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>

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>

Clinicopathological study of 145 childhood rhabdomyosarcoma cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathological, immunohistochemical and electron microscopic characteristics of pediatric rhabdomyosarcomas (RMS).</p><p><b>METHODS</b>One hundred and forty-five cases of pediatric rhabdomyosarcomas were studied by routine histological, immunohistochemical and electron microscopic studies.</p><p><b>RESULTS</b>There were 97 male and 48 female patients with ages ranging from 4 months to 13 years and a mean of 4.2 years. The follow-up period of 100 patients was from 1 year to 20 years with a mean of 5 years after diagnosis. All cases were subtyped into the following histological categories: embryonal RMS, botryoid RMS, spindle cell RMS, alveolar RMS and solid RMS. Histopathological subtypes, tumor site and tumor stage correlated significantly with the patients' 5 years survival. The best prognosis was observed in spindle cell and botryoid RMS. Embryonal RMS carried an intermediate prognosis. Patients with alveolar RMS and solid RMS had the worst prognosis. Tumors involving bladder, head and neck carried a favorable clinical outcome. Patients with tumors involving trunk extremities retroperitoneum and pelvis did poorly. Immunohistochemically, all cases were positive for Vimentin. The positive staining rates for desmin, SMA and myoglobin were 78%, 75% and 37%, respectively. All tumors were negative for NSE, CD99 and LCA. Electron microscopy study showed features of myofilament and sarcomere in 10 of 15 cases.</p><p><b>CONCLUSIONS</b>RMS is the most common soft tissue sarcoma of childhood. Immunohistochemistry and electron microscopy are helpful in diagnosis and classification of RMS.</p>