{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T20:22:22Z","timestamp":1791145342616,"version":"4.1.0"},"reference-count":34,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[1998,1,1]],"date-time":"1998-01-01T00:00:00Z","timestamp":883612800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Cutan Med Surg"],"published-print":{"date-parts":[[1998,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Patients with xeroderma pigmentosum (XP) frequently develop sunlight-induced skin cancer. Infrequently, internal neoplasms may also occur. A 21-year-old patient with XP, who had many skin cancers, developed a rare internal tumour \u2014 a grade II diffuse fibrillary spinal cord astrocytoma \u2014 during a break in a therapeutic trial of isotretinoin for skin cancer prevention. Treatment of neoplasms in XP patients presents special difficulties because of their defect in DNA repair.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>The study objective was to raise awareness of the cancer surveillance process in XP patients and the concerns involved in choice of therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Since the spinal cord tumour was inoperable, the patient was treated with x-radiation, continued on isotretinoin treatment and was followed closely for tumour response.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Despite sensitivity to sunlight, the patient had a normal acute response to the x-ray treatment without excessive skin reaction. Serial examinations by magnetic resonance imaging (MRI) starting 8 months after x-ray treatment was initiated, showed a marked gadolinium enhancement followed by regression. This clearing was first seen at 2 years after biopsy and persisted to at least 9 years after treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>In contrast to the exaggerated sensitivity to UV radiation, XP patients may tolerate therapeutic doses of x-radiation. Isotretinoin treatment may have contributed to the good response of this spinal cord astrocytoma.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/120347549800200308","type":"journal-article","created":{"date-parts":[[2017,7,9]],"date-time":"2017-07-09T19:44:58Z","timestamp":1499629498000},"page":"153-158","source":"Crossref","is-referenced-by-count":38,"title":["Xeroderma Pigmentosum: Spinal Cord Astrocytoma with 9-Year Survival after Radiation and Isotretinoin Therapy"],"prefix":"10.1177","volume":"2","author":[{"given":"John J.","family":"DiGiovanna","sequence":"first","affiliation":[{"name":"Division of Dermatopharmacology, Department of Dermatology, Brown University School of Medicine and Laboratory of Skin Biology, National Institute of Arthritis Musculoskeletal and Skin Diseases, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas","family":"Patronas","sequence":"additional","affiliation":[{"name":"Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Katz","sequence":"additional","affiliation":[{"name":"Laboratory of Pathology, National Institutes of Health, Bethesda, Maryland, USA"},{"name":"Dermatology Branch, National Institutes of Health, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donita","family":"Abangan","sequence":"additional","affiliation":[{"name":"Office of the Clinical Director, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth H.","family":"Kraemer","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Carcinogenesis, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[1998,1,1]]},"reference":[{"key":"bibr1-120347549800200308","first-page":"1974","volume-title":"Dermatology in general medicine","author":"Kraemer KH","year":"1993","edition":"4"},{"key":"bibr2-120347549800200308","first-page":"4393","volume-title":"The metabolic and molecular bases of inherited disease","author":"Cleaver JE","year":"1995","edition":"7"},{"key":"bibr3-120347549800200308","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(91)70202-D"},{"key":"bibr4-120347549800200308","doi-asserted-by":"publisher","DOI":"10.2176\/nmc.34.817"},{"key":"bibr5-120347549800200308","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1994.01690080084012"},{"key":"bibr6-120347549800200308","doi-asserted-by":"publisher","DOI":"10.1093\/carcin\/5.4.511"},{"key":"bibr7-120347549800200308","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198806233182501"},{"key":"bibr8-120347549800200308","unstructured":"Burger PC, Scheithauer BW, Vogel FS. 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