Long‐term remission of bilateral Wilms tumors that developed from premature separation of chromatids/mosaic variegated aneuploidy syndrome due to bilateral nephrectomy and peritoneal dialysis
We report a 38‐month‐old Japanese male with premature chromatid separation/mosaic variegated aneuploidy syndrome bearing biallelic BUB1B germline mutations who suffered from bilateral Wilms tumor. After right nephrectomy, dactinomycin monotherapy was administered for the left Wilms tumor; however, s...
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| Veröffentlicht in: | Pediatric blood & cancer 2019-08, Vol.66 (8), p.e27804-n/a |
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| container_title | Pediatric blood & cancer |
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| creator | Ochiai, Kayo Yamada, Ai Kimoto, Yasuhiro Imamura, Hideaki Ikeda, Toshio Matsukubo, Makoto Ieiri, Satoshi Moritake, Hiroshi |
| description | We report a 38‐month‐old Japanese male with premature chromatid separation/mosaic variegated aneuploidy syndrome bearing biallelic BUB1B germline mutations who suffered from bilateral Wilms tumor. After right nephrectomy, dactinomycin monotherapy was administered for the left Wilms tumor; however, severe adverse reaction prevented the patient from receiving further chemotherapy. Left nephrectomy was then performed without postoperative chemotherapy. The patient survived for 15 months after bilateral nephrectomy without peritoneal relapse, metastasis of Wilms tumor, or the occurrence of rhabdomyosarcoma and maintained a good quality of life while receiving peritoneal dialysis at home. |
| doi_str_mv | 10.1002/pbc.27804 |
| format | Article |
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After right nephrectomy, dactinomycin monotherapy was administered for the left Wilms tumor; however, severe adverse reaction prevented the patient from receiving further chemotherapy. Left nephrectomy was then performed without postoperative chemotherapy. The patient survived for 15 months after bilateral nephrectomy without peritoneal relapse, metastasis of Wilms tumor, or the occurrence of rhabdomyosarcoma and maintained a good quality of life while receiving peritoneal dialysis at home.</description><identifier>ISSN: 1545-5009</identifier><identifier>EISSN: 1545-5017</identifier><identifier>DOI: 10.1002/pbc.27804</identifier><identifier>PMID: 31081598</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Aneuploidy ; bilateral nephrectomy ; bilateral Wilms tumor ; Chemotherapy ; Child, Preschool ; Chromatids ; Chromatids - pathology ; Chromosome Disorders - complications ; Chromosome Disorders - pathology ; Chromosome Disorders - therapy ; Dactinomycin ; Hematology ; Humans ; Kidney Neoplasms - complications ; Kidney Neoplasms - pathology ; Kidney Neoplasms - therapy ; Male ; Metastases ; Mosaicism ; Nephrectomy ; Nephrectomy - mortality ; Oncology ; PCS/MVA syndrome ; Pediatrics ; Peritoneal dialysis ; Peritoneal Dialysis - mortality ; Peritoneum ; Prognosis ; Quality of Life ; Remission ; Remission Induction ; Rhabdomyosarcoma ; Survival Rate ; Tumors ; Wilms Tumor - complications ; Wilms Tumor - pathology ; Wilms Tumor - therapy</subject><ispartof>Pediatric blood & cancer, 2019-08, Vol.66 (8), p.e27804-n/a</ispartof><rights>2019 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3534-5268e0c959c496c432571cf557e9cb57d50ed31fd67adb97075d85f27e5e82bd3</citedby><cites>FETCH-LOGICAL-c3534-5268e0c959c496c432571cf557e9cb57d50ed31fd67adb97075d85f27e5e82bd3</cites><orcidid>0000-0001-5471-7646 ; 0000-0003-0055-9157</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fpbc.27804$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fpbc.27804$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31081598$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ochiai, Kayo</creatorcontrib><creatorcontrib>Yamada, Ai</creatorcontrib><creatorcontrib>Kimoto, Yasuhiro</creatorcontrib><creatorcontrib>Imamura, Hideaki</creatorcontrib><creatorcontrib>Ikeda, Toshio</creatorcontrib><creatorcontrib>Matsukubo, Makoto</creatorcontrib><creatorcontrib>Ieiri, Satoshi</creatorcontrib><creatorcontrib>Moritake, Hiroshi</creatorcontrib><title>Long‐term remission of bilateral Wilms tumors that developed from premature separation of chromatids/mosaic variegated aneuploidy syndrome due to bilateral nephrectomy and peritoneal dialysis</title><title>Pediatric blood & cancer</title><addtitle>Pediatr Blood Cancer</addtitle><description>We report a 38‐month‐old Japanese male with premature chromatid separation/mosaic variegated aneuploidy syndrome bearing biallelic BUB1B germline mutations who suffered from bilateral Wilms tumor. After right nephrectomy, dactinomycin monotherapy was administered for the left Wilms tumor; however, severe adverse reaction prevented the patient from receiving further chemotherapy. Left nephrectomy was then performed without postoperative chemotherapy. The patient survived for 15 months after bilateral nephrectomy without peritoneal relapse, metastasis of Wilms tumor, or the occurrence of rhabdomyosarcoma and maintained a good quality of life while receiving peritoneal dialysis at home.</description><subject>Aneuploidy</subject><subject>bilateral nephrectomy</subject><subject>bilateral Wilms tumor</subject><subject>Chemotherapy</subject><subject>Child, Preschool</subject><subject>Chromatids</subject><subject>Chromatids - pathology</subject><subject>Chromosome Disorders - complications</subject><subject>Chromosome Disorders - pathology</subject><subject>Chromosome Disorders - therapy</subject><subject>Dactinomycin</subject><subject>Hematology</subject><subject>Humans</subject><subject>Kidney Neoplasms - complications</subject><subject>Kidney Neoplasms - pathology</subject><subject>Kidney Neoplasms - therapy</subject><subject>Male</subject><subject>Metastases</subject><subject>Mosaicism</subject><subject>Nephrectomy</subject><subject>Nephrectomy - mortality</subject><subject>Oncology</subject><subject>PCS/MVA syndrome</subject><subject>Pediatrics</subject><subject>Peritoneal dialysis</subject><subject>Peritoneal Dialysis - mortality</subject><subject>Peritoneum</subject><subject>Prognosis</subject><subject>Quality of Life</subject><subject>Remission</subject><subject>Remission Induction</subject><subject>Rhabdomyosarcoma</subject><subject>Survival Rate</subject><subject>Tumors</subject><subject>Wilms Tumor - complications</subject><subject>Wilms Tumor - pathology</subject><subject>Wilms Tumor - therapy</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kc1u1DAUhSNERUthwQsgS2xgMR3_xHGypCMKlUaCBYhl5Ng3HVdOHGynKDseoa_Eq_AkXDrTCiGxurbvd861fYriBaNnjFK-njpzxlVNy0fFCZOlXEnK1OOHNW2Oi6cpXSNaUVk_KY4FozWTTX1S_NyG8erXj9sMcSARBpeSCyMJPemc13iqPfnq_JBInocQsex0JhZuwIcJLOljGMiEQp3nCCTBpKPOBwuzwy7ubFoPIWlnyI2ODq7Q1xI9wjz54OxC0jJaJIHYGUgOf40eYdpFMDkMCwosmSC6HEbAlnXaL8mlZ8VRr32C54d6Wny5ePd582G1_fj-cvN2uzJCinIleVUDNY1sTNlUphRcKmZ6KRU0ppPKSgpWsN5WStuuUVRJW8ueK5BQ886K0-L13neK4dsMKbf4WQa8x4eEObWcC4ayWjBEX_2DXoc5jng7pMqSKV4xgdSbPWViSClC307RDTouLaPtn1xbzLW9yxXZlwfHuRvAPpD3QSKw3gPfnYfl_07tp_PN3vI3TlqyuA</recordid><startdate>201908</startdate><enddate>201908</enddate><creator>Ochiai, Kayo</creator><creator>Yamada, Ai</creator><creator>Kimoto, Yasuhiro</creator><creator>Imamura, Hideaki</creator><creator>Ikeda, Toshio</creator><creator>Matsukubo, Makoto</creator><creator>Ieiri, Satoshi</creator><creator>Moritake, Hiroshi</creator><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7TO</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>K9.</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-5471-7646</orcidid><orcidid>https://orcid.org/0000-0003-0055-9157</orcidid></search><sort><creationdate>201908</creationdate><title>Long‐term remission of bilateral Wilms tumors that developed from premature separation of chromatids/mosaic variegated aneuploidy syndrome due to bilateral nephrectomy and peritoneal dialysis</title><author>Ochiai, Kayo ; Yamada, Ai ; Kimoto, Yasuhiro ; Imamura, Hideaki ; Ikeda, Toshio ; Matsukubo, Makoto ; Ieiri, Satoshi ; Moritake, Hiroshi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3534-5268e0c959c496c432571cf557e9cb57d50ed31fd67adb97075d85f27e5e82bd3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Aneuploidy</topic><topic>bilateral nephrectomy</topic><topic>bilateral Wilms tumor</topic><topic>Chemotherapy</topic><topic>Child, Preschool</topic><topic>Chromatids</topic><topic>Chromatids - pathology</topic><topic>Chromosome Disorders - complications</topic><topic>Chromosome Disorders - pathology</topic><topic>Chromosome Disorders - therapy</topic><topic>Dactinomycin</topic><topic>Hematology</topic><topic>Humans</topic><topic>Kidney Neoplasms - complications</topic><topic>Kidney Neoplasms - pathology</topic><topic>Kidney Neoplasms - therapy</topic><topic>Male</topic><topic>Metastases</topic><topic>Mosaicism</topic><topic>Nephrectomy</topic><topic>Nephrectomy - mortality</topic><topic>Oncology</topic><topic>PCS/MVA syndrome</topic><topic>Pediatrics</topic><topic>Peritoneal dialysis</topic><topic>Peritoneal Dialysis - mortality</topic><topic>Peritoneum</topic><topic>Prognosis</topic><topic>Quality of Life</topic><topic>Remission</topic><topic>Remission Induction</topic><topic>Rhabdomyosarcoma</topic><topic>Survival Rate</topic><topic>Tumors</topic><topic>Wilms Tumor - complications</topic><topic>Wilms Tumor - pathology</topic><topic>Wilms Tumor - therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ochiai, Kayo</creatorcontrib><creatorcontrib>Yamada, Ai</creatorcontrib><creatorcontrib>Kimoto, Yasuhiro</creatorcontrib><creatorcontrib>Imamura, Hideaki</creatorcontrib><creatorcontrib>Ikeda, Toshio</creatorcontrib><creatorcontrib>Matsukubo, Makoto</creatorcontrib><creatorcontrib>Ieiri, Satoshi</creatorcontrib><creatorcontrib>Moritake, Hiroshi</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric blood & cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ochiai, Kayo</au><au>Yamada, Ai</au><au>Kimoto, Yasuhiro</au><au>Imamura, Hideaki</au><au>Ikeda, Toshio</au><au>Matsukubo, Makoto</au><au>Ieiri, Satoshi</au><au>Moritake, Hiroshi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Long‐term remission of bilateral Wilms tumors that developed from premature separation of chromatids/mosaic variegated aneuploidy syndrome due to bilateral nephrectomy and peritoneal dialysis</atitle><jtitle>Pediatric blood & cancer</jtitle><addtitle>Pediatr Blood Cancer</addtitle><date>2019-08</date><risdate>2019</risdate><volume>66</volume><issue>8</issue><spage>e27804</spage><epage>n/a</epage><pages>e27804-n/a</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><abstract>We report a 38‐month‐old Japanese male with premature chromatid separation/mosaic variegated aneuploidy syndrome bearing biallelic BUB1B germline mutations who suffered from bilateral Wilms tumor. After right nephrectomy, dactinomycin monotherapy was administered for the left Wilms tumor; however, severe adverse reaction prevented the patient from receiving further chemotherapy. Left nephrectomy was then performed without postoperative chemotherapy. The patient survived for 15 months after bilateral nephrectomy without peritoneal relapse, metastasis of Wilms tumor, or the occurrence of rhabdomyosarcoma and maintained a good quality of life while receiving peritoneal dialysis at home.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>31081598</pmid><doi>10.1002/pbc.27804</doi><tpages>3</tpages><orcidid>https://orcid.org/0000-0001-5471-7646</orcidid><orcidid>https://orcid.org/0000-0003-0055-9157</orcidid></addata></record> |
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| subjects | Aneuploidy bilateral nephrectomy bilateral Wilms tumor Chemotherapy Child, Preschool Chromatids Chromatids - pathology Chromosome Disorders - complications Chromosome Disorders - pathology Chromosome Disorders - therapy Dactinomycin Hematology Humans Kidney Neoplasms - complications Kidney Neoplasms - pathology Kidney Neoplasms - therapy Male Metastases Mosaicism Nephrectomy Nephrectomy - mortality Oncology PCS/MVA syndrome Pediatrics Peritoneal dialysis Peritoneal Dialysis - mortality Peritoneum Prognosis Quality of Life Remission Remission Induction Rhabdomyosarcoma Survival Rate Tumors Wilms Tumor - complications Wilms Tumor - pathology Wilms Tumor - therapy |
| title | Long‐term remission of bilateral Wilms tumors that developed from premature separation of chromatids/mosaic variegated aneuploidy syndrome due to bilateral nephrectomy and peritoneal dialysis |
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