[Pediatric Multiple Endocrine Neoplasia Type 2B with Pheochromocytoma Diagnosed after Perforation of a Colonic Diverticulum : A Case Report].

A 12-year-old girl was found to have decending colon diverticulum perforation and retroperitoneal abscess on computed tomography (CT) carried out to determine the cause of fever and stomachache. CT-guided drainage tube placement was performed. She was suspected of having MEN2B from her specific facial appearance, Marfan-like body shape and lingual mucosa neuroma. Cervical ultrasonography and serum tumor marker revealed medullary thyroid carcinoma and metastasis to cervical lymph node. Genetic examination revealed a mutation of RET gene codon 918. Therefore, she was diagnosed as having MEN2B. Laboratory data showed elevated urinary catecholamines. Metaiodobenzylguanidine (MIBG) adrenal scintigraphy showed bilateral adrenal uptake and a definitive diagnosis of bilateral adrenal pheochromocytomas was made. Discharge from the drainage tube persisted and it was difficult to continue conservative treatment. Therefore, laparoscopic bilateral adrenalectomy and transverse colon colostomy were performed. Subsequently, total thyroidectomy and cervical lymph node dissection were performed. At five years of follow up, bilateral lung metastases were observed, but the serum calcitonin level was normal and the patient is under observation.

Ethanol lock therapy in pediatric patients: A multicenter prospective study.

BACKGROUND: Ethanol lock therapy (ELT) has been performed for the purpose of preserving central venous catheters (CVC) in central venous catheter-related blood stream infection (CRBSI), but evidence for its effectiveness is not established. We conducted a multicenter, prospective study on the ELT protocol to ascertain its safety and effectiveness against CRBSI. METHODS: The subjects were patients aged over 1 year with potential for developing CRBSI who had long-term indwelling silicone CVCs. After culturing the catheterized blood, a 70% ethanol lock was performed daily for 2-4 h for 7 days. The effectiveness rate of ELT for single and multiple courses, the presence or absence of relapse of CRBSI within 4 weeks of treatment, and whether the CVC could be salvaged after 4 weeks were examined. RESULTS: From September 2014 to August 2018, 49 cases from six hospitals were enrolled in the study. Catheter blockage was seen in one case and the CVC was removed. A single course of ELT was effective in episodes 88% (42/48). In the remaining three episodes that failed after a single course of ELT, a second ELT was performed; however, all were ineffective. In episodes 93% (40/42), no CRBSI relapse was seen up to 4 weeks after the end of treatment. In episodes 84% (41/49), the catheter could be preserved for 4 weeks or more after the end of treatment. Facial flushing was seen in two cases as an adverse event; however, this was transient and soon disappeared. CONCLUSION: ELT is effective for 88% of CRBSI and 84% of catheters can be salvaged; therefore, this protocol is considered useful. TRIAL REGISTRATION: UMIN000013677.

A case of pediatric virilizing adrenocortical tumor resulting in hypothalamic-pituitary activation and central precocious puberty following surgical removal.

We present a 6-year-old boy with a virilizing adrenocortical tumor who initially presented with peripheral precocious puberty. Development of facial acne, pubic hair and a growth spurt were noted at the age of five. A low-pitched voice as well as maturation of external genitalia was noted at the age of six. Both serum and urinary levels of adrenal androgens were elevated. Abdominal computed tomography revealed a large right suprarenal mass and he underwent surgical resection without any complications. The histological diagnosis was adrenocortical carcinoma according to the criteria of Weiss. Following surgical removal of the androgen-producing tumor, the patient subsequently developed hypothalamic-pituitary activation and demonstrated central precocious puberty. He was treated with a gonadotropin-releasing hormone agonist in order to delay further pubertal progression. Clinical follow-up of potential secondary effects of excess hormone secretion after removal is important in some pediatric patients with virilizing adrenocortical tumor.