The Representational approach as described in the detailed description.

The Representational approach as described in the detailed description. Each participating parent receives three rounds that consist of two meetings. Each round starts with a meeting where the parent identifies an area where he/she needs more information. The nurse and the parent jointly survey the parent's representation of the area and discusses consequences of knowledge gaps or misunderstandings. Then, new information is introduced and benefits from the new information is discussed. After some days, a follow-up meeting takes place.

Representational approach

Ulla Hällgren Graneheim, Associate professor

The aim of this study is to explore whether an intensified informational intervention built upon the Representational approach framework can reduce stress in parents of children with cancer.

BACKGROUND

Parents of children with cancer have great information needs and report that these are not always met. Psychosocial suffering such as stress and anxiety is also common in this group.

HYPOTHESIS

An informational intervention emanating from the needs identified by parents is associated to decreased perceived stress, decreased depressive symptoms, decreased anxiety, increased satisfaction with information and decreased use of health care contacts in parents.

INTERVENTION

The intervention builds upon the Representational approach. This approach emanates from Leventhal's theories of illness representations and theories of conceptual change. Central qualities in the approach is that parents identify the area where information is needed themselves and that a thorough assessment of their current representation of that area is performed before information is given.

Each participating parent receives three rounds that consist of two meetings. Each round starts with a meeting where the parent identifies an area where he/she needs more information. The nurse and the parent jointly survey the parent's representation of the area and discusses consequences of knowledge gaps or misunderstandings. Then, new information is introduced and benefits from the new information is discussed. After some days, a follow-up meeting takes place.

DESIGN AND METHODS

The intervention will be evaluated using a single-case design (A-B-A-B-A-B-A) with 10 parents. All parents will receive the intervention, and repeated measures of the outcome variables both before and after will be used to assess the effect of the intervention. Data will be collected by web questionnaires with SMS- and e-mail reminders.

In parallel, a process evaluation aiming at describing treatment fidelity, experiences of participation and impact of contextual factors on the results. For this aim, qualitative interviews with participants and audio recording of intervention sessions will be used.

Information to Parents of Children With Cancer. An Exploratory Study

As measured by the Perceived stress scale (PSS). 10 items answered on a 5-point Likert scale.

Includes headache, sleep disturbances, palpitations, and heartburn, each measured on a 4-point scale.

Measured on a 7-point visual digital scale.

Includes general satisfaction with the information from the health care professionals as well as information from the intervention nurses. Measured on a 7-point visual digital scale.

Number of contacts with the health care system concerning the ill child and the parent's own health problems.

Umeå University

Associate professor

Neoplasms

Blood and Lymph Conditions

Immune System Diseases

All Conditions

Rare Diseases

Lymphoma

Lymphosarcoma

Individual interview + Self-report questionnaire + long-term follow-up medical consultation + At 1 year : Assessment of prescription's adherence

Individual interview + Self-report questionnaire

The qualitative interview aims to identify the supportive care needs of patients and parents as well as their expectations with regard to a long-term follow-up.

Interview

To assess the quality of life, anxiety/depression of former patients and relatives:

For child and adolescent patients (less than 15 years old at inclusion):

* Pediatric Quality of Life Inventory Cancer module
* Screen for Child Anxiety Related Emotional Disorders Revised
* Children's Depression Inventory

For patients over 15 years of age at the time of inclusion and parents:

* 36-Item Short Form Health Survey
* Hospital Anxiety and Depression Scale

To describe and evaluate the offer and use of the network of health professionals who are aware of post-treatment issues: number of consultations, speciality and location of the professionals requested following the long-term follow-up consultation, time between referral and first consultation, evaluation of the geographical accessibility of care around the patient's home.

Self-report questionnaire

To assess adherence to long-term follow-up medical consultation:

- number of patients summoned/ number of consultations carried out ; reason for refusal of long-term follow-up medical consultation To assess medical complications through clinical data collection

Medical consultation

Veronique CHRISTOPHE, Pr

Amandine BERTRAND, MD

Cécile Conter, MD

Isabelle Ray-Coquard, MD

Perrine Marec Bérard, MD

Amandine Bertrand, MD

Pierre Leblond, MD

Nadège Corradini, MD

Each year, there are 2100 new cases of cancer in children and adolescents/young adults (AYA) in France. Due to a significant improvement in the effectiveness of therapies, the survival rate of all types of cancer combined after 5 years is 80-85%. This is leading to the emergence of new problems, which require an adaptation of the long-term care of these former patients. Nevertheless, although some medical complications have been widely described and are the subject of recommendations, many questions remain unanswered regarding the real long-term needs of patients and of their main caregivers.

The main objective of this study is to assess not only the specific supportive care needs of former onco-hematology patients treated before the age of 25 years, but also those of their parents up to 6 months after the end of the oncological follow-up, i.e. 3 to 5 years after the diagnosis.

The secondary objectives aim to:

1. Assess adherence to long-term follow-up medical consultation
2. Assess the early complications presented by the patients
3. Assess the quality of life and the anxiety-depression of former patients and their parents
4. Describe and evaluate the offer and use of the network of health professionals aware of the post-treatment issues; assess the referral to the network of health professionals aware of the post-treatment issues.

Depending on the objectives, the methodology used in this study is mixed, qualitative and quantitative. The study will be proposed systematically to all former patients of the hospital department diagnosed before the age of 25 years and at least 6 years of age at the time of inclusion as well as to their parents. A sample of 60 former pediatric patients (20 per age group) and 60-120 parents (ideally 40 per age group) is expected.

At the scientific level, this study will provide a better understanding of the cognitive and emotional processes involved in the long-term follow-up, in particular by identifying the supportive care needs of different participants (former patients and parents) and their experiences and quality of life during the long-term follow-up phase. At the individual level, this pilot study will enable the development of a multiprofessional structure, expert in cancer after-care from the end of the oncological follow-up.

Rational Each year, there are 2100 new cases of cancer in children and adolescents/young adults (AYA) in France. Due to a significant improvement in the effectiveness of therapies, the survival rate of all types of cancer combined after 5 years is 80-85%. This is leading to the emergence of new problems, which require an adaptation of the long-term care of these former patients. Many studies highlight that 60 to 65% of pediatric oncology patients will present medical and/or psychosocial complications in the 20 years following their oncological treatment, with a cumulative incidence of a serious adverse event of 40% 30 years after the cancer diagnosis. Nevertheless, although some medical complications have been widely described and are the subject of recommendations, many questions remain unanswered regarding the real long-term needs of patients and of their main caregivers, in this project their parents. It is therefore necessary to identify the risk factors by determining the expectations and the supportive care needs of patients and their caregivers in the long-term follow-up in order to intervene early and thus reduce the incidence of these later complications.

However, only 30 to 50% of former patients in pediatric oncology-hematology and their family attend a long-term follow-up medical consultation. We hypothesize that this lack of commitment is multifactorial (e.g. unmet supportive care needs, geographical distance from home, lack of information about the importance of long-term follow-up and follow-up structures nearby) and that a precise study of the needs expressed by former patients and their family should lead to an improved attendance at these consultations.

A better understanding of the supportive care needs of these patients and their families, as well as the brakes/obstacles or lers of their compliance with a long-term follow-up, is therefore essential to improve their quality of life, prevent or detect the sequela of therapies and reduce the risk of morbidity/mortality.

Objectives The main objective of this study is to assess not only the specific supportive care needs of former onco-hematology patients treated before the age of 25 years, but also those of their parents up to 6 months after the end of the oncological follow-up, i.e. 3 to 5 years after the diagnosis.

The secondary objectives aim to:

1. Assess adherence to long-term follow-up medical consultation
2. Assess the early complications presented by the patients
3. Assess the quality of life and the anxiety-depression of former patients and their parents
4. Describe and evaluate the offer and use of the network of health professionals aware of the post-treatment issues; assess the referral to the network of health professionals aware of the post-treatment issues.

Methodology Depending on the objectives, the methodology used in this study is mixed, qualitative and quantitative. The study will be proposed systematically to all former patients of the hospital department diagnosed before the age of 25 years and at least 6 years of age at the time of inclusion as well as to their parents. The experiences and the specific needs of each person (patients, mothers and fathers) will be assessed independently. Three independent groups will be formed based on the age of the patient when included in the study. For each group, the parents may or may not be paired with the patients. A sample of 60 former pediatric patients (20 per age group) and 60-120 parents (ideally 40 per age group) is expected.

Expected results At the scientific level, this study will provide a better understanding of the cognitive and emotional processes involved in the long-term follow-up, in particular by identifying the supportive care needs of different participants (former patients and parents) and their experiences and quality of life during the long-term follow-up phase. This first stage will be followed by more powerful studies on the modalities of following-up patients after cancer, in order to add to previously published research on the long-term medical and psychological side effects.

At the individual level, this pilot study will enable the development of a multiprofessional structure, expert in cancer after-care from the end of the oncological follow-up. This support could be initiated early and the link with the local network established quickly. It should reduce the experience of being abandoned presented by former patients and their families. At the family level, studying the needs expressed by parents could lead to a family psychological approach in order to restore the family links impacted by the disease.

At the organizational level, this study should serve as a basis for developing a structure that can carry out tertiary prevention, and thus reduce the costs attributable to long-term side effects (e.g. educational and professional absenteeism, use of treatments, private consultations or even hospitalization).

Supportive Care Needs of Former Child, AYA Cancer Patients, and of Their Parents: Evaluation During Long-term Follow-up

Supportive Care Needs of Former Child, Adolescent and Young Adult Cancer Patients, and of Their Parents: Evaluation During Long-term Follow-up

To identify the supportive care needs of patients and parents as well as their expectations with regard to a long-term follow-up

Number of patients summoned/ number of consultations carried out ; reason for refusal of long-term follow-up medical consultation

Clinical data : cardiac/renal/bones/digestive/hepatic/endocrine/dermatological/sexual/pulmonary/nutritional damages, pain, physical activity, addictology, tiredness, psychological follow-up

For child and adolescent patients (less than 15 years old at inclusion):

- Pediatric Quality of Life Inventory Cancer module

For patients over 15 years of age at the time of inclusion and parents:

- 36-Item Short Form Health Survey

For child and adolescent patients (less than 15 years old at inclusion):

* Screen for Child Anxiety Related Emotional Disorders Revised
* Children's Depression Inventory

For patients over 15 years of age at the time of inclusion and parents:

- Hospital Anxiety and Depression Scale

number of consultations, speciality and location of the professionals requested following the long-term follow-up consultation, time between referral and first consultation, evaluation of the geographical accessibility of care around the patient's home

University of Paris 5 - Rene Descartes

Institut d'Hématologie et d'Oncologie Pédiatrique

Centre Leon Berard

Symptoms and General Pathology

Recurrence

Granuloma

Granuloma, Plasma Cell

Inflammatory Myofibroblastic Tumor

Antineoplastic Agents

All Drugs and Chemicals

Amino Acids

Crizotinib

Tyrosine Kinase Inhibitors

Protein Kinase Inhibitors

Enzyme Inhibitors

Tyrosine

Patients with unresectable, relapsed or refractory inflammatory myofibroblastic tumor, receiving crizotinib

Crizotinib 280mg/m2 twice a day (for 24 months maximum)

Marina Tikhonova

Eugene Pashanov, Prof. PhD

Amina Suleimanova, MD

Targeted therapy based on target identification by genetic examinations is a promising direction in the treatment of patients with a complicated course of inflammatory myofibroblastic tumor. Recently, the main work covered in foreign publications, aimed at finding additional methods of treatment, by identifying new targets for targeted therapy in patients with unresectable IMT, but currently there is no standardized approach to the treatment of IMT in children worldwide.

This study can show the benefits of the usage of crizotinib as targeted therapy in children with ALK/ROS1 positive unresectable, progressive or reccurent inflammatory myofibroblastic tumors. The main hypothesis is that crizotinib would increase an objective response rate in this group of patients.

Inflammatory myofibroblastic tumor (IMT) is a rare type of childhood neoplasm characterized by intermediate biological behavior. The main method of treatment of this tumor is a complete surgical removal. It should be said, that in 2-5% of patients the presence of distant metastases was noted at the time of diagnosis, in addition, the complex anatomical localization of the process sometimes does not allow a radical surgical intervention. In patients with unresectable IMT, the presence of distant metastases, residual tumor after initial resection or progression of the disease, attempts have been made to prescribe therapy with steroids , nonsteroidal anti-inflammatory drugs (NSAIDs), cytostatic drugs. However, the effectiveness of this therapy was low.

Since the late 90s, it is known that with the identification of a number of specific cytogenetic changes, this type of neoplasm is a true neoplastic process. In 50% of all cases of IMT there are clonal translocations of the ALK gene located at the 2p23 encoding the anaplastic lymphoma kinase (ALK). It was shown that ALK gene activation is based on translocations involving a large number of partner genes with tyrosine kinase activity. Currently, there are more than 21 partner genes involved in the formation of chimeric transcripts. The most frequent are TPM3, TPM4, CLTC, CARS, RANBP, EML4, TFG.

The introduction of modern molecular genetic methods, including the next generation sequencing (NGS), has expanded the understanding of the mechanisms of carcinogenesis in IMT. Along with ALK gene rearrangements, the ROS1 gene (YWHAE-ROS1 and TFG-ROS1) and PDGFRB (NAB2) genes were detected in the group of patients with ALK-negative IMT.

The therapy with tyrosine kinase inhibitors (crizotinib) has become possible not only in patients with ALK-positive tumor, but also in a group of patients who do not have an ALK gene rearrangement, but in whom a rearrangement of the ROS1 gene has been detected \[4\].

Previously, large-scale studies using tyrosine kinase inhibitors (crizotinib) have not been conducted in patients under 18 with IMT.

The Phase 1 study, conducted by the Children's Cancer Group (USA), showed the response rate on therapy with crizotinib in 3/7 patients, and . disease stabilization in 4/7 patients (Mosse Y., 2013).

The median age of patients with IMT, included in the study, was 8.4 years (range 1.1 - 21.4). In this study, it was noted that the use of crizotinib in a dose regimen of 280 mg / m2 twice a day in this group of patients does not lead to an increase in the incidence of side effects of grade 3 and 4. Thereby, the maximum permissible dose was determined, which amounted to 560 mg / m2 / day in two doses.

In the phase 2 study, it was shown that in the group of patients with IMO a response in 86% of cases has been achieved, with complete response in 36% (5/14), partial response in 50% (7/14), and stabilization of the disease in 14% (Mosse Y., 2017).

In the recently published single-drug, open-label, non-randomized phase 2 study the objective response rate on crizotinib in ALK+ patients was 50% (n=6/12) Schöffski P., et al. Lancet Respir Med. 2018 Jun;6(6):431-441. doi: 10.1016/S2213-2600(18)30116-4. Epub 2018 Apr 15).

The investigator's study is designed in order to show if the treatment with ALK-inhibitor crizotinib is able to improve the outcome of pediatric IMTs in Russia.

Study of Crizotinib in Children and Adolescents With Myofibroblastic Tumors

Targeted Therapy in Children and Adolescents With Recurrent, Progressive and Unresectable Inflammatory Myofibroblastic Tumor With the Inhibitor of Tyrosine Kinase -Crizotinib

The frequency of response to therapy with Crizotinib in patients with translocation of the ALK / ROS1 genes and their partners.

Time after treatment without any signs or symptoms of inflammatory myofibroblastic tumor

The length of time from the start of treatment with crizotinib, that patients diagnosed with the inflammatory myofibroblastic tumor are still alive

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Information to Parents of Children With Cancer. An Exploratory Study	NCT01502189	Stress Health Knowledg...	Representationa...	18 Years -	Umeå University
Information to Parents of Children With Cancer. An Exploratory Study	NCT01502189	Stress Health Knowledg...	Representationa...	18 Years -	Umeå University
Supportive Care Needs of Former Child, AYA Cancer Patients, and of Their Parents: Evaluation During Long-term Follow-up	NCT04711733	Solid Tumor or ...	Interview Self-report que... Medical consult...	6 Years - 31 Years	Centre Leon Berard
Study of Crizotinib in Children and Adolescents With Myofibroblastic Tumors	NCT03874273	Inflammatory My...	Crizotinib	- 18 Years	Federal Research Institute of Pediatric Hematology, Oncology and Immunology
Supportive Care Needs of Former Child, AYA Cancer Patients, and of Their Parents: Evaluation During Long-term Follow-up	NCT04711733	Solid Tumor or ...	Interview Self-report que... Medical consult...	6 Years - 31 Years	Centre Leon Berard

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