Presentación de casos clínicos RESUMENEl lipoblastoma forma parte de los tumores derivados del tejido adiposo. Es una neoplasia benigna que se puede presentar de forma localizada o difusa, que afecta a niños en la primera infancia y que, generalmente, se localiza en el tronco y las extremidades. Estos tumores son poco frecuentes en la edad pediátrica, pero cuando uno de ellos se presenta, constituye un desafío que se debe resolver rápidamente por el riesgo de malignidad que implican otras lesiones neoplásicas, de similares características. El diagnóstico preoperatorio es dificultoso, ya que los métodos por imágenes no aportan información específica que permita diferenciarlos de otros tumores, como los liposarcomas. El diagnóstico definitivo es anatomopalógico y, en casos dudosos, es necesaria la confirmación citogenética. Se presenta el caso de un niño de 14 meses de edad con diagnóstico de lipoblastoma de localización retroperitoneal de gran tamaño.
Palabras clave: neoplasias retroperitoneales, lipoblastoma, pediatría.ABSTRACT Lipoblastoma is part of tumors derived from adipose tissue. It is a benign neoplasm that can be localized or diffuse, affecting children in early childhood and usually located in the trunk and extremities. These tumors are uncommon in the pediatric age, but when one of them is present, it is a challenge that we must resolve quickly due to the risk of malignancy that involves other similar neoplastic lesions. The preoperative diagnosis is difficult, because the imaging methods do not provide specific information that allows us to differentiate them from other tumors, such as liposarcomas. The definitive diagnosis is anatomopathological and, in doubtful cases, cytogenetic confirmation is necessary. We present the case of a 14-month-old boy with diagnosis of large sized lipoblastoma of retroperitoneal localization.
Lipoblastoma retroperitoneal gigante. Caso clínico
Giant retroperitoneal lipoblastoma. Case report
INTRODUCCIÓNLos tumores adiposos se clasifican como b e n i g n o s : l i p o m a , l i p o m a h e t e r o t ó p i c o , lipomatosis, hibernoma y variantes de lipoma, como lipoblastoma y lipoblastomatosis (LBS); o malignos: liposarcoma, que es extremadamente raro en la infancia.
Background
Epstein‐Barr virus (EBV) is a DNA virus with oncogenic potential, especially in immunocompromised patients. EBV can promote smooth muscle proliferation, resulting in EBV‐associated smooth muscle tumors (EBV‐SMT).
Methods
We report a case of a 10‐year‐old child with end‐stage renal disease secondary to hypoplastic crossed and fused kidneys who underwent kidney transplantation. EBV serology was unknown for the donor and negative for the recipient; three months after he had a primary EBV infection. Two years after the transplantation, percutaneous nephrostomy was performed because of a drop in the estimated glomerular filtration rate and severe dilatation of the graft. Nephrography showed contrast enhancement of the pelvis of the graft kidney and proximal ureter, with a clear blockage at the level of the mid ureter and no passage towards the bladder. A 1.5‐cm tumor was found causing intraluminal compression of the mid ureter.
Results
Complete resection of the tumor and distal ureter was performed leaving a short proximal ureter. A tension‐free uretero‐ureteroanastomoses was achieved using the native ureter. There were no surgical complications. Histologic evaluation showed spindle‐shaped muscle cells, moderate pleomorphism, and inflammatory infiltration. Immunohistochemical staining was positive for muscle‐specific actin. Epstein‐Barr encoding region (EBER) in situ hybridization was positive, confirming the diagnosis of EBV‐associated SMT.
Conclusions
EBV–SMT is an exceedingly rare oncological entity that may develop in either the graft or any other organ. The clinical findings are location related. EBV seroconversion following transplantation might be a risk factor for the development of SMT in solid organ recipients.
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