young adults (primitive neuroectodermal tumors or PNETs). Hepatic PNETs are very rare neoplasms with an aggressive course, and the tumor also develops in the biliary tract. Other hepatobiliary SCTs include desmoplastic small round cell tumor, NUT midline carcinoma, and hepatic neuroblastoma and its variants. As all hepatobiliary SCTs are very rare neoplasms, they have to be distinguished from the more common metastatic lesions of these tumors.

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Desmoplastic Small Round Cell Tumors in a Young Man

Cited by 5 publications

References 13 publications

Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

Metastasized Intraabdominal Desmoplastic Small Round Cell Tumor

Undifferentiated Small Cell Tumors of the Hepatobiliary Tract

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