Role of radiation therapy for pediatric upper extremity extraskeletal osteosarcoma: A case series.

BACKGROUND: Extraskeletal osteosarcoma is an extremely rare disease, comprising less than 0.1% of all cancers diagnosed in the United States, of which less than 5% occur in the upper extremities. The management of two cases of pediatric upper extremity extraskeletal osteosarcoma is discussed. CASE DESCRIPTION: Two children initially noticed painless left upper extremity masses at the ages of 16 and 13, respectively. Following a period of several months, both lesions became symptomatic, necessitating operative intervention, which revealed giant cell-rich extraskeletal osteosarcoma; PET staging following gross total resection revealed no residual or metastatic disease in either patient. After extensive discussion with the patients and family, adjuvant chemotherapy was initiated for one patient, and adjuvant radiation therapy was initiated in both patients. CONCLUSIONS: Despite the rarity of these tumors, the importance of radiation therapy has been established by current and ongoing studies such as the Children's Oncology Group study ARST0332. Radiation therapy remains an important component of the multimodality therapy comprising optimal treatment of this disease, despite the relative paucity of long-term outcome data derived from level I evidence.

Impact of socioeconomic markers on parents' retention of pediatric oncology home care education: A pilot study.

Little is known about the extent to which parents retain the education on how to manage home medical emergencies. We sought to describe retention of pediatric oncology home care education (POHCE) in a cohort of 24 parents of newly diagnosed children with cancer and investigate sociodemographic disparities in this retention. We measured retention using a vignette-based survey instrument. The mean score was 4 (range 0-6, SD = 1.6) and parents with high school only education and those with limited cancer health literacy scored lowest (2.5 and 2.8, respectively). Future POHCE interventions can focus on parents' literacy and education levels as predictors to tailor alternative education strategies.

Parent-centered communication at time of pediatric cancer diagnosis: A systematic review.

There are limited data focused on parental communication needs surrounding the time when a child is diagnosed with cancer. In this systematic review, we synthesized current literature on communication preferences of parents at the time of their child's diagnosis of cancer. We identified 16 studies that yielded 4 major themes parents recognized as important: communication style, content, logistics, and healthcare team. We further identified several concepts that inform parent-centered communication practice. The ensuing pediatric oncology parent-centered communication concept map is meant as a tool to expand providers' communication experience at the time of a new cancer diagnosis.

The Salmonella virulence protein SifA is a G protein antagonist.

Salmonella's success at proliferating intracellularly and causing disease depends on the translocation of a major virulence protein, SifA, into the host cell. SifA recruits membranes enriched in lysosome associated membrane protein 1 (LAMP1) and is needed for growth of Salmonella induced filaments (Sifs) and the Salmonella containing vacuole (SCV). It directly binds a host protein called SKIP (SifA and kinesin interacting protein) which is critical for membrane stability and motor dynamics at the SCV. SifA also contains a WxxxE motif, predictive of G protein mimicry in bacterial effectors, but whether and how it mimics the action of a host G protein is not known. We show that SKIP's pleckstrin homology domain, which directly binds SifA, also binds to the late endosomal GTPase Rab9. Knockdown studies suggest that both SKIP and Rab9 function to maintain peripheral LAMP1 distribution in cells. The Rab9:SKIP interaction is GTP-dependent and is inhibited by SifA binding to the SKIP pleckstrin homology domain, suggesting that SifA may be a Rab9 antagonist. SifA:SKIP binding is significantly tighter than Rab9:SKIP binding and may thus allow SifA to bring SKIP to the SCV via SKIP's Rab9-binding site. Rab9 can measurably reverse SifA-dependent LAMP1 recruitment and the perinuclear location of the SCV in cells. Importantly, binding to SKIP requires SifA residues W197 and E201 of the conserved WxxxE signature sequence, leading to the speculation that bacterial G protein mimicry may result in G protein antagonism.