Unusual Manifestation of Extraosseous Ewing Sarcoma: Report of 3 Cases.

Ewing sarcoma (ES), described as a diffuse endothelioma of the bone, is divided into two categories: osseous and extraosseous, which mainly affects adolescents. Extraosseous Ewing Sarcomas (EES) are rare tumors originating from soft tissues. Their clinical presentation depends mainly on the primary location of the tumor and are highly chemosensitive and radiosensitive. The purpose of this study was to describe the clinical characteristics and outcomes of 3 children with EES and uncommon presentation treated in our Unit. The diagnosis of EES was confirmed by biopsy and cytogenetic analysis with fluorescence in situ hybridization (FISH). Surgical excision was planned as primary treatment, followed by adjuvant chemotherapy according to EURO-E.W.I.N.G protocol. To date, all patients are alive, 1, 3 and 4 years after completion of treatment, with no signs of recurrence or metastasis.

Lipid profile of children with acute lymphoblastic leukemia during L-asparaginase treatment.

Background: L-asparaginase is valuable in treating pediatric acute lymphoblastic leukemia (ALL), yet its use has been associated with lipid profile disturbances. Methods: We compared the lipid profile [high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, total cholesterol, triglycerides, apolipoprotein-α1 (Apo-Α1), apolipoprotein-B100 (Αpo-B100), lipoprotein-α (Lp-α), glucose, amylase, and lipase] between newly diagnosed ALL patients, ALL survivors, and healthy controls. We also assessed alterations of the parameters mentioned earlier during induction and consolidation treatment. Results: We recorded significant differences in the lipid profile at diagnosis of children with ALL compared to controls (HDL cholesterol, triglycerides, Apo-A1, and Apo-B100 levels). HDL cholesterol, total cholesterol, and Apo-Α1 levels increased significantly during induction at most time points. Levels of Αpo-B100, triglycerides, and Lp-α exhibited a downward trend. During re-induction, no change was observed. During the treatment of high-risk patients, we found no statistically significant difference for any of the examined variables. Conclusion: To confirm our preliminary results, the role of the administration of L-asparaginase and other medications in the variations in the lipid profile at diagnosis of children with ALL needs to be further elucidated with larger multicentre studies, including more patients from diverse ethnic backgrounds. HIPPOKRATIA 2023, 27 (2):41-47.

Osteopetrorickets in An Infant with Coexistent Congenital Cytomegalovirus Infection.

Osteopetrosis refers to a group of rare hereditary disorders characterized by generalized skeletal densification due to limited bone resorption by osteoclasts. The infantile autosomal recessive form represents the most malignant one with onset early in infancy and life expectancy less than 1-2 years without therapy. Frequently, osteopetrosis is complicated by rickets, a condition called osteopetrorickets. Currently, bone marrow transplantation remains the only treatment option. We present a case of infantile autosomal recessive osteopetrosis complicated by rickets in a 2 and a half-month-old female infant with coexistent congenital cytomegalovirus (CMV) infection, successfully treated by hematopoietic stem cell transplantation (HSCT). Diagnostic procedure and differential diagnosis are discussed along with a short review of the literature. Diagnosis of osteopetrosis requires high clinical suspicion, which is enhanced by radiology and confirmed by bone biopsy and molecular analysis. Our patient has been successfully treated by HSCT and has remained in a good general condition thereafter.

Bone involvement at diagnosis as a predictive factor in children with acute lymphoblastic leukemia.

BACKGROUND: Bone involvement represents a common symptom at diagnosis in children with acute lymphoblastic leukemia, and its prognostic value is not entirely clarified. The aim of this study was to evaluate bone involvement at diagnosis in children with acute lymphoblastic leukemia as a predictive factor and to correlate its presence with other demographic, clinical, and laboratory findings. METHODS: We retrospectively reviewed the medical records of 97 children with acute lymphoblastic leukemia diagnosed from January 2005 to December 2014. The mean age of patients was 5.7 years, and 83 (85.6 %) of them were diagnosed with B-acute lymphoblastic leukemia. RESULTS: Among the 97 children, 46 (47.4 %) reported bone involvement at the time of diagnosis. Among children with B-acute lymphoblastic leukemia 43/83 (51.8 %) reported bone involvement, while among children with T-acute lymphoblastic leukemia only 3/14 (21.4 %) (p =0.04). Bone involvement was registered more frequently among males (30/59; 50.8 %) in comparison to females (16/38; 42.2 %) (p =0.414). The mean white blood cell count at diagnosis was lower among children with bone involvement (109,800/mm3 vs. 184,700/mm3) (p =0.092). The mean age of patients with bone involvement was four years, which differs significantly from those without bone involvement (p =0.029). Moreover, children with bone involvement at diagnosis were prednisone "good responders" (79.5 %) when compared with those without bone involvement (58.8 %) (p =0.046). Additionally, mean serum phosphate values were higher at diagnosis among children with bone involvement (5.3 mg/dl vs. 4.8 mg/dl, p =0.035). CONCLUSIONS: The presence of bone involvement at diagnosis is related with immunophenotype of B-acute lymphoblastic leukemia, lower mean age, lower mean white blood cell count and good prednisone response. According to presented data, we conclude that the presence of bone involvement at diagnosis represents a positive predictive factor for outcome/survival. Hippokratia 2016, 20(3): 227-230.

History of Maternal Fetal Loss and Childhood Leukaemia Risk in Subsequent Offspring: Differentials by Miscarriage or Stillbirth History and Disease Subtype.

BACKGROUND: Despite the putative intrauterine origins of childhood (0-14 years) leukaemia, it is complex to assess the impact of perinatal factors on disease onset. Results on the association of maternal history of fetal loss (miscarriage/stillbirth) with specific disease subtypes in the subsequent offspring are in conflict. We sought to investigate whether miscarriage and stillbirth may have different impacts on the risk of acute lymphoblastic leukaemia (ALL) and of its main immunophenotypes (B-cell and T-cell ALL), as contrasted to acute myeloid leukaemia (AML). METHODS: One thousand ninety-nine ALL incidents (957 B-ALL) and 131 AML cases along with 1:1 age and gender-matched controls derived from the Nationwide Registry for Childhood Hematological Malignancies and Brain Tumors (1996-2013) were studied. Multivariable regression models were used to assess the roles of previous miscarriage(s) and stillbirth(s) on ALL (overall, B-, T-ALL) and AML, controlling for potential confounders. RESULTS: Statistically significant exposure and disease subtype-specific associations of previous miscarriage(s) exclusively with AML [odds ratio (OR) 1.67, 95% confidence interval (CI) 1.00, 2.81] and stillbirth(s) with ALL [OR 4.82, 95% CI 1.63, 14.24] and B-ALL particularly, emerged. CONCLUSION: Differential pathophysiological pathways pertaining to genetic polymorphisms or cytogenetic aberrations are likely to create hostile environments leading either to fetal loss or the development of specific leukaemia subtypes in subsequent offspring, notably distinct associations of maternal miscarriage history confined to AML and stillbirth history confined to ALL (specifically B-ALL). If confirmed and further supported by studies revealing underlying mechanisms, these results may shed light on the divergent leukemogenesis processes.

Socioeconomic disparities in survival from childhood leukemia in the United States and globally: a meta-analysis.

BACKGROUND: Despite advancements in the treatment of childhood leukemia, socioeconomic status (SES) may potentially affect disease prognosis. This study aims to evaluate whether SES is associated with survival from childhood leukemia. METHODS: The US National Cancer Institute Surveillance, Epidemiology and End Results Program (SEER) 1973-2010 data were analyzed; thereafter, results were meta-analyzed along with those from survival (cohort) studies examining the association between SES indices and survival from childhood leukemia (end-of-search date: 31 March 2014). Random-effects models were used to calculate pooled effect estimates (relative risks, RRs); meta-regression was also used. RESULTS: We included 29 studies yielding 28 804 acute lymphoblastic leukemia (ALL), 3208 acute myeloblastic leukemia (AML) and 27 650 'any' leukemia (denoting joint reporting of all subtypes) cases. According to individual-level composite SES indices, children from low SES suffered from nearly twofold higher death rates from ALL (pooled RR: 1.83, 95% confidence interval 1.00-3.34, based on four study arms); likewise, death RRs derived from an array of lower area-level SES indices ranged between 1.17 and 1.33 (based on 11 study arms). Importantly, the survival gap between higher and lower SES seemed wider in the United States, with considerably (by 20%-82%) increased RRs for death from ALL in lower SES. Regarding AML, poorer survival was evident only when area-level SES indices were used. Lastly, remoteness indices were not associated with survival from childhood leukemia. CONCLUSION: Children with lower SES suffering childhood leukemia do not seem to equally enjoy the impressive recent survival gains. Special health policy strategies and increased awareness of health providers might minimize the effects of socioeconomic disparities.

Association of fetal loss with recent parvovirus infection and other demographic prognostic risk factors.

A total of 206 pregnant women were prospectively enrolled to this case-control study, from which 71 and 65 women had 1st and 2nd trimester miscarriage, respectively. The remaining 70 women with uneventful pregnancy were the control group. The serological profile of parvo B19 infection was confirmed with ELISA. Electron microscopy was selectively conducted in the patients' group. Recent infection rate in women with 1st and 2nd trimester miscarriage was 3.68% and 5.8%, respectively. Univariate analysis revealed significant association between miscarriage and ethnicity (p = 0.04), type of work (p = 0.019), children attending school (p = 0.012) and recent parvovirus B19 infection (p = 0.013). Pregnant women with recent infection had a two-fold higher risk (OR = 1.94) for miscarriage. The association between 1st and 2nd trimester miscarriage rates in the women with recent parvovirus B19 infection, was not significant (p = 0.29). Multivariate analysis showed that recent parvovirus B19 infection was higher in women with children at school (OR = 3.5, p = 0.036). Placental tissues and specific histological findings were only detectable in the patients group.

Epileptic seizures after octreotide administration in a 6.5-year-old female with ALL and L-asparaginase associated pancreatitis: a possible drug interaction.

INTRODUCTION: Octreotide is a synthetic somatostatin analogue which has been suggested for use in the management of acute pancreatitis, though its safety and effectiveness in the pediatric setting has not been extensively studied. CASE REPORT: we present a rare case of a 6.5-year-old female with acute lymphoblastic leukemia (ALL) and L-asparaginase (L-asp) induced pancreatitis, who developed epileptic seizures, possibly associated with octreotide administration. Her imaging and laboratory findings ruled out a leukemic involvement or infection of CNS. The EEG revealed repetitive sharp waves maximal on the frontal and temporal areas of the right hemisphere. The child was treated with diazepam and she continued with systemic anticonvulsant treatment with levetiracetam. After 2 weeks of conservative treatment, pancreatitis resolved and she continued her chemotherapy protocol. Levetiracetam treatment lasted 8 months. 7 months after the first episode, EEG was reported as normal, and the child completed the chemotherapy protocol without any further severe complications. CONCLUSIONS: Larger and well designed studies are needed to warrant the safety of octreotide in pediatric population.

Ovarian tumours in childhood and adolescence.

Malignant ovarian tumors are very rare during childhood and adolescence where their incidence is approximately 0.71 per 100,000. We reviewed the symptoms, histologic subtypes, diagnostic evaluation and management of ovarian tumours in children and adolescents with emphasis on malignant tumours. The histology of these tumours is complex and their diagnosis frequently poses problems. Surgery and chemotherapy yield high cure rates in patients with malignant germ cell tumours whereas prognosis is poorer in ovarian carcinomas. Elucidation of the mechanisms underlying the pathogenesis of ovarian tumors might further improve the management of these patients.

Alveolar soft-part sarcoma of the extremity: a case report.

Alveolar soft-part sarcoma (ASPS) is a rare form of soft tissue sarcoma and is most often seen in adolescents and young adults. Surgical excision of the primary tumor and pulmonary metastases has resulted in prolonged survival in some patients while the benefit of adjuvant chemotherapy and/or radiotherapy has been disputed. An 11- year-old boy with ASPS which presented with a markedly vascular tumor in the left thigh, and multiple bilateral pulmonary metastases 8 months after diagnosis is described. The patient has remained disease-free for over 5 years since the initial diagnosis.

Knowledge and practice of contraception among Greek female medical students.

OBJECTIVE: In Greece, modern contraceptive methods are used on a limited scale. This study aimed to investigate the knowledge as well as the practice of contraception among female medical students in Greece. STUDY DESIGN: Knowledge and practice of contraception of 102 female graduating medical students were assessed with a self-administered, anonymous questionnaire. RESULTS: Most students were using condoms as the only contraceptive method (45.1%) and 16.7% were not applying any contraceptive method at all. Oral contraceptives were used as only contraceptive means by 4.9% of the students and in combination with condoms by another 4.9%. Condoms were thought to be the most effective contraceptive method available by 28.4% of the students, whereas 4.9% responded that they did not consider any contraceptive method to be effective. Only 55.9% of the students had ever asked their gynaecologist about contraception. CONCLUSION: Much more time should be spent in teaching contraception in the Greek medical schools to ensure the delivery of adequate family planning guidance by future practitioners.

Use of amifostine in the treatment of recurrent solid tumours in children.

AIM: Preclinical and clinical evaluation of amifostine (AMI) administration in conjunction with systemic chemotherapy supports its role as a cytoprotective agent of normal tissues without loss of impairing the antitumour effectiveness of chemotherapeutic agents. Since only a limited number of clinical studies has been performed using AMI in paediatric pts with malignancies we investigated the protective effect of AMI against carboplatin-induced myelotoxicity and nephrotoxicity in a paediatric group of patients. MATERIAL AND RESULTS: AMI was administered in 18/28 paediatric patients with reccurent solid tumours along with ICE (ifosfamide, carboplatin, etoposide) chemotherapy. A significant (p<0.05) decrease in GFR was observed in the control group whereas it was maintained at pre-treatment levels in the AMI-treated group. Leukopenia and neutropenia were significantly (p<0.05) less in AMI-group. No protective effect of AMI was shown concerning thrombocytopenia. CONCLUSIONS: AMI was generally well tolerated at the dose of 740 mg/m2. Side effects including nausea, vomiting, hypotension, flushing and rigors were moderate and reversible and the interruption of infusion was never required.