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.

Schwannoma Localized Retroperitoneally in a 14-Year-Old Boy.

Schwannomas usually occur in adults being between the second and fifth decades, and such neoplasms are extremely rare in a pediatric population. In addition, they are not normally found in the retroperitoneal region. Here, we present a pediatric case of a retroperitoneal schwannoma in an adrenal location where the tumor was not able to be preoperatively differentiated from other benign or malign adrenal gland tumors. In our opinion, this tumor can be included in the differential diagnosis of a nonfunctioning retroperitoneal adrenal mass in children.

Outcome in neuroblastoma.

OBJECTIVE: To determine outcome of neuroblastoma (NBL) in children under 18 mo of age who had been treated with national protocols. METHODS: The characteristics and treatment outcomes of 27 children were evaluated retrospectively. RESULTS: The event-free survival (EFS) at 60 and 108 mo were 84.7 % ± 7.7 and 72.6 % ± 7.7, respectively. The overall survival (OS) was 91.7 % ± 8 at 108 mo. The only significant risk factor for OS in children with neuroblastoma was the treatment response at the end of therapy (p = 0.001). "Wait and see" policy was applied to two infants with low risk NBL and one infant with stage 4S neuroblastoma and all 3 of these infants have been in remission at last followup. Four of the five patients with MYCN-amplified neuroblastoma were alive at a median follow-up time of 54 mo (range: 5-108 mo). CONCLUSIONS: The EFS and OS of the present group were similar to that of the previous series which included children under 18 mo of age with neuroblastoma. Well known prognostic factors did not affect EFS and OS significantly; this may be related to the retrospective design of the present study and the small number of patients reviewed. High survival rate in infants with MYCN-amplified tumors suggests the difference in the biology of infant neuroblastoma.

Neurofibromatosis type 1 and diabetes mellitus: an unusual association.

Neurofibromatosis type 1 is a multisystemic disease. It may manifest as abnormalities of the nervous tissue, bones, soft tissue, or skin. Autoimmune disease associated with NF1 can be seen. Diabetes mellitus is rarely seen in association with NF1. Here, we report a case with established NF1 who also had a diagnosis of diabetes mellitus.

Ewing's sarcoma metastasis into the breast.

The metastasis of extramammary malignancies into the breast is very unusual. Lymphoma, malignant melanoma, and rhabdomyosarcoma are the most common tumors that metastasize into breast tissue. The histological spectrum of breast masses in children and adolescents is different from that of adults. Imaging findings are useful for performing a diagnosis, but in a patient with a known malignancy, any enlarging breast mass, even one with a benign radiological appearance, should be investigated with a biopsy. In this article, we present the imaging findings of a 12-year-old female patient with breast metastasis of Ewing's sarcoma.

Soft tissue sarcomas and central nervous system tumors in children with neurofibromatosis type 1.

OBJECTS: We aim to evaluate the characteristics of pediatric patients with neurofibromatosis type 1 (NF1) who developed soft tissue sarcomas (STSs) and central nervous system (CNS) tumors that have been followed up in our center. MATERIALS AND METHODS: Medical records of children with NF1 were retrospectively analyzed. RESULTS: There were 78 patients who met at least two diagnostic criteria for NF1. The median age of patients was 10 years (0.5-18), and M/F ratio was 1.3. The prevalance of the optic glioma was 11.5% (n = 9), and one patient with optic glioma also had cystic astrocytoma, one patient had brain stem tumor, and one patient had a CNS tumor (without histopathologic diagnosis). Seven of nine children were ≥ 7 years old at the time of the diagnosis of optic glioma. Visual impairment developed in four patients, and two of them were treated with radiotherapy solely on the basis of evidence of clinical and radiological progression of the tumors. Four patients developed STSs. Two of them had malignant peripheral nerve sheath tumors (MPNST), and the remaining two had bladder rhabdomyosarcoma. Three of the four patients with STSs died with progressive disease. CONCLUSION: The clinical course of malignancy in NF1 is often different from that of similar tumor types in the general population. Careful follow-up in patients with NF1 is required to enable the early diagnosis of malignancies, and the developments of new targeted therapies are needed for improvement of the outcome for patients of this group, especially with MPNST.

Evaluation of nutritional status in Turkish adolescents as related to gender and socioeconomic status.

OBJECTIVE: To evaluate the nutritional status of Turkish high school adolescents using anthropometric indicators and to determine the relationship of nutritional status with gender and socioeconomic status (SES) in adolescents. METHODS: Six hundred eighty adolescent students (n=284 males, 396 females) aged 14-18 years were selected from 6 high schools of different regions. Nutritional status was evaluated according to the anthropometric indicators, which were based on the WHO criteria. Adolescents were grouped into three SES categories. RESULTS: The rates of being stunted, underweight, and overweight/obesity were 4.4%, 5.0% and 16.8%, respectively. Height and weight standard deviation scores (SDS) were significantly lower in adolescents with low SES (p<0.05). The frequency of stunting was significantly higher in adolescents with low SES (p=0.012). Frequency of underweight, overweight and obesity did not differ significantly between socioeconomic groups and genders (p>0.05). CONCLUSION: Adolescents of low SES were shorter and thinner than those of other SES categories. Undernutrition needs to be addressed in low SES. Among all Turkish adolescents, the major nutritional problems were overweight and obesity. There were no SES and gender differences in prevalence of overweight and obesity among the Turkish school adolescents living in urban areas. Prevalence of obesity is rising, regardless of differences in SES and gender, in developing countries too.

Delayed renal excretion of methotrexate after a severe anaphylactic reaction to methotrexate in a child with osteosarcoma.

Although methotrexate is an agent widely used in the practice of pediatric oncology, allergic reactions to methotrexate are most unusual. Most of these reactions typically occur after repeated administration. Here, we report a severe anaphylactoid reaction to the first dose of high-dose methotrexate infusion in a child with osteosarcoma who has also experienced a delayed excretion of methotrexate. Clinicians must be aware of the possibility of a systemic, near-fatal anaphylactic reactions with methotrexate and patients who experience severe anaphylactic reactions should be followed carefully because of the possibility of delayed methotrexate excretion.

Pilomatricoma in children: a frequently misdiagnosed superficial tumor.

Pilomatricomas are the most common superficial tumors in children, but they are frequently misdiagnosed preoperatively. There are some characteristic features of pilomatricomas that can help clinicians differentiate it from other tumors. The authors report 3 children with head and neck pilomatricomas, one with multiple tumors. They emphasize some clinical features that may help in differential diagnosis to avoid unnecessary investigations before surgical removal.

Skin and soft tissue complications in pediatric leukemia patients with and without central venous catheters.

We aimed to retrospectively evaluate the skin and soft tissue complications secondary to procedures in acute leukemia patients with and without catheters. Eighty-seven acute leukemia patients (75 acute lymphoblastic leukemia, 12 acute myeloid leukemia ) were included. There were 30 patients with 37 catheter use (6 port, 31 Hickman catheter) and 57 patients without catheter. In patients with catheters, skin and soft tissue complications were seen in 20 (66%) children. The most frequent complication was cellulitis (55%). In the patients without catheter, skin and soft tissue complications were seen in 37 (65%) patients. Cellulitis (37.8%) and extravasation (37.8%) were the most frequent causes. When the frequency of skin and soft tissue complications in patients with and without catheters were compared with each other, there was statistically no significant difference (P=0.792). The duration of chemotherapy was significantly longer in patients who developed skin and soft tissue complications with or without catheters when compared with the duration of the therapy in patients without any skin and soft tissue complications (259.2+/-36.3 and 218.3+/-58.3 d, respectively; P<0.0001). In pediatric leukemia patients, with or without catheters, skin and soft tissue complications are common and these complications may prolong the duration of chemotherapy.