RESUMEN
BACKGROUND: Wilms tumor is associated in 7 to 10% of patients with congenital abnormalities. Among those, aniridia is the most constant feature of the WAGR syndrome that includes, in one third of cases. Wilms tumor. We report two cases of aniridia associated with fetal rhabdomyomatous nephroblastoma. CASE REPORTS: Case 1. A one-year old girl with congenital aniridia was admitted for macroscopic hematuria. Abnormal ultrasonography and tomodensitometry revealed a large, bilateral, kidney tumor. The patient was given actinomycin and vincristine, without efficacy. Bilateral tumorectomy was performed 6 months later and the histological study showed a fetal rhabdomyomatous nephroblastoma. This patient is in remission at the age of 5. Case 2. A boy, also with congenital aniridia, presented with macroscopic hematuria at the age of 2 years revealing a nephroblastoma located on his right kidney. Preoperative chemotherapy remained uneffective and the nephrectomy performed 1 month later permitted the diagnosis of fetal rhabdomyomatous nephroblastoma. The patient is well 4 years later. CONCLUSION: Both cases of fetal rhabdomyomatous nephroblastoma, a histological variant of Wilms tumor, seem to be the first reported in the WAGR syndrome.
Asunto(s)
Aniridia/complicaciones , Neoplasias Renales/complicaciones , Rabdomioma/patología , Tumor de Wilms/complicaciones , Femenino , Humanos , Lactante , Neoplasias Renales/patología , Neoplasias Renales/terapia , Masculino , Síndrome WAGR/diagnóstico , Tumor de Wilms/patología , Tumor de Wilms/terapiaRESUMEN
Two children (4 and 12 years old) presented with a Stevens-Johnson syndrome. The symptoms and their evolution were remarkably similar. In both cases, a Mycoplasma pneumoniae infection was suspected after a serolgic study. This etiology, still unfamiliar, must be suspected in presence of severe stomatitis in children.