Background. Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder of UV radiation‐induced damage repair that is characterized by photosensitivity and a propensity for developing, among many others, skin cancers at an early age. This systematic review focused on the correlation between the clinical, pathological, and genetic aspects of XP and skin cancer. Methods. A systematic review was conducted through a literature search of online databases PubMed, Cochrane Library, SciELO, and Google Scholar. Search terms were “Xeroderma pigmentosum”, “XP”, “XPC”, “Nucleotide excision repair”, “NER”, “POLH”, “Dry pigmented skin”, and “UV sensitive syndrome” meshed with the terms “Skin cancer”, “Melanoma”, and “NMSC”. Results. After 504 abstracts screening, 13 full‐text articles were assessed for eligibility, and 3 of them were excluded. Ten articles were selected for qualitative assessment. Conclusions. Patients with XP usually suffer shorter lives due to skin cancer and neurodegenerative disease. Deletion/alteration of a distinct gene allele can produce different types of cancer. The XPC and XP‐E variants are more likely to have skin cancer than patients in other complement groups, and the most common cause of death for these patients is skin cancer (metastatic melanoma or invasive SCC). Still, aggressive preventative measures to minimize UV radiation exposure can retard the course of the disease and improve the quality of life.

Schwannomas are mainly benign tumors arising from the Schwann cells of the peripheral nerve sheath. These tumors can often be associated with non-specific symptoms, such as abdominal heaviness. In this article, we present a detailed description of the surgical management of a giant sacral schwannoma in an elderly patient, for which intraoperative neuromonitoring made it possible to distinguish easily the nerves of the sacral plexus from which the tumor originated and to remove it without complications. Treatment of these rare and symptomatic giant tumors is still a challenge for surgeons; to treat adequately these tumors; a multidisciplinary approach is required to ensure an optimal therapeutic approach to reduce the risk of recurrence and, on the other hand, is not associated with unnecessary iatrogenic neurological damage.