A Rare Case of BRAF-mutated Metastatic Pleomorphic Xanthoastrocytoma Patient who Developed Radiodermatitis After Receiving Anti-BRAF Treatment.

Pleomorphic xanthoastrocytoma (PXA) is a rare type of grade 2 or 3 brain tumor that usually occurs in children and young adults. The standard treatment for PXA is maximally safe resection, usually with adjuvant radiation therapy, for high-grade tumors. BRAF V600E mutation is one of the most common molecular alterations in these tumors, with nearly 70% of cases carrying this mutation. Although BRAF inhibitors have shown promise in treating progressive or refractory disease, their use has been associated with various adverse effects, including radiodermatitis, which is a relatively common complication. This paper presents a case of a 16-year-old male patient with BRAF-mutated metastatic PXA, who developed mild radiodermatitis after receiving BRAF inhibitors with concurrent radiation therapy.

Preliminary findings of German-sourced ONC201 treatment in H3K27 altered pediatric pontine diffuse midline gliomas.

PURPOSE: H3K27 altered pediatric pontine diffuse midline gliomas (pDMG) have a poor prognosis, and conventional treatments offer limited benefits. However, recent advancements in molecular evaluations and targeted therapies have shown promise. The aim of this retrospective analysis was to evaluate the effectiveness of German-sourced ONC201, a selective antagonist of dopamine receptor DRD2, for the treatment of pediatric H3K27 altered pDMGs. METHODS: Pediatric patients with H3K27 altered pDMG treated between January 2016 and July 2022 were included in this retrospective analysis. Tissue samples were acquired from all patients via stereotactic biopsy for immunohistochemistry and molecular profiling. All patients received radiation treatment with concurrent temozolomide, and those who could acquire GsONC201 received it as a single agent until progression. Patients who could not obtain GsONC201 received other chemotherapy protocols. RESULTS: Among 27 patients with a median age of 5.6 years old (range 3.4-17.9), 18 received GsONC201. During the follow-up period, 16 patients (59.3%) had progression, although not statistically significant, the incidence of progression tended to be lower in the GsONC201 group. The median overall survival (OS) of the GsONC201 group was considerably longer than of the non-GsONC201 group (19.9 vs. 10.9 months). Only two patients receiving GsONC201 experienced fatigue as a side effect. 4 out of 18 patients in the GsONC201 group underwent reirradiation after progression. CONCLUSION: In conclusion, this study suggests that GsONC201 may improve OS in pediatric H3K27-altered pDMG patients without significant side effects. However, caution is warranted due to retrospective design and biases, highlighting the need for further randomized clinical studies to validate these findings.

Psychosocial Impact of the War in Ukraine on Pediatric Cancer Patients and Their Families Receiving Oncological Care Outside Their Country at the Onset of Hostilities.

Psychosocial care of pediatric cancer patients and their families is as critical as the medical and surgical components of their therapies. Strains on family communication and structure and financial need are linked to poorer psychological outcomes for both patients and families. It is critical that children remain as connected as possible to their communities and extended families during therapy. For Ukrainian pediatric cancer patients receiving care outside of their nation's borders on February 24, 2022, the Russian invasion of Ukraine compounded these problems. Based on conversations with patients and parents, we evaluated the psychosocial impact of war on pediatric Ukrainian cancer patients and their families who had left their country before the onset of the conflict to undergo treatment of pediatric malignancies at our medical center. These families shared with us the problems they have experienced after the Russian invasion of Ukraine. Their concerns can be summarized in 4 categories: (1) emotional stress experienced by the patients, families and relatives related to the dangers of war; (2) difficulties in obtaining previous hospital records in Ukraine; (3) medical expenses; and (4) uncertainty regarding the patient's and their family's future and the ability of the children to ever return to their homes. Psychosocial distress relating to the violence of war will hopefully pass in near future, but our pediatric patients and their families will continue to face stressors related to displacement and financial concerns for some time to come.

Magnetic resonance image-guided stereotactic body radiation therapy for liver rhabdoid tumor in infancy: A case report.

Extracranial malignant rhabdoid tumors are rare and aggressive tumors that typically occur in the pediatric age group and have a poor prognosis. Herein, we report a case of a one year and five months old male infant who was referred with the diagnosis of malignant rhabdoid tumor of the liver. Magnetic resonance guided stereotactic body radiotherapy was administered with concomitant chemotherapy. Treatment was well tolerated with no severe acute side effects. A 40.8% volumetric reduction of the tumor was observed at the last fraction of MR guided radiotherapy.

Clinical practice of a genetics referral selection tool in pediatric cancer patients.

There are few guidelines for genetic counseling and management of pediatric cancer patients with probable cancer predisposition. In this study, we used a previously proposed patient selection tool by Jongmans and discussed the findings in regard to pediatric cancer patients we treated. Pediatric solid tumor patients who were treated in Kocaeli University Department of Pediatric Oncology were evaluated with the five main questions in Jongmans' referral tool. All of the patients and records of diagnostic imaging were examined and analyzed. One-hundred-twenty-three patients participated in the study. The most common indication for genetic counseling was 'consanguinity of the parents' with '≥2 malignancies at childhood age' following it. Fifty-two (42.28%) patients had indication for genetic counseling. We recommend developing and using genetics counseling selection tools such as Jongmans' which helps clinicians differentiate patients with probable cancer predisposition.

EWSR1(22q12) Translocation Positive Pediatric Adrenal Tumor with Loss of 1p, 11q, and Unbalanced Gain of 17q: Neuroblastoma or Ewing Sarcoma?

Background Although neuroblastoma and Ewing sarcoma/Primitive neuroectodermal tumor are different clinical entities, they are both a member of small round blue cell tumors and can mimic each other's behavior in clinical and molecular aspects. Case report: A 3 year-old girl with an abdominal mass was found to have a small round blue cell tumor originating from the right adrenal gland. High level of neuron specific enolase, initial genetic test results (N-Myc amplification: negative, loss of 1p, 11q, and unbalanced gain of 17q) and characteristic radiological appearance of the tumor suggested a preliminary diagnosis of neuroblastoma but further analysis showed CD99 expression and presence of EWSR1 rearrangement, which are mostly observed in Ewing sarcoma. Conclusion: Adrenal gland tumors of childhood with complex immunophenotypic features requires distinguishing two discrete tumors in the small round blue cell tumor group, neuroblastoma and Ewing sarcoma. Although no exact diagnosis of the tumor was made, we reached a good response with neuroblastoma treatment protocol.

Evaluation of Clinical Properties and Treatment Responses of Infantile Hemangioma.

BACKGROUND: Infantile hemangiomas are the most common vascular tumors in childhood. Although spontaneous regression is common; several infantile hemangioma patients need treatment due to possible morbidities. The aim of this study was to investigate the medical methods used in the treatment of infantile hemangiomas and to evaluate the factors affecting treatment response. METHODS: Clinical and demographic characteristics, risk factors, treatment indications, modalities, duration, and responses of 100 patients between January 2007 and January 2017 were evaluated. RESULTS: The most common form of hemangiomas was superficial lesions. Sixty three per cent of the patients were female. Ulceration and hemorrhage were found in 26% of the cases and ocular problems were detected in 3% of the cases. Among the indications for treatment were cosmetic reasons with 56%, ulcer and bleeding with 25% and risk of vision problems with 13%. Propranolol with/without steroid was used as first line treatment and response rates were: 84 patients with more than 50% response, 9 patients with less than 50% response and 7 patients with treatment refractory. The most important factor affecting the treatment response was age at the beginning of the treatment. Duration of treatment, presence of ulceration, location, and size of hemangioma were also found to have significant effects on responses. CONCLUSIONS: This study demonstrated the importance of the kind and initiation time of infantile hemangioma treatment. A strong positive effect can be reached by starting treatment before the end of the proliferation phase. J Drugs Dermatol. 2020;19(12): doi:10.36849/JDD.2020.5009.

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.

PHACE Syndrome: A Rare Case.

PHACE syndrome (OMIM 606519) is a rare neurocutaneous vascular disorder, characterized by posterior fossa malformations, large cervicofacial infantile hemangiomas, arterial anomalies, aortic coarctation, cardiac abnormalities, and eye abnormalities. The long-term outcome of PHACE syndrome patients is unclear; however, it seems that they are at risk for childhood stroke. The radiologist has an important role on diagnosis of PHACE syndrome and in the assessment of potential complications. Investigation of infants with segmental craniofacial hemangiomas should include cranial magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the cerebral and cervical arteries. Brain MRI and MRA findings of a 5-year-old female patient with PHACE syndrome are presented.

Nuclear protein in testis midline carcinoma in a Turkish boy: a case report.

Background: Nuclear protein in testis (NUT) midline carcinoma (NMC) is an undifferentiated carcinoma, usually localized to the midline and presenting a translocation in the gene for bromodomain containing protein 4. Here, we report a rare case of NMC in an 8-year-old Turkish boy. Case report: There were masses in the lung, liver, and iliac wing representing metastases. Abdominal lymph node sampling revealed epithelial tumor infiltration with cellular pleomorphism. Immunohistochemistry was strongly positive for cytokeratin and epithelial membrane antigen protein. Because of undifferentiated carcinoma morphology, the tumor was considered to be a NMC. Immunoreactivity with antibodies to NUT and the presence of NUT clarified by fluorescence in situ hybridization (FISH) supported the diagnosis. Despite initial response to chemotherapy, the patient died 7 months after the diagnosis. Conclusions: Immunoreactivity for NUT antibodies along with a dual-color FISH and karyotype analysis was suggestive for diagnosis of NMC. In differential diagnosis of undifferentiated carcinomas that occur particularly at midline localization, NMC should be considered.

More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma.

Demirsoy U, Alparslan B, Sen MC, Anik Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçioglu F. More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma. Turk J Pediatr 2019; 61: 139-141. Paraneoplastic syndromes in Hodgkin lymphoma (HL) can be seen with different symptoms and organ findings within a significant time before definite diagnosis of the primary disease. Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported paraneoplastic components in pediatric HL. In this report, we present an 11-year-old girl who had all these three paraneoplastic components synchronously before HL was identified.

The Performance of Radiographic Criteria for Bone Malignancy When Applied to Computed Tomography and Magnetic Resonance Imaging.

BACKGROUND: The conventional radiologic features that differentiate benign from malignant bone lesions were originally described using radiography (x-ray [XR]). When evaluating sectional imaging studies such as magnetic resonance imaging (MRI) and computed tomography (CT), one may apply these principles to identify malignant bone lesions. The aim of this study was to evaluate the performances of these radiographic features for detecting malignity when applied to CT and MRI. MATERIALS AND METHODS: This retrospective study was approved by our institutional ethical board. Thirty-nine patients with histopathologic proof of a high-grade bone malignancy and preoperative imaging data obtained with a minimum of two different modalities were included in the study. Four radiologists reviewed the images and scored the lesions for distinctness of margins, presence and type of periosteal reaction, matrix mineralization, and presence of soft tissue mass. The average score for each modality was then tested for accuracy with regard to the histopathology. RESULTS: When lesion margins were considered, XR was the best modality to detect a high-grade malignancy. MRI, especially postcontrast T1-weighted sequence, was the least helpful in this regard. There was no significant difference between CT and XR and between CT and MRI. When the periosteal reaction was considered, XR was the best modality to detect the malignant type of periosteal reaction. In this regard, MRI and CT were misleading; either by not detecting or undergrading periosteal reaction. MRI was the best modality to detect soft tissue mass. CONCLUSION: Conventional imaging criteria for bone malignancy can be misleading when applied to MRI or CT. When cross-sectional imaging features contradict those from XR, the latter should be the guide for clinical management.

Malignant triton tumor of trigeminal nerve-case report.

INTRODUCTION: Here we are presenting a unique case of malignant triton tumor of the trigeminal nerve in a 4-year-old boy who presented with diplopia and ptosis. INTERVENTION: Near total excision of the tumor was performed, and adjuvant chemotherapy and radiotherapy were administered. RESULTS: The patient is in good health and has no evidence of clinical and radiological tumor recurrence for 22  months.

Subclinical anthracycline-induced cardiotoxicity in long-term follow-up of asymptomatic childhood cancer survivors: Assessment by speckle tracking echocardiography.

OBJECTIVE: Survivors of childhood cancer treated with anthracyclines carry the risk for developing late-onset cardiotoxicity. The purpose of this study was to evaluate left ventricular (LV) function in this patient group and compare it with healthy controls by means of conventional and speckle tracking echocardiography (STE) after exposure to chemotherapy. MATERIAL AND METHODS: Conventional and STE were performed in 45 childhood cancer survivors (mean age 11 ± 4.6; 26 male) treated with anthracyclines (median cumulative dosage 240 mg/m2 ; range, 100-460) and compared with age, gender and body surface area matched healthy controls. Follow-up period after chemotherapy was 21.9 ± 17.8 months. Blood samples were taken from survivors and controls to determine brain natriuretic peptide (BNP). RESULTS: Following anthracycline exposure, pediatric cancer survivors had lower longitudinal, radial anteroseptal, and radial anterior strain values compared to controls (P < .05). The calculated global longitudinal and global radial strain values were lower compared to the control group (P < .05). Both groups had normal ejection fraction (EF) and fractional shortening (FS). Brain natriuretic peptide (BNP) levels of both groups were in the normal range. CONCLUSION: Despite normal EF and FS, children exposed to anthracycline therapy may have late-onset subtle changes of LV strain values measured by STE. Whether these changes of strain can predict future risk of developing heart failure needs to be explored in further studies.

Post-traumatic stress disorder in mothers of children who have undergone cancer surgery.

BACKGROUND: The aim of this study was to investigate the rate of post-traumatic stress disorder (PTSD) and associated risk factors among mothers of children who underwent cancer surgery. METHOD: This cross-sectional, multi-center study included a total of 60 mothers whose children underwent major thoraco-abdominal surgery and were under follow up in the outpatient setting between February 2016 and May 2016. Clinical Data Form, Hospital Anxiety and Depression Scale (HADS), and Clinician-Administered PTSD scale were used. RESULTS: Of all participants, 13 (21.7%) were diagnosed with PTSD. These mothers had shorter duration of marriage, longer duration of hospital stay after surgery, and higher HADS scores, compared with the others without PTSD. Thoughts of guilt such as "I am being punished or tested" were more frequent in mothers with PTSD. Insomnia, irritability, concentration problems, and psychological reactivity were the most common symptoms. CONCLUSION: Post-traumatic stress disorder is a severe disorder that may worsen the daily functioning of mothers and may also have an unfavorable effect on child. It is therefore of utmost importance for clinicians to recognize PTSD and the associated risk factors in order to guide these parents.

Endobronchial recurrence of non-Hodgkin's lymphoma mimicking foreign body aspiration.

A 2-year-old patient with a history of non-Hodgkin's lymphoma (NHL) was presented to our hospital with the complaint of shortness of breath and wheezing. Posteroanterior chest radiograph revealed hyperlucency and hyperexpansion of the right hemithorax. We performed computed tomography (CT) because of a suspicion of foreign body aspiration. CT revealed right main bronchus occlusion by a hypodense lesion. Bronchoscopy revealed a mass lesion in the right main bronchus which was histopathologically diagnosed as NHL. Only a few cases of endobronchial recurrence of lymphoma have been reported in the current literature, but there is no reported case in a pediatric patient.

Prognostic factors and treatment results of pediatric Hodgkin's lymphoma: A single center experience.

The aim of this study was to assess the demographic, clinic data, prognostic factors and treatment/follow-up results of children who were diagnosed with Hodgkin lymphoma and followed in our center of Pediatric Oncology, Kocaeli University Medical Faculty, Kocaeli, Turkey, for 10 years. This retrospective study evaluated 41 patients with Hodgkin lymphoma who were younger than 18 years-old. All patients were treated with risked adapted ABVD (Adriamycin, Bleomycin, Vincristine, Dacarbazine) chemotherapy and also received involved field radiotherapy. Thirty-two patients (78%) were males and 9 (22%) were females, with a mean age of 10.7±4.0 years. The histopathological diagnosis was mixed cellular type in 51.2% of the patients. B symptoms (unexplained fever, unexplained weight loss, drenching night sweats) were present in 53.7% of the patients and 36.6% of the patients were at advanced stage at the time of the diagnosis. The 3-year overall and event-free survival rates were 88% and 5-year overall and event-free survival rates were 88%, 78%. Age, stage, treatment risk groups, presence of B symptoms and hematological parameters had no significant effect on overall and event-free survival in univariate analysis while bulky disease was the only significant factor on overall survival. Our treatment policy was succesful regarding the similar survival rates in the treatment risk groups, however novel treatment strategies adopting the early response with the reduction of adverse effects are planned in the near future.

The effect of childhood cancer therapy on ovarian reserve and pubertal development.

The effects of childhood cancer therapy on ovarian reserve tests and on pubertal development within 5 years were compared with a control group. The study group was composed of 41 patients who underwent chemotherapy during pre-menarche (subgroup A; n = 15) and after menarche (subgroup B; n = 26); the control group was composed of 44 patients admitted with non-cancer related diseases (in total n = 85). Mean total ovarian volume and total antral follicle counts on ultrasound examination were significantly lower in the study group compared with the control group (3.5 ± 2.3 versus 5.2 ± 2.4 ml; P = 0.001; and 3.4 ± 3.3 versus 8.6 ± 3.5; P < 0.001, respectively). Mean FSH level was significantly higher in the study group (13.5 ± 16.2 versus 7.3 ± 2.7 mIU/ml; P = 0.017). Anti-Müllerian hormone levels in subgroup A were significantly higher than in subgroup B (1.8 ± 0.1 versus 1.5 ± 0.08 pg/dl; P = 0.034). In conclusion ovarian volume, antral follicle count and FSH can be used for evaluating the harmful effect of cancer chemotherapy on ovarian follicles. Post-menarche, Anti-Müllerian values reveal that ovarian follicles are more sensitive to the devastating effects of cytotoxic treatment.