The clone wars - revenge of the metastatic rogue state: the sarcoma paradigm.

Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other "hits." These possibilities and the evidence base to support them are explored

The clone wars - revenge of the metastatic rogue state: the sarcoma paradigm.

Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other "hits." These possibilities and the evidence base to support them are explored

Patient and clinician beliefs about potential barriers to treatment of neuropathic pain for adolescents with sickle cell disease

Abstract Pain is the hallmark symptom causing morbidity for people with sickle cell disease (SCD) and may present as nociceptive, neuropathic, or mixed type pain. Neuropathic pain (NP) is underrecognized and undertreated in patients with SCD and is associated with decreased patient‐reported quality of life. Surveys were completed by clinicians caring for adolescents with SCD in the outpatient setting. SCD patients ages 1418 at increased risk of NP completed a patient‐facing survey at a scheduled clinic visit. Ninety‐four percent of responding clinicians agreed that NP significantly contributes to reported pain in SCD. Clinicians believed that NP medications are effective for reducing chronic pain (62%) and decreasing opioid utilization (44%). Clinician‐identified barriers to prescribing NP medications included concerns about medication adherence (82%), lack of pediatric guidelines for NP medications (70%), and perceived patient concern about side effects (65%). More than 1/3 (35%) of clinicians reported that they were not comfortable managing NP medications. Clinician‐identified barriers to referral to a pain management specialist included scheduling concerns (88%) and perceived patient/family lack of interest (77%). Most patients expressed willingness to take a medication for NP (78%), see a pain management specialist (84%), or learn more about nonpharmacologic interventions (72%), although most (51%) also reported some concerns about taking a medication for NP, citing insufficient knowledge (34%), and potential for side effects (32%). A minority of respondents (15%) worried about referral to a pain management specialist. Clinician and patient perspectives provide insights that may guide education efforts or other interventions to improve treatment of SCD‐related NP

Supporting Adolescents and Young Adults with Cancer: Oncology Provider Perceptions of Adolescent and Young Adult Unmet Needs

Few studies have assessed oncology providers' perceptions of adolescent and young adult (AYA) cancer patients' unmet needs. In this statewide survey (N = 91), we found that most providers recognize the financial, insurance, and late effects needs of their AYA cancer patients. However, employment, education, behavioral health, sexuality, and social and family issues were endorsed by less than half of providers as areas where AYAs needed assistance. Few providers felt that their AYA cancer patients had unmet needs for these same concerns. Educational efforts are needed to improve providers' awareness of the impact of cancer for AYA cancer patients

Patient Navigation Preferences for Adolescent and Young Adult Cancer Services by Distance to Treatment Location

PurposeTo describe how distance to treatment location influences patient navigation preferences for adolescent and young adult (AYA) cancer patients and survivors.MethodsThis study is part of a statewide needs assessment to inform the development of an AYA cancer patient and survivor navigation program. Participants were recruited from outpatient oncology clinics in Utah. Eligible participants had been diagnosed with cancer between ages 15-39 and had completed at least 1 month of treatment. Participants completed a semi-structured interview on preferences for patient navigation. Summary statistics of demographic and cancer characteristics were generated. Thematic content analysis was used to describe navigation preferences among participants classified as distance (≥20 miles) and local (<20 miles), to explain differences in their needs based on distance from their treatment center.ResultsThe top three patient navigation needs were general information, financial, and emotional support. More local patients were interested in patient navigation services (95.2%) compared to distance participants (77.8%). Fewer local (38.1%) than distance participants (61.1%) reported challenges getting to appointments, and distance patients needed specific financial support to support their travel (e.g., fuel, lodging). Both local and distance patients desired to connect with a navigator in person before using another form of communication and wanted to connect with a patient navigator at the time of initial diagnosis.ConclusionDistance from treatment center is an important patient navigation consideration for AYA cancer patients and survivors. After initially connecting with AYAs in person, patient navigators can provide resources remotely to help reduce travel burden

Molecular inversion probe analysis detects novel copy number alterations in Ewing sarcoma

Ewing sarcoma (ES) is the second most common bone tumor in children and young adults, with dismal outcomes for metastatic and relapsed disease. To better understand the molecular pathogenesis of ES and to identify new prognostic markers, we used molecular inversion probes (MIPs) to evaluate copy number alterations (CNAs) and loss of heterozygosity (LOH) in formalin-fixed paraffin-embedded (FFPE) samples, which included 40 ES primary tumors and 12 ES metastatic lesions. CNAs were correlated with clinical features and outcome, and validated by immunohistochemistry (IHC). We identified previously reported CNAs, in addition to SMARCB1 (INI1/SNF5) homozygous loss and copy neutral LOH. IHC confirmed SMARCB1 protein loss in 7–10% of clinically diagnosed ES tumors in three separate cohorts (University of Utah [N = 40], Children's Oncology Group [N = 31], and University of Michigan [N = 55]). A multifactor copy number (MCN)-index was highly predictive of overall survival (39% vs. 100%, P < 0.001). We also identified RELN gene deletions unique to 25% of ES metastatic samples. In summary, we identified both known and novel CNAs using MIP technology for the first time in FFPE samples from patients with ES. CNAs detected by microarray correlate with outcome and may be useful for risk stratification in future clinical trials

Leveraging Grief: Involving Bereaved Parents in Pediatric Palliative Oncology Program Planning and Development

As pediatric palliative care (PPC) became a recognized medical specialty, our developing clinical PPC team longitudinally partnered with bereaved parents to understand the care that their children received as they transitioned towards end of life. Families developed Eight Priorities, shared within, to improve care for children with a poor chance of survival based on their experience of losing a child to cancer. In this paper, we delineate the top eight PPC needs from a parent perspective to offer multi-layered, individually tailored resources for patients and families. One of these Eight Priorities noted that bereavement care for the remaining family members is vital for healing after the death of a child to promote meaning making and resilience in bereaved families. Here, we outline the creation of a bereaved parent-designed bereavement support program as one example of how we have partnered with parents to fulfill their Eight Priorities for quality care