Wilms tumour in a two day old neonate: A rare and challenging case

Wilms tumor or nephroblastoma is the second most common renal tumor occurring in the neonatal age group next to congenital mesoblastic nephroma. The most common age group of presentation of Wilms tumor is between 3 and 4 years of age. Here, we report a case of right-sided Wilms tumor in a 2-day-old neonate. A term-born female baby presented on day 2 of birth with abdominal distension and physical examination revealed a large palpable mass in the right lumbar region crossing the midline. Imaging features were suggestive of a retroperitoneal mass of probable right renal origin. The patient was taken up for right radical nephroureterectomy and Per-operatively, there was anticipated tumor spillage. Post-operative histopathological examination was suggestive of a triphasic Wilms tumor with no anaplasia and favorable histology. The patient was diagnosed with Wilms tumor of the right kidney, COG stage III, SIOP (“International Society of Pediatric Oncology”) intermediate risk. Loss of heterozygosity testing for 1p and 16q was negative. She was started on chemotherapy with a DDA4 regimen. She completed whole abdominal radiotherapy and tolerated treatment well. The child is disease free and is under regular follow-up. This case of neonatal Wilms tumor was indeed a challenge to the pediatric surgeons and the oncologists but the final results were worth the challenge that was faced

Neoplasias malignas en el neonato / Malignant neoplasms in the neonate

El cáncer en la edad pediátrica presenta características que lo diferencian de otros tipos reportados en edades posteriores. La supervivencia global a 3 años es de hasta el 70%, dependiendo de la neoplasia estudiada. Los principales aparatos y sistemas afectados son el sistema hematopoyético, el sistema nervioso central y simpático, así como tejidos mesenquimatosos. El incremento en la incidencia de tumores neonatales observado en este y otros estudios se basa en el aumento del número de tumores sólidos (teratomas y neuroblastomas), ya que los casos de tumores en el sistema nervioso central y leucemias han permanecido constantes. La ultrasonografía es la primera línea de abordaje y puede detectar hasta el 70% de las anomalías fetales. La fisiología del neonato hace que el tratamiento multidisciplinario necesario en las enfermedades neoplásicas sea modificado sustancialmente en este grupo de edad, para evitar toxicidad y secuelas. El tratamiento más utilizado es la cirugía. Logrando el diagnóstico oportuno existen opciones terapéuticas efectivas para mejorar la supervivencia de estos pacientes.

Cancer in children has characteristics that differentiate it from other types reported in later ages. Overall survival at 3 years is up to 70% depending on the tumor studied. Major organs and systems affected are the hematopoietic system, central nervous system and sympathetic and mesenchymal tissues. The increased incidence of neonatal tumors observed in this and other studies is based on the increasing number of solid tumors (teratomas and neuroblastomas) because cases of central nervous system tumors and leukemias have remained constant. Ultrasonography is the first line of approach and can detect up to 70% of fetal anomalies. The physiology of the newborn causes the necessary multidisciplinary treatment in neoplastic disease to be modified substantially in this age group to avoid toxicity and sequelae. The most common treatment is surgery. Achieving timely diagnostic treatment options are effective in improving the survival of these patients.