Transurethral resection for botryoid bladder rhabdomyosarcoma

The outcome of multimodal therapy for localized bladder rhabdomyosarcoma is quite good in terms of morbidity, and conservative surgery is generally recommended. However, in cases originating in the bladder neck, tumorectomy or partial cystectomy has adverse effects on bladder function. A 2-year-old girl underwent transurethral resection of a bladder tumor (TUR-BT), chemotherapy consisting of vincristine, actinomycin-D, and cyclophosphamide, and radiotherapy. She was in remission for 3 years when frequent urination became evident. Her bladder capacity and compliance were low; however, her urinary symptom was controlled using anticholinergic medication. Accordingly, TUR-BT could be an optional approach for bladder rhabdomyosarcoma

Amylase Levels Are Useful for Diagnosing Omphalomesenteric Cysts: A Case Report

Omphalomesenteric cysts are an exceedingly rare type of embryologic remnant of the omphalomesenteric duct. Owing to its rarity and unspecific imaging findings, it is occasionally difficult to diagnose preoperatively. Herein, we report the case of a 15-month-old female with an omphalomesenteric cyst that presented as a painful abdominal mass. Imaging showed a 4 cm cystic lesion just beneath the umbilicus, which also contained a 1 cm enhanced lesion. On the immediate right side of this cyst, a 7 cm hematoma was found within the abdominal wall. Aspiration revealed bloody fluid with an amylase level of 38,250 U/L. She was then diagnosed with an omphalomesenteric cyst, with aberrant pancreas and associated hematoma of the abdominal wall. These findings were confirmed with laparotomy and subsequent pathological examinations. The high level of amylase in the cyst led us to speculate the existence of ectopic pancreatic tissue. Thus, amylase measurement may be considered for the diagnosis of an omphalomesenteric cyst

Larger Physique as a Risk Factor for Infantile Appendicitis: A Retrospective Study

The clinical features and risk factors of acute appendicitis in infants are unclear. Our aim was to evaluate the association between anthropometrics and the occurrence of infantile appendicitis. This was a retrospective study of infants (<6 years of age) and school-age children (6–10 years of age) of Asian ethnicity who required hospitalization for appendicitis at our two participating institutions between 2004 and 2018. The Z-score for height, body weight, and body mass index (BMI) was compared between the two groups, as well as between patients presenting with perforated and non-perforated appendicitis. The analysis included data from 73 infants and 362 school-age children. Z-scores were greater in infants than in school-age children for height (0.37 versus −0.03, p = 0.003) and body weight (0.12 versus −0.36, p = 0.023), with no between-group difference for the Z-score of BMI. There was no difference in Z-scores for height, weight, and BMI between the perforated and non-perforated appendicitis infant groups. Infants presenting with acute appendicitis were characterized by a larger physique but with normal proportion. This trend was not observed in school-age children. Therefore, larger infants presenting with abdominal pain should be screened for appendicitis

Malignant rhabdoid tumor of the liver: a case report and literature review

Malignant rhabdoid tumor (MRT) is a rare and aggressive malignancy associated with poor outcomes. MRT of the liver is even rarer, and little information has been described. We report the case of an 8-month-old boy with MRT of the liver. The tumor showed aggressive progression despite a multidisciplinary approach, and the patient died due to multiple organ failure 14 days after admission. Autopsy revealed the liver tumor and multiple metastases with negative immunohistochemistry for INI1/BAF47. A review of 53 cases of pediatric MRT of the liver is provided

Combined Use of Three-Dimensional Construction and Indocyanine Green-Fluorescent Imaging for Resection of Multiple Lung Metastases in Hepatoblastoma

It is essential to accurately and safely resect all tumors during surgery for multiple lung metastases. Here, we report a case of hepatoblastoma (HB) with multiple pulmonary nodules that ultimately underwent complete resection using combined three-dimensional image reconstruction and indocyanine green (ICG) fluorescence guidance. A 1-year-old boy was diagnosed with HB and multiple lung metastases. After intensive chemotherapy, complete resection with subsegmentectomy (S5 + 6) and partial resection (S3, S8) were performed. More than 100 pulmonary nodules, which remained visible on computed tomography (CT) despite additional postoperative chemotherapy, were subjected to pulmonary resection. We used the SYNAPSE VINCENT software (Fujifilm Medical, Tokyo, Japan) to obtain three-dimensional images of the nodules. We numbered each nodule, and 33 lesions of the right lung were resected by multiple wedge resections through a right thoracotomy, with the aid of palpation and ICG fluorescence guidance. One month after the right metastasectomy, resection of 64 lesions in the left lung was performed via left thoracotomy. Postoperative CT showed complete clearance of the lung lesions, and the patient remained disease-free for 15 months after the treatment. This case study confirms that the combination of three-dimensional localization and ICG fluorescence guidance allows for accurate and safe resection of nearly 100 lung metastases

The prognostic evaluation of marginal positive resection in hepatoblastoma: Japanese experience

Aim: In the Japanese study group for Pediatric Liver Tumor (JPLT) studies, the survival of patients with hepatoblastoma (HB) was improved by cisplatin/pirarubicin-based chemotherapy with combined surgical resection. We aimed to clarify whether marginal positive resection is correlated with the prognosis of HB patients from the JPLT-2 study (1999-2012).Methods: Of the 361 JPLT-2 patients, we excluded 4 who died before surgery, 14 inoperable following preoperative chemotherapy, and 6 macroscopically positive resections and analyzed local recurrence and survival rates in 337 patients who underwent primary resection including liver transplantation.Results: The five-year event-free survival (EFS) and overall survival (OS) rates were 76.0% and 87.7% in patients (n = 312) with complete resection of their primary tumors and 59.1% and 83.0% in those (n = 25) with microscopically margin-positive resection (microMPR), respectively. Among patients without distant metastasis, the five-year EFS and OS rates were 81.4% and 90.8% in those (n = 263) with complete resection vs. 62.5% and 90.9% in those (n = 22) with microMPR, respectively. The EFS, but not OS, was significantly lower (P < 0.05) in patients with microMPR vs. complete resection. The local recurrence rate was significantly different (chi-square = 12.11, P < 0.01) between the two groups.Conclusion: In patients administered cisplatin/pirarubicin-based chemotherapy, the presence of microMPR influenced local recurrence but not outcome. Advance of liver surgery including LT correlated with improving of resection rates. The presence of microMPR influenced the local recurrence but not the outcome in the JPLT-2 study. The outcome of patients with microMPR might depend on the postoperative treatment and/or tumor biology rather than occurrence of recurrence

Acute pancreatitis caused by a duodenal duplication cyst covering the ampulla of Vater

Duodenal duplication cyst is not a common congenital anomaly and the pathophysiology may become very complicated if the cyst is situated at the ampulla of Vater. Here we report a very rare female case of duodenal duplication cyst at the ampulla of Vater, which caused acute pancreatitis due to massive protein plaques in the pancreatic duct. She had a past history of double duodenal atresia and underwent surgery as a neonate. The correct diagnosis could not be determined before the second operation at four years of age and the exact pathophysiology finally became apparent during the operation with a contrast medium study and duodenotomy. We discuss the complicated clinical features and diagnostic and treatment procedures before and during the operation