Closed spinal dysraphism (CSD) encompasses a heterogeneous group of spinal cord deformities, which can be accompanied by several types of skin stigmata. These skin stigmata may include inconspicuous features, such as sacral dimples and deformed gluteal clefts, but the association between such mild skin stigmata and CSD is uncertain. This study aimed to reevaluate the indication for magnetic resonance imaging (MRI) in patients with skin stigmata while considering the indication for surgery. A retrospective analysis was conducted on magnetic resonance images of 1255 asymptomatic children with skin stigmata between 2003 and 2015. Skin stigmata classification was based on medical chart data. All subtypes of CSDs except for filum terminale lipomas (FTL), FTL thicker than 2 mm or with low conus medullaris, were considered to meet the surgical indication. CSD prevalence was estimated while considering the surgical indications and assessed after excluding all FTL cases. Skin stigmata were classified into seven types, dimple, deformed gluteal cleft, hair, subcutaneous mass, appendage, discoloration, and protruding bone, and included 1056 isolated and 199 complex ones. The prevalence of CSD was 19.5%, 6.8%, and 0.5% among patients with isolated dimples (n = 881) and 13.9%, 5.8%, and 0.7% among those with isolated deformed gluteal clefts (n = 136) for all cases, surgical indications, and patients without FTL, respectively. Dimples and deformed gluteal clefts had a low prevalence of CSD requiring surgical intervention, and cases without FTL were rare. Asymptomatic patients with mild skin stigmata may not require immediate MRI.
Neuroblastoma is the most common extracranial solid tumor in children. When metastasis to the falx cerebri is present, it is classified as stage M; however, its behavior has not been well characterized. Here we present a case of stage M infantile neuroblastoma (NB) with involvement of the falx cerebri, and also summarize the clinical profiles of previously reported cases. Notably, all of the tumors resolved with low-dose chemotherapy alone. Although further study is needed to distinguish NBs presenting at these different intracranial locations, NB with metastasis to the falx cerebri may be categorized as MS when diagnosed at less than 18 months of age.
Pleuropulmonary blastoma (PPB) is a rare tumor that arises from mesenchymal cells of the lung or pleura. PPB is classified as type I (cystic), type Ir (regressed), type II (cystic and solid), or type III (solid). Here, we report the case of a 2-year-old boy with type II PPB. The patient was treated with neoadjuvant chemotherapy, and the tumor was completely surgically resected. A DICER1 mutation was detected in the tumor specimen. The risk of recurrence is high, and long–term follow–up is required.
A 7-year-6-month-old female was diagnosed with a pelvic malignant peripheral nerve sheath tumor and lymph node metastases. Tumorectomy was performed after four cycles of chemotherapy. A 33-mm cystic lesion was observed around the left iliac muscle after three cycles of postoperative chemotherapy, and proton beam therapy (PBT) was recommended. She was referred for absorbable spacer (AS) placement. The left ovarian appendage (OA) was resected due to the direct tumor infiltration. The right OA was fixed to the uterosacral ligament. The AS was fixed to the lateral pelvis. The PBT (70.3 Gy relative biological effectiveness) was performed successfully with the AS, and she also had the reproducing possibility due to prevention of severe irradiation damage of the right OA. AS eliminated the surgical removal of spacers and enabled us high-dose PBT for residual tumor without severe irradiation damage including infertility.
Pseudo-Meigs syndrome (PMS) and gliomatosis peritonei (GP) are rare conditions associated with ovarian teratomas. We report the case of an 11-year-old female patient suffering from immature ovarian teratoma with PMS and GP. Enhanced computed tomography (CT) revealed a tumor measuring 240 × 150 × 100 mm in the lower abdomen, ascites, and right pleural effusion. Before the operation, the right pleural effusion increased and required chest tube drainage. Right adnexectomy was performed, and peritoneal nodules were detected during surgery. A biopsy was performed and pathological examination revealed a right immature ovarian teratoma (Grade 3) and glial tissue (Grade 0), thus confirming the diagnosis of GP. Postoperatively, ascites and pleural effusion disappeared, confirming the diagnosis of PMS. Eight months after the operation, a metachronous contralateral mature ovarian teratoma developed, for which fertility-sparing surgery was performed. Therefore, long-term follow-up is required in this patient.
Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors that arise from chromaffin cells. We report the case of an 11-year-old boy with no history of hypertension who presented with an abdominal mass arising from the retroperitoneum. During laparoscopic surgery, the patient developed a hypertensive crisis with a peak systolic blood pressure of over 270 mmHg, although we were able to continue to perform laparoscopic resection. Even if no concomitant symptoms due to catecholamine production are observed before surgery, as in this case, blood pressure may fluctuate during surgery. Intraoperative and postoperative management should be considered in cooperation with anesthesiologists.
A two-year-old girl presented with an abdominal mass, abdominal pain, and fever. A quail egg-sized elastic-hard mass was palpable in the upper left abdomen. The patient's growth was impaired. Laboratory data showed an inflammatory response and elevated pancreatic enzymes. The mass showed a cystic structure adjacent to the caudal side of the bifid tail of the pancreas. A fluid collection was observed around the spleen. Surgery was planned under the diagnosis of pancreatic foregut cystic malformation (FCM) associated with a bifid pancreatic tail. However, as the inflammation worsened, parenteral nutrition (PN) was introduced and cyst drainage was performed first, obtaining 10–14 mL/day, and her symptoms subsided. Cyst drainage continued for six weeks at home. Laparoscopic surgery was performed 47 days after the initiation of drainage. Cystography showed communication between the cyst and the slightly dilated pancreatic duct of the bifid tail during surgery. A partial pancreatectomy with cystectomy was performed. The diagnosis of FCM originating from the pancreas, associated with the bifid tail, was confirmed. The postoperative course was uneventful, and the patient was discharged eight days post-surgery. Pancreatic FCM with a bifid pancreatic tail is rare, and we successfully treated it using minimally invasive surgery.