A large retroperitoneal lymphatic malformation successfully treated with traditional Japanese Kampo medicine in combination with surgery

BackgroundCurrent treatment options for lymphatic malformations (LMs) are multimodal. Recently, the effectiveness of treating LMs with Eppikajyutsuto (TJ-28) has been reported. TJ-28 is a kind of oral herbal medicine classified as the traditional Japanese Kampo medicine.Case presentationA 12-year-old girl was admitted to our hospital for intermittent upper abdominal pain. Radiological examinations revealed a large (9.5 × 5.8 × 10.0 cm) retroperitoneal LM, which was suspected to adhering and stretching both pancreas head and duodenum. The large retroperitoneal tumor resection might induce involving complications because of the size and the location. Therefore, we used TJ-28 in order to diminish the tumor size before surgery. The patient received oral doses of 7.5 g/day (2.5 g × 3 times/day) of TJ-28. Six months after the medication, the tumor decreased markedly to 3.5 × 1.5 × 1.2 cm in size. Thereafter, the mass was sub-totally resected (95%) via a 3 cm trans-umbilical incision without any surgical complications.ConclusionsWe reported a case of successfully treated retroperitoneal LM with the combination treatment of TJ-28 and surgery. Based on our experience, this TJ-28 treatment option may be very useful in treating cases of LMs having surgical difficulties because of size and/or location

Proton beam therapy with concurrent chemotherapy is feasible in children with newly diagnosed rhabdomyosarcoma

BACKGROUND: The optimal treatment for rhabdomyosarcoma (RMS) requires multidisciplinary treatment with chemotherapy, surgery, and radiotherapy. Surgery and radiotherapy are integral to the local control (LC) of RMS. However, postsurgical and radiotherapy-related complications could develop according to the local therapy and tumor location. In this study, we conducted a single-center analysis of the outcomes and toxicity of multidisciplinary treatment using proton beam therapy (PBT) for pediatric RMS. MATERIALS AND METHODS: RMS patients aged younger than 20 years whose RMS was newly diagnosed and who underwent PBT at University of Tsukuba Hospital (UTH) during the period from 2009 to 2019 were enrolled in this study. The patients’ clinical information was collected by retrospective medical record review. RESULTS: Forty-eight patients were included. The 3-year progression-free survival (PFS) and overall survival (OS) rates of all the patients were 68.8% and 94.2%, respectively. The 3-year PFS rates achieved with radical resection, conservative resection, and biopsy only were 65.3%, 83.3%, and 67.6%, respectively (p = 0.721). The 3-year LC rates achieved with radical resection, conservative resection, and biopsy only were 90.9%, 83.3%, and 72.9%, respectively (p = 0.548). Grade 3 or higher mucositis/dermatitis occurred in 14 patients. Although the days of opioid use due to mucositis/dermatitis during the chemotherapy with PBT were longer than those during the chemotherapy without PBT [6.1 and 1.6 (mean), respectively, p = 0.001], the frequencies of fever and elevation of C-reactive protein were equivalent. CONCLUSIONS: Multidisciplinary therapy containing PBT was feasible and provided a relatively fair 3-year PFS, even in children with newly diagnosed RMS without severe toxicity

A rare mechanism of delayed splenic rupture following the nonoperative management of blunt splenic injury in a child

BackgroundNonoperative management (NOM) has been established as the standard treatment for isolated blunt organ injury in hemodynamically stable pediatric patients. Although delayed splenic rupture or bleeding is a rare complication in NOM, it is an issue that many pediatric surgeons are greatly concerned about. We herein report a rare pediatric case concerning the mechanisms involved in delayed splenic rupture after NOM.Case presentationA 9-year-old boy with severe abdominal pain was transferred to our hospital. Twenty-one hours before the admission, he had been kicked in the region of his left lateral abdomen. Contrast-enhanced abdominal computed tomography revealed a severe intra-parenchymal hematoma and multiple lacerations of the spleen with a large amount of hemoperitoneum without active bleeding. His condition was diagnosed as a grade III injury on the AAST splenic injury scale. After fluid resuscitation, his vital signs became stable. The patient was treated with NOM in our intensive care unit. However, suddenly after defecation (72 h after the injury), he started complaining of severe abdominal pain and left shoulder pain. His blood pressure dropped to 70/35 mmHg, and he started to lose consciousness. Abdominal ultrasonography (US) revealed increased ascites. Fluid resuscitation and blood transfusion were performed. His symptoms and abdominal US findings suggested that splenic re-bleeding had caused delayed splenic rupture to occur. Emergency splenectomy was performed. The resected spleen was enlarged with a large parenchymal hematoma. The posterior-lateral side of the splenic capsule was ruptured.ConclusionsThe mechanism of delayed splenic rupture in our case was considered to be the result of a tear in the subcapsular hematoma caused by stretching the splenocolic ligament related to a bowel movement during defecation. Although delayed splenic rupture or bleeding is unpredictable, it is very important to understand the mechanisms and to educate the family of the children with splenic injuries of the warning signs of delayed rupture or bleeding

A case of HER-2-positive recurrent breast cancer showing a clinically complete response to trastuzumab-containing chemotherapy after primary treatment of triple-negative breast cancer

We report a case of HER-2-positive recurrent breast cancer showing a clinically complete response to trastuzumab-containing chemotherapy 6 years after primary treatment of triple-negative breast cancer. The primary tumor was negative for HER-2 as determined by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) (1+, and ratio, 1.1), but examination of the recurrent lymph node metastasis showed positivity for HER-2 by FISH (ratio, 5.2). No lesions were detected in either her left breast or in other organs, and the patient was diagnosed as having HER-2-positive recurrent disease. Combination chemotherapy using weekly paclitaxel and trastuzumab was initiated, and a clinically complete response was achieved. This report suggests the benefit of routine evaluation of HER-2 status in recurrent breast cancer with the introduction of HER-2-targeting agents

Analysis of risk factors of long-term complications in congenital diaphragmatic hernia: A single institution\u27s experience

ObjectiveTo establish better management practices to reduce morbidities in survivors with congenital diaphragmatic hernia (CDH).MethodsOf 60 patients treated for CDH at our institution between 1991 and 2011, 49 patients without severe anomalies were retrospectively reviewed.ResultsSince 2004, gentle ventilation (GV) has been the main treatment for CDH. Patients were divided into the following two groups: the non-GV group (n = 29) who were treated before GV treatment was implemented, and the GV group (n = 20). The overall survival rate was 62.1% (18/29) and 95% (19/20) in the non-GV and GV groups, respectively (p = 0.016). Despite the high survival rate, the incidence of long-term complications in survivors was still high (14/19, 73.7%) in the GV group. In the GV group, liver-up (p = 0.106) and the need for patch repair (p = 0.257) tended to be associated with the development of long-term complications, but did not reach statistical significance. The presence of perioperative complications was associated with the development of long-term complications (p = 0.045) in the GV group.ConclusionPatients who developed short-term complications seemed to be at risk of long-term complications. Therefore, to minimize long-term morbidities in CDH survivors, the prevention of short-term complications might be important

Hypergastrinemia and a duodenal ulcer caused by gastric duplication

BackgroundHypergastrinemia and the resultant peptic ulcer related to an enteric duplication has been quite rarely reported in the literature.Case presentationWe herein report the case of a 4-year-old girl who presented with hypergastrinemia and a duodenal ulcer at 2 years of age. She had been followed up with a proton pump inhibitor, which resulted in resolution of the ulcer; however, unexplained hypergastrinemia had continued. A cystic lesion at the antrum was discovered at 4 years of age, which we suspected to be a gastric duplication. After we resected the lesion, the hypergastrinemia resolved without recurrence of the duodenal ulcer. The histology was compatible with a gastric duplication, and the lumen was lined with antral mucosa that strongly stained positive for gastrin. We presumed that the antral mucosa inside the duplication in our case had no hydrogen ion feedback inhibition of gastrin release from gastrin cells and increased release of gastrin from the mucosa inside the duplication led to the duodenal ulcer. Only two cases have been reported in the literature that had hypergastrinemia related to enteric duplication.ConclusionGastric duplication should be included in the differential diagnosis of sustained hypergastrinemia in children

Colonic volvulus detected by CT scan in a case with mental retardation and prune belly syndrome

Colonic volvulus is a rare disease in children. Delayed diagnosis of the condition can often be fatal, especially in pediatric patients with mental retardation. We herein present the case of a female pediatric patient with colonic volvulus, prune belly syndrome, and mental retardation. Preoperative CT scans showed the characteristic signs of this disease. The volvulus occurred in the proximal colon of the colostomy. The release of the colonic volvulus and reconstruction of the colostomy were performed without the resection of the ischemic colon. The postoperative clinical course was uneventful

Colonic volvulus detected by CT scan in a case with mental retardation and prune belly syndrome

Colonic volvulus is a rare disease in children. Delayed diagnosis of the condition can often be fatal, especially in pediatric patients with mental retardation. We herein present the case of a female pediatric patient with colonic volvulus, prune belly syndrome, and mental retardation. Preoperative CT scans showed the characteristic signs of this disease. The volvulus occurred in the proximal colon of the colostomy. The release of the colonic volvulus and reconstruction of the colostomy were performed without the resection of the ischemic colon. The postoperative clinical course was uneventful