Laparoscopic Removal of Extraosseous Ewing's Sarcoma of the Kidney in a Pediatric Patient

Abstract

In the pediatric population, to the best of our knowledge, only 2 cases of renal extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) have been published. We report the initial case of renal EES/PNET occurring in a 10-year-old girl treated by a laparoscopic radical nephrectomy. The regimen used is the first documented use of neoadjuvant chemotherapy prior to laparoscopic radical nephrectomy for PNET. This approach obviated the need for a large incision and a prolonged postsurgical recovery. The minimally invasive nature of the laparoscopic procedure allowed for a rapid convalescence and resumption of her chemotherapy regimen within 14 days of the surgery.

An Approach to Renal Masses in Pediatrics

“…[118][119][120][121] They are very aggressive, usually large and invasive, 19 and typically have poorly defined margins with areas of hemorrhage and necrosis. 122,123 The distinct vascular nature is manifested by an arborizing vascular pattern, perithelial arrangement of tumor cells, and perivascular pseudorosettes. 124 More than 90% of renal Ewing sarcoma/ PNETs have characteristic translocations involving rearrangement of the ESWR1 gene on chromosome 22, t [11;22][q24;q12].…”

Renal Primitive Neuroectodermal Tumors

“…4,6 Laparoscopic surgical intervention has been shown to allow a rapid resumption of neoadjuvant chemotherapy. 15 Of interest, a case of spontaneous regression of metastatic lung nodules from renal PNET postnephrectomy has been documented. 28 Chemotherapy recommendations to treat PNET have included alternating cycles of ifosfamide and etoposide with vincristine, doxorubicin, and d-actinomycin, although the addition of ifosfamide and etoposide has not been shown to be beneficial in patients with metastasis.…”

“…10 Despite this, case reports of renal PNETs 20 cm in size or greater have also been reported. 5,15 As both the size and coloration of these tumors features considerable overlap with more common renal neoplasms, gross examination alone cannot confer a diagnosis of renal PNET. 1,3-5,10 Some cases may also feature vesicular nuclei and small nucleoli.…”

Retroperitoneoskopische Nephrektomie bei einem Neugeborenen wegen Faktor-V-Leiden-Mutation und in utero vena cava inferior und Nierenvenenthrombose: erster Fallbericht

“…In 1992 Figenshau et al performed the initial pediatric laparoscopic nephroureterectomy [17], while the first pediatric laparoscopic partial nephrectomy with ureterectomy was described in 1993 by Jordan and Winslow [19]. With major advances in endoscopic instrumentation and technology the laparoscopic approach has evolved to a level where it challenges the standard open approach for nephrectomy and nephroureterectomy [20][21][22][23][24][25][26][27]. More recently the retroperitoneoscopic approach to the kidney, which involves direct retroperitoneal access without entering the peritoneal cavity, appears to be increasingly popular with urologists interested in minimally invasive surgery.…”