Abstract Background MYCN-amplified neuroblastoma almost always expresses the MYCN protein. MYCN amplification is one of the crucial risk factors for a poor prognosis. However, there are scattered cases in which MYCN protein expression is absent despite MYCN amplification, making it difficult to evaluate the prognostic importance of MYCN amplification. Recently, the usefulness of MYCN protein expression in the histological diagnosis of neuroblastoma has been reported, but there are only a few cases of treatment intensity reduction based on negative protein expression despite MYCN amplification. Case presentation: Herein, we report two cases of infantile neuroblastoma—a 3-month-old girl and 10-month-old boy with stage 4S and 2B neuroblastoma, respectively—with MYCN amplification or gain without MYCN expression. The 3-month-old girl with stage 4S disease was initially treated with one course of chemotherapy before biopsy due to hepatomegaly causing abdominal compartment syndrome. MYCN amplification was evident, but MYCN expression was negative; therefore, treatment intensity was reduced and autologous stem cell transplantation was not performed. The 10-month-old boy with stage 2B showed MYCN gain on initial biopsy, but was negative for MYCN expression. Therefore, he was treated with conventional chemotherapy and surgery. Both patients were treated with conventional chemotherapy and 13-cis-retinoic acid without autologous stem cell transplantation. They remained disease free for 10 and 7 years post-resection, respectively. These two cases led us to speculate that MYCN protein expression more closely reflects the nature of a tumor than MYCN amplification or gain. Conclusions Nevertheless, chemotherapy could be optimized based on histological features and MYCN expression rather than MYCN amplification or gain. It is expected that MYCN expression will be considered as a factor in determining treatment intensity for MYCN-amplified or -gain tumors.

Appendiceal intussusception in children is a rare entity characterized by the invagination of the appendix into the cecum. The early diagnosis and optimal surgical management of appendiceal intussusception have not been achieved because of its rarity. Here, we report a case of a 7-year-old girl with appendiceal intussusception presenting with recurrent ileocecal invagination. She was treated by simple appendectomy without using a stapling device. The imaging findings in patients with appendiceal intussusception were reported, and the optimal surgical treatment for appendiceal intussusception was discussed.

MYCN-amplified neuroblastoma demonstrating favorable histology, which is genotype–phenotype discordant, is extremely rare. This study reports two cases of peripheral neuroblastic tumors with genotype–phenotype discordance: a 3-month-old female and 10-month-old male patients with stage 4S and 2B neuroblastoma, respectively, harboring MYCN-amplification and favorable histology. Immunohistochemical staining was negative for N-myc. Both patients were treated with conventional chemotherapy and 13-cis-retinoic acid without autologous stem-cell rescue, and have been disease-free for 74 and 38 months post-resection, respectively. Nevertheless, chemotherapy could have been optimized on the basis of histological features of the tumors, showing no expression of N-myc.

Because of their rarity, the treatment strategy for pediatric ovarian epithelial tumors is controversial, especially for a giant cystadenoma. We report the largest mucinous cystadenoma (MCA) case in the pediatric literature thus far. A 12-year-old girl had abdominal distention and visited our hospital. She had a multilocular cyst with some protuberance on the inside and high values of CA 19-9 and CA-125. We diagnosed her with a left MCA and performed a left oophorectomy. The tumor was the stage IA borderline malignant MCA and weighed 11.8 kg. Five years have passed, the patient has not experienced recurrence or metastasis. The resection of giant tumors can affect respiration and circulation. However, pre- or intra-operative drainage may lead to dissemination and adhesion. When we treat pediatric giant ovarian epithelial tumors, we must understand the findings that suggest the possibility of malignancy to decide appropriately as to whether drainage should be performed.

1,492,349 4/1924 Brown et al. ...................... 73/322.5 2,003,869 6/1935 Stegemann ....................... 141/94 X 3,556,682 1/1971 Sakamoto et al. ... 73/322.5 X 4,031,581 6/1977 Baugh ................................... 441/29 4,037,992 7/1977 Uchida et al. ...... ... 406/109 X 4,193,057 3/1980 Bennett et al. ................... 441/29 X 4,231,686 11/1980 Sakamoto et al. .................... 406/14 4,239,423 12/1980 Sakamoto et al. .............. 406/109 X 4,854,783 Aug. 8, 1989 11 Patent Number:

To clarify the role of interval appendectomy (IA) in pediatric patients with acute appendicitis with an appendiceal inflammatory mass or abscess, we histologically analyzed the appendices removed during IA.

A 5-month-old female infant was admitted to hospital because of a slowly growing solid mass in the right side of the chest. The mass was located on the right fifth rib and did not involve the skin. Ultrasound (US) revealed that the mass was echogenic with a central high echogenicity, suggesting calcification. Computed tomography (CT) and magnetic resonance imaging (MRI) showed that the calcifying tumor possibly originated from the periosteum of the rib. The tumor was successfully removed and histological diagnosed as a calcifying aponeurotic fibroma (CAF). The patient is free from the tumor 118 months after the surgery. Usually, a CAF develops in the distal extremities and often recurs following excision. To the best of our knowledge, there are no reports in the English literature of a CAF that originated from the chest wall. Herein, we review pediatric cases of CAF that occurred in uncommon sites such as the head, face, neck, and trunk, and discuss the clinical characteristics and treatment of CAF.

症例は精神運動発達遅滞を有する5p-症候群の27歳男性．20歳頃から固形物摂取が困難となり，27歳時にコーヒー残渣様嘔吐と貧血が出現し当科紹介．食道裂孔ヘルニア，高度食道狭窄の診断で，噴門形成，胃瘻造設を施行．術後1カ月で食道ブジーを行ったが，狭窄は治療抵抗性であり，4回目のブジー時に食道気管支瘻が判明し保存的治療施行．一時瘻孔が閉鎖したが経過中に瘻孔再発し手術（中下部食道切除，瘻閉鎖，食道胃接合部閉鎖，Roux-en-Y脚を挙上し口側食道空腸吻合）を施行した．胃をリザーバーとして用いるtotal gastroesophageal dissociation での再建となった．術後はすぐに胃瘻使用が可能であり栄養管理容易で経過良好であった．逆流性食道炎による著明な食道狭窄は治療に難渋することが多い．各症例のQOLや病態を考慮した治療戦略を練ることが重要である．

Arai, M.; Nagahara, Y.; Kosaka, Y.; Toda, M.; Kuroiwa, M.; Okamoto, H. Author Information

Using magnetic resonance imaging (MRI), changes in the livers of postoperative biliary atresia (BA) patients were investigated.Periodic MRI was performed in 32 postoperative BA patients. The findings were evaluated by calculating the near-normal liver tissue area that corresponded with normal- or high-signal regions on T1-weighted imaging. The patients were divided into three groups based on the extent of near-normal liver tissue on the final MRI: group A, n = 14; group B, n = 13; and group C, n = 5, included patients with >40%, 20-40%, and <20% area of near-normal liver tissue, respectively. The relationship among the macroscopic and histological findings in the liver at orthotopic living donor liver transplantation (OLDLT), patient outcomes, and MRI findings were investigated.In group A, 11 patients had no evidence of liver dysfunction. In group B, six patients either had undergone or were awaiting OLDLT. In group C, all patients had undergone OLDLT. All patients had either adequate or impaired bile drainage in each liver segment. The segmental changes corresponded with the liver architecture at OLDLT. The changes could be evaluated on MRI at 1-2 years after surgery.Adequate and restricted areas of liver tissue with near-normal structure were indicative of good and poor prognoses, respectively. Shortly after portoenterostomy, these segmental changes occurred and/or developed in each liver segment and could be detected on MRI. It is emphasized that patients with >40% area of near-normal liver architecture at the initial stages did not require OLDLT, while those with <20% area did require OLDLT.

We report a rare case of neonatal Beckwith-Wiedemann syndrome (BWS) associated with alveolar rhabdomyosarcoma (RMS). Alveolar RMS was diagnosed on the basis of excisional biopsy. Chemotherapy was initiated and followed by bone marrow transplantation. The patient, who is now 3 years and 11 months of age, is alive 46 months after the initial diagnosis, albeit with disease. We could not detect the PAX3-FKHR or PAX7-FKHR transcripts; however, we could observe hypomethylation of the differentially methylated region of the long QT intronic transcript 1. Thus, neonatal alveolar RMS with BWS may result from an alternate molecular pathway.

A 3-year-old girl with recurrent chylous ascites was successfully treated by laparoscopic ligation of the ruptured lymphatic trunk. She was referred to our hospital at 16 days of age because of marked abdominal distension. Imaging methods showed massive ascites of unknown origin, and analysis of the ascites revealed its chylous nature. Conservative treatments were started. Her condition improved to some extent, and she was discharged. Two years later, she was readmitted with abdominal distension and loss of appetite. Laparoscopic surgery was planned to clarify the etiology and to treat intractable ascites. Sudan black B was orally administered, and laparoscopy revealed the presence of a whitish-gray fluid in the abdominal cavity, and a dark-blue stream of the dye was noticed. The responsible lesion of the chylous ascites was detected by tracking the stream. The lesion was ligated twice with an endoloop. She has been completely free from the symptoms for 3 years and 9 months. This experience indicates the usefulness of laparoscopic surgery in investigating the etiology of chylous ascites and treating it. The concomitant use of a lipophilic dye is mandatory to find the responsible lesion at surgery. Laparoscopic surgery, instead of open surgery, should be considered as a treatment of choice for intractable chylous ascites.

Background. The clinical outcome of neuroblastoma (NB) depends on age, stage, and MYCN amplification. Receptor tyrosine kinases (RTKs) promote cell growth, migration, and metastasis in cancer cells, including NB. However, the correlation of the expression profile of RTKs with prognosis in NB remains controversial. Procedure. Expression and mutation analysis of KIT, PDGFR, FLT3, RET, and TRKA mRNAs were performed in 24 NB cell lines and 40 tumor samples using RT-PCR followed by direct sequencing. Immunohistochemical analysis of KIT and PDGFR protein expression was also examined in 38 paraffin sections of NB turner samples. Results. The expression of KIT, PDGFR beta, and FLT3 mRNA was associated with NB in patients under I year (P<0.02) and TRKA expression (P< 0.001). The loss of expression of these kinases was associated with MYCN amplification (P< 0.02) and advanced stages of disease in patients over I year of age (P < 0.005). PDGFR alpha mRNA expression was detected in all cell lines and tumor samples, and RET mRNA expression was not associated with any clinical parameters. Immunohistochemistry results showed the similar findings. We did not find any activating mutations in KIT, PDGFR, FLT3, or RET. Notably, the GNNK(-) isoform of KIT was predominant in all cell lines and clinical samples. Conclusion. Expression of KIT, PDGFR beta, and FLT3 was associated with a good prognosis in NB. The loss of expression of these RTKs might correlate to the disease progression of NB.

Pediatrics InternationalVolume 48, Issue 2 p. 181-184 Patient Report Bilateral ovarian teratomas successfully treated by ovary-conserving technique HAYATO YAMAUCHI, Corresponding Author HAYATO YAMAUCHI Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanHayato Yamauchi, Department of General Surgical Science, Gunma University, Graduate School of Medicine, 3–39–22 Showa-machi, Maebashi, Gunma 371–8511, Japan.Search for more papers by this author 1 ATSUSHI TAKAHASHI, ATSUSHI TAKAHASHI Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 1 TOMOKO HIRAKATA, TOMOKO HIRAKATA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 1 KAZUMICHI ONIGATA, KAZUMICHI ONIGATA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 TAKASHI KANAZAWA, TAKASHI KANAZAWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 CHITOSE OGAWA, CHITOSE OGAWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 MINORU KUROIWA, MINORU KUROIWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 3 AKIHIRO MORIKAWA, AKIHIRO MORIKAWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 HIROYUKI KUWANO, HIROYUKI KUWANO Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 1 HAYATO YAMAUCHI, Corresponding Author HAYATO YAMAUCHI Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanHayato Yamauchi, Department of General Surgical Science, Gunma University, Graduate School of Medicine, 3–39–22 Showa-machi, Maebashi, Gunma 371–8511, Japan.Search for more papers by this author 1 ATSUSHI TAKAHASHI, ATSUSHI TAKAHASHI Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 1 TOMOKO HIRAKATA, TOMOKO HIRAKATA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 1 KAZUMICHI ONIGATA, KAZUMICHI ONIGATA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 TAKASHI KANAZAWA, TAKASHI KANAZAWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 CHITOSE OGAWA, CHITOSE OGAWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 MINORU KUROIWA, MINORU KUROIWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 3 AKIHIRO MORIKAWA, AKIHIRO MORIKAWA Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 2 HIROYUKI KUWANO, HIROYUKI KUWANO Departments of1General Surgical Science (Surgery I)and2Pediatrics and Developmental Medicine, Gunma University, Graduate School of Medicine , 3Department of Surgery, Gunma Children's Medical Center, Gunma, JapanSearch for more papers by this author 1 First published: 05 April 2006 https://doi.org/10.1111/j.1442-200X.2006.02190.xCitations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I 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We investigated enteric innervations in 15 isolated and five syndromic cases of Hirschsprung disease (HSCR) with immunohistochemistry for the S100 protein (S100), class III α‐tubulin (TUJ1), peripherin, neuronal nitric oxide synthase (nNOS) and CD34. The number of neurites per smooth muscle unit of the circular muscle layer (CML) was counted in the longitudinal sections. TUJ1 was the best marker to detect whole neuritic networks of the enteric nervous system. There were differences in the innervation patterns between isolated rectosigmoid aganglionosis (RS) and long segment aganglionosis (LS) including total colonic aganglionosis and extensive aganglionosis. In the aganglionic bowel (AGB) of LS, no nerve fibers innervated smooth muscle units in the CML in the area from the small bowel to the terminal descending colon. In the rectosigmoid region of every type of isolated HSCR, we observed transmural nerve fibers forming meshworks in the CML with TUJ1 and S100 antibodies. In RS, the neurites running parallel with smooth muscle cells gradually decreased in number in the distal portion. However, in the rectosigmoid AGB in LS, those neurites were absent and most neurites perpendicularly crossed the CML. Hypertrophic nerve trunks (HNT) in the submucous and myenteric plexuses were observed more frequently in the rectosigmoid region than in the rostral portion. Based on these data, it is suggested that the neuritic meshworks in the CML of the rectosigmoid AGB might derive from not only the sacral plexus, via HNT, but also intrinsic neurons in the oligoganglionic bowel. All of the syndromic HSCR were RS. In the AGB of RS with Down syndrome, the distribution of neurite meshworks in the CML is markedly reduced. In the AGB of RS with mental retardation suspected of having Mowat–Wilson syndrome, the density of intramuscular innervation was comparatively higher. In the rostral portion to the AGB of syndromic HSCR, myenteric ganglia were clearly small in size, and more numerous per smooth muscle unit with scarce internodal strands. These dysplastic features fall under neither hyperganglionosis nor hypoganglionosis classifications. We considered that syndromic HSCR might occur on the basis of a dysplastic enteric nervous system caused by genetic alteration.

Background. Histology after intensive induction chemotherapy is expected to become a beacon indicating when and how extensively radical surgery and lymph node dissection should be performed in advanced neuroblastoma. A thorough histologic review of surgical specimens was undertaken. Procedure. All specimens from 34 patients who were pretreated intensively (>= 3 cycles) with recent chemotherapy were reviewed. Thirty patients were > 12 months of age with stage 3/4 disease, and 4 were < 12 months of age but with MYCN-amplified stage 4 diseases. After 3 to 7 cycles (mean, 4.3 cycles) of induction chemotherapy, patients underwent radical surgery of the primary tumor and lymph nodes in all retroperitoneal sections. A single pathologist reviewed all of the specimens, and histologic chemotherapeutic effects were graded as: (+++), < 1% viable tumor;(++), 1%-10% viable tumor;(+), 11%-50% viable tumor; (+/-), 51%-90% viable tumor; and (-), > 91% viable tumor. Results. Grade (+++) effects were observed in 56% of patients treated with the new regimens, whereas grade (+++) was seen in only 20% treated with regimens before 1991. Operation time and blood loss were 7 hr and 6 min (P=0.087) and 646 ml (P=0.064), respectively, in patients with > 5 cycles (mean, 5.3 cycles) of chemotherapy, while they were 7 hr and 50 min and 1,168 ml, respectively, in those with approximately 3 cycles (mean, 3.2 cycles). Histologically, metastases were found in the contralateral nodes beyond the aorta in 92% of those whose tumor originated on the left, and in 80% of those with tumors occurring on the right. Conclusions. Five cycles of induction chemotherapy did not improve histologic chemotherapeutic effects, but helped to facilitate a shorter operation time and less blood loss than 3 cycles of chemotherapy. Surgery after 5 cycles Of (98)A(3) also appears to be easier to perform than that after 3 cycles of A(1)/new A(1). Only 14% of the children treated before 1985 with the St. Jude protocols experienced grade (+++) chemotherapeutic effects, and 22% of the patients treated before 1991 with regimen A(1), or new A(1) of the Study Group of Japan showed grade (+++) effects, whereas 56% of the patients treated after 1991 with either regimen A(3) or (98)A(3) exhibited grade (+++) chemotherapeutic effects. Histologic chemotherapeutic effects were roughly parallel with a good prognosis.