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In this report, we present an autopsy case of LNSS in a Japanese boy. The affected neonate had hydrops fetalis and was born at 28 weeks and 4 days of gestation, weighing 2104\u2009g. He had bilateral inverted eyelids, verrucous linear nevus separated along Blaschko's line, myocardial hypertrophy, and pharyngeal constriction, and underwent intensive treatment in NICU for arrhythmia, hydrocephalus, and respiratory distress. The hydrocephalus progressed gradually and he died at the age of 181 days, 12 days after a sudden cardiac arrest and recovery. <jats:italic>KRAS<\/jats:italic> G12D mutation was found in a skin biopsy specimen but not in blood cells, suggesting a postzygotic mosaicism. Autopsy revealed novel pathological findings related to LNSS, including intracranial lipomatous hamartoma and mesenteric lymphangioma, in addition to previously reported findings such as multicystic dysplastic kidney. There was the limited expression of mutated <jats:italic>KRAS<\/jats:italic> protein in kidneys.<\/jats:p>","DOI":"10.1111\/pin.13463","type":"journal-article","created":{"date-parts":[[2024,7,12]],"date-time":"2024-07-12T11:39:35Z","timestamp":1720784375000},"page":"538-545","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Autopsy case of linear nevus sebaceous syndrome with <i>KRAS<\/i> (G12D) mutation"],"prefix":"10.1111","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7168-8493","authenticated-orcid":false,"given":"Akira","family":"Ohishi","sequence":"first","affiliation":[{"name":"Maternal\u2010Fetal and Neonatal Care Center Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasunori","family":"Enomoto","sequence":"additional","affiliation":[{"name":"Department of Regenerative and Infectious Pathology Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideto","family":"Iwafuchi","sequence":"additional","affiliation":[{"name":"Department of Pathology Shizuoka Children's Hospital Shizuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5473-2608","authenticated-orcid":false,"given":"Shiori","family":"Meguro","sequence":"additional","affiliation":[{"name":"Department of Regenerative and Infectious Pathology Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isao","family":"Kosugi","sequence":"additional","affiliation":[{"name":"Department of Regenerative and Infectious Pathology Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Baba","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshihide","family":"Iwashita","sequence":"additional","affiliation":[{"name":"Department of Regenerative and Infectious Pathology Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuki","family":"Segawa","sequence":"additional","affiliation":[{"name":"Maternal\u2010Fetal and Neonatal Care Center Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daizo","family":"Ueno","sequence":"additional","affiliation":[{"name":"Department of Regional Neonatal and Perinatal Medicine Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shigeo","family":"Iijima","sequence":"additional","affiliation":[{"name":"Department of Regional Neonatal and Perinatal Medicine Hamamatsu University School of Medicine Hamamatsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,7,12]]},"reference":[{"key":"e_1_2_12_2_1","unstructured":"OMIM. 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