Neuroblastoma is one of the most common solid tumors in children and has a diverse clinical behavior that largely depends on the tumor biology. Neuroblastoma exhibits unique features, such as early age of onset, high frequency of metastatic disease at diagnosis in patients over 1 year of age and the tendency for spontaneous regression of tumors in infants. The high-risk tumors frequently have amplification of the MYCN oncogene as well as segmental chromosome alterations with poor survival. Recent advanced genomic sequencing technology has revealed that mutation of ALK, which is present in ~10% of primary tumors, often causes familial neuroblastoma with germline mutation. However, the frequency of gene mutations is relatively small and other aberrations, such as epigenetic abnormalities, have also been proposed. The risk-stratified therapy was introduced by the Japan Neuroblastoma Study Group (JNBSG), which is now moving to the Neuroblastoma Committee of Japan Children's Cancer Group (JCCG). Several clinical studies have facilitated the reduction of therapy for children with low-risk neuroblastoma disease and the significant improvement of cure rates for patients with intermediate-risk as well as high-risk disease. Therapy for patients with high-risk disease includes intensive induction chemotherapy and myeloablative chemotherapy, followed by the treatment of minimal residual disease using differentiation therapy and immunotherapy. The JCCG aims for better cures and long-term quality of life for children with cancer by facilitating new approaches targeting novel driver proteins, genetic pathways and the tumor microenvironment.
Most cases of abdominal lymphangioma are asymptomatic. However, patients may occasionally present with acute abdomen because of an intestinal obstruction or peritonitis caused by infected cysts, hemorrhaging, and/or torsion. These conditions may differ based on the location of the lymphangioma and do not always require emergency surgery. This report presents two cases of abdominal lymphangioma derived from the omentum, which required emergency surgery because of severe abdominal distension and peritonitis caused by torsion, respectively. In contrast, another case of retroperitoneal lymphangioma presented with acute abdomen and underwent elective surgery after conservative therapy for peritonitis. Elective surgery is acceptable if the symptoms are controllable under conservative therapy. However, it is important not to overlook the possible lethal complications such as intestinal and/or urological obstruction, aggressive peritonitis, and torsion of the cyst, which require emergency surgery.
Objective. The purposes of this study were to evaluate the accuracy of the ultrasonographic measurements used during diagnosis of hypertrophic pyloric stenosis (HPS), to examine the relationship between the palpability and the morphologic features of the pyloric mass, and to show the morphologic resolution of the pylorus after pyloromyotomy. Methods. One hundred three infants with the diagnosis of HPS by clinical findings and ultrasonographic measurements were treated surgically by Ramstedt pyloromyotomy. The measurement parameters were the muscle thickness, diameter, and channel length of the pylorus. The serial measurements were performed postoperatively for 1 year after surgery. Results. The preoperative ultrasonographic measurements of the pylorus were significantly correlated with the actual measurements obtained by surgery (P < .05). The diameter correlated most significantly with the palpability of the pylorus. In contrast to the prompt improvement of clinical symptoms, the muscle thickness did not reach the normal range (≈2 mm) until 8 months after surgery. The diameter had not decreased to its normal range (≈10 mm) by the end of the 1-year postoperative follow up period. The channel length decreased more rapidly than the other 2 parameters. Conclusions. The preoperative ultrasonographic measurements of the pylorus reliably evaluated the pyloric muscle thickness and diameter in HPS. The actual pyloric diameter correlated with the palpability of the pyloric mass. The morphologic resolution of the pylorus after surgery did not correlate with the prompt improvement of symptoms. Key words: hypertrophic pyloric stenosis; pylorus; ultrasonography. Surgery (K.M., A.N.) and Radiology (T.K.), Fukuoka Children's Hospital, Fukuoka, Japan. Revision requested June 7, 2007. Revised manuscript accepted for publication August 15, 2007. Address correspondence to Katsumi Muramori, MD, PhD, Department of Pediatric Surgery, Matsuyama Red Cross Hospital, Matsuyama City, Abbreviations HPS, hypertrophic pyloric stenosis ypertrophic pyloric stenosis (HPS) is diagnosed by palpation of the abdominal mass measurement of the hypertrophied pylorus by ultrasonographic examination alone. Although conservative therapy with atropine sulfate has been reported to be effective in some cases, 1-3 almost all cases of HPS have been treated surgically by pyloromyotomy. It is important to know the postoperative changes of the pylorus for consideration of the relationship between the improvement of symptoms and the morphologic changes and for discussion about the necessity for surgery. Some reports have examined postoperative changes of the pylorus, but these study cases were small in number. [4][5][6] Received May 21, 2007, from the Departments of
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