Acute leukemia after radiotherapy in a patient with Turcot's syndrome. Impaired colony formation in skin fibroblast cultures after irradiation.
Colonic polyposis and carcinoma developed in a woman with Turcot's syndrome at the age of 31 years; astrocytoma developed when she was 37. Her brother and sister had died of astrocytoma at the ages of 18 and 33 years, respectively. Progressive neutropenia developed in the patient three months after radiotherapy for her brain tumor and acute myelomonocytic leukemia 19 months after treatment. Three laboratories independently evaluated cultures of her skin fibroblasts for in vitro sensitivity to cell killing (loss of colony-forming ability) by x-rays. Survival assays consistently revealed slight but significant radiosensitivity in an early-passage (six to 10 doublings) fibroblast subculture. A later subculture (21 to 29 doublings) showed no abnormality, a possible effect of selective in vitro loss of radiosensitive cells.
D000230:Adenocarcinoma / Q000235:genetics; D000328:Adult; D001254:Astrocytoma / Q000532:radiotherapy*; D001932:Brain Neoplasms / Q000235:genetics / Q000532:radiotherapy*; D003114:Colony-Forming Units Assay; D005260:Female; D005347:Fibroblasts / Q000528:radiation effects*; D006801:Humans; D007417:Intestinal Polyps / Q000235:genetics; D007951:Leukemia, Myeloid / Q000209:etiology* / Q000235:genetics; D007953:Leukemia, Radiation-Induced / Q000209:etiology*; D009378:Neoplasms, Multiple Primary / Q000235:genetics; D010375:Pedigree; D011836:Radiation Tolerance; D011878:Radiotherapy / Q000009:adverse effects; D012004:Rectal Neoplasms / Q000235:genetics; D012811:Sigmoid Neoplasms / Q000235:genetics; D012867:Skin / Q000166:cytology; D013577:Syndrome