Concomitant variants in NF1, LZTR1 and GNAZ genes probably contribute to the aggressiveness of plexiform neurofibroma and warrant treatment with MEK inhibitor.

Neurofibromatosis 1 (NF1) is caused by germline mutations in the NF1 gene and manifests as proliferation of various tissues, including plexiform neurofibromas. The plexiform neurofibroma phenotype varies from indolent to locally aggressive, suggesting contributions of other modifiers in addition to somatic loss of NF1. In this study, we investigated a life-threatening plexiform neurofibroma in a 9-month-old female infant with NF1.

Polypharmacy among pediatric cancer patients dying in the hospital.

Background: Decisions on medication treatment in children dying from cancer are often complex and may result in polypharmacy and increased medication burden. There is no information on medication burden in pediatric cancer patients at the end of life (EOL).

Objectives: To characterize medication burden during the last hospitalization in children dying from cancer.

Ultrasound-guided core biopsy with on-site cytology-immediate diagnosis in pediatric oncology.

Background: Accurate and swift tissue diagnosis is extremely important for the timely initiation of treatment in pediatric oncology. In our department, ultrasound-guided core needle biopsy (US-guided CNB) is used for tissue diagnosis. In 2016, we added on-site cytology, allowing for an immediate primary diagnosis.

Ultrasound-Guided Core Biopsy for Tissue Diagnosis in Pediatric Oncology: 16-Year Experience With 597 Biopsies.

Percutaneous imaging-guided core needle biopsies (CNBs) for cancer diagnosis in pediatric patients are gaining interest because of their availability, lower rate of complications, and high diagnostic power compared with traditional surgical biopsies. Nevertheless, their precise role in the diagnostic algorithm of pediatric oncology is still unknown. The purpose of this study was to report our accumulated 16-year experience with CNB; discuss the availability, safety, and diagnostic accuracy of the procedure and the adequacy of ancillary testing; and compare our findings with the available literature.

Rare haematogenous sarcoma metastasis to the heart in a child.

Haematogenous non-contiguous metastatic spread of remote solid tumours to the heart is rare. We describe a previously healthy 5-year-old girl who presented with extensive intracardiac involvement by metastatic pelvic sarcoma.

Medical marijuana use for pediatric oncology patients: single institution experience.

Medical marijuana (MM) is widespread in many medical fields, including oncology, with limited use in pediatric oncology where research is scarce and often shows conflicting results. This research focuses on alleviating side effects of anticancer treatment as an integral part of supportive and palliative care of children with cancer. We report our experience with MM treatment in 50 children, adolescents, and young adults with different types of cancer during 2010-2017.

Correction to: Human Development Index and its association with staff spiritual care provision: a Middle Eastern oncology study.

Incorrect family name of Layth Mula-Hussain.

Human Development Index and its association with staff spiritual care provision: a Middle Eastern oncology study.

Background: Although staff spiritual care provision plays a key role in patient-centered care, there is insufficient information on international variance in attitudes toward spiritual care and its actual provision.

Methods: A cross-sectional survey of the attitudes of Middle Eastern oncology physicians and nurses toward eight examples of staff provision of spiritual care: two questionnaire items concerned prayer, while six items related to applied information gathering, such as spiritual history taking, referrals, and encouraging patients in their spirituality. In addition, respondents reported on spiritual care provision for their last three advanced cancer patients.

Factors Influencing Do-Not-Resuscitate Status in Children During Last Month of Life: Single Institution Experience.

Background: It is currently expected that about 20% of children with cancer will ultimately die. Writing advanced life directives sufficiently long before the actual death of a child ensues allows both parents and medical staff to develop optimal treatment plans in the best interests of the child.

Aim Of The Study: The aim of the study was to evaluate factors that may influence the process of decision-making regarding Do-Not-Resuscitate (DNR) status.

Training for awareness of one's own spirituality: A key factor in overcoming barriers to the provision of spiritual care to advanced cancer patients by doctors and nurses.

Objective: When patients feel spiritually supported by staff, we find increased use of hospice and reduced use of aggressive treatments at end of life, yet substantial barriers to staff spiritual care provision still exist. We aimed to study these barriers in a new cultural context and analyzed a new subgroup with "unrealized potential" for improved spiritual care provision: those who are positively inclined toward spiritual care yet do not themselves provide it.

Method: We distributed the Religion and Spirituality in Cancer Care Study via the Middle East Cancer Consortium to physicians and nurses caring for advanced cancer patients.

Medical students' attitudes towards participating in a palliative medicine course: A new specialty in Israel.

Background: Palliative medicine is a growing field in Israel, and its training program is still in process. The current study aimed to evaluate students' attitudes regarding a course in palliative care established in a division of oncology.

Method: Some 45 medical students in their 5th to 6th years participated in a one-week course on palliative care.

STAT3 gain-of-function mutations associated with autoimmune lymphoproliferative syndrome like disease deregulate lymphocyte apoptosis and can be targeted by BH3 mimetic compounds.

Autoimmune lymphoproliferative syndrome (ALPS) is typically caused by mutations in genes of the extrinsic FAS mediated apoptotic pathway, but for about 30% of ALPS-like patients the genetic diagnosis is lacking. We analyzed 30 children with ALPS-like disease of unknown cause and identified two dominant gain-of-function mutations of the Signal Transducer And Activator Of Transcription 3 (STAT3, p.R278H, p.

An 8-Year-Old Child with Malignant Deciduoid Mesothelioma of the Abdomen: Report of a Case and Review of the Literature.

Malignant mesothelioma is an uncommon tumor that usually arises in the pleural cavity of adults with a history of asbestos exposure. Less frequently, it appears in the peritoneum or other mesothelial surfaces. Deciduoid mesothelioma is a rare subtype that has been found at both sites.

Metastatic rhabdoid meningioma with BRAF V600E mutation and good response to personalized therapy: case report and review of the literature.

Rhabdoid meningioma is an aggressive phenotype of meningioma, associated with a poor prognosis. We present a very rare case of high-grade meningioma with rhabdoid features that eventually expressed in a coma state. Comprehensive genomic profiling using a Next Generation Sequencing (NGS) assay revealed three genomic alterations: activating BRAF mutation (V600E), loss of CDKN2A/2B, and APC I1307K.

Alkaline phosphatase level change in patients with osteosarcoma: its role as a predictive factor of tumor necrosis and clinical outcome.

Background: In osteosarcoma the histological response, measured by the percentage of tumor necrosis, constitutes one of the most significant predictive factors, with better survival in patients whose tumor necrosis is > or = 90%.

Objectives: To determine if the decrease rate of serum alkaline phosphatase (SAP) levels during the first month of neoadjuvant chemotherapy could serve as a predictive indicator of tumor necrosis and clinical outcome.

Methods: We analyzed the medical files of 53 osteosarcoma patients (19 females, 34 males) (median age 16 years, range 8-24); the disease was metastatic in 12 and localized in the other 41.

Association between religious and socio-economic background of parents of children with solid tumors and DNR orders.

Background: The influence of socio-economic and religious background on decisions made by parents of children with incurable cancer regarding DNR orders is not fully understood.

Procedure: A retrospective analysis of medical charts of patients who died between January 2000 and January 2011 was performed. The following data were sought: written evidence of DNR discussion with parents, religious background, educational level, monthly income.

Anaplastic ganglioglioma in the spinal cord: case report and literature review.

Anaplastic ganglioglioma (AGG) is a rare tumor. A PubMed database search yielded only a few case reports and fewer case series. An even rarer entity is AGG arising in the spinal cord.

Role of 18F-FDG PET/CT in the staging of pediatric peritoneal mesothelioma.

A 7-year-old girl with a 1-month history of diffuse abdominal pain underwent an ultrasound which showed a pelvic mass with multiple peritoneal implants and ascites. An US-guided core biopsy of one of the implants as well as a transrectal biopsy of the pelvic tumor showed pathological findings consistent with epithelioid mesothelioma. We describe the findings on (18)F-FDG PET/CT in pediatric peritoneal mesothelioma.

Ultrasound-guided core biopsy as the primary tool for tissue diagnosis in pediatric oncology.

Introduction: Traditionally in pediatric oncology, biopsies were incisional, with a recent alternative of percutaneous imaging-guided biopsies. In our department, ultrasound (US)-guided core biopsy is the first choice for tissue diagnosis in the pediatric population. We retrospectively reviewed our experience and assessed the accuracy rate, safety, and availability of the procedure.

Value of routine bone scans in patients with bone sarcomas before local treatment.

During modern treatment of children with bone sarcomas, the children undergo multiple diagnostic imaging procedures, including bone scan (BS) with Tc99m. The aim of the BS is to establish the metastatic status of the skeletal system at the time of initial diagnosis and thereafter. We retrospectively reviewed 85 medical charts of patients with osteosarcoma (n = 40) and Ewing sarcoma (n = 45) who had been treated in our department between 01.

Osteogenic sarcoma in a child with familial expansile osteolysis syndrome: an accidental association?

We present the first reported case of a child with familial expansile osteolysis syndrome (FEO) who developed osteogenic sarcoma (OS) of the iliac bone. A 17-year-old adolescent presented with pain and a mass on the left pelvis. He was from a family with several members who had been diagnosed with FEO, from which he also suffered.

Undifferentiated sarcoma of the thyroid in a child.

The most common malignant tumor of the thyroid is papillary carcinoma. Sarcoma of the thyroid is encountered very rarely; its therapy is complex and poses significant problems due to the problematic location of the tumor. A 14-year-old female was diagnosed with undifferentiated sarcoma of the thyroid and received combined therapy comprising surgery, chemo- and radiotherapy without significant side effects.