Successful treatment of Neuroblastoma in an adolescent with intra-arterial embolization before surgery

Abstract Introduction Neuroblastoma in the adolescent is characterized by indolent growth and poor outcome. Surgical resection of the tumor is an essential part of the multimodality treatment. Surgical complications depend on the presence of Image Defined Risk Factors (IDRFs). Methods We present an adolescent with pelvic neuroblastoma and epidural compression. To facilitate tumor resection, the patient underwent preoperative selective embolization. Results After selective embolization a subsequent complete resection etraspinal localisation was performed without complication with complete remission after 2 years. Conclusions Preoperative embolization is a safe and feasible technique that can help pediatric surgical oncologist to reduce complications IDRFs-related

Multiple endocrine neoplasias type 2B and RET proto-oncogene

Multiple Endocrine Neoplasia type 2B (MEN 2B) is an autosomal dominant complex oncologic neurocristopathy including medullary thyroid carcinoma, pheochromocytoma, gastrointestinal disorders, marphanoid face, and mucosal multiple ganglioneuromas. Medullary thyroid carcinoma is the major cause of mortality in MEN 2B syndrome, and it often appears during the first years of life. RET proto-oncogene germline activating mutations are causative for MEN 2B. The 95% of MEN 2B patients are associated with a point mutation in exon 16 (M918/T). A second point mutation at codon 883 has been found in 2%-3% of MEN 2B cases. RET proto-oncogene is also involved in different neoplastic and not neoplastic neurocristopathies. Other RET mutations cause MEN 2A syndrome, familial medullary thyroid carcinoma, or Hirschsprung's disease. RET gene expression is also involved in Neuroblastoma. The main diagnosis standards are the acetylcholinesterase study of rectal mucosa and the molecular analysis of RET. In our protocol the rectal biopsy is, therefore, the first approach. RET mutation detection offers the possibility to diagnose MEN 2B predisposition at a pre-clinical stage in familial cases, and to perform an early total prophylactic thyroidectomy. The surgical treatment of MEN 2B is total thyroidectomy with cervical limphadenectomy of the central compartment of the neck. When possible, this intervention should be performed with prophylactic aim before 1 year of age in patients with molecular genetic diagnosis. Recent advances into the mechanisms of RET proto-oncogene signaling and pathways of RET signal transduction in the development of MEN 2 and MTC will allow new treatment possibilities

Tunable Schottky barrier and high responsivity in graphene/Si-nanotip optoelectronic device

We demonstrate tunable Schottky barrier height and record photo-responsivity in a new-concept device made of a single-layer CVD graphene transferred onto a matrix of nanotips patterned on n-type Si wafer. The original layout, where nano-sized graphene/Si heterojunctions alternate to graphene areas exposed to the electric field of the Si substrate, which acts both as diode cathode and transistor gate, results in a two-terminal barristor with single-bias control of the Schottky barrier. The nanotip patterning favors light absorption, and the enhancement of the electric field at the tip apex improves photo-charge separation and enables internal gain by impact ionization. These features render the device a photodetector with responsivity (3 A/W for white LED light at 3 mW/cm2 intensity) almost an order of magnitude higher than commercial photodiodes. We extensively characterize the voltage and the temperature dependence of the device parameters and prove that the multi-junction approach does not add extra-inhomogeneity to the Schottky barrier height distribution. This work represents a significant advance in the realization of graphene/Si Schottky devices for optoelectronic applications.Comment: Research paper, 22 pages, 7 figure

Surgical Advances in Paediatric Neuroblastoma

As an introduction to this “Special Issue” launched by Children (ISSN 2227-9067), I would firstly like to highlight those neoplasms should be labeled with the more appropriate term “Neuroblastomas” (NBs) [...

Genetics of anorectal malformations.

Anorectal malformations (ARM) present an incidence rate ranging from 1:1,500 to 1:5,000 live births, and have variable clinical presentations ranging from mild forms that might require only minor surgical interventions to more complicated cases that need to be managed with multi-staged operations

Ingested magnets.

A 9-year-old boy ingested 23 magnets . Four days later, he had clinical and surgical evidence of intestinal perforation and peritonitis due to pressure necrosis of the bowel. In an unrelated incident, a developmentally delayed 13-year-old boy ingested 15 magnets. Ten days later, volvulus and intestinal occlusion developed. Both patients were operated on without complications, and all magnets were removed. Although ingested nonmagnetic foreign bodies are likely to be passed spontaneously without consequence, ingested magnets may attract each other through the intestinal wall and cause severe damage, such as pressure necrosis, perforation, intestinal fistulas, volvulus, and obstruction. Thus, close observation and early intervention are warranted after ingestion of magnets