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Purpose: To determine long-term outcomes of a cohort of children with germinoma treated with chemotherapy and radiation therapy without primary tumor boost even in the absence of complete response to chemotherapy. Methods: This retrospective study analyzed the outcome of patients with germinoma consecutively diagnosed and treated at a tertiary care center from January 2000 to December 2021. MRIs were reviewed by two radiologists, blinded to patient data. Tumor location at diagnosis, tumor response to chemotherapy and at completion of radiation therapy and site of relapse were assessed. Tumor response was assessed radiologically by determining the tumor size and response on diffusion-weighted imaging, in addition to biochemical, cytological parameters and neurological status. Results: Of 46 pediatric germinoma patients, 29 children (14 male; median age 12.8 years) received no primary tumor boost. Median follow-up was 63 months (range 9-187 months). Twenty-five children had localized disease and tumor location was suprasellar (n= 11), pineal (n= 10), bifocal (n= 3) and basal ganglia (n= 1) while 4 children had metastatic disease at presentation. All patients completed multi-agent chemotherapy followed by either ventricular irradiation (VI) (23.4 Gy) (n = 23), whole brain (WBI) (23.4 Gy) (n = 5) or craniospinal radiation (CSI) (23.4 Gy) (n= 1). Two children, who had localized disease at presentation and received VI after chemotherapy, relapsed 9 months and 32 months after completion of treatment respectively. No patient had a local relapse. Location of relapse was distant, outside (n= 1) and inside (n= 1) the irradiation field. Five-year PFS was 91% and overall survival (OS) was 100%. Conclusions: In this case series, excellent 5-year PFS and OS rates were achieved with chemotherapy followed by radiation therapy of 23.4 Gy delivered without primary tumor boost. No local relapse was observed despite omitting primary tumor boost in patients with localized and metastatic germinoma.

Relapsed or refractory central nervous system (CNS) tumors still have poor prognosis, and, therefore, new treatment options are required. We retrospectively researched treatment results of patients with CNS tumors treated with nimotuzumab from 2010 to 2015. The study included nine patients with the diffuse intrinsic pontine glioma; eight with medulloblastoma; three each with anaplastic ependymoma, glioblastoma multiforme, and central nervous system primitive neuroectodermal tumor (CNS PNET); two patients with gliomatosis cerebri; and one patient each with other tumor types, including atypical teratoid rhabdoid tumor, thalamic astrocytoma, low-grade glial tumor, high-grade glial tumor, and cribriform neuroepithelial tumor. An objective response was observed in 10 of 33 patients with four patients showing a complete response, three a partial response, and three patients had stable disease. The 2-year overall survival (OS) and progression-free survival (PFS) rates were 35 ±9% and 19 ±8%, respectively. Due to the objective response in medulloblastoma, CNS PNET, and anaplastic ependymoma (MED group), survival rates of this group were analyzed. The 2-year OS and PFS for the MED group were 71 ±12% and 30 ±13%, respectively. The treatment was well tolerated. The treatment responses for medulloblastoma, CNS PNET, and anaplastic ependymoma have been promising. Likewise, some patients with relapsed or progressive CNS tumors may benefit through nimotuzumab-containing regimen.

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.

In this case report; a 12-year-old male with ALL who developed transient left hemiparesis associated with dysphasia and central facial paralysis 14 days after high dose methotrexate (5g/m2) and intrathecal MTX (12 mg, according to age) treatment has been reported. Cranial magnetic resonance imaging (MRI) revealed restricted diffusion in bilateral centrum semiovale compatible with MTX-induced acute encephalopathy. All clinical symptoms resolved completely without any treatment. Clinical findings including headache, nausea, emesis, lethargy, altered mental status, blurred vision, aphasia, dysphasia, hemiparesis and cranial MRI findings of restricted diffusion that does not comply with the territory of ​​any artery seizure should alert the physician for MTX-induced acute encephalopathy.

Ifosfamide is an important chemotherapeutic agent used in the therapeutic protocols of many malignant tumors. Central nervous system toxicity of ifosfamide manifests with encephalopathy in 10% to 30% of patients treated with ifosfamide. Thiamine and methylene blue have been reported beneficial in the treatment and prevention of ifosfamide-induced encephalopathy (IIE). We describe an episode of encephalopathy developed at the third cycle of ifosfamide treatment in a child with Ewing sarcoma. With the administration of thiamin, the encephalopathy resolved and no episode was noted during subsequent courses of ifosfamide. Previous use of cisplatin, concomitant use of opioids, low levels of serum albumin and hemoglobin, and elevated levels of serum creatinine are potential risk factors for IIE. The current case illustrates the possibility of IIE even in the absence of such additional risk factors, treated successfully with thiamin and draws attention to the need for close neurological monitorization of patients treated with ifosfamide.

Castleman disease is a rare, heterogeneous disorder that driven by proinflammatory responses. Human herpes virus-8 has a major role in pathogenesis of multicentric Castleman disease. There is also a subgroup of cases, human herpes virus-8 negative, idiopathic multicentric Castleman disease. The role of primary immunodeficiencies in idiopathic Castleman disease are poorly described. DOCK8 deficiency is a combined primary immunodeficiency. It has a broad clinic spectrum including atopy, autoimmunity and cancer. We present a 10-year-old, DOCK8 deficient patient. He had giant lobular capillary hemangiomas on his neck, iliac and gluteal regions and multiple lymphadenopathies. Abdominal lymph node pathology revealed hyaline vascular type Castleman disease and human herpes virus-8 staining was negative. His lesions were shown to be infected with orf virus. Our case is the first case to relate idiopathic multicentric Castleman disease and DOCK8 deficiency; also, very unusual presentation of orf virus infection in humans.

Burkitt lymphoma manifesting as an intracardiac mass is a rare entity. This report describes the case of a 10-year-old boy who presented with an intracardiac mass and tumor thrombosis in the anterior mediastinum that proved to be Burkitt lymphoma. The LMB-96 chemotherapy protocol was given and at the end of the treatment there was still residual mass. A biopsy was performed and the pathology revealed thymus tissue. The patient has been in complete remission for 3 months. Burkitt lymphoma has a short doubling time and an intracardiac lesion can become life threatening. Early recognition and prompt treatment are crucial in achieving optimal outcomes.

BACKGROUND: Response-based radiation therapy has been the approach for germinoma after chemotherapy. However, the presence of residual lesions at the end of chemotherapy did not demonstrate a negative impact on progression-free survival (PFS). Similarly, resection of residual tumors after chemotherapy did not show a survival benefit. AIM: Our study objective was to determine long-term outcomes of a cohort who received chemotherapy and radiation therapy without primary tumor boost even in the absence of complete response to chemotherapy. METHOD: This retrospective study analyzed the outcome of germinoma patients diagnosed and treated at a tertiary care center from January 2006 to December 2021. RESULTS: Twenty-nine children (14 male; median age 12.8 years) were identified. Median follow-up was 63 months (range 9-187 months). Twenty children had localized disease and tumor location was suprasellar (n= 9), pineal (n= 10), and bifocal (n= 1) while 9 children had metastatic disease at presentation. All patients completed multi-agent chemotherapy followed by either whole ventricular (WVI) (23.4 Gy) (n = 23), whole brain (WBI) (23.4 Gy) (n = 5) or craniospinal radiation (CSI) (23.4 Gy) (n= 1). Two children, who had localized disease at presentation and received WVI after chemotherapy, relapsed 9 months and 32 months after completion of treatment respectively. None of them had local relapses. Location of relapse was distant, outside (n= 1) and inside (n= 1) the radiation field. Five-year PFS was 93% and overall survival (OS) was 100%. CONCLUSION: In this limited experience, excellent 5-year PFS and OS rates were achieved with chemotherapy followed by radiation therapy delivered without primary tumor boost. This study also demonstrated the absence of local relapse despite omitting primary tumor boost in patients with localized and metastatic germinoma.

The COVID-19 pandemic has disrupted cancer care. An audit at a major Paediatric Oncology Department in Turkey was performed to determine its impact on paediatric cancer care. A comparison was made among the number of daily paediatric cancer patients, diagnostic and treatment procedures. The data for the ‘COVID-19 period’ (10 March to 31 October 2020) were compared with the corresponding ‘prior year control period’ (10 March to 31 October 2019). Moreover, presentation delay (duration between first symptoms to healthcare visit) was calculated for new cases. The findings indicate that the mean 34.7 outpatients per day during ‘COVID-19 period’ was significantly lower than the ‘prior year control period’ (52.2). There were 17.7 inpatients per day during the ‘COVID-19 period’ which was significantly lower than 23.8 inpatients per day during the ‘prior year control period’. Significant reduction in the daily mean number of patients undergoing chemotherapy, radiotherapy, surgery and imaging studies during the ‘COVID-19 period’ was also evident. A negative trend in the diagnosis of new paediatric cancers was evident with 128 new cancer cases during the ‘COVID-19 period’, whereas the corresponding number was 212 for the ‘prior year control period’. The presentation delay (median 31 days) remain unchanged during the ‘COVID-19 period’. The findings suggest significant damage to paediatric cancer care during the COVID-19 pandemic. Appropriate obligatory actions by oncology societies and policymakers can minimise longer term negative impacts.

Yaman Bajin I, Demir H, Saltik Temizel IN, Ozen H, Yuce A. Long term follow-up of children with chronic hepatitis B: a single center experience. Turk J Pediatr 2019; 61: 846-851. Chronic Hepatitis B infection is an important clinical issue because of the associated risk of developing cirrhosis and hepatocellular carcinoma. Especially in children, there is no consensus about the optimal treatment. Clinical features and long-term outcomes of 165 children diagnosed with chronic hepatitis B at our institution between January 1993 and June 2012 were analysed retrospectively. Patients were divided into four groups according to their treatment protocols. The first group received Interferon (IFN) only, the second group started lamivudine (LMV) first then IFN+LMV combined and then continued with LMV only, the third group started with IFN+LMV combined then continued with LMV only and the fourth group received LMV only. After a median follow-up period of 7 years (1-19 years) the highest e-seroconversion (the loss of HBeAg followed by gain of anti- HBe antibody) rate, biochemical and virological response was observed with combined (IFN+LMV) treatment regimens. Patients with higher ALT levels were better treatment responders (p: 0.003). Identification of the patients who need to be treated in order to determine the most effective therapy with optimal treatment duration is important to reduce the risk of developing future complications like cirrhosis and hepatocellular carcinoma.

Yaman-Bajin İ, Aytaç S, Kuşkonmaz B, Uçkan-Çetinkaya D, Ünal Ş, Gümrük F, Çetin M. Infant lymphoblastic leukemia: a single centers 10 year experience. Turk J Pediatr 2019; 61: 325-329. Infant acute lymphoblastic leukemia (ALL) is a rare disease and consists of 4-5% of all childhood ALL. Despite improved survival rates in childhood ALL, infants with ALL have a worse prognosis. We aimed to evaluate the clinical features and treatment outcomes of our patients diagnosed with infant ALL at Hacettepe University, Pediatric Hematology Department between 1 January 2008 and 1 January 2018 retrospectively. There were 13 patients with a median age of 7 months. Three of the patients were triplets born from a spontaneous monozygotic triplet pregnancy. Relapse were observed in 4 patients. Hematopoietic stem cell transplantation (HSCT) was performed for five patients. Relapse after HSCT was observed in 3 patients. After a median follow-up period of 18 months, 6 patients (45%) (3 after HSCT and 3 who only received chemotherapy) were alive and in remission. Prognosis of infant ALL is poor in that only half of the patients survive. Our results suggest that bone marrow transplantation seems to be a good and efficient choice of treatment for selected patients. However, there is still a big issue to decide which patient should undergo transplantation and more studies are needed to reevaluate the eligibility criteria for HSCT in this group of patients.

e14042 Background: Optic gliomas are the most common tumors of the optic pathway, comprising about 1% of all intracranial tumours. Here we present the clinical characteristics and the outcome of patients with optic glioma at our institution. Methods: Seventy two patients diagnosed and followed up at a pediatric cancer center in Ankara, Turkey between January 1, 2008 and December 31, 2020 were included in this analysis. The clinical features and outcome of patients were recorded from patient files and hospital information system retrospectively. Results: The median age at the time of diagnosis was 3.5 years (4-157 months), the female/male ratio was 1.3. Fourteen children (19%) were asymptomatic and tumors were detected with routine surveillance, and 58 (81%) had symptoms (the most common being proptosis in 16 patients (22%)). The most common site of optic glioma was the intraorbital region (31%). Fourty patients (55%) had neurofibromatosis type 1 (NF-1). Five patients had histopathological diagnoses; 3 pilomyxoid astrocytoma, 2 pilocytic astrocytoma. Children were treated if they had tumor progression on MRI and/or worsening visual acuity. Twenty seven (38%) children were observed without any therapy, 6 patients (8%) received radiation and chemotherapy, 4 patients (5%) received radiation only, and 35 patients (49%) received chemotherapy only. Treatment regimens for the 41 children who received chemotherapy included carboplatin/etoposide (17), cisplatin/etoposide (18), carboplatin/vincristine (5), and temozolomide (1). The 5-year progression-free survival (PFS) and overall survival (OS) were 64% and 90%, respectively with a 31 month median follow-up. The 5 year PFS was significantly lower in patients with optic tract-hypothalamic-chiasmatic involvement (34%) than orbital involvement (83%) (p=0.013). Five year PFS was significantly higher in patients with NF-1 (80%) than patients without NF-1(46%) (p=0.027) and significantly lower in patients diagnosed at

e22518 Background: Rhabdomyosarcoma is a rare tumor in children and adolescents, presenting 3% to 4% of all pediatric cancers. The female genital tract is considered as a favorable site of childhood RMS. The outcome has improved significantly during the last two decades, attributed to risk stratification and multimodality management of these challenging tumors. Here we present the clinical features and treatment results of girls with genital tract rhabdomyosarcoma to discuss. Methods: Fourteen girls with vaginal and uterine servical rhabdomyosarcoma younger than 18 years of age diagnosed and followed up between the years of 1995 and 2019 were included in this analysis. The clinical features and treatment results of patients were recorded from patient files and hospital information system retrospectively. Results: There were seven cases with vaginal and seven with uterin cervical carcinoma. All patients presented with polypoid masses protruding from the vagina. Median age of patients at diagnosis was 71 months (range 8-200 months). The IRS modified TNM staging was stage I for all the patients. All cases had embryonic type of RMS except one with alveolar type. All patients were treated with surgery and adjuvant chemotherapy, three received radiotherapy additionally. All achieved complete remission. Four patients experienced tumor relapse, 1 patient died with progressive disease. The median follow-up time was 75 months (7-271 months) for 13 patients who were alive with remission. Conclusions: Rhabdomyosarcoma is a rare sarcoma with a higher incidence in children and adolescents. With current treatment strategies, female genital tract rhabdomyosarcomas have a good prognosis. Favorable prognostic factors such as early stage at diagnosis and a favorable histology may contribute to the excellent observed survival. All parties who had involved on the care of these girls must be aware of the high survival with proper treatment to avoid treatment related morbidities and mortalities. Because of the rareness of the disease we wanted to share our experience.

Osteosarcoma is the most common primary malignant tumor of the bone. The most common sites of osteosarcoma in children are the metaphysis of long bones, especially the distal femur, proximal tibia, and proximal humerus. It occurs very rarely in flat bones. Here we report a 14-year-old adolescent boy with primary osteosarcoma of the fifth rib and a review of literature.

With improvements in molecular diagnostic methods, report of Human bocavirus (HBoV) as an etiologic agent in many studies on viral respiratory and gastrointestinal infections has been increasing. Two pediatric patients who presented with secondary hemophagocytic lymphohistiocytosis were examined for etiologic causes, including viruses. Whole bacterial and fungal cultures and viral serological studies were negative. Viral polymerase chain reaction of nasopharyngeal secretions showed HBoV. One was successfully treated with intravenous immunoglobulins, whereas the other died with multiorgan failure. Here we report 2 pediatric patients with secondary hemophagocytic lymphohistiocytosis and detection of HBoV as the sole agent, predicting an association.

Although response to colchicine has been proposed as one of the diagnostic criteria in patients with Familial Mediterranean fever (FMF), the validity of this response has not been validated. The aim of this study was to assess the efficacy of the response to colchicine and to evaluate the extent of the effect of placebo. A double-blind randomized placebo-controlled trial with a cross-over design was conducted. The frequency of FMF attacks, the disease score, physical examination, and acute phase reactants were assessed at 0, 3, and 6 months. Blood samples were collected for complete blood count (CBC), erythrocyte sedimentation rate (ESR), levels of serum C-reactive protein (CRP) and serum amyloid A (SAA), and MEFV mutation analysis in 79 patients with a preliminary diagnosis of FMF. Patients were randomly allocated to receive either drug A or drug B in a double-blind fashion. The designated drug was switched at 3 months. Patients taking colchicine had less frequent FMF attacks (median 0) and lower FMF disease score (median 0) when compared to those on placebo (median 1 and 3, respectively) (p = 0.002 and p = 0.007, respectively). In genetically confirmed FMF patients, median attack number and median disease score was 0 under colchicine treatment, whereas these parameters were significantly higher in the placebo group (median 2 and 8, respectively) (p = 0.007 and p = 0.02, respectively) suggesting that colchicine is more effective than placebo in reducing attacks and disease score. Positive and negative predictive values were 70.2 and 37.5 %, respectively. During the placebo period, patients had less FMF attacks when compared to that of the pre-study period (median 2 vs 6, respectively) (p

Infantacute lymphoblastic leukemia (ALL) is a rare disease and consist of 4-5% of all childhood ALL. Despite improved survival rates of childhood ALL, infants with ALL have a worse prognosis . There were 206 patients diagnosed with ALL at Hacettepe University, Pediatric Hematology Department between 1 January 2008 and 31 December 2016, 11 out of 206 (5.3%) were diagnosed as infant ALL. We aimed to evaluate clinical findings, laboratory results and treatment outcome of our infant ALL patients retrospectively. There were 9 boys and two girls with a median age of 8 months (range 1-12 months) and nine out of 11 (82%) were older than 6 months of age at the initial diagnosis. Interestingly 2 of 3 siblings born from spontaneous triplet pregnancy diagnosed at the same time when they were 7 months old while the other sibling diagnosed 4 months later when he was 11 months old. Physical examination revealed hepatosplenomegaly in 10 out of 11 patients and 1 patient had extramedullary involvement with subcutaneous nodules on the forehead. On admission median WBC count was found 202x10-9/L (range 37.4-739x10-9/L) with a median hemoglobin level 9 g/dL (range 2.6-12.9 g/dL) and median platelet count 81x10-9/L (range10- 314x10-9/L). Immunophenotyping revealed CALLA+ B cell leukemia in 3 patients, CALLA- B cell in 6 patients and T cell leukemia in 2 patients. Cytogenetic analysis showed t(4;11) positivity in 4 patients and 3 of them were spontaneous triplets. Initial CNS involvement were positive in 2 of the patients. Eight patients (73%) were treated with Interfant protocols (Interfant 99 (n=4), Interfant 06 (n=4)) and 3 patients received Modified St. Jude Total XV protocol. Relapse were observed in 2 patients (one had both CNS and bone marrow relapse one year after the completion of chemotherapy and the other experienced an early bone marrow relapse at the 7thmonth of the treatment). Hematopoetic stem cell transplantation (HSCT) was performed in five patients from matched related donor (n=2), unmatched related donor(n=1), and matched unrelated donor(n=2). Moreover, relapse after HSCT was observed in 2 patients 3 and 4 months later; one received second transplantation and survived while the other died because of progressive disease. Four of the five patients who had HSCT survived 74 months, 45, 18 and 13 months after the initial diagnosis, respectively. After a median of 18 months (range 4- 74 months ) follow-up period 6 patients are alive (45 %) and on remission 4 after HSCT and 2 with only chemotherapy. Infant leukemia usually presents with high hyperleucocytosis and extramedullary involvement. The prognosis is poor and international colloborative studies reprorted that 4 year -event free survival rates were between 28-54%. Our results suggest that bone marrow transplantation seems to be a good and efficient choice of treatment. However there is still a big issue to decide which patient should go under transplantation and more studies are needed to reevaluate the eligibility criteria for HSCT in this group of patients. Moreover to the best of our knowledge infant leukemia in triplets without monozygocity were first time reported. Disclosures No relevant conflicts of interest to declare.

Ozyoruk D, Kocayozgat A, Yaman-Bajin I, Cetindag F, Oguz- Erdogan AS, Gunes A. A synchronous occurrence of bifocal intracranial germinoma and bilateral testicular epidermoid cyst in an adolescent patient with Klinefelter`s syndrome. Turk J Pediatr 2019; 61: 456-459. Klinefelter syndrome (KS) is characterized by an additional X chromosome in males leading to a karyotype of 47,XXY. It is associated with an increased risk of certain malignancies, including leukemia, breast cancer and extragonadal germ cell tumor such as mediastinal germ cell tumors and rarely intracranial germ cell tumors. It is possible that the increased risk of developing certain cancers can be attributed to a direct effect of the chromosomal abnormality or the combined action of the abnormal chromosomes and hormonal imbalances. Here we describe a synchronous occurrence of bifocal intracranial germinoma and bilateral testicular epidermoid cyst in an adolescent patient with Klinefelter`s syndrome. The synchronous occurrence of the dual tumors in this patient with Klinefelter`s syndrome might be resulted from the migration defect during embriyogenesis due to underlying genetic disease or it is a coincidental condition, yet there has been no case reported in the literature, so far.

Secretory breast carcinoma (SBC) is a rare type of breast neoplasia that was originally described in children. SBC is an indolent breast tumor with good clinical outcome and rare systemic involvement. Since, majority of studies concerning pediatric SBC have been case reports, it has been difficult to clearly elucidate the characteristics and optimal treatment strategies for SBC in children. Although treatment recommendations vary, surgical excision is the primary mode of treatment. Also, necessity of axillary and/or sentinel lymph node dissection is another matter of discussion in children. We report a 6-year-old girl who was diagnosed as SBC was reported to discuss the use of mastectomy with sentinel lymph node dissection in the treatment of this rare tumor in children.