Cancer Research and Treatment

Original Article

Extra-cranial Malignant Rhabdoid Tumor in Children: A Single Institute Experience: Che Ry Hong, Hyoung Jin Kang, Hee Young Ju, Ji Won Lee, Hyery Kim, Sung-Hye Park, Il Han Kim, Kyung Duk Park, Hee Young Shin; Cancer Res Treat. 2015;47(4):889-896. Published online January 2, 2015; DOI: https://doi.org/10.4143/crt.2013.176

Purpose
Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. Due to its extreme rarity, most of the available data are based on retrospective case series. To add to the current knowledge of this disease, we reviewed the patients treated for extra-cranial MRT in our institute. Materials and Methods A retrospective medical record review was conducted on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children’s Hospital between January 2003 and May 2013. Results Eleven patients (7 boys, 4 girls) were diagnosed with extra-cranial MRT at a median age of 9 months old. INI1 staining was important in the pathological confirmation. Six patients (55%) had renal MRT and five (45%) had soft tissue MRT. Five patients (45%) had metastases at diagnosis. All patients underwent chemotherapy, eight patients (73%) underwent surgery, six patients (55%) received therapeutic radiotherapy, and four patients (36%) underwent high dose chemotherapy with autologous stem cell rescue (HDCT/ASCR) with melphalan, etoposide, and carboplatin. Five patients (45%) died of disease following progression (n=3) or relapse (n=2), however, there was no treatment related mortality. The overall survival of the cohort was 53.0% and the event-free survival was 54.5% with a median follow-up duration of 17.8 months (range, 2.3 to 112.3 months). Conclusion Extra-cranial MRT is still a highly aggressive tumor in young children. However, the improved survival of our cohort is promising and HDCT/ASCR with melphalan, etoposide, and carboplatin may be a promising treatment option.

Citations

Citations to this article as recorded by

Extracranial malignant rhabdoid tumors in children: high mortality even with the help of an aggressive clinical approach
Siqi Xie, Yuanyuan Fang, Yingying Yang, Lan Liu, Jianxi Bai, Sheng Lin, Bing Zhang, Yifan Fang
European Journal of Pediatrics.2023; 183(2): 557. CrossRef
Infantile Extracranial Rhabdoid Tumor of the Scalp
Sura Al Rawabdeh, Deifallah Alsharari, Hayat Khasawneh, Ola M. Al Waqfi, Qamar Yaser Malabeh, Hiathem Abu Alhaija, Raed Mohammad Aljubour, Hamzeh M. Alkhawaldeh, Pablo Fernandez Pe as
Case Reports in Medicine.2021; 2021: 1. CrossRef
Pharmacokinetics of high-dose carboplatin in children undergoing high-dose chemotherapy and autologous stem cell transplantation with BSA-based dosing
Che Ry Hong, Hyoung Jin Kang, Seol Ju Moon, Jaeseong Oh, Kyung Taek Hong, Jung Yoon Choi, Kyung-Sang Yu, Hee Young Shin
Bone Marrow Transplantation.2020; 55(1): 137. CrossRef
Successful Treatment of Extra‐Renal Noncerebral Rhabdoid Tumors with VIDE
Naoko Yasui, Akihiko Yoshida, Eisuke Kobayashi, Fumihiko Nakatani, Hiroshi Kawamoto
Pediatric Blood & Cancer.2016; 63(2): 352. CrossRef

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Case Report

Clear Cell Carcinoma of the Pancreas -A Case Report and Review of the Literature-: Hui-Young Lee, Dong-Gyu Lee, Kwangjin Chun, Seungkoo Lee, Seo-Young Song; Cancer Res Treat. 2009;41(3):175-181. Published online September 29, 2009; DOI: https://doi.org/10.4143/crt.2009.41.3.175

Abstract PDF PubReader ePub

Most of the malignant neoplasms of the pancreas demonstrate features that are consistent with adenocarcinoma. According to the WHO classification, primary clear cell carcinoma of the pancreas is rare and it is classified as a "miscellaneous" carcinoma. In addition, there is not an adequate systematic overview that can demonstrate its true existence as a definable entity. We report here on an unusual case of primary pancreatic clear cell carcinoma, which is the first such reported case in Korea. A 66 year old woman presented with abdominal pain and significant weight loss over the previous three weeks. On the abdominal computed tomography (CT), we detected an abdominal mass involving the pancreas tail and liver, and clear cell carcinoma with rhabdoid feature was seen on the histologic evaluation. The tumor cells showed well defined cell membranes, clear cytoplasm and prominent cell boundaries. The immunohistochemical stains showed positive reactions to antibodies against pan-cytokeratin, cytokeratin 7, carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). On the other hand, there was a negative reaction for cytokeratin 20, chromogranin, synaptophysin, smooth muscle actin and HMB-45. She was diagnosed with a primary pancreatic clear cell carcinoma with hepatic metastasis and she received palliative gemcitabine chemotherapy. The patient died one month later of pancreatic cancer progression.

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