Undifferentiated Malignant Epithelial Tumors Involving Serosal Surfaces of Scrotum and Abdomen in Young Males

Ia, Sesterhenn; Davis, Christine; Mostofi, F. K.

doi:10.1016/s0022-5347(17)75592-9

Cited by 22 publications

(15 citation statements)

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“…Desmoplastic small round cell tumour (DSRCT) has been described by Sesterhenn et al [1] and Gerald and Rosai [2]. It is a rare neoplasm of small round cells located in the abdomen, mainly affecting male adolescents and young adults.…”

Section: Introductionmentioning

confidence: 99%

Intraabdominal desmoplastic small round cell tumour

2005

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Desmoplastic small round cell tumour (DSRCT) is an extremely rare neoplasm. Adolescent males and young adults are most frequently affected. It is highly malignant, with only 29% of patients surviving up to 3 years. This paper documents two cases, one of which, at 4 years old, is the second youngest case documented. Case 1, a 10-year old boy, presented with a 20-day history of choluria, acholia, asthenia, anorexia, and right abdominal pain. Laboratory values were altered, and imaging showed multiples masses in the liver and retroperitoneum. A minilaparotomy was carried out, and a biopsy showed a stage III DSRCT. He was treated with chemotherapy but died of hepatic failure. Case 2, a 4-year-old boy, presented with a 2-month history of abdominal distension. Several hard masses were palpated in the abdomen, and a right inguinal mass that compressed the right testis was observed. Biopsy of the inguinal tumour showed a DSRCT. After treatment with chemotherapy, two operations were carried out to resect different intraabdominal masses. The patient died with peritoneal carcinomatosis 2 months after the last operation. The first patient died due to the advanced stage of the disease, and the second died after chemotherapy, peripheral blood stem transplantation, and multiple operations. The occurrence of this type of tumour in the paediatric age group as well as its high malignancy is noteworthy. Until more effective forms of treatment are found, we recommend treatment with chemotherapy, surgery, and radiotherapy, with close monitoring of the patient.

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Section: Introductionmentioning

confidence: 99%

Intraabdominal desmoplastic small round cell tumour

2005

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“…Multiple EWS exons are involved in forming the EWS/FLI1 hybrid transcript expressed in Ewing's tumors and PNETs, although biological differences among the various chimeric proteins have not been established . Recently, another EWS fusion partner has been described in desmoplastic small round cell tumor (DSRCT), a primitive sarcoma with distinctive clinicopathologic and cytogenetic characteristics (Sesterhenn et al, 1987;Gonzalez-Crusssi, 1990;Gerald et al, 1991). DSRCT usually consists of an intra-abdominal mass in pediatric and adolescent patients, with a preponderance of male cases (Parkash et al, 1995;Tison et al, 1996).…”

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LINE-I element insertion at the t(11;22) translocation breakpoint of a desmoplastic small round cell tumor

Nau

Zucman‐Rossi

et al. 1997

Genes Chromosom. Cancer

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“…Tipik olarak ilk abdominal kavitede özellikle de pelviste prezente olur ve köken aldığı viseral bir organın bulunmaması ve periton yüzeyinde değişik boyutta çok sayıda nodül içermesi ile karakterizedir (5,8,9). Kadın-larda DKYHT ile over tutulumu arasında bir ilişkinin bulunduğu ileri sürülmektedir (10).…”

Section: Discussionunclassified

Primer Over Tümörü Gibi Prezentasyon Gösteren Dezmoplastik Küçük Yuvarlak Hücreli Tümör

Ebru;PERÇİNEL¹

2008

Ankara Üniversitesi Tıp Fakültesi Mecmuası

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Undifferentiated Malignant Epithelial Tumors Involving Serosal Surfaces of Scrotum and Abdomen in Young Males

Cited by 22 publications

References 0 publications

Intraabdominal desmoplastic small round cell tumour

Intraabdominal desmoplastic small round cell tumour

LINE-I element insertion at the t(11;22) translocation breakpoint of a desmoplastic small round cell tumor

Primer Over Tümörü Gibi Prezentasyon Gösteren Dezmoplastik Küçük Yuvarlak Hücreli Tümör

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