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The outcome of Ewing's sarcoma depends on the anatomical site of the tumor. Studies conducted in high-risk patients are limited. We evaluated the outcome of high-risk Ewing's sarcoma patients that received long-term treatment protocol. Twenty-five patients (22 males, 3 females) with poor prognostic features were treated according to long-term Ewing's sarcoma protocol. Central-axis localization, inadequacy or unavailability of surgical resection, older than 15 years of age, are accepted as high-risk factors. The median age of patients was 23 years (range, 18-55). The tumor localization was pelvis (9), femur (1), tibia (1), fibula (1), maxilla (1), clavicle (1), vertebrae (5), metatarse (1), and ribs (5). Neoadjuvant chemotherapy was applied between weeks 0 and 6, local therapy on week 9, and adjuvant maintenance chemotherapy between weeks 11 and 41. All patients received neoadjuvant and adjuvant maintenance chemotherapy. Local therapy consisted of radiotherapy (32%), surgery alone (12%), or surgery and radiotherapy (56%). The median total treatment period was 10 months. The median follow-up was 25 months (range, 7-89). Three-year cumulative OS and DFS rates were 43% (95% CI, 28.5-57.85) and 40% (95% CI 23.63-52.19), respectively. The most common grade III/IV toxicities observed during the treatment protocol were neutropenia (16%) and gastrointestinal toxicities (16%). Our study indicated that long-term multiagent combination chemotherapy may result in better outcome in adult high-risk patients undergoing adequate surgical resection of the tumor and local radiotherapy. Further randomized studies are needed to assess the efficacy of this treatment protocol in patients with adequate surgical margins.

Multiple myeloma (MM) is a malignancy arising from mature plasma cells in the bone marrow and usually presents with bone destruction, hypercalcemia, anemia, renal damage, and increased susceptibility to infection. The etiology of MM is unknown, with no established lifestyle, occupational, or environmental risk factors. Because MM is an uncommon disease, etiologic assessments can be difficult. It has been reported to be in association with sarcoidosis, and in a few cases, rheumatoid arthritis. Familial type of MM with an autosomal dominant heredity pattern has also been reported. The genetic loci affected in these cases are still unknown. Herein we present a family with 3 affected cases in an autosomal dominant inheritance pattern. The first case was a man diagnosed to have immunoglobulin (Ig)A-type myeloma at the age of 50. The history revealed 2 more cases in the family: an uncle diagnosed to have unsecretory-type myeloma at the age of 76 and a cousin (the daughter of the affected uncle) who was diagnosed at the age of 48 years to have IgG-type myeloma and did not respond to therapy. This patient also had a history of sarcoidosis preceding the diagnosis of myeloma. All other affected family members had been treated for dental-oral infection (including chronic gingivitis) for 3 and 4 years before the diagnosis of myeloma. Karyotype analysis revealed pseudohypodiploidy and deletion of chromosome 13q in only the patient with coexisting sarcoidosis. To our knowledge, this is the first report on familial myeloma from Turkey. This family enhances the role of hereditary factors and chronic inflammation in the etiology of MM.

Haploidentical hematopoietic stem cell transplantation (HSCT) is currently one of the alternative curative treatment options for some nonmalignant but also for malignant diseases. However, concerns regarding its safety cause delays in time and a successful outcome. Between 2000 and 2005, twenty-one children with poor prognostic nonmalignant disorders, 13 boys and 8 girls, with a median age of 12 months, underwent 28 haploidentical peripheral HSCT. Immunomagnetic bead depletion device (CliniMACS) was used for indirect T-cell depletion. Indications for transplant were severe combined immunodeficiency (n=16), osteopetrosis (n=2), MDS (n=1), amegakaryocytic thrombocytopenia (n=1), and aplastic anemia (n=1). Five patients (24%) had lung infection at the time of transplantation. The patients received a median of 25.67 x 10(6) G-CSF-mobilized peripheral CD34(+) progenitor cells and a median of 4.19 x 10(4) T-lymphocytes per kilogram of body weight with a T-cell depletion rate of median 4.59 logs. The rate of total engraftment was 66.6%. Median times for leukocyte and platelet engraftment were 14 and 16 days, respectively. The 6-year projected survival was 32% for all patients and 29.76% for patients with severe combined immunodeficiency (SCID). The rates of transplant-related mortality, graft failure, and severe GvHD were 14.2, 33.4%, and 8.3%, respectively. Infection was the main cause of death. The poor outcome may be explained with the poor prognostic factors of our patients such as the type of SCID in most cases (T-B- SCID), the median age over 6 months and the presence of lung infection in some children at the time of transplantation.

Aims and Background: The role of high-dose chemotherapy in breast cancer has not been fully defined. It has been concluded that new trials should focus on defining potential subgroups that are more likely to benefit from high-dose chemotherapy. We compared survival differences in patients receiving human granulocyte-colony stimulating factor (G-CSF) or granulocyte-monocyte colony stimulating factor (GM-CSF) after high-dose chemotherapy with stem cell support., Methods: High-risk non-metastatic breast cancer patients (axillary lymph node involvement more than 8) aged 16 to 65 years and with a performance status < or = 1 underwent high-dose chemotherapy with autograft. Written informed consent was obtained from every patient, and the study was approved by the local ethics committee., Results: For 54 eligible women, the median follow-up was 41.4 months. The five-year disease-free survival was 45.7%. The five-year projected overall survival rate was 53.9%. Among them, patients who received GM-CSF (n = 12) posttransplant lived longer than the patients who received G-CSF (n = 15) (five year survival rates, 46.6% vs 75%, P < 0.050). The patients who received GM-CSF posttransplant had fewer relapses (5 vs 9). However, between the two groups there was no statistically significant difference regarding disease-free survival rates calculated with the Kaplan-Meier method (58.8% vs 40%; P = 0.121)., Conclusions: Patients receiving GM-CSF posttransplant lived longer and they had fewer relapses than those who received G-CSF. This result merits consideration. The antitumor activity of GM-CSF should be investigated further in prospective randomized trials.

Objective: To investigate the impact of c-erb2 status on survival after high-dose chemotherapy., Methods: Between March 1997 and June 2004, a total of 54 women with breast cancer who has at least 8 metastatic lymph nodes underwent high-dose chemotherapy with hematopoietic stem cell transplantation in Gulhane Military Medical School, Ankara, Turkey. Archival specimens were analyzed by fluorescent in situ hybridization to determine the impact of c-erb2 status after peripheral blood stem cell transplantation on survival. The patients were divided into c-erb2 negative (n=20) and positive (n=11) groups., Results: No statistically significant differences were detected between c-erb2 negative and positive groups regarding 5-year disease-free survival (41 and 27%, log rank p=0.11), and overall survival (60 and 45%, p=0.33). Transplant related mortality did not differ between groups., Conclusion: We found no differences between c-erb2 negative and positive groups regarding disease-free and overall survival. To clarify the value of the c-erb2 status in predicting outcome after high-dose chemotherapy, prospective randomized studies are needed.

Background: Adiponectin is a new adipocyte-secreted protein and associated with insulin-resistant status, such as type 2 diabetes mellitus and obesity. The inverse correlation between serum adiponectin levels and breast cancer risk was recently documented. On the other hand, the association of tissue adiponectin levels with breast cancer has not been previously reported. Thus, in the present study, the relationship between tissue adiponectin levels and breast cancer was evaluated., Methods: We analyzed the correlation between tissue adiponectin levels and the occurrence of breast cancer in a case-control study comprising 27 women with diagnosed and histologically confirmed breast cancer and 33 women with fibroadenoma. In addition, the association of tissue adiponectin levels with the various classical risk factors, such as body mass index, menopausal status and, tumor size, stage, lymph node status, hormonal status were also studied., Results: Tissue adiponectin levels in patients with breast cancer (0.75 +/- 0.06) were significantly higher than those in controls (0.68 +/- 0.1) (P = 0.02). The high tissue adiponectin levels were associated with significantly (P = 0.001) an increased risk for breast cancer compared with those in the low tissue adiponectin levels (OR, 1.34; 95% CI, 1.12-1.84) in breast cancer patients. In addition, postmenopausal women with the high tissue adiponectin levels showed a significantly (P = 0.003) an increased risk for breast cancer compared with women in low tissue adiponectin levels (OR, 1.63; 95% CI, 1.23-1.90). The correlation between BMI and breast cancer was not found (P > 0.05). Furthermore, the status of estrogen receptor, progesterone receptor, HER-2/neu receptor and lymph nodes involvement were established, no effect on the tissue adiponectin levels in patients with breast cancer and no correlations were detected among tumor stage, tumor size and the levels of tissue adiponectin (P > 0.05)., Conclusion: Our results suggest that the high tissue adiponectin levels significantly detected in breast cancer patients and associated with an increased risk for breast cancer.

Purpose/objectives: To determine whether a relationship exists between quality of life (QOL) and the sociodemographic characteristics of gender, age, marital status, educational level, occupation, and level of income in patients with cancer in Turkey., Design: Exploratory study using a convenience sample., Setting: Five hospitals in the capital city of Turkey., Sample: 620 patients with cancer (44 inpatients and 576 outpatients)., Methods: The study was carried out using face-to-face interviews. A questionnaire was used as a tool for collecting data on the sociodemographic characteristics of patients, and the Rolls-Royce Quality-of-Life Scale was employed to measure QOL., Main Research Variables: Gender, age, marital status, educational level, occupation, level of income, and QOL., Findings: Men, older adults, widowed spouses, patients with lower levels of education, housewives, and those with lower income had lower QOL scores., Conclusions: Considering the results and giving the groups referred to in the study priority in the planning of patient care would be useful., Implications for Nursing: In the field of nursing in Turkey, a limited number of studies have examined the relationship between the sociodemographic characteristics of patients with cancer and QOL. The results will help nurses assess patient needs and engage in nursing interventions that are appropriate to the needs.

Breast cancer is the most common malignancy which affects women. In 5-10% of all cases, breast cancer presents as a hereditary cancer syndrome. Since 1996, 68 families with suspicion of familial breast cancer have been referred to our department. In 5 of the 68 families (7.4%), the clinical diagnosis was hereditary breast ovarian cancer syndrome. In 17 families (25%), two or more breast cancer cases were present. Mutation screening of BRCA1 and BRCA2 in these families revealed a BRCA1 mutation (185delAG) in one family. Three families (4.4%) had a diagnosis of Li-Fraumeni syndrome and germline mutations in TP53 (Lys292Ile, Pro278Ser and Pro278Thr). Breast cancer occurred in a family with hereditary nonpolyposis colon carcinoma (HNPCC; Lynch syndrome) carrying an MLH1 mutation (IVS17-3G>C). Most of our families (41 families; 60.2%) had only one case with breast cancer or cystic adenoma (or both) and did not need counseling and DNA testing. In summary, in 10 of the 68 families in our series (14.7%), a germline mutation in a breast cancer predisposing gene was detected. Our data show the importance of detailed examination of clinical data, pedigree analyses, and molecular germline diagnostics for the counseling of breast cancer cases.

Primary cutaneous B-cell lymphomas (PCBCL) are rare and constitute 5-10% of all cutaneous lymphomas. In patients with PCBCL presenting with solitary or localized skin lesions, radiotherapy is the preferred treatment. Two patients who were treated with 4 MeV electrons, both obtained remission for 51 months. Unfortunately, at the last visit one patient relapsed on the border of the radiotherapy field and was re-treated with a generous irradiation field in 2004. Complete response was obtained again. Thus, for localized PCBCL, radiotherapy alone is an effective treatment.

This prospective study was carried out in healthy donors and patients. The performance of the CliniMACS was evaluated with the comparison of the numbers of total nucleated cell (TNC) within and over the capacity of the normal scale column. In addition, large vs. normal scale column and manual vs. automated washing systems were also compared. A total of 44 selections were done. Eighteen normal scale selections were done with initial TNC numbers over 6 x 10(10) and 14 selections were performed below this number. None of the cases had CD34+ cell numbers over the capacity. Flow cytometry was used to check each separation performance for purity and recovery of CD34+ cells along with T- and B-cell depletion level parameters. All healthy donors had significantly better mean purity and recovery of CD34+ cells, and T- and B-cell depletion status than that of patients with values 95 vs. 85%, P: 0.006; 77 vs. 58%, P: 0.004; 4.55 log vs. 4.06 log, P: 0.004; 3.19 log vs. 2.63 log, P: 0.01, respectively. However, the performance of the system was not dependent on using the normal or large-scale column; automated or manual washing systems; and initial TNC numbers above (>6 x 10(10), range: 7.05-21.84 x 10(10), mean: 12.32 x 10(10)) or within (<6 x 10(10), range: 0.86-5.89 x 10(10), mean: 4.15 x 10(10)) the column capacity. In conclusion, the performance of the CliniMACS is more efficient in healthy donors than in patients. However, the performance of the system did not change as long as the numbers of CD34+ cells (range: 0.34-5.87 x 10(8)) were not exceeding the column capacity despite that more than 6 x 10(10) TNCs were used.

In the past, variable results were reported for single daily and two divided daily doses of granulocyte colony-stimulating factor (G-CSF) in stem cell collection where no study exists investigating the effect of body mass index (BMI) on mobilization. The numbers of CD34(+) cells collected were compared in 86 patients with solid or hematological malignancies receiving either single daily (14 mug/kg/day) G-CSF (filgrastim) as group I (n=36) or two divided doses of G-CSF daily (2 x 7 mug/kg/day) as group II (n = 50). Both groups were divided into subgroups according to their BMI as group a (BMI 25 kg/m(2)). Two groups were similar in terms of BMI, gender, and disease characteristics. All patients have received G-CSF as a single or two divided doses subcutaneously and aphereses have been done on the 5th day. No significant difference in numbers of CD34(+) cells between groups Ia and Ib, groups IIa and IIb, and groups Ia and IIa was found. On the other hand, the mean ratio and the number of CD34(+) cells in group Ib were significantly higher than those of group IIb (0.58 +/- 0.06% vs. 0.37 +/- 0.26%, P = 0.01 and 3.67 +/- 0.65 x 10(4)/kg/ml vs. 1.92 +/- 0.37 x 10(4)/kg/ml, P= 0.02). In conclusion, in patients with BMI >25 kg/m(2), once daily G-CSF compared to split dose administration induces a greater number of CD34(+) stem cell mobilization, which suggests the presence of a different pharmacokinetics in obese patients., (Copyright 2003 Wiley-Liss, Inc.)

Objectives: One of the major challenges in the post-transplant period is nutrition. In this prospective, non-randomized study, total parenteral nutrition (TPN) was given to 31 patients and partial parenteral nutrition (PPN) was given to 30 patients undergoing autologous hematopoietic stem cell transplantation for solid tumors or hematologic malignancies to compare the effects of these parenteral nutrition modalities on post-transplant hematological engraftment, blood chemistry, and supportive therapy requirements., Methods: All patients in the TPN group and 17 patients in the PPN group received growth factor in the post-transplant period. Both groups did not differ with respect to sex, age, and reinfused CD34(+) cell numbers., Results: After transplantation body mass index and body weight decreased significantly in both groups (P < 0.001). Whereas serum albumin concentrations did not decrease significantly in the TPN group, it fell markedly in the PPN group at the end of parenteral nutrition (P = 0.019). After parenteral nutrition, blood chemistry was also remarkable for serum urea and glucose levels, which were elevated significantly in the TPN group (P < 0.001 and P = 0.03, respectively). Patients receiving TPN had a higher incidence of positive microbial cultures and clinical infection than did patients receiving PPN (64.5% versus 40%, P = 0.05). The most striking result was a delay in platelet engraftment for the TPN group compared with the PPN group (15.54 and 12.93 d, respectively; P = 0.014). This difference was also noted in patients using growth factor in the PPN group (P = 0.017). Parallel to these results, platelet transfusion requirement increased in the TPN group compared with the PPN group (1.93 versus 1.16 U, P = 0.004). Both groups were unremarkable for leukocyte recovery and red blood cell transfusion requirement., Conclusions: Consequently, TPN has some pitfalls of hyperglycemia, infection tendency, delayed platelet engraftment, and increased platelet transfusion requirement. Therefore, it should not be used as a standard nutrition support for patients undergoing autotransplantation.