Neurofibromatosis type I is a complex, multisystem cancer predisposition syndrome of a number of tumors, which commonly arise in the central and peripheral nervous system, the gastrointestinal tract (GIT), and breast and soft tissues, yet the association with endometrial cancer is rare. Herein, we report the case of a 67-year-old female, never married, who had been followed since puberty for neurofibromatosis type I clinically manifested by pigmentary abnormalities such as café-au-lait macules, neurofibromas, and Lisch nodules with various comorbidities, such as diabetes type I, hypertension, renal failure, and ophthalmic disorders (poor vision). One year earlier, she developed a locally advanced endometrial cancer, for which chemotherapy would not have been optimal because of these comorbidities; due to the pathology of neurofibromatosis, the lifespan of such individuals is typically around fifteen years shorter than that of the general population and it negatively impacts their quality of life.
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