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Primary immune deficiencies are a group of heterogenous genetic disorders characterized by frequent infections, autoimmunity and malignancy. In this study, we aimed to evaluate clinical characteristics, outcomes of children with malignancy developed on background of primary immunodeficiency and compare survival rates of patients between malignant lymphoma with primary immunodeficiency and without immunodeficiency from tertiary oncology center in a developing country. A total 23 patients with primary immunodeficiency and malignancy were evaluated retrospectively. A total of 26 malignancies (first or second) in 23 patients were determined. The median age at the time of the first malignancy was 8 years (ranges 2–18 years) with increased male ratio (M/F:14/9). Non-Hodgkin lymphoma (n = 17; 65%) was the most common malignancy, followed by Hodgkin lymphoma (n = 5), anaplastic ependymoma (n = 1), spinal glioblastoma multiforme (n = 1), retinoblastoma (n = 1) and intracranial hemangiopericytoma (n = 1). The median follow-up time of patients was 25 months (ranges between 1 and 189 months). The 5-year overall survival rate of patients with malignant lymphoma associated with primary immunodeficiency (41%) were lower than immunocompetent patients with malignant lymphoma (80%) (p = 0.000). The 5-year overall survival of patients was diagnosed between 2021 and 2013 years (62%) was higher than previous years (22%) (p = 0.03). In conclusion, non-Hodgkin lymphomas were the most common histopathologic type in patients with malignancy associated with primary immunodeficiency in the present study. The survival of patients with malignant lymphoma associated with primary immunodeficiency has improved in recent years, yet it is still lower than immunocompetent patients with lymphoma and new targeted drugs are required for better survival rates. [ABSTRACT FROM AUTHOR]

Malnutrition is a common consequence of cancer in children, but the most effective methods of nutrition intervention are under debate. We aimed to evaluate the nutritional status of children diagnosed with cancer, and to investigate the effect of oral nutritional supplements on anthropometric measurements, biochemical parameters, and outcome. A randomized clinical study of 45 newly diagnosed cancer patients was performed. Anthropometric and biochemical data and related factors were assessed at 0, 3, and 6 months after diagnosis. On initial anthropometric assessment, prevalence of malnutrition by weight or height was found to be lower as compared with body mass index (BMI), or weight for height (WFH), or arm anthropometry. Twenty-six of the patients (55%) received oral nutritional supplement. During the second 3 months after diagnosis, there was a statistically significant decrease in number of the patients with WFH <90th percentile and BMI <5th percentile (P =.003 and P =.04, respectively). Infectious complications occurred more frequently in malnourished patients during first 3 months, and survival of children who were malnourished at the 6th month was significantly lower than that of well-nourished children (P =.003). On laboratory assessment, serum prealbumin levels of the all subjects were below normal ranges, but no relation was found for serum prealbumin or albumin levels in patients who were malnourished or not at diagnosis. Nutritional intervention is necessary to promote normal development and increase functional status as a child receives intensive treatment. Protein- and energy-dense oral nutritional supplements are effective for preventing weight loss in malnourished children. [ABSTRACT FROM AUTHOR]

Primary ovarian malignant melanoma arising in teratomatous component of germ cell tumors is seen extremely rare with most reports being only of single cases and small series in reproductive aged woman and mostly from cystic teratoma, whereas information on pediatric presentation is sparse. This case is reported for being extremely rare tumor. [ABSTRACT FROM AUTHOR]

Background: An increased incidence of non-Hodgkin lymphoma (NHL) has been seen in various primary immune deficiency (PID) cases. The present study aimed to evaluate the clinical characteristics and treatment outcomes of five cases with NHL associated with primary immunodeficiency. Methods: We retrospectively evaluated five patients with primary immunodeficiency who developed NHL. Two patients had ataxia-telangiectasia (A-T), one patient had common variable immunodeficiency (CVID), one patient had Bloom's Syndrome, and one patient had Wiskott-Aldrich syndrome (WAS). Results: All patients were male (median age, 8 years). Stage distribution was stage III in three patients and stage IV in two patients. Three patients had B-cell lymphoma and two had T-cell lymphoma. Reduced doses of Berlin-Frankfurt-Münster (BFM) and French Society of Pediatric Oncology (SFOP) regimens were used in four patients according to histopathological subtype. The two patients with ataxia and one patient with Bloom's Syndrome died of progressive/relapsed disease at months 5, 19, and 6, respectively. The patient with CVID associated with T-cell lymphoma has been in remission for 7 years. A full-dosage regimen of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) was successfully used in the patient with WAS and B-cell lymphoma; he was still in remission after 3 years. Conclusion: Primary immunodeficiency diseases are one of the strongest known risk factors for the development of NHL. Management of these patients remains problematic. There is a great need to develop new therapeutic approaches in this group. The use of rituximab in combination with CHOP may provide a promising treatment option for B-cell lymphomas associated with immunodeficiency. [ABSTRACT FROM AUTHOR]

Cardiac rhabdomyomas (CRs) are the most common heart tumors in children and closely associated with tuberous sclerosis complex (TSC). This study was performed to assess the presentation type, clinical course, treatment modalities, and outcome of the patients with rhabdomyoma, associated with TSC. We reviewed our patients with cardiac rhabdomyomas (CRs), who had received a diagnosis of TSC previously or during the follow-up period between June 1996 and January 2012, retrospectively. Thirty-two patients with TSC were evaluated and among them 11 patients (34%) were associated with CRs. Five patients (45%) had multiple tumors and consequently a total of 29 CRs were analyzed in our study. The median follow-up period was 2 years (range: 1 week-15 years). Clinical presentation was cardiac murmur in three patients, cyanosis in two patients and arrhythmia in one patient. Five patients were asymptomatic at the diagnosis and CRs were detected during routine cardiac evaluation for TSC. Cardiac tumors were diagnosed prenatally in two patients. Spontaneous regression rate was 31% and we experienced a complete regression of a tumor with an echogenic bordered tissue defect and septal thinning in a patient. Three patients had hemodynamically significant tumor obstruction; two of them underwent surgery. The other patient, who had multiple CRs, was treated medically with everolimus because of high-risk potential of surgery. Although surgical resection is the preferred treatment in most of the patients with hemodynamic instability, we need novel alternative medical therapies in some critically ill patients who cannot be operated due to various reasons. [ABSTRACT FROM AUTHOR]

Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure. [ABSTRACT FROM AUTHOR]

Although both Hodgkin lymphoma and cyst hydatic disease in children have been seen with an increased frequency, there is no previously reported case of Hodgkin lymphoma associated with cyst hydatic disease from Turkey. The authors report such a case of Hodgkin lymphoma. Intrahepatic cystic masses were diagnosed during ultrasound examination for clinical staging on admission. The diagnosis of cyst hydatic of the liver was confirmed by surgery. Although there was no residual and/or new cyst formation on radiologic follow-up, elevated antibody titers (indirect hemagglutination test) persisted following surgical excision at least for 2 years of follow-up. [ABSTRACT FROM AUTHOR]

Veno-occlusive disease (VOD) is a rare complication of chemotherapy in children with cancer. In the literature successful treatment of severe VOD has not been well determined. The authors report a 5-year-old boy with Willms tumor whose condition was complicated with VOD while receiving chemotherapy. He was treated successfully with high-dose methylprednisolone. After therapy, the clinical signs of VOD, such as abdominal ascites, oliguria, and jaundice, disappeared without any adverse effects of therapy. [ABSTRACT FROM AUTHOR]

Children with cancer are at an increased risk of hepatitis B infection and chronic liver disease. Since hepatitis B vaccines containing pre-S2 antigen has been recently reported as being more efficient in providing immunization in healthy individuals, the authors compared antibody response to pre-S2-containing vaccine with no-pre-S2-containing hepatitis B vaccine, when given in double doses to 100 children receiving chemotherapy. Patients, aged 1 to 16 years with negative HBV serology, were vaccinated with 2 different types of HBV vaccines between 1997 and 1999. Group 1 received Gen Hevac B containing pre-S2 (n = 41) in a dose of 20 μg for patients younger than 10 years old and 40 μg for older patients. Group 2 was vaccinated at the same dose with hepatitis B vaccines not containing pre-S2 antigen. All vaccinations were repeated at 0, 1, and 6 months. Serum samples were drawn for determination of anti-HBs titers at 1, 3, 6, and 8 months. After the third dose of vaccine, the seroconversion rate was 72% in group 1 and 62% in group 2. The anti-HBs levels were higher in the group receiving pre-S2-containing hepatitis B vaccine. However, the difference between groups was not statistically significant (p > .05). The administration of pre-S2-containing hepatitis B vaccines may give a better seroconversion and higher antibody response to vaccination in children with cancer. But a further large-scale study is needed to confirm this finding. [ABSTRACT FROM AUTHOR]

Peripheral neuropathy is one of the rarely reported neurological complications of interferon therapy. The authors report such a case in a 15-year-old boy during alpha-interferon therapy for Hodgkin's disease. He received alpha-interferon at a dose of 1.8 million units/day 5 times a week by subcutaneous injections as adjuvant immunotherapy post autologous stem cell transplant. Twenty months after the initiation of therapy, he complained of severe pain in his lower distal extremities. Neurological examination revealed the absence of deep tendon reflexes. A nerve conduction study showed a sensorial, polyneuropathic involvement in the lower extremities. Within 4 weeks after the stopping of interferon, his pain improved, and recovery was also seen by nerve conduction studies. Symptoms reappeared at the resumption of interferon treatment. This study suggests that peripheral neuropathy may rarely occur in patients given long-term interferon treatment at high cumulative dosage. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses the case study of a six-year-old girl who was diagnosed with Wilms tumor (WT) with urofacial syndrome (UFS) or ochoa syndrome.