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Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors?

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Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors? / Vokuhl, Christian; Oyen, Florian; Häberle, Beate; von Schweinitz, Dietrich; Schneppenheim, Reinhard; Leuschner, Ivo.

In: GENE CHROMOSOME CANC, Vol. 55, No. 12, 12.2016, p. 925-931.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Vokuhl, C, Oyen, F, Häberle, B, von Schweinitz, D, Schneppenheim, R & Leuschner, I 2016, 'Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors?', GENE CHROMOSOME CANC, vol. 55, no. 12, pp. 925-931. https://doi.org/10.1002/gcc.22390

APA

Vokuhl, C., Oyen, F., Häberle, B., von Schweinitz, D., Schneppenheim, R., & Leuschner, I. (2016). Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors? GENE CHROMOSOME CANC, 55(12), 925-931. https://doi.org/10.1002/gcc.22390

Vancouver

Vokuhl C, Oyen F, Häberle B, von Schweinitz D, Schneppenheim R, Leuschner I. Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors? GENE CHROMOSOME CANC. 2016 Dec;55(12):925-931. https://doi.org/10.1002/gcc.22390

Bibtex

@article{9362fdddf61749bdac93afca5247e5f4,
title = "Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors?",
abstract = "Small cell undifferentiated (SCUD) hepatoblastoma is a rare variant of hepatoblastoma with poor outcome and loss of INI1 expression, sharing this with malignant rhabdoid tumors (MRT). We studied all tumors from the files of the Kiel Pediatric Tumor Registry (KTR) with the initial diagnosis of SCUD and MRT. After re-review, we performed immunistochemistry, fluorescence in situ hybridization, and multiplex ligation dependent probe amplification for loss of expression and deletion of INI1/SMARCB1 in 23 tumors. Morphologically, 12 of the tumors had a small cell morphology, 9 showed the typical picture of MRT, and 2 were composed of both small cells and rhabdoid cells. All but 1 of the 23 tumors showed loss of INI1 protein expression by immunohistochemistry. Nineteen of the INI1 negative tumors were analyzed by FISH technique and all showed a deletion of the INI1/SMARCB1 gene (17 homozygous deletions, 2 heterozygous deletions). We investigated 14 of these cases by multiplex ligation dependent probe amplification and verified the deletions in all cases. In conclusion, we postulate that SCUD hepatoblastoma is not a hepatoblastoma but represents a malignant rhabdoid tumor of the liver. {\textcopyright} 2016 Wiley Periodicals, Inc.",
author = "Christian Vokuhl and Florian Oyen and Beate H{\"a}berle and {von Schweinitz}, Dietrich and Reinhard Schneppenheim and Ivo Leuschner",
note = "{\textcopyright} 2016 Wiley Periodicals, Inc.",
year = "2016",
month = dec,
doi = "10.1002/gcc.22390",
language = "English",
volume = "55",
pages = "925--931",
journal = "GENE CHROMOSOME CANC",
issn = "1045-2257",
publisher = "Wiley-Liss Inc.",
number = "12",

}

RIS

TY - JOUR

T1 - Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors?

AU - Vokuhl, Christian

AU - Oyen, Florian

AU - Häberle, Beate

AU - von Schweinitz, Dietrich

AU - Schneppenheim, Reinhard

AU - Leuschner, Ivo

N1 - © 2016 Wiley Periodicals, Inc.

PY - 2016/12

Y1 - 2016/12

N2 - Small cell undifferentiated (SCUD) hepatoblastoma is a rare variant of hepatoblastoma with poor outcome and loss of INI1 expression, sharing this with malignant rhabdoid tumors (MRT). We studied all tumors from the files of the Kiel Pediatric Tumor Registry (KTR) with the initial diagnosis of SCUD and MRT. After re-review, we performed immunistochemistry, fluorescence in situ hybridization, and multiplex ligation dependent probe amplification for loss of expression and deletion of INI1/SMARCB1 in 23 tumors. Morphologically, 12 of the tumors had a small cell morphology, 9 showed the typical picture of MRT, and 2 were composed of both small cells and rhabdoid cells. All but 1 of the 23 tumors showed loss of INI1 protein expression by immunohistochemistry. Nineteen of the INI1 negative tumors were analyzed by FISH technique and all showed a deletion of the INI1/SMARCB1 gene (17 homozygous deletions, 2 heterozygous deletions). We investigated 14 of these cases by multiplex ligation dependent probe amplification and verified the deletions in all cases. In conclusion, we postulate that SCUD hepatoblastoma is not a hepatoblastoma but represents a malignant rhabdoid tumor of the liver. © 2016 Wiley Periodicals, Inc.

AB - Small cell undifferentiated (SCUD) hepatoblastoma is a rare variant of hepatoblastoma with poor outcome and loss of INI1 expression, sharing this with malignant rhabdoid tumors (MRT). We studied all tumors from the files of the Kiel Pediatric Tumor Registry (KTR) with the initial diagnosis of SCUD and MRT. After re-review, we performed immunistochemistry, fluorescence in situ hybridization, and multiplex ligation dependent probe amplification for loss of expression and deletion of INI1/SMARCB1 in 23 tumors. Morphologically, 12 of the tumors had a small cell morphology, 9 showed the typical picture of MRT, and 2 were composed of both small cells and rhabdoid cells. All but 1 of the 23 tumors showed loss of INI1 protein expression by immunohistochemistry. Nineteen of the INI1 negative tumors were analyzed by FISH technique and all showed a deletion of the INI1/SMARCB1 gene (17 homozygous deletions, 2 heterozygous deletions). We investigated 14 of these cases by multiplex ligation dependent probe amplification and verified the deletions in all cases. In conclusion, we postulate that SCUD hepatoblastoma is not a hepatoblastoma but represents a malignant rhabdoid tumor of the liver. © 2016 Wiley Periodicals, Inc.

U2 - 10.1002/gcc.22390

DO - 10.1002/gcc.22390

M3 - SCORING: Journal article

C2 - 27356182

VL - 55

SP - 925

EP - 931

JO - GENE CHROMOSOME CANC

JF - GENE CHROMOSOME CANC

SN - 1045-2257

IS - 12

ER -