Xeroderma pigmentosum and renal leiomyosarcoma: A very rare case report association

•The occurrence of extra-dermatological cancers has been rarely reported in Xeroderma Pigmentosum, we present an exceedingly rare association of XP and renal Leiomyosarcoma in 14-year-old patient.•Radical nephrectomy remains the treatment of choice in order to confirm the diagnosis and guide a multidisciplinary approach.•The potential benefits of adjuvant therapy should be discussed with selected patients to improve the poor prognosis of this condition.•Physicians must be aware of this rare association and renal imaging should be performed at the least clinical sign. Xeroderma pigmentosum (XP) is a rare genetic disorder causing extreme sensitivity to ultraviolet (UV) rays. It is transmitted by an autosomal recessive mode. This disease is classically characterized by a high risk of skin cancer. The occurrence of extra-dermatological cancers in patients followed for XP has been rarely reported in the literature. We present the case of a 14-year-old adolescent with an exceptional association of XP and renal leiomyosarcoma. A 14-year-old adolescent was presented with an abdominal mass revealed by hematuria and abdominal pain. Computed tomography (CT) of the abdomen showed an enhancing, heterogeneous right renal mass, consistent with the features of renal tumor. After non conclusive biopsy, a total nephrectomy of the right kidney was performed. Based on gross specimen observation and immunochemical analysis, the patient was diagnosed with high-grade renal leiomyosarcoma. An adjuvant chemotherapy was indicated and the patient was referred to medical oncology. At 12 months follow-up, the patient is in total remission; The CT scan did not show any locoregional or distant recurrence The risk of renal tumor is increased in patients followed for XP. Attending physicians should ask for renal imaging at the slightest sign of clinical call.