Radiation-induced Desmoid Tumor Development in the Radiotherapy Field in a Child With Pineoblastoma: A Case Report.

Although treatment-related secondary malignancies are rare, they are important problems after the treatment of childhood malignant diseases. Irradiation-induced sarcomas are the development of sarcoma different from the primary tumor after a latent period of ≥3 years or more in the radiotherapy field. Desmoid tumor is extremely rare as irradiation-induced tumor. A 7.5-year-old girl was referred to our hospital after a subtotal mass excision for a solid lesion with a cystic component located in the pineal gland. Pathologic examination revealed pineoblastoma. After surgery, craniospinal radiotherapy, and chemotherapy consisting of vincristine, cisplatin, and etoposide were performed. Painless swelling in the left parieto-occipital region ~75 months after the end of the treatment developed in the patient. A mass was detected in the intracranial but extra-axial region by radiologic imaging methods. Due to the total removal of the mass and the absence of a tumor in the surgical margins, she was followed up without additional treatment. The pathologic diagnosis was a desmoid tumor. She was followed up disease free for ~7 years after the primary tumor and ~7 months after the secondary tumor. Treatment-related desmoid tumor development after treatment for a central nervous system tumor in a child is extremely rare.

Correlation of myeloid-derived suppressor cells with C-reactive protein, ferritin and lactate dehydrogenase levels in patients with severe COVID-19.

The novel coronavirus disease 2019 (COVID-19) remains a global health emergency, and understanding the interactions between the virus and host immune responses is crucial to preventing its lethal effects. The expansion of myeloid-derived suppressor cells (MDSCs) in COVID-19, thereby suppressing immune responses, has been described as responsible for the severity of the disease, but the correlation between MDSC subsets and COVID-19 severity remains elusive. Therefore, we classified patients according to clinical and laboratory findings-aiming to investigate the relationship between MDSC subsets and laboratory findings such as high C-reactive protein, ferritin and lactate dehydrogenase levels, which indicate the severity of the disease. Forty-one patients with COVID-19 (26 mild and 15 severe; mean age of 49.7 ± 15 years) and 26 healthy controls were included in this study. MDSCs were grouped into two major subsets-polymorphonuclear MDSCs (PMN-MDSCs) and monocytic MDSCs-by flow cytometric immunophenotyping, and PMN-MDSCs were defined as mature and immature, according to CD16 expressions, for the first time in COVID-19. Total MDSCs, PMN-MDSCs, mature PMN-MDSCs and monocytic MDSCs were significantly higher in patients with COVID-19 compared with the healthy controls (P < .05). Only PMN-MDSCs and their immature PMN-MDSC subsets were higher in the severe subgroup than in the mild subgroup. In addition, a significant correlation was found between C-reactive protein, ferritin and lactate dehydrogenase levels and MDSCs in patients with COVID-19. These findings suggest that MDSCs play a role in the pathogenesis of COVID-19, while PMN-MDSCs, especially immature PMN-MDSCs, are associated with the severity of the disease.

Clinical Utility of Total Oxidative Stress and Total Antioxidant Capacity in Childhood Febrile Neutropenia.

The aim is to determine the oxidative status of children with febrile neutropenia (FEN). Blood samples were collected to determine the total antioxidant capacity (TAC) and total oxidative status (TOS) of healthy children (once) and children with FEN after 0, 48, and 96 hours. Eighteen patients with FEN were evaluated. The baseline TAC level of patients was significantly higher than that of the controls (P<0.0001). The TAC levels of patients with FEN with and without antibiotic modification were higher than those of the controls (P=0.002 and 0.02, respectively). The TAC levels of the patients with FEN with antibiotic modification were lower than those of the patients without antibiotic modification (P=0.0224). The oxidative stress index (OSI), calculated TOS/TAS, value of the children with FEN was lower than that of the controls (P<0.0001). The OSI values of the patients with FEN with and without antibiotic modification were lower than those of the control group (P=0.001 and <0.0001, respectively). The TAC values of the patients with antibiotic modification were higher than those of the patients without antibiotic modification (P=0.02). In conclusion, the oxidative status of the children with FEN was affected, and it can give information about the follow-up of FEN.

A Prolonged Activated Partial Thromboplastin Time in a Child With Hodgkin Lymphoma.

A 3-year-old girl presented with fever and left-sided neck mass that did not resolve despite antibiotic treatment. Physical examination was normal except lymphadenopathy in the left cervical region. Complete blood count was Hb: 8.01 g/dL, leukocyte count: 4034/mm3, platelet count: 286,000/mm3. On preoperative period, coagulation studies revealed a prolonged activated partial thromboplastin time (aPTT, 46.1 s [N: 21 to 36]), corrected mixing test for aPTT. The prothrombin time and international normalized ratio were in normal limits. Factor VIII levels, von Willebrand factor antigen, and ristocetin cofactor were normal. After the patient was given fresh frozen plasma, lymph node excision was performed. As a result of pathologic examination, Hodgkin lymphoma was diagnosed as classic type. The patient was instituted ABVD chemotherapy protocol for Hodgkin lymphoma. The aPTT at the sixth day of treatment was within normal limits. Coagulation test abnormalities are extremely rare in Hodgkin lymphoma. To our knowledge, this is the first report of prolonged aPTT in a child with Hodgkin lymphoma.

Delayed Radiation Myelopathy in a Child With Hodgkin Lymphoma and ARTEMIS Mutation.

The authors present a case of delayed radiation myelopathy in a 12-year-old girl with Hodgkin lymphoma and Artemis mutation. This is the first of such a case presented in the literature.

Pediatric Lymphoma and Solid Tumors Associated With Cancer Susceptibility Syndromes.

The aim of this study was to determine cancer susceptibility syndromes (CSSs) in children with lymphoma and solid tumors and to evaluate their effects on overall survival rates. Between 2006 and 2019, the oncologic charts of 672 patients who were followed-up with a diagnosis of lymphoma and solid tumor in our clinic were retrospectively reviewed. CSSs were detected in 42 children (6.25%). The most common CSS was DNA damage repair defects/genetic instability (in 18 of 42 patients with CSSs, 42.8%). In the patients with CSSs, 48 different cancers developed. The most common types of cancer were lymphoma (n: 10, 21%) and high-grade glial tumor (n: 9, 19%). The lymphoma subgroups were very rare childhood lymphomas, such as gray zone lymphoma and marginal zone lymphoma. The overall survival rates for patients with DNA damage repair defects/genetic instability; with CSSs other than DNA damage repair defects/patients with genetic instability syndrome; and without any CSS, were 9.7%, 65.1%, and 68.7%, respectively. The overall survival rate for patients with DNA damage repair defects/patients with genetic instability syndrome was lower than both patients with CSSs other than DNA damage repair defects/genetic instability syndrome (P=0.002) and those without any CSS (P<0.0001). CSSs should be kept in mind in children with cafe au lait spots; syndromic features; a family history of cancer, especially in siblings; and rare childhood cancers.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children With Non-Hodgkin Lymphoma.

The aim of this study is to evaluate the clinical and laboratory findings of pediatric patients with non-Hodgkin lymphoma (NHL) who developed Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Between 2006 and 2018, the medical records of child patients with NHL who developed SJS and TEN were reviewed retrospectively. SJS/TEN developed in 7 of 70 patients with NHL (10%). The pathologic subgroups of the patients with SJS/TEN were ALK-negative anaplastic large cell lymphoma (n: 3), Burkitt lymphoma (n: 2), lymphoblastic lymphoma (n: 1), and primary mediastinal B-cell lymphoma (n: 1). Five patients had TEN, 1 patient had SJS/TEN, and 1 patient developed only SJS. In 5 patients, both steroids and intravenous immunoglobulin were administered for treatment, and clinical improvement was achieved in 3 of these patients. Only steroid treatment was used for 1 patient, whereas for the other patient, intravenous immunoglobin was preferred. In addition, N-acetylcysteine treatment was administered for these 2 patients. Four patients with acute renal failure died, and it was found that SJS/TEN is observed more frequently in patients with NHL in which intensive treatment protocols with high-dose methotrexate are used more than with other childhood malignant diseases. Early diagnosis and administration of appropriate and supportive treatment approaches may improve the prognosis.

Comparison of the Radiologic and Clinical Findings of Adolescents With Breast Complaints.

INTRODUCTION: The American College of Radiology's Breast Imaging Reporting and Data System (BI-RADS) was developed to guide imaging-based surgical treatment in patients with breast cancer. Studies confirming the BI-RADS did not include adolescents. To evaluate the validity of this classification system in adolescents, we aim to investigate the relationship between the BI-RADS and pathology findings in adolescents. METHODS: The medical data of 67 female adolescent patients, aged 12 to 18, referred to our clinic for breast-related complaints between 2013 and 2016 were reviewed retrospectively for demographic data and the results of radiologic and surgical pathologies. RESULTS: The main underlying reasons for the visit at the clinic were fullness, tenderness, pain, and palpable masses. Of the 67 patients, 46 were enrolled in the study. After breast ultrasonography, patients whose complaints had diminished were subtracted from the follow-up. The mean age of the patients was 16 years (12 to 18 y). The mean mass diameter size was 3.69 cm (0.9 to 15 cm), and the mean clinical follow-up was 65.3 days (11 to 1095 d). All the patients who were surgically intervened had benign pathology. Of the 21 surgically intervened patients with BI-RADS levels of ≥3, most had fibroadenomas. CONCLUSIONS: The BI-RADS classification-based treatment algorithm may not be valid in adolescents. In the present study, all patients with lesions with BI-RADS ≥3 levels had revealed benign pathologies. The BI-RADS classification may show an increased risk. However, to determine the need for a biopsy in adolescents, there is a need for larger-scale pediatric and adolescent studies using the BI-RADS classification.

An aggressive parameningeal rhabdomyosarcoma with multiple spinal cord metastases: a case report and review of the literature.

PURPOSE: Spinal cord metastasis from rhabdomyosarcoma (RMS) is extremely rare, with three cases reported to date. Herein, we report an aggressive case of RMS of the infratemporal fossa who which developed spinal cord metastases during treatment. CASE PRESENTATION: A 6-year-old girl presented with an enlarging painless mass around her right ear for 3 months. An enhanced magnetic resonance imaging (MRI) revealed a 5 × x4 × x4.5 5 cm mass on her right infratemporal fossa. A tru-cut biopsy was performed, and histopathologic examination revealed the diagnosis of rhabdomyosarcoma. At the time of the diagnosis, cerebrospinal fluid cytology was negative for malignant cells. The patient underwent induction chemotherapy. There was minimal response to chemotherapy, and the patient underwent curative radiotherapy. However, by 12th fraction of RT, the patient developed a progressive weakness on her lower extremity. Spinal MRI revealed multiple gross masses in different parts of the spinal cord. The local radiotherapy was changed toas craniospinal radiotherapy. However, two 2 weeks after the completion of the RT, the patient developed sepsis and expired because of septic shock. CONCLUSION: Parameningeal RMS is a peculiar subgroup of RMS, which needs an aggressive approach. Despite aggressive approach, meningeal spread is the most important cause of the treatment failure. We should keept in mind that during the treatment, there can be meningeal spread towards to either the brain or spinal cord; therefore, we should follow -up the patients closely from this aspect.

Growth-differentiation Factor-15 and Tissue Doppler Imaging in Detection of Anthracycline-induced Cardiomyopathy During Therapy of Childhood Cancers.

OBJECTIVES: The aim of this study was to evaluate the importance of growth-differentiation factor-15 (GDF-15) levels and tissue Doppler imaging (TDI) in the early detection of anthracycline-induced cardiomyopathy during the treatment of childhood cancers. PATIENTS AND METHODS: Twenty patients (13 males and 7 females) newly diagnosed with childhood cancer whose treatment protocol included anthracycline were included in the study. Echocardiography, including M-mode, pulse Doppler, and TDI, was performed after the first anthracycline treatment at cumulative doses of 100, 200, and 300 mg/m and at least 6 months after the last treatment. GDF-15 and troponin-I were also measured at these time points. RESULTS: The median age of the patients was 14 years (range, 3 to 18 y). The median cumulative anthracycline dose was 220 mg/m (range, 60 to 400 mg/m). Conventional pulse wave and pulse wave tissue Doppler methods revealed significant differences in the right ventricular myocardial performance indices of the patients who received cumulative anthracycline doses of 300 mg/m compared with their indices at least 6 months after the last treatment. The serum GDF-15 levels after the cumulative anthracycline dose of 200 mg/m were also higher than the patients' pretreatment levels. CONCLUSIONS: Doppler/TDI and GDF-15 levels may be used in the early determination of anthracycline-induced cardiomyopathy during the treatment of childhood cancers.

Effects of Prednisolone, L-Asparaginase, Gemfibrozil, and Combinations of These Elements on Mice Lipid Profile, Liver, and Pancreas.

The aim of this study is to determine the effects of L-asparaginase (L-ASP), corticosteroids (CSs), and antilipidemics, separately and in combination, on the lipid profiles and the liver and pancreas histology in mice. This study included 8 groups of 7 mice each. Before any drug administration, serum samples were taken from all of the mice. Then, normal saline was applied to the control group, and a medication or combination of medications was applied to the other groups. Levels of triglycerides, cholesterol (COL), and high-density lipoprotein (HDL) and low-density lipoprotein (LDL) were determined, and the livers and pancreases were evaluated histologically at the end of the study. Triglycerides increased significantly in the CS-only and the L-ASP-only groups, COL increased significantly in the CS-only group, and HDL increased significantly in the CS-only and the antilipidemic-only groups. LDL was significantly lower in the CS-only and the L-ASP-only groups. CSs and L-ASP were significantly effective in liver necrosis, L-ASP was significantly effective in liver balloon degeneration, and CS were significantly effective in pancreas vacuolization. Triglyceride measurement is recommended before/during CS and/or L-ASP treatment. Starting with an antilipidemic agent can be considered to avoid possible complications in patients with significantly high rates. Indicators of a possible liver or pancreas injury should also be considered.

Neurological complication of non Hodgkin lymphoma in childhood: experience from a single center in Turkey.

PURPOSE: Lymphomas are the third most common childhood malignant disease after leukemia and central nervous system (CNS) tumors. Early diagnosis of these complications will reduce mortality and morbidity. In this study we aimed to review the neurological complications of childhood non Hodgkin Lymphoma (NHL). PATIENTS AND METHODS: Forty four children with NHL between 2006 and 2012 were investigated retrospectively and 14 cases with neurological complications were identified. RESULTS: The most common symptom was alteration of the consciousness (10 patients, 71.4 %) followed by convulsion (5 patients, 35.7 %), and hallucination (4 patients, 28.5 %); headache, eye pain, neurogenic bladder, speech disability and facial paralysis, and hemiplegia, were less common and each of them was seen in 1 (7.1 %) of the patients. The neurological complications were mostly seen in children with precursor T lymphoblastic lymphoma followed by anaplastic large cell lymphoma. The complications were secondary to medications (Eight patients) infection (two patients); CNS relapse (two patients); or CNS involvement of the primary disease (two patients). Chemotherapy-related neurologic complications were secondary to intrathecal methotrexate, L-asparaginase, vincristine, and ifosfamide CONCLUSION: Advanced disease and PTLL subtype can be suggested as predictors of neurological complication. The survival rates of neurological complications are fairly good unless it is secondary to involvement of the primary disease. In patients with drug-induced neurological complications, the treatment can be safely re-administered after controlling the neurological complications. Therefore, clinicians managing children with NHL must be informative about neurological complications.

Synovial sarcoma in a premature newborn.

Synovial sarcoma is rarely detected in infants, with an annual incidence of 0.5 per million. Synovial sarcoma occurs more frequently in adolescents and young adults, with the majority of patients presenting between 15 and 40 years of age. It is extremely rare, however, in pediatric patients under 2 years of age. In the present study we examined a 3-day-old male infant born at 32 weeks who had a mass on his left arm. Synovial sarcoma was identified on histopathological and immunohistochemical analysis of biopsy material acquired from the mass. On whole body magnetic resonance imaging, diffuse metastases were detected in the bilateral lungs in the retroperitoneal zone, in bilateral suprarenal glands, the right liver lobe, the right kidney, and the brain. To our knowledge this is the youngest patient to be diagnosed with synovial sarcoma in the literature.

Intracranial metastasis of neuroblastoma: in two different areas at the same time.

INTRODUCTION: Neuroblastoma (NB) is the most common extracranial solid malignancy in children. The major cause of death from this cancer is metastasis of tumors, and metastasis can be seen in different areas of the body. Metastasis of NB occurs by hematogenous and lymphatic routes. Generally, brain metastases have been reported in only one area of the brain parenchyma. CASE REPORT: A 4-year-old male patient was treated in our clinic due to the NB that settled in the intra-abdominal region, but the patient presented with headache and nausea approximately 5 months after completion of the treatment. Whereupon, cranial imaging was performed and two masses were detected in the bilateral frontal lobe, and then the patient underwent surgery. Metastatic NB diagnosis was confirmed histopathologically. The patient's chemotherapy treatment is still ongoing. CONCLUSION: We report the case of a male patient with two separate metastatic masses in the brain parenchyma, which occurred in two different areas at the same time.

The activity of valproic Acid in the treatment of refractory hyperleukocytosis in a child with acute lymphoblastic leukemia.

Hyperleukocytosis may be associated with an early morbidity and mortality due to leukostasis. Cytoreductive therapies-such as steroids, hydroxiurea, exchange transfusion, and leukapheresis-have been used for the prevention of leukostasis. Herein, the valproic acid used for the treatment of the hyperleukocytosis did not respond to known therapies in a child; lymphoblastic lymphoma developed bone marrow relapse under the treatment is discussed.

Impact on the survival of neutrophil-lymphocyte ratio, platelet-lymphocyte ratio, and monocyte-lymphocyte ratio on prognosis in children with Hodgkin lymphoma.

OBJECTIVES: To evaluate the clinical utility of the recently described absolute neutrophil counts to absolute lymphocyte counts ratio (NLR), absolute platelet counts to absolute lymphocyte counts ratio (PLR), and absolute monocyte counts to absolute lymphocyte counts ratio (MLR) on prognosis in children with Hodgkin lymphoma (HL). METHODS: We retrospectively evaluated the clinical characteristics, laboratory features (lymphocyte counts, NLR, PLR, and MLR), treatment, and results of 52 children with HL in the Department of Pediatric Hematology and Oncology, Selcuk University, konya, Turkey, from January 2006 until December 2021. RESULTS: The patients included 27 (51.9%) females and 25 (48.1%) males. The age of the patients ranged between 3-17.5 years old (median: 9 years). There were 22 patients in stage II, 24 in stage III, and 6 in stage IV. The most prevalent histopathological subgroup was the nodular sclerosing type (53.8%). The 5-year overall survival rate was 93.7%. The overall survival rate differed based on lymphocyte counts (p<0.0001), NLR (p=0.018), and PLR (p=0.009). However, none of the prognostic factors in the univariate analysis were not prognostic risk factors (p>0.05) in the multivariate analysis. CONCLUSION: Lymphocyte counts, NLR, and PLR may be useful markers for determining the outcomes in children with HL.

A pediatric bithalamic high grade glioma with concomitant H3K27M and EGFR mutations.

BACKGROUND: Despite many treatment approaches, survival rates in high grade glial tumors are still not at the desired level. One of the cause of this failure might be that although having similar histologic features, they may display different biological behaviors depending on molecular heterogeneity. CASE: A 10-year-old girl presented with sudden onset left sided hemiparesis, headache, and ataxia. Physical examination was normal except for left sided hemiparesis and ataxia. A hyperintense mass lesion involving the bilateral thalamus was detected in the axial T2-weighted and coronal FLAIR sequences on brain MRI. There was no enhancement in axial T1-weighted contrast-enhanced sequences. Due to the size and location of the tumor, the patient was considered inoperable. Intensity modulated radiotherapy was intended for curative treatment to the patient because the radiological findings suggested a low-grade glial tumor. Tumor was unresponsive to radiotherapy but biopsy could be performed. The histopathological examination revealed a diffuse glial tumor with increased cellularity, mild nuclear atypia and rare mitosis. Due to the infiltrative pattern of the tumor, it was accepted as a high grade diffuse glial tumor. A chemotherapy protocol including cisplatin and etoposide in the first cycle, vincristine and cyclophosphamide in the second cycle, and carboplatin and vincristine in the third cycle were instituted to the patient. After the third cycle of chemotherapy, the tumor progressed radiologically. H3.1 K27M c.83A > T (HIST1H3C p.Lys28Met), ATRX c.2169_2170del (p.Glu723AspfsTer9), TP53 c.338T > C (p.Phe113Ser), and EGFR c.2300_2308dup (p.Ala767_va1769dup) were detected in the genetic assessment of tumor tissue. The patient`s treatment was changed to vincristine, temozolomide, and irinotecan. Unfortunately, MRI showed progression after three cycles of second-line chemotherapy. The patient`s family refused any further treatment, and the patient died with progressive disease in a short time. CONCLUSIONS: EGFR mutation along with H3.1 K27M mutation is extremely rare in children to our knowledge. It should be kept in mind that if there is a possibility of targeted therapy, there may be a treatment option in this malignant disease with a poor prognosis.

Familial clustering of nasopharyngeal carcinoma in the family of an adolescent with nasopharyngeal carcinoma.

BACKGROUND: Nasopharyngeal carcinoma (NPC) is one of the rare malignant diseases of childhood, of which only 1% occurs in children. In recent years, genetic factors have attracted attention in NPC. A very limited data have been reported about clustering within families. CASE: Herein, the familial clustering of nasopharyngeal carcinoma in the family of an adolescent with nasopharyngeal carcinoma is presented. CONCLUSIONS: There is familial clustering in nasopharyngeal carcinoma (NPC), but our knowledge on this subject is limited, especially in children or adolescent populations. Therefore, we should be more careful in NPC in childhood, especially in first-degree relatives.

Clinical Features, Treatment and Outcome of Childhood Glial Tumors.

AIM: To evaluate the clinical features, treatment approaches, and outcomes of glial tumors in children. MATERIAL AND METHODS: Files (2006 to 2020) of children diagnosed with glial tumors and followed-up were reviewed retrospectively. Information regarding demographic and clinical characteristics, treatment approaches, and outcomes were retrieved from the patients? files. RESULTS: Of the total of 180 pediatric patients diagnosed with brain tumors, 73 (40.6%) had glial tumors. The children with astrocytoma were in the age range of 2?18 years (median age: 8.7 years), while the ages of children with ependymoma ranged from three months to 10 years (median age: 3 years). This difference was statistically significant (p < 0.0001). The male to female ratio was 1.6. The most common symptoms or signs were headaches (n=34, 46.6%), abnormal gait or coordination (n=22, 30.2%), vomiting (n=21, 28.8%), and cranial nerve palsies (n=20, 27.4%). The pathological diagnoses were astrocytomas (n=53, 72.6%), oligodendroglial tumors (n=2, 2.7%), ependymoma (n=15, 20.7%), and other glial tumors (n=3, 4.1%). The most common tumor location was supratentorial (n=42, 57.5%), while midline glioma was detected in seven patients. The 5-year overall survival (OS) rate of all glial tumors, astrocytoma, and ependymoma was 42%, 40%, and 55%, respectively. The 5-year OS rate of the tumor Grade I, II, III, and IV was 77.2%, 45%, 32%, and 0%, respectively (p < 0.0001). The 5-year OS rate of supratentorial, infratentorial, and spinal tumors was 25.6%, 63.6%, and 50%, respectively (p=0.021). In Cox regression analysis, it was found that the tumor resection and grade had an effect on the tumor prognosis. CONCLUSION: Treatment results are not satisfactory in high-grade astrocytomas. There is a need for new treatment approaches that would take cognizance of molecular features and adopt multidisciplinary approaches.