Cobeñas Carlos J, Pereyra Priscila, Spizzirri Ana Paula, Gauto Santacruz Cristina, Del Carmen Suarez Angela, Altamirano Eugenia, Pérez Paula, Tello Nicolas
Nephrology Department, Hospital de Niños "Sup. Sor María Ludovica", La Plata, Buenos Aires, Argentina.
Pathology Department, Hospital de Niños "Sup. Sor María Ludovica", La Plata, Argentina.
Pediatr Nephrol. 2025 Feb;40(2):389-391. doi: 10.1007/s00467-024-06493-4. Epub 2024 Sep 4.
We report on a 14-year-old girl who developed post-transplantation smooth muscle tumours (PTSMT) located in the spleen, lungs, liver, and central nervous system (CNS), 4 years after kidney transplantation. She was asymptomatic, and the disease was detected during the work-up for a urinary tract infection. Diagnosis was performed by the analysis of a tissue specimen, through the biopsy of a lung tumour, which revealed a proliferation of spindle-shaped cells which were positive for actin and vimentin. In situ hybridization studies were positive for Epstein-Barr virus, and her serologic status was negative prior to transplantation. We reduced immunosuppression by stopping mycophenolate and switching tacrolimus for sirolimus. After 18 months of follow-up, she remains asymptomatic, and the CNS tumour reduced its diameter from 24 × 21 mm to 14 × 13 mm. PTSMT should be considered in the differential diagnosis of transplanted patients who develop neoplastic complications associated with immunosuppression.
我们报告了一名14岁女孩,她在肾移植4年后出现了位于脾脏、肺、肝脏和中枢神经系统(CNS)的移植后平滑肌肿瘤(PTSMT)。她没有症状,该病是在对尿路感染进行检查时被发现的。通过对肺肿瘤活检的组织标本进行分析来进行诊断,结果显示梭形细胞增殖,肌动蛋白和波形蛋白呈阳性。原位杂交研究显示爱泼斯坦-巴尔病毒呈阳性,且她在移植前的血清学状态为阴性。我们通过停用霉酚酸酯并将他克莫司换为西罗莫司来减少免疫抑制。经过18个月的随访,她仍然没有症状,中枢神经系统肿瘤的直径从24×21毫米缩小到了14×13毫米。对于出现与免疫抑制相关的肿瘤并发症的移植患者,鉴别诊断时应考虑PTSMT。