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Parents of adolescents with cancer (AWC) provide critical support throughout the cancer journey and could offer key insights into support needs. This prospective study aimed to obtain parent perspectives on peer support needs of AWC. Ten individual parents (9 mothers and 1 father) completed a survey and a semistructured interview. Four themes were identified: cancer journey challenges; emotions, reactions, and coping; personal support preferences; and AWC's support needs. Parents recognized that AWC require various support, but lacked insight into their specific peer support desires. Next step interventions should focus on peer support for AWC, while also incorporating peer support for parents. [ABSTRACT FROM AUTHOR]

Background: There is a lack of self-management tools for adolescents with cancer (AWC). This study evaluated the feasibility of Teens Taking Charge Cancer, a web-based self-management program. Methods: A pilot randomized control trial (RCT) was conducted across 4 pediatric oncology clinics. AWC (12–18 years) and their caregivers were randomized to either the intervention or control group. All were asked to complete 12 website modules over 12 weeks (at their own pace) and received monthly calls from health coaches. The intervention website was based on cognitive behavioral principals, designed as an interactive self-guided online program, while the control consisted of education and included links to 12 general cancer websites. Outcome assessments occurred at enrollment and 12 weeks post-intervention. The primary outcomes included rate of accrual and retention, adherence to the protocol, acceptability and satisfaction with intervention using questionnaire and semi-structured interviews, adverse events and engagement with the intervention. Results: Eighty-one teen-caregiver dyads were enrolled with a retention rate of 33%. In the intervention group 46% (n = 18) logged in at least once over the 12-week period. A mean of 2.4 of 12 modules (SD 3.0) were completed; and no one completed the program. Thirty-three percent of caregivers in the intervention logged into the website at least once and none completed the full program. Discussion: The results from this pilot study suggest that the current design of the Teens Taking Charge Cancer RCT lacks feasiblity. Future web-based interventions for this group should include additional features to promote uptake and engagement with the program. [ABSTRACT FROM AUTHOR]

An area of concern affecting the quality of life of childhood cancer survivors (CCS) is that of sexual dysfunction (SD), which may be a result of both physical and psychosexual challenges associated with cancer and its treatment. This is especially pertinent as CCS are known to experience diminished quality of life compared to peers. Relevant to SD, cancer and its associated treatment are associated with negative effects on body image and romantic relationships, as well as overall physical and mental health. Although CCS have been shown to have SD at higher rates than the general population, this is often under-recognized and CCS commonly report that it is not addressed by their health care providers. To guide future research and improve clinical screening and treatment practices for SD, we performed a narrative review of this understudied topic to summarize existing knowledge of the incidence, risk factors, pathophysiology, and rates of screening for SD in CCS. We also outline current gaps in knowledge and directions for future research. [ABSTRACT FROM AUTHOR]

Malignant hepatic tumors in children are rare, comprising 1.3% of all pediatric malignancies. Following hepatoblastoma, hepatocellular carcinoma is the second most common. Other malignant hepatic tumors seen in childhood include those of mesenchymal origin including undifferentiated embryonal sarcoma, angiosarcoma, rhabdomyosarcoma and epithelioid hemangioendothelioma, as well as biliary tumors such as cholangiocarcinoma. Diagnosis can be challenging because of their rarity, and the recognition of distinctive imaging features for certain tumors such as epithelioid hemangioendothelioma and biliary rhabdomyosarcoma can focus the differential diagnosis and expedite the diagnostic process. A complete MRI examination with hepatocyte-specific contrast media and diffusion-weighted imaging helps to focus the differential diagnosis, and, although findings are often nonspecific, in some cases typical features on MRI can be helpful in diagnosis. Histopathological analysis is usually required for definitive diagnosis. Hepatic tumors tend to be aggressive, and full staging is imperative to establish disease extent. Significant proportions are not amenable to upfront surgical resection and often require a multimodality approach including neoadjuvant chemotherapy within a multidisciplinary setting. Facilitating complete surgical resection is usually required for better survival. In this review, we emphasize pathology and imaging features for rare liver tumors that are useful in reaching a prompt diagnosis. We also discuss general clinical findings, prognosis and management of these tumors. [ABSTRACT FROM AUTHOR]

Cancer therapy can result in infertility in childhood cancer survivors; however, little is known about how young adolescent males make sense of infertility and potential future parenthood. What's more, research shows that many healthcare professionals in Canada do not discuss fertility preservation (FP) with this vulnerable population. This study examines how male adolescent childhood cancer survivors understand infertility as a long-term effect of cancer treatments. We used a narrative analysis to examine 16 interviews with 14–18-year-old males. The findings from this research illustrate that FP discussions may raise meaningful questions for young men about how they see themselves and construct their identities. The findings also demonstrate that young men's beliefs about FP may be informed by family narratives, and it shows that biological parenthood may be important to adolescent males. [ABSTRACT FROM AUTHOR]

Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in the pediatric age group. While RMS has been traditionally classified on the basis of its histological appearance (with embryonal and alveolar being most common), it is now clear that the PAX-FOXO1 fusion product drives prognosis. We report here a case of pelvic embryonal RMS in a 3-month-old male who was subsequently found to have developed brain metastases during the course of chemotherapy. Cytogenetic analysis of the brain metastases at the time of autopsy as well as next-generation sequencing analysis revealed a reciprocal translocation involving the SH3 domain containing ring finger 3 gene (SH3RF3, on chromosome 2q13) and the Lipase C gene (LIPC, on chromosome 15q21.3). Due to the poor quality of the pretreatment and postresection samples, cytogenetics and NGS analysis looking for the presence of this balanced translocation in these specimens could not be performed. To the authors' knowledge, this translocation has never been described in RMS. Further studies are needed to determine the biological and clinical implications of this novel translocation. [ABSTRACT FROM AUTHOR]

Sexual health is an important consideration for young adults with cancer; however, oncology healthcare providers are often not equipped with strategies to approach these problems in a systematic way. To address this gap in adolescents and young adults (AYA) care, a one-day international Sexual Health Symposium was held before the Global AYA Cancer Congress (Edinburgh, December 2016). The goals of the symposium were to (1) provide a forum, where international AYA experts had the opportunity to share their knowledge regarding AYA sexual health and (2) develop a guideline for healthcare professionals to screen for and intervene on sexual health issues. This review focused on commonly encountered concerns: (1) management of climacteric symptoms, (2) sexual dysfunction in young men, (3) contraception during and after cancer therapy, and (4) psychosocial issues and care. [ABSTRACT FROM AUTHOR]

In the article, the author discusses the issue of fertility preservation (FP) among adolescent and young adult (AYA) members of the lesbian, gay, bisexual, and transgender (LGBT) population who are suffering from cancer, particularly in the U.S.

Background: Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature. Objective: To retrospectively review the imaging features of histologically proven congenital infantile fibrosarcoma. Materials and methods: The list of histologically confirmed congenital infantile fibrosarcomas between November 1999 and June 2013 was obtained from the oncology-pathology database. Imaging features and pathology reports of these tumors were reviewed. Patient charts were reviewed and clinical features, management and outcomes were recorded. Results: During the study period, 13 children (9 girls and 4 boys; age range: 0 day-16 months, median age: 2.5 months) with congenital infantile fibrosarcomas were available for complete radiological review. The translocation (t[12;15]) was present in 11/13 (84.6%) and absent in 2/13. Eight/thirteen (61.5%) tumors were located in extremities (5 in lower and 3 in upper), 3/13 in thoracolumbar paraspinal regions, and one each in abdomen and sternocleidomastoid muscle. Imaging features included iso- to hyperintensity on T1-W, hyperintensity on T2-W as compared to skeletal muscles and heterogeneous enhancement. Six (37.5%) tumors showed hemorrhagic components and 2 (15.4%) showed low intensity foci. None of the patients had evidence of regional or distant metastases at diagnosis. Management included surgical resection only (1/13) and combined surgery and chemotherapy (10/13). Overall survival was 100% with a median follow-up of 49.3 months. Conclusion: Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a soft-tissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. Patients have a favorable outcome. [ABSTRACT FROM AUTHOR]

Improving access to clinical trials for adolescents with cancer remains an important issue for pediatric health care providers. In this brief report, we highlight barriers to increasing the number of clinical trials as a mechanism for addressing this problem. The challenges discussed include: (1) engaging stakeholders to increase funding; (2) increasing cooperation between clinical trial cooperative groups; and (3) permitting delivery of novel drugs to postpubertal adolescents, in the absence of formal pediatric Phase I evaluation. [ABSTRACT FROM AUTHOR]

Abstract: Background: Pancreatic tumors in children represent a very rare entity. We reviewed the clinical and pathologic features of pediatric patients with pancreatic tumors at a single institution. Methods: We conducted a retrospective review of cases diagnosed at the Hospital for Sick Children between 1986 and 2010. Results: Twenty-seven patients were diagnosed during the study period: 18 with solid pseudopapillary neoplasm (SPN), 6 with pancreatic endocrine neoplasia, and 3 with other tumors. Of the 27 children, 3 had associated syndromes, in specific tuberous sclerosis, von Hippel-Lindau, and polycystic ovarian syndrome. The most common symptoms were pain and vomiting in SPN and hypoglycemia and seizures in insulinomas. Magnetic resonance imaging and computed tomographic scan were equally accurate in determining size and site of origin of the tumor, and both were better than ultrasound. All patients underwent surgery (distal pancreatectomy in 14 cases, Whipple procedure in 8, other procedures in 5), which represented the only treatment for all but 3 cases. Seventeen patients (94%) with SPN are alive (median follow-up, 32 months), 3 of whom had positive margins. Two patients died: 1 male with SPN with malignant transformation and 1 with fibrosarcoma. Conclusions: We describe the largest single-institution study of pediatric pancreatic tumors. Females with SPN have an excellent outcome, even in presence of positive margins, suggesting that limited surgical resection may be appropriate for these patients. [Copyright &y& Elsevier]

The article focuses on a study which examined if differences in therapeutic strategy in pediatric and adult specialty sarcoma centers are associated with clinical outcome. The study reviewed the medical data of patients with Ewing sarcoma (EWS), who were treated between 1990 and 2005 at tertiary care pediatric and adult institutions. It discovered that the doses of alkylating agents and timing of local therapy infleunce clinical outcome in patients with EWS.

Simple Summary: Adolescents and young adults (AYAs) with cancer experience a high level of distress and have unique unmet palliative and supportive care needs. There is limited knowledge about the symptom burden, quality of life, and type of care that AYA patients receive. In 2017, a dedicated AYA-specialized palliative care clinic was established at Princess Margaret Cancer Centre in Canada, with a collaborative approach between palliative care and psychiatry. This study aims to describe the demographics and symptoms burden of AYA cancer patients who attended the integrated palliative care and psychiatry clinic, measure the impact of the clinic on AYAs' symptom control, and examine their end-of-life outcomes. Clinical guidelines aimed at cancer care for adolescents and young adults (AYAs) encourage early integration of palliative care, yet there are scarce data to support these recommendations. We conducted a retrospective chart review of AYA patients, aged 15 to 39 years, who were referred to the Integrated AYA Palliative Care and Psychiatry Clinic (IAPCPC) at the Princess Margaret Cancer Centre between May 2017 and November 2019 (n = 69). Demographic data, symptom prevalence, change in symptom scores between baseline consultation and first follow-up, and intensity of end-of-life care were collected from the patients' medical charts, analyzed, and reported. Of the 69 patients, 59% were female, and sarcoma was the most common cancer. A majority of patients had at least one symptom scored as moderate to severe; tiredness, pain, and sleep problems were the highest scored symptoms. More than one-third used medical cannabis to manage their symptoms. Symptom scores improved in 61% after the first clinic visit. Out of the 69 patients, 50 (72.5%) had died by October 2020, with a median time between the initial clinic referral and death of 5 months (range 1–32). Three patients (6%) received chemotherapy, and eight (16%) were admitted to an intensive care unit during the last month of life. In conclusion, AYAs with advanced cancer have a high burden of palliative and psychosocial symptoms. Creating a specialized AYA palliative care clinic integrated with psychiatry showed promising results in improving symptom scores and end-of-life planning. [ABSTRACT FROM AUTHOR]

Phosphatidylinositol 3-kinase (PI3-K) signalling pathway is a crucial path in cancer for cell survival and thus represents an intriguing target for new paediatric anti-cancer drugs. However, the unique clinical toxicities of targeting this pathway (resulting in hyperglycaemia) difficulties combining with chemotherapy, rarity of mutations in childhood tumours and concomitant mutations have resulted in major barriers to clinical translation of these inhibitors in treating both adults and children. Mutations in PIK3CA predict response to PI3-K inhibitors in adult cancers. The same mutations occur in children as in adults, but they are significantly less frequent in paediatrics. In children, high-grade gliomas, especially diffuse midline gliomas (DMG), have the highest incidence of PIK3CA mutations. New mutation-specific PI3-K inhibitors reduce toxicity from on-target PI3-Kα wild-type activity. The mTOR inhibitor everolimus is approved for subependymal giant cell astrocytomas. In paediatric cancers, mTOR inhibitors have been predominantly evaluated by academia, without an overall strategy, in empiric, mutation-agnostic clinical trials with very low response rates to monotherapy. Therefore, future trials of single agent or combination strategies of mTOR inhibitors in childhood cancer should be supported by very strong biological rationale and preclinical data. Further preclinical evaluation of glycogen synthase kinase-3 beta inhibitors is required. Similarly, even where there is an AKT mutation (∼0.1 %), the role of AKT inhibitors in paediatric cancers remains unclear. Patient advocates strongly urged analysing and conserving data from every child participating in a clinical trial. A priority is to evaluate mutation-specific, central nervous system-penetrant PI3-K inhibitors in children with DMG in a rational biological combination. The choice of combination, should be based on the genomic landscape e.g. PTEN loss and resistance mechanisms supported by preclinical data. However, in view of the very rare populations involved, innovative regulatory approaches are needed to generate data for an indication. • PI3-K pathway mutations are rare in paediatrics, with the highest frequency in DMG. • New trials of mTOR inhibitors should be supported by strong biological rationale. • Toxicities and lack of activity have been major barriers to clinical translation. • New mutation-specific inhibitors in combination, may greatly increase activity. • Evaluating mutation-specific, CNS penetrant, inhibitors in DMG is a priority. [ABSTRACT FROM AUTHOR]