Late effects of osteosarcoma and its treatment in pediatric patients: A single-center experience.

PURPOSE: The success of osteosarcoma treatment strategies improved survival rates. The need of diagnosing and managing adverse effects is increasing. We aimed to investigate the outcomes and late results of pediatric osteosarcoma treatment in the survivors. METHODS: Out of osteosarcoma patients (n=54), we assessed the long-term outcomes of survivors (n=39) diagnosed from 2002-2018. We compared the survivors' (n=39) health status (cardiac, renal, neurologic, psychiatric, physical limitations), pain, and psychosocial outcomes (education level, smoking history, and alcohol consumption, marital status, parenthood, health care services usage) with their siblings (n=77). The quality of life and overall survival of amputee and nonamputee survivors are also compared. We provided the retrospective data from the files and phone calls and used Kaplan Meier survival analysis, Ki-Kare, and t-test. Results The overall survival (OS) of children with osteosarcoma (n=54) who survived at 2 years and 5 years from the diagnosis was 90.7 and 77.8%, respectively. These patients achieved 2- year event-free survival (EFS) of 70.4% and 5-year EFS of 57.4%. Thirty-nine survivors of osteosarcoma were compared with 77 sibling controls. Osteosarcoma survivors were more likely than the sibling cohort to report adverse health status containing nephrotoxicity (5.1 vs 0%) (p=0.045), cardiotoxicity (10.3 vs 0%)(p<0.01), neurotoxicity (5.1 vs 1.7%) (p=0.045), activity limitations (64 vs 1.3%)(p<0.01) and pain (12.8 vs 0%) (p=0.002). Survivors' educational status (p=0.014), marital status (5.1 vs 32.5%)(p=0.001), employment (2.6 vs 28.6)(p < 0.001), parenthood (0 vs 29.9%)(p < 0.001) were negatively affected compared with the control group. The prevalence of smoking, alcohol use, psychiatric treatment, and deafness were similar. The amputees (n=9) had an OS rate of 55.6%, and the nonamputees (n=45) had 75.6%. We found similar quality of life results between them. CONCLUSION: Long-term survivors of pediatric osteosarcoma are at significant risk of chronic health conditions, physical limitations, and pain up to 16 years follow up. Follow-up clinics and clinical guidelines are required for the survivors of children with osteosarcoma.

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.

The Efficiency and Toxicity of Mifamurtide in Childhood Osteosarcoma.

The aim of the present study was to evaluate the efficiency and side effects of mifamurtide in childhood osteosarcoma (OS). In total, 477 doses of 2 mg/m intravenous (IV) mifamurtide, along with paracetamol as a premedication, were given to 15 patients with primary nonmetastatic OS after complete surgical resection and to 3 patients with progressive OS. The most common side effects encountered in the patients were chills and fever (17/18). These reactions were observed in 4 patients during the administration of each dose, in a single patient during the last administration, and in the remaining 12 patients during the first or initial 2 administrations. Headache, myalgia, and arthralgia were observed in 2 patients during each infusion. Headache was observed in 1 patient with additional hearing loss during the first 2 infusions. One patient had back pain occuring within the first infusion. Of the 15 patients with primary nonmetastatic OS and treated with the addition of mifamurtide to chemotherapy, 13 showed a complete remission, and 2 patients were still under treatment with a complete remission. Of 3 patients with progressive disease, 2 died while the disease progressed further in the third case over a 51-month period. The 3-year overall survival and event-free survival distributions were 87.5% (mean follow-up time, 46.12; 95% confidence interval, 37.79-52.45 mo) and 75.6% (mean follow-up time, 31.30; 95% confidence interval, 26.54-36.06 mo), respectively. We consider that mifamurtide therapy is a safe and well-tolerated agent in childhood OS.

Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country.

Incesoy-Özdemir S, Ertem U, Sahin G, Bozkurt C, Yüksek N, Ören AC, Balkaya E, Alkan A. Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country. Turk J Pediatr 2017; 59: 410-417. Germ cell tumor (GCT) is a rare malignancy accounting for 2-3% of all pediatric tumors. The overall survival rate of children and adolescents with GCT is more than 80% after adopting combined therapy. The aim of this study is to review clinical presentation, management, and outcome in a single-center series with extracranial GCT. Clinical characteristics, pathologic presentations, and survival outcomes of 101 children with GCT, treated at our hospital from 1988 to 2011, were analyzed. Sixty-two of patients were female and 39 of them were male. Fifty-eight (57%) patients had gonadal tumor (24 testicular, 34 ovarian), 43 (43%) extragonadal. Histologically, teratomas were found most frequently (26 mature, 10 immature), followed by yolk sac tumors (n: 33), mixed malignant tumors (n: 13), embryonal carcinoma (n: 10), disgerminoma (n: 8) and seminoma (n: 1). Twenty-six patients were diagnosed as mature teratoma and we excluded them in the evaluation of staging and survival. Five-year overall and relaps-free survival were 80.3% (mean follow-up time: 215.8 months) and 73.4% (mean follow-up time: 176.2 months), respectively. Five-year survival rates were 93.2% and 90.2% in malign GCTs diagnosed after 1999.

Unilateral nevoid telangiectesia: report of two pediatric cases.

Unilateral nevoid telangiectasia (UNT) is characterized by the dermatomal distribution of telangiectasia, which are distributed unilaterally and frequently found in C3-C4 or the trigeminal dermatomal areas. It may be congenital or acquired and has a 2:1 female: male ratio. Several theories where developed in order to explain its etiopathogenesis. The most widely accepted is the one which establishes its probable association with an increase in estrogen levels. Its incidence is unknown, although it's probably subdiagnosed and more frequent than previously recognized. We present two pediatric cases of UNT followed at our instution during the past year.

A rare type of cancer in children: extranodal marginal zone B-cell (MALT) lymphoma of the ocular adnexa.

Primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphomas (OAMLs) are mostly seen in the 5th-7th decades of life, with female predominance, and they occur rarely in children. Thus, knowledge about this cancer type is obtained from adult data in the literature, while the data regarding OAMLs in the pediatric population are limited to a few case reports. Herein, we report a 10-year-old boy with OAML who was treated successfully with radiotherapy, and we discuss this uncommon lymphoma in children.

A rare type of secondary cancer in a child with acute lymphoblastic leukemia: malignant fibrous histiocytoma.

Secondary cancers which are related with treatment of childhood acute lymphoblastic leukemia (ALL) is a significant problem with longer term. For development of secondary cancer after treatment, the latency period varies between 5 and 10 years. In this case, a 13 year-old-boy diagnosed as high-risk ALL was treated with chemotherapy and prophylactic cranial radiotherapy at a dose of 1800 cGy. Six years after the end of treatment he developed a 5 × 5 × 4 cm mass at the right temporal region of the cranium. The mass was excised totally with clear surgical margin. Pathology of mass has been diagnosed as malignant fibrous histiocytoma (MFH), recently referred to as an undifferentiated pleomorphic sarcoma (UPS). After treatment of childhood ALL, reported cases of secondary MFH is extremely rare in the literature. Herein we present a case of MFH/UPS that developed as a secondary cancer 6 years after the end of ALL treatment.

The relationship between cerebrospinal fluid osteopontin level and central nervous system involvement in childhood acute leukemia.

The aim of this study was to evaluate the relationship between cerebrospinal fluid (CSF) osteopontin (OPN) levels and central nervous system (CNS) involvement in children with a diagnosis of acute leukemia. The study sample consisted of 62 patients who had been diagnosed with acute leukemia. The control group consisted of 16 patients that had presented and had no malignant disease, CNS infection or chronic disease. CSF OPN levels were studied with enzyme-linked immunosorbent assay (ELISA) method. The mean CSF OPN level was 32.76±49.22 ng/ml in the patients at the time of diagnosis and 14.93±6.84 ng/ml in the control group (p>0.05). The mean CSF OPN level was 27.68±32.73 ng/ml at the time of diagnosis in the group without CNS involvement and 53.48±89.21 ng/ml in the group with CNS involvement (p>0.05). However, the CSF OPN level at the time of CNS relapse in patients who developed CNS involvement during follow-up (127.4±52 ng/ml) was significantly higher than in the group without CNS involvement at diagnosis and follow-up (mean CSF OPN level: 27.68±32.73 ng/ml) (p<0.001). The analysis of CSF OPN levels at the time of diagnosis-before relapse and at the periods of relapse and remission in patients who had CNS involvement at diagnosis and/or follow-up revealed statistically significant differences between the time points (p<0.001). High CSF OPN levels in childhood acute leukemia patients may be used as evidence for CNS involvement, and any increases found in CSF OPN levels may be a preliminary predictor for CNS involvement.

Oropharyngeal tularemia mimicking tumoral relapse in a patient with Hodgkin lymphoma in remission.

Tularemia is a zoonotic disease caused by Francisella tularensis. The clinical forms mostly depend on the port of entry into humans. Ingestion typically results in the oropharyngeal form and is associated with symptoms such as fever, pharyngitis, cervical lymphadenitis, and suppuration. In this report, we describe a child treated for Hodgkin's disease presenting six years later with a left cervical lymphadenopathy mimicking a relapse.

Secondary childhood acute myeloid leukemia with complex karyotypic anomalies including monosomy 7, monosomy 5 and translocation (1;10) after 131I-metaiodobenzylguanidine therapy for relapsed neuroblastoma.

The prognosis for relapsing or refractory neuroblastoma (NB) remains dismal, with a five-year disease-free survival of < 20%, and no effective salvage treatment has been identified so far. 131I-metaiodobenzylguanidine (131I-MIBG) has come to play an essential role in the imaging and therapy of NB over the past 30 years. The role of 131I-MIBG in the treatment of NB is continually expanding. 131I-MIBG treatment together with cumulative doses of other alkylating agents has potential serious late side effects such as myelodysplasia and leukemia, although rare. We describe a secondary acute myeloid leukemia case with complex karyotypic anomalies that included monosomy 5, monosomy 7 and translocation (1;10) in a child with relapsed NB who received therapeutic 131I-MIBG.