Primary Ewing's sarcoma/primitive neuroectodermal tumor of the urogenital tract in children / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study, we summarize the clinical presentation, pathological features, therapeutic strategies, and prognosis of ES/PNET.</p><p><b>METHODS</b>Clinical information on two cases of ES/PNET in the penis and ureter was analyzed, and relevant literature was reviewed.</p><p><b>RESULTS</b>ES/PNET was confirmed pathologically, immunohistochemically and via molecular biology techniques in the penis (n=1) and ureter (n=1). In one case, a tumor was found at the base of penis, which had invaded the corpus cavernosum, and resulted in a massive enlargement of the penis. This tumor was initially diagnosed as an endocrine disorder. However, a confirmed diagnosis was made 11 months later when massive metastases in both lungs were noted. A tumor biopsy was performed to confirm the diagnosis, and chemotherapy with a CAV (cyclophosphamide+doxorubicin+vincristine)+IE (ifosfamide+etoposide) regimen for 9 months was prescribed. In the second case, a child was admitted due to abdominal pain and a hydroureter in the right kidney, as determined by ultrasonography. A tumor was found in the right ureter at the level of iliac vessels. Removal of the tumor and ureteral anastomosis were performed, and chemotherapy with CAV+IE for 8 months were prescribed. Both patients are currently being followed-up closely.</p><p><b>CONCLUSIONS</b>ES/PNET is a highly malignant tumor and has poor prognosis. Pre-operative diagnosis of ES/PNET of urogenital tract is difficult and largely depends on pathology, immunohistochemistry, and, if applicable, molecular biology. Comprehensive therapy may include surgery, chemotherapy and radiotherapy.</p>

Primitive neuroectodermal tumor/Ewing's sarcoma of the penis in children: a case report and review of the literature / 中华男科学杂志

<p><b>OBJECTIVE</b>To investigate the clinical manifestations, pathological characteristics and treatment of primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) of the penis in children.</p><p><b>METHODS</b>We analyzed the clinical data of a case of PNET/EWS and reviewed relevant literature.</p><p><b>RESULTS</b>The patient was a 5-year-old boy, admitted for penis swelling with pain for 11 months. Biopsy showed a small round cell tumor, CD99 positive by immunohistochemical staining, with EWS translocation by fluorescence in situ hybridization on molecular biological examination. The tumor was confirmed to be PNET/EWS of the penis, and disappeared after 45 weeks of chemotherapy and local radiotherapy.</p><p><b>CONCLUSION</b>PNET/EWS of the penis is an extremely rare disease, with no specific clinical symptoms except penis enlargement with pain. Immunohistochemistry and molecular biological examination contribute to its diagnosis.</p>

Preservation of the kidney with delayed diagnosis of traumatic pelvi-ureteric junction disruption secondary to blunt abdominal trauma in children / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The delayed diagnosis of pelvi-ureteric junction (PUJ) disruption in children following blunt abdominal trauma can result in loss of function of the involved kidney. We examined the potential for kidney preservation and the limits of diagnostic delays.</p><p><b>METHODS</b>A retrospective review of 17 cases of PUJ disruption at Beijing Children's Hospital from 1993 to 2009 was done with respect to diagnosis, treatment and follow-up.</p><p><b>RESULTS</b>The interval from trauma to diagnosis of PUJ disruption was (52 ± 52) days. If one case with nephrectomy was excluded, the interval from trauma to diagnosis was (40 ± 20) days. The average time between injury and first treatment was (49 ± 25) days. Pelvi-ureteric reanastomosis and caliceal ureterostomy were performed separately in 11 and 4 patients, respectively. Ileal replacement for ureter injuries was finally performed in one patient. Hydronephrosis of the injured kidney was reduced and the function improved in 15 out of 17 patients (88%). Only one patient received nephrectomy and the nephrectomy rate was 5.9%.</p><p><b>CONCLUSION</b>Differential renal function at the PUJ disruption side can be saved and the rate of nephrectomy reduced by appropriate surgery if the time to diagnosis and first treatment is limited to within two months.</p>