Adolescent- and adult-onset neuroblastic tumor: A retrospective multicenter observational study of patients diagnosed in France between 2000 and 2020.

BACKGROUND: Adult- and adolescent-onset neuroblastomas are rare, with no established therapy. In addition, rare pheochromocytomas may harbor neuroblastic components. This study was designed to collect epidemiological, diagnostic and therapeutic data in order to better define the characteristics of malignant peripheral neuroblastic tumors (MPNT) and composite pheochromocytomas (CP) with MPNT. PROCEDURE: Fifty-nine adults and adolescents (aged over 15 years) diagnosed with a peripheral or composite neuroblastic tumor, who were treated in one of 17 institutions between 2000 and 2020, were retrospectively studied. RESULTS: Eighteen patients with neuroblastoma (NB) or ganglioneuroblastoma (GNB) had locoregional disease, and 28 patients had metastatic stage 4 NB. Among the 13 patients with CP, 12 had locoregional disease. Fifty-eight percent of the population were adolescents and young adults under 24 years of age. The probability of 5-year event-free survival (EFS) was 40% (confidence interval: 27%-53%). CONCLUSIONS: Outcomes were better for patients with localized tumor than for patients with metastases. For patients with localized tumor, in terms of survival, surgical treatment was the best therapeutic option. Multimodal treatment with chemotherapy, surgery, radiotherapy, and immunotherapy-based maintenance allowed long-term survival for some patients. Adolescent- and adult-onset neuroblastoma appeared to have specific characteristics associated with poorer outcomes compared to pediatric neuroblastoma. Nevertheless, complete disease control improved survival. The presence of a neuroblastic component in pheochromocytoma should be considered when making therapeutic management decisions. The development of specific tools/resources (Tumor Referral Board, Registry, biology, and trials with new agents or strategies) may help to improve outcomes for patients.

Prognostic value of hemogram parameters in osteosarcoma: The French OS2006 experience.

BACKGROUND: Previous studies have shown that neutrophil-to-lymphocyte (NLR) ratio at diagnosis and early lymphocytes recovery on doxorubicin-based chemotherapy, may impact the outcome in patients with osteosarcoma (OST). This study aimed to evaluate the prognostic value of hemogram parameters in patients with OST treated with high-dose methotrexate and etoposide/ifosfamide (M-EI) chemotherapy. MATERIALS AND METHODS: We retrospectively analyzed the prognostic value of various hemogram parameters at diagnosis and during therapy in a large consecutive cohort of patients with OST included in the French OS2006 trial and treated with M-EI chemotherapy. RESULTS: A total of 164 patients were analyzed. The median age was 14.7 years (interquartile range [IQR]: 11.7-17). Median follow-up was 5.6 years (IQR: 3.3-7.7 years). Three-year event-free survival (EFS) and overall survival (OS) were 71.5% (95% confidence interval [CI]: 64%-78%) and 86.4% (95% CI: 80%-91%), respectively. In univariate analysis, blood count parameters at diagnosis and early lymphocyte recovery at Day 14 were not found prognostic of survival outcomes. By contrast, an increase of NLR ratio at Day 1 of the first EI chemotherapy (NLR-W4) was associated with reduced OS in univariate (p = .0044) and multivariate analysis (hazards ratio [HR] = 1.3, 95% CI: 1.1-1.5; p = .002), although not with EFS. After adjustment on histological response and metastatic status, an increase of the ratio NLR-W4 of 1 was associated with an increased risk of death of 30%. CONCLUSIONS: We identified NLR-W4 as a potential early biomarker for survival in patients with OST treated with M-EI chemotherapy. Further studies are required to confirm the prognostic value of NLR and better identify immune mechanisms involved in disease surveillance.

Subcutaneous implantable pleural port catheter in the management of malignant pleural effusions in young patients with solid tumors: A new option in the armamentarium of symptomatic treatment.

The contribution of subcutaneous implantable pleural port catheter (SIPP) for malignant pleural effusions (MPE) management in young patients with cancer, in a palliative care setting is not well explored. This monocentric series analyzed 38 patients, median age 18 years (range 3-25) with significant pleural effusion. SIPP were considered efficient for eight of 12 evaluable patients (67%) and allowed MPE outpatient management in five cases. SIPPs are safe and efficient device to manage MPE patients with solid tumors, in the palliative care setting. This original tool could help health care providers for thoracic symptom management in alternative to iterative pleural punctures.

The Place of Sick Peers in Adolescents and Young Adults with Cancer: Advantage, Disadvantage, and What Makes Barriers to the Encounter.

Purpose: The appreciation of peer support can vary from one country to another due to the cultural and relational differences. This study explores what perceptions French adolescents and young adults (AYAs) in post-treatment for cancer have of the place of sick peers during their treatment and what can make barriers to meet them. Methods: A semistructured interview has been proposed 6 months after the end of cancer treatments. A thematic analysis has been conducted to highlight the major themes and subthemes identified through the participants' discourses. Results: Twelve AYAs (mean age 23 y.o., standard deviation = 2.8; min = 19; max = 26) from two French cancer centers were interviewed. Five major themes were identified, but only two were presented in this article: the place of peers and the impact of coronavirus disease 2019 (COVID-19) epidemic on AYA facilities. AYA peers with cancer major theme demonstrated that meeting sick peers has benefits (e.g., identification, understanding, support, feeling of normalcy) but also has disadvantages (e.g., negative emotional influence). The benefits of peer-to-peer meetings seem to outweigh the disadvantages. Nevertheless, AYAs can face social barriers to this kind of relationship (e.g., fatigue, need to focus on oneself, confrontation to cancer and negative events, feeling of unnatural meeting). Finally, patients' encounters and the normal functioning of AYA facilities have been hampered by the COVID-19 pandemic. Conclusion: Even if AYA services systematically suggest a meeting with other sick peers, it is important to reiterate this proposal since the needs can evolve over time. It can also be interesting to propose places of life outside the hospital to make the encounters more comfortable and natural for AYAs. Clinical Trial Registration number: NCT03964116.

Psychological Adjustment, Adaptation, and Perception of Social Support in French Adolescents and Young Adults After the Diagnosis of Cancer.

Purpose: The third Cancer Plan (2014-2019) has helped with the recognition of adolescents and young adults (AYAs) with cancer's medical and psychosocial specificities and has enabled the creation of dedicated structures in France. Methods: The study involved 43 AYA patients (Nmen = 21; Nwomen = 22) between 15 and 27 years old (Mage = 19.9), diagnosed with all types of cancer, and were recruited in two French cancer centers. Online questionnaires were filled in 2 months after the beginning of treatment. AYAs completed measures of depressive and anxiety symptoms, perceived social support, and coping strategies. Results: Results demonstrated moderate depressive symptoms (M = 10.7, standard deviation [SD] = 7.0) and suggested a good satisfaction (M = 30, SD = 9.5) and a mild availability (M = 27, SD = 10.3) of the social support. Spearman's correlations demonstrated that coping strategies are related to depressive symptoms, for which acceptance (p < 0.01) of the disease played a key role in their psychological adjustment. Perceived social support subscales were positively correlated with the use of distraction as a coping strategy (p < 0.05). Kruskal-Wallis test demonstrated the preferential use of instrumental (p < 0.05) and emotional support (p < 0.01), denial (p < 0.01), and self-blame (p < 0.01) for women and the use of acceptance (p < 0.05) and humor (p < 0.05) for men; and there were no significant differences between patients hospitalized in the two cancer center facilities. Conclusion: Finally, a better understanding of the psychological adjustment and processes among French AYAs with cancer will help families and processionals to better adjust AYA-specific needs at the beginning of cancer treatment. ClinicalTrials.gov.: NCT03964116.

Latissimus dorsi free flap reconstruction of a temporal defect following pediatric oncologic surgery: Case report + literature review.

INTRODUCTION: Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children and adolescents. Around 35 % of pediatric sarcomas occur in the head and neck region. Consequently, RMS is considered the most common type of childhood malignancy diagnosed in this region. OBSERVATION: We report the clinical case of a 6 years old patient, who presented a large temporal hollowing following oncological excision surgery for temporal rhabdomyosarcoma. He underwent surgical reconstruction to fill the right temporalis fossa using a latissimus dorsi muscle free flap micro-anastomosed to the lingual vessels. DISCUSSION: This clinical case highlights the value of plastic surgery in oncological reconstruction, which, combined with a multidisciplinary and collective approach, enables a holistic approach and facilitates socio-psychological integration after oncological surgery in the pediatric population.

Fat-Containing Soft Tissue Tumors in Children, Adolescents, and Young Adults: Which Require Biopsy?

PURPOSE: To confirm the overall benignity of fat-containing soft tissue tumors (STT) on a pediatric cohort and to define the clinical and imaging features that warrant a biopsy. METHODS: A retrospective monocentric study was conducted on patients aged less than 25 years consecutively referred for fat-containing STT to our Comprehensive Cancer Center between 1998 and 2022. Tumor imaging characteristics at diagnosis (US, CT, or MRI) were correlated with pathology. RESULTS: The database extraction identified 63 fat-containing tumors with clinical, histologic, and imaging data available for review. In total, 58 (92%) were benign tumors: 36 lipoblastomas and lipomas, 12 fibrous hamartomas of infancy (FHI), 5 lipofibromatosis, 2 lipomas arborescens, 2 lipomatosis and 1 spindle-cell lipoma. Five patients (8%) were diagnosed with liposarcoma. Factors significantly correlated with malignancy were age >10 years old (p < 0.001), having a cancer-predisposing condition (p < 0.001), a percentage of fat <25% (p = 0.002), and a presence of myxoid zones (p < 0.001) on imaging. CONCLUSION: Most fat-containing STT in children may be classified as benign tumors based on clinics and imaging. The indication for biopsy could be limited to patients aged 10 years or more with either a cancer-predisposing condition or imaging features demonstrating either a low-fat component (<25%) or the presence of myxoid zones.

NUT carcinoma in children, adolescents and young adults.

BACKGROUND: NUT carcinoma (NC), defined by the presence of the NUTM1 rearrangement, is an aggressive tumour associated with poor prognosis. This rare cancer is underdiagnosed and difficult to treat. OBJECTIVE AND METHODS: The primary objective of this review is to describe the clinical, radiological and laboratory features of NC in young patients. The secondary objective is to propose a consensual strategy for the French very Rare Tumour group (FRACTURE group). RESULTS: NUT-specific antibody immunostaining in cases of undifferentiated or poorly differentiated carcinoma may demonstrate the specific NUT gene rearrangement. NCs are frequently advanced stage at diagnosis and the outcome remains poor despite a global strategy that generally includes conventional combination chemotherapy with wide local therapy (surgery, radiotherapy). Chemosensitivity is frequently only transient. CONCLUSION: Recent data have shown that new targeted drugs (histone deacetylase and bromodomain and extra-terminal protein inhibitors) are promising, but their role has yet to be evaluated in NC. Centralized data review is necessary to improve our knowledge of paediatric NC. We propose a multimodal strategy based on published data and their personal experience.

Esthesioneuroblastoma in children, adolescents and young adults.

The esthesioneuroblastoma (ENB) is characterized as a rare malignant sinonasal tumor of neuroectodermal origin. Its starting point is the olfactory epithelium located in the upper part of the nasal cavities. Different nomenclatures have been proposed, but the most common are "esthesioneuroblastoma" and "olfactory neuroblastoma". ENBs have a bimodal distribution and mainly occur in teenagers, young adults and people aged 50-60. It is a very rare tumor in pediatrics since only around 100 cases have been reported so far. Within ENBs, we can distinguish tumors with different biological behavior ranging from localized forms with slow evolution to aggressive and metastatic forms at onset. In addition, precisely diagnosing undifferentiated tumors and distinguishing them from other etiologies of sinonasal tumors are sometime difficult. Added to its very low incidence, these characteristics make the study of ENB complicated. The standard treatment currently includes broad surgery followed by radiation therapy in localized resectable tumors. Neoadjuvant chemotherapy is indicated in large unresectable tumors and in metastatic forms. However, in certain indications, such as high-grade operable tumors, the role of perioperative chemotherapy remains to be defined. The objective of this analysis is to detail current knowledge regarding ENBs' epidemiological, biological, clinical and radiological characteristics as well as how to manage ENB in young patients.

[Diagnostic strategy in pediatrics soft tissue sarcomas]. / Stratégie diagnostique des sarcomes des tissus mous de l'enfant et adolescent.

Soft tissue sarcomas in children are rare tumor, representing around 6 to 7% of children cancer. They spread mostly sporadically (90%) and therefore are rarely associated to an underlying constitutional genetic disease (10%). About half of those sarcomas are rhabdomyosarcomas and the others are a very heterogenous histologic group with various bio-pathologies and prognosis. Clinical presentation is mainly a soft tissue lump often difficult to distinguish from more frequent benign causes (malformative, infectious, benign, or pseudotumor). Inappropriate initial diagnosis work-up has a strong impact on soft tissue sarcomas' prognosis. Adapted complementary investigations (first ultrasound and MRI) are important to compile arguments for a malign origin and to indicate a biopsy. However, predictive value of imaging exams still remains imperfect, and histological analysis by percutaneous image-guided biopsy and sometimes by surgical biopsy is often necessary. Authors realize an update on optimal diagnostic pathway including molecular tests in presence of a soft tissue mass in a child.