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Pineoblastomas (PB) are rare tumors of the central nervous system and are more common in children. There is no consensus about standard of care. The objective of this study is to analyze the outcome of children with PB.Six patients with PB who were diagnosed between 1990-2012 were evaluated retrospectively. Demographics, age of diagnosis, first complaint, tumor region, diagnosis type, seeding metastasis to the spinal axis or cerebrospinal fluid (CSF), treatment and survival of these patients were recorded.Three patients had subtotal resection and all patients received chemotherapy and craniospinal irradiation (CSI) after diagnosis. Median follow-up after treatment was 5.5 (range:1-19) years. Two patients are alive with no evidence of disease for 7.5 and 10 years, one of whom was diagnosed with papillary thyroid carcinoma 9.5 years after treatment. One of the patients who died had lived for 19 years after diagnosis.Pineoblastomas are rare but very aggressive tumors; more effective treatment strategies are needed. Survivors should be followed up for late effects such as second malignancies and endocrine deficiencies.

Nimotuzumab is an IgG1 antibody that targets epidermal growth factor receptor (EGFR). Overexpression of EGFR is detected in some pediatric brain tumors including diffuse intrinsic pontine gliomas (DIPG)s. Since May 2010, nimotuzumab, combined with carboplatin or vinorelbine or Temozolomide (TMZ), was administered during progressive disease (PD) after the use of the institutional protocol consisting of radiotherapy (RT) + TMZ and adjuvant TMZ. After May 2012, children with newly diagnosed disease received TMZ during RT, and nimotuzumab and TMZ after RT. Nimotuzumab was given as 150 mg/m2/dose once a week for 12 weeks, and then every other week with TMZ until PD. PD patients were switched to nimotuzumab + vinorelbine combination until death. Nimotuzumab was used in 24 children with DIPG (seven in the PD group, 17 in the newly diagnosed patient group). In the PD group, median survival time was 12 months (7–42 months); 1-year and 2-year overall survival (OS) rates were 42.9 ± 18% and 14.3 ± 13%, respectively. The median survival in this group, after the initiation of nimotuzumab was 6 months (3–8 months). In the newly diagnosed patient group, median survival time was 11 months (3–35 months) and median progression free survival was 4 months (1–21 months). The 1-year OS in this group was 35.3 ± 11% and 2 year OS was 11.8 ± 7%. Nimotuzumab ± chemotherapy was well tolerated with no major adverse effect. Nimotuzumab-containing regimens are feasible and tolerable; it might be that some patients either with newly diagnosed DIPG or with progressive disease may benefit modestly from nimotuzumab-containing combinations.

In children and adolescents with chest pain and dyspnea, pneumonia, pleural effusion, and empyema are the frequent causes in the differential diagnosis. Malignant tumors of the chest wall are rare and most originate from the ribs. In children, the most frequent malignant tumor of the rib is Ewing’s sarcoma. Osteosarcomas of the rib are very rare. Osteosarcoma has a predilection for rapidly growing long bones including the femur, tibia and humerus in adolescents. In this paper, we present an adolescent girl who presented with chest pain and dyspnea with osteosarcoma that originated from the rib and extended to the right hemithorax.

Background: Primary breast rhabdomyosarcoma (RMS) can occur in children. There is a lack of knowledge regarding radiologic findings and added diffusion-weighted magnetic resonance imaging (MRI) features of RMS in the literature. Case Report: A 12-year-old girl was diagnosed with primary alveolar RMS of the breast. Gray scale ultrasound revealed posterior acoustic enhancement behind a well-circumscribed, multilobulated hypoechoic mass. Doppler ultrasound revealed increased peripheral and central vascularity. Hypointense septations on T2-weighted image exhibiting more enhancement than the stroma on late gadolinium-enhanced images were striking within a hyperintense mass. A hyperintense hemorrhagic focus on T1-weighted image was present in the absence of any necrosis. Avid enhancement on early postcontrast images proceeding from the periphery to the center was depicted. Conclusion: A rapidly enlarging mass with an echogenic peripheral rim together with posterior acoustic enhancement on gray scale ultrasound, intense vascularity on Doppler ultrasound, axillary lymphadenopathy, and satellite nodules on MRI should raise suspicion. Enhancing central and peripheral septations are suggestive of RMS. Dynamic contrast-enhanced MRI in suspected cases can provide valuable data in the differential diagnosis.

To investigate the relationship between serum PSA level, Gleason score of PCa and the outcomes of Ga68-PSMA PET/CT in patients with recurrent PCa. A total of 109 consecutive patients (median age 71 years; range 48–89 years) who had PSA recurrence after RP and/or hormonotherapy and/or radiotherapy were included in this study. Local recurrences, lymph node metastasis (pelvic, abdominal and/or supradiaphragmatic), bone metastases (oligometastatic/multimetastatic) and other metastatic sites (lung, liver, brain, etc) were documented. In 91(83.4%) patients at least one lesion characteristic for PCa was detected by68Ga-PSMA PET/CT. The median serum total PSA (tPSA) was 6.5 (0.2–640) ng/ml.There was a significant difference between 68Ga-PSMA PET/CT positive and negative patients in terms of serum total PSA value. No statistical significance was found between positive and negative 68Ga-PSMA PET/CT findings in terms of Gleason score. Local recurrence was detected in 56 patients. whereas lymph node metastases were demonstrated in 46 patients. Pelvic nodal disease was the most frequent presentation followed by abdominal and supradiaphragmaticnodal involvement. Bone metastases [oligometastasis, (n = 20); multimetastasis, (n = 35)⦌ were also detected in 55 patients. In the ROC analysis for the study cohort, the optimal cut-off value of total serum PSA was determined as 0.67 ng/ml for distinguishing between positive and negative 68Ga-PSMA PET/CT images, with an area under curve of 0.952 (95% CI 0.911–0.993). 68Ga-PSMA PET/CT was found to be an effective tool for the detection of recurrent PCa. Even though no relationship was detected between the GS and 68Ga-PSMA PET/CT findings, serum total PSA values may be used for estimating the likelihood of positive 68Ga-PSMA PET/CT results.

Breast metastasis is rare in childhood malignancies. Soft tissue sarcomas, especially rhabdomyosarcomas (RMS), and hematologic neoplasms, such as lymphomas, are the most common tumors that metastasize to the breast, albeit rare.All cases with breast metastasis within a cohort of 200 RMS patients followed in our institution during 1990 to 2014 were assessed retrospectively and the literature was reviewed.There were 3 adolescent female patients with breast metastasis. All had alveolar histology. The primary tumors were in the parameningeal sites, extremities, and the perineum, respectively. Two patients had breast metastasis at diagnosis, and 1 during follow-up. In 1 breast lesion, there was a complete response to chemotherapy, and in another there was no response to chemotherapy, and the patient underwent radical mastectomy. In the third patient, there was partial response, and lesions progressed. All patients died with recurrent/progressive disease, 2 with no recurrence in the breast. In the English literature, there are 70 cases including our cases. All but 1 involve female patients, all adolescents, most have alveolar histology and poor prognosis. All had chemotherapy, whereas some had surgery and/or radiotherapy for local treatment.Breast metastasis should be considered in adolescent female patients with RMS. Optimal management is not clear. Besides chemotherapy, mastectomy and radiotherapy should be considered on a case basis.

Objectives To compare conventionally fractionationed volumetric arc therapy (VMAT) and hypofractionated stereotactic body radiotherapy (SBRT) modalities in terms of prostate-specific antigen (PSA) kinetics, toxicity, and quality of life (QOL) in patients with localized prostate cancer. Methods Patients received radical radiotherapy as either 33.5 Gy/5 fr for SBRT or 75.6 Gy/35 fr for VMAT. International Prostate Symptom Score (IPSS) and European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire Prostate Cancer Module (QLQ-PR25) forms were used to assess QOL. Results Of the 48 patients (28 in SBRT and 20 in VMAT) included in the study, 40 (20 in SBRT and 20 in VMAT) were evaluated for QOL status. PSA control rate was 100% and PSA nadir value was 0.5 ng/dl in both arms during the median follow-up period of 23 months. The magnitude of PSA bounce was higher in the SBRT arm than in the VMAT arm ( P = 0.01). The PSA decline rate in the VMAT arm was higher than in the SBRT arm ( P = 0.028). Three (10.7%) patients treated with SBRT who had a history of transurethral resection of the prostate (TURP) experienced grade 3 urinary toxicity. No significant difference was observed concerning sexual activity and sexual functioning scores, whereas scores at 10.5 and 13.5 months were decreased in both arms. The SBRT and VMAT arms had similar urinary incontinence, bowel symptoms, and IPSS obstruction scores. The magnitude of increase in IPSS scores at treatment completion was higher in the VMAT arm than in the SBRT arm ( P = 0.046). The decrease in hormonal symptom scores at 4.5, 10.5, and 13.5 months was higher in the VMAT arm than in the SBRT arm ( P = 0.007, 0.027, and 0.021, respectively). Conclusions Both treatment modalities had similar effectiveness and provided acceptable outcomes in terms of toxicity and QOL. Grade 3 urinary toxicities might be eliminated with careful patient selection for SBRT.

We designed our study to analyze the prognostic factors and treatment outcomes of adult medulloblastoma patients who received postoperative craniospinal irradiation. Fourty-three patients who were treated due to medulloblastoma at Istanbul University, Institute of Oncology between 1990 and 2013 were retrospectively analyzed. All of the patients were older than 18 years, with a median age of 27 years (range, 18–51 years). In 40 (93%) patients, total resection of the tumor was achieved, and 3 (7%) patients had undergone a subtotal tumoral resection. Risk assessment revealed 7 high-risk and 36 standard-risk patients. All patients received postoperative craniospinal irradiation, delivering a median craniospinal dose of 36 Gy, with an additional boost to the posterior fossa up to 54 Gy. Fifteen patients received chemotherapy. The median follow-up was 62 months (range, 3–213 months). The 5-year, 10-year, overall, and disease-free survival rates were 63%, 51%, 66%, and 55%, respectively. Univariate analysis revealed that hydrocephalus, initial local recurrence, subtotal resection in primary surgery, initial Karnofsky performance status ≤70, duration of symptoms shorter than 30 days, and primary site dose < 54 Gy were found to be negative prognostic factors. Toxicity was moderate. The main therapy in adult medulloblatoms is craniospinal irradiation following surgery. The prognostic factors and outcomes of the patients in our study are concordant with previous reports in the literature.

To validate the clinical outcomes and prognostic factors in prostate cancer (PCa) patients with Gleason score (GS) 8–10 disease treated with external beam radiotherapy (EBRT) + androgen deprivation therapy (ADT) in the modern era. Institutional databases of biopsy proven 641 patients with GS 8–10 PCa treated between 2000 and 2015 were collected from 11 institutions. In this multi-institutional Turkish Radiation Oncology Group study, a standard database sheet was sent to each institution for patient enrollment. The inclusion criteria were, T1–T3N0M0 disease according to AJCC (American Joint Committee on Cancer) 2010 Staging System, no prior diagnosis of malignancy, at least 70 Gy total irradiation dose to prostate ± seminal vesicles delivered with either three-dimensional conformal RT or intensity-modulated RT and patients receiving ADT. The median follow-up time was 5.9 years (range 0.4–18.2 years); 5‑year overall survival (OS), biochemical relapse-free survival (BRFS) and distant metastases-free survival (DMFS) rates were 88%, 78%, and 79%, respectively. Higher RT doses (≥78 Gy) and longer ADT duration (≥2 years) were significant predictors for improved DMFS, whereas advanced stage was a negative prognosticator for DMFS in patients with GS 9–10. Our results validated the fact that oncologic outcomes after radical EBRT significantly differ in men with GS 8 versus those with GS 9–10 prostate cancer. We found that EBRT dose was important predictive factor regardless of ADT period. Patients receiving ‘non-optimal treatment’ (RT doses

10538 Background: Rhabdomyosarcoma (RMS) of the female genital tract is rare, accounting for 3.5% of cases of rhabdomyosarcomas. Germline DICER1 mutations are associated with predisposition to pleuropulmonary blastoma and other tumors including sarcomas. Recently DICER1-associated RMS of the uterus/ovary has ben reported.. The aim of this study is to evaluate demographic characteristics, molecular pathogenesis, treatment and long term outcome of female genital tract rhabdomyosarcoma. Methods: Files of children with RMS of the female genital tract diagnosed at the Istanbul University, Oncology Institute during 1990-2019 were reviewed. Molecular genetic sequencing was performed by polymerase chain reaction amplification of genomic DNA extracted from the formalin-fixed, paraffin embedded tumors, followed by Sanger sequencing. Genetic testing for DICER1 variants of the proband and family members was performed if DICER1 mutation was detected in the tumor of the proband. Results: Of 210 RMS cases, 11 arose from the female genital tract. The median age at diagnosis was 52 months (10 months-15 years). Primary sites were vaginal (n = 5), uterus (n = 4), and ovary(n = 2). Presenting symptoms included vaginal mass (n = 6), vaginal bleeding (n = 5), and abdominal pain (n = 3). Four had group 1, five group 3, two group 2 disease and all received chemotherapy (vincristine, actinomycinD + cyclophosphamide).Three received radiotherapy; three underwent hysterectomy. DICER1 mutation was detected in tumor tissue in three patients:[ c.5113G > A (p.E1705K); c.5428G > T (p.D1810Y); c.1870C > T (p.Arg624Ter)] Genetic testing revealed germline DICER1 pathogenic variation in two patients and their family members, one of whom had cystic nephroma in infancy and history of Wilms tumor in an uncle. They were referred for surveillance for the DICER1 related diseases. A patient with metachronous bilateral ovarian RMS died. Two are married, one has children. The 5 year survival for RMS of the female genital tract was 85.7 % at a median follow-up of 34 (4-298) months. Conclusions: Rhabdomyosarcoma of the female genital tract is associated with a favorable prognosis, however some individuals undergo aggressive local therapies. Individuals with RMS of the female genital tract should be tested for DICER1 pathogenic variation. The detection of a DICER1 mutation in an individual or family members is important to facilitate surveillance for related tumors, so that they may be detected at the earliest possible stage, potentially increasing survival and decreasing risks of late effects.

Amac: Cocukluk cagi Hodgkin Lenfoma tedavisinde sagkalim oran ve suresi uzadikca, tedavi sonucu gelisen yan etkilerin gorulme sikligi artmakta ve tedavinin gec etkileri giderek onem kazanmaktadir. Bu calismada cocukluk caginda Hodgkin Lenfoma nedeniyle tedavi goren hastalarda, kemoterapi ve radyoterapinin tiroid fonksiyonlari uzerine gec yan etkilerinin degerlendirilmesi amaclandi. Yontem: Istanbul Saglik Bilimleri Universitesi, Kanuni Sultan Suleyman Egitim ve Arastirma Hastanesi, Cocuk Hematoloji ve Onkoloji Klinigi’nde, 1994-2013 yillari arasinda Hodgkin Lenfoma tanisi ile tedavi gorup, remisyonda olarak izlenen, en az 6 aydir tedavi almayan, 34’u erkek, 6’si kiz olmak uzere toplam 40 hastanin tiroid fonksiyonlari retrospektif olarak incelendi. Bulgular: Tani alan hastalarin yas ortalamasi 14.13±6.081 (dagilim: 4-31), erkek/kiz orani 34/6 (%85/%15) idi. Ortalama takip suresi 5.54±3.68 yil (0,92-13,92) idi. Tum hastalara kemoterapi verildi. Boyun bolgesine radyoterapi 37 hasta (%92,5) almisti. Ortalama radyoterapi dozu 21.4875±4.48726 Gy idi. Hastalarimizda %72.5 oraninda primer hastalik bas-boyun bolgesine lokalize idi. B semptomu 7 hastada (%17,5) vardi. Kirk hastanin 12’inde (%30 hasta) hipotiroidi saptandi, 5 hastaya (%41) klinik hipotiroidi tanisiyla replasman tedavisine baslandi. Bu hastalarin 11’i erkek (%91), 1’i kizdi (%9). Hastalarimizin 5’inde (%12.5) yapilan tiroid USG’sinde tiroid bezi nodulu saptandi. Tiroid nodulu saptanan toplam 5 hastanin 3’u erkek (%60), 2’si kizdi (%40). Hipotiroidi gelisme zamani 3.10±2.02 yil, tiroid nodulu olusma zamani 8.90±7.10 yil olarak saptandi. Tiroid sintigrafisi ve tiroid ultrasonografi sonuclarina gore secilen uc hasta tiroid biyopsisine yonlendirildi, malinite saptanmadi. Sonuc: Pediatrik Hodgkin Lenfoma hastalarinda radyasyona bagli tiroid hastaliklari, tedaviden sonraki yillarda, hastalik remisyonda iken gelisebilir. Ozellikle boyun radyoterapisine maruz kalmis hastalar, tiroid fonksiyon bozukluklarinin ortaya cikmasi acisindan yakindan takip edilmelidir.

Background Although the survival of pediatric cancer has increased dramatically in the last decades, the survival of refractory, relapsed, and metastatic cases is still dismal. The combination of irinotecan and temozolomide has shown activity against refractory/relapsed pediatric solid tumors. Method Thirty-four children with refractory/relapsed solid tumors who had previously been heavily pretreated and who were given vincristine, irinotecan, and temozolomide as third- or further line chemotherapy during 2004–2015 were evaluated. Results Patients were diagnosed with Ewing sarcoma (n = 15), rhabdomyosarcoma (n = 8), neuroblastoma (n = 8), osteosarcoma (n = 2), and Wilms’ tumor (n = 1). Thirty patients presented with disease progression on therapy and the other four presented with relapsing. A total of 141 cycles were administered. Radiotherapy was used in 17 patients and surgery in 4 as local therapy. Among all patients, 6 had complete response, 3 had partial response, 14 had stable disease, and 11 had progressive disease. The objective response was 26.4% (complete response + partial response) and median survival duration was six months. The first and second year overall survival rates were 22.3% and 16.8%. The objective response in Ewing sarcoma patients was 40%. Diarrhea was the most common toxicity and 14 (10%) courses were associated with grade 3–4 diarrhea. Conclusions In heavily pretreated patients with refractory/relapsed solid tumors, the vincristine, irinotecan, and temozolomide regimen seemed promising in Ewing sarcoma patients and was well tolerated.

Background/aim Prostate cancer (PCa) is an androgen-dependent disease. Novel anti-androgens (i.e. enzalutamide) have recently been developed for the treatment of patients with metastatic castration-resistant prostate cancer (CRPC). Evidence is accumulating that prostate cancer antigen 3 (PCA3) is involved in androgen receptor (AR) signaling. Here, in combination with enzalutamide-mediated AR blockade, we investigated the effect of PCA3 targeting on the viability of PCa cells. Materials and methods In hormone-sensitive LNCaP cells, AR-overexpressing LNCaP-AR+ cells and VCaP cells (representing CRPC), PCA3 was silenced using siRNA oligonucleotides. Gene expression and cell viability was assessed in PCA3-silenced and/or AR-blocked cells. Results PCA3 targeting reduced the expression of AR-related genes (i.e. prostate-specific antigen (PSA) and prostate-specific transcript 1 (non-protein coding) (PCGEM1)) and potentiated the effect of enzalutamide. Proliferation of PCa cells was suppressed upon PCA3 silencing with a greater effect in LNCaP-AR+ cells. Furthermore, PCA3 silencing sensitized PCa cells to enzalutamide-induced loss of cell growth. Conclusion PCA3, as a therapeutic target in PCa, might be used to potentiate AR antagonists.

Aim of the study : Patients with large and high-grade extremity soft-tissue sarcoma are at significant risk for distant metastasis and sarcoma-related death. There is no randomized trial comparing chemoradiotherapy to radiotherapy in the neoadjuvant setting for high risk extremity soft-tissue sarcoma. The aim of this study is to evaluate the outcomes of patients treated with two different modalities (neoadjuvant sequential chemoradiotherapy vs. radiotherapy alone) in a single center. Material and methods : Data of 67 patients were analyzed retrospectively. Thirty-four patients received neoadjuvant sequential chemoradiotherapy (2–3 cycles of doxorubicin (75 mg/m 2 ) and ifosfamide (6 g/m 2 ) followed by radiotherapy of 28 Grays (Gy) administered as 8 fractions of 35 Gy) and 33 patients received radiotherapy alone. R0 resection rates and 3-year survival estimates were evaluated. Results : Median follow-up time was 37 months. The estimated 3-year overall and disease-free survival rates for the whole patient group were 79% (95% CI: 67.0–86.4) and 57.9% (95% CI: 46.3–69.0), respectively. The most common side effects were nausea and leucopenia. Three-year overall, disease-free, local recurrence-free and distant recurrence-free survival rates did not differ significantly. All patients except one underwent wide excision or compartmental resection. R0 resection rate for the whole patient group was 92.5% (n = 62). Sites of progression were similar across both treatment arms. Conclusions : Preoperative hypofractionated radiotherapy alone or sequentially with chemotherapy result in high rates of limb salvage and acceptable toxicity. Our study results did not show a statistically significant treatment effect regarding survival and patterns of failure.

Aim This study aims at examining absolute dose verification of step-and-shoot intensity modulated radiation treatment (IMRT) of prostate and brain patients by use of ion chambers of two different volumes and thermoluminescent detectors (TLD). Background The volume of the ion chamber (IC) is very important for absolute dose verification of IMRT plans since the IC has a volume average effect. With TLD detectors absolute dose verification can be done measuring the dose of multiple points simultaneously. Materials and methods Ion chambers FC65-P of volume 0.65 cc and semiflex of volume 0.125 cc as well as TLDs were used to measure the central axis absolute dose of IMRT quality assurance (QA) plans. The results were compared with doses calculated by a treatment planning system (TPS). The absolute doses of off axis points located 2 cm and 4 cm away from the isocenter were measured with TLDs. Results The measurements of the 0.125 cc ion chamber were found to be closer to TPS calculations compared to the 0.65 cc ion chamber, for both patient groups. For both groups the root mean square (RMS) differences between doses of the TPS and the TLD detectors are within 3.0% for the central axis and points 2 cm away from the isocenter of each axis. Larger deviations were found at the field edges, which have steep dose gradient. Conclusions The 0.125 cc ion chamber measures the absolute dose of the isocenter more accurately compared to the 0.65 cc chamber. TLDs have good accuracy (within 3.0%) for absolute dose measurements of in-field points.

Diagnosis of secondary malignancies began with the increasing survival in childhood cancer. Children treated for acute lymphoblastic leukemia (ALL) have an increased risk for developing mucoepidermoid carcinoma (MEC) of the parotid gland. The latent period ranges from 5 to 16 years. A 2 6/12-year-old girl was treated for pro-B ALL. Treatment included multidrug chemotherapy, prophylactic intrathecal methotrexate, and cranial radiotherapy. MEC of the left parotid gland was diagnosed at the age of 8 years, 3 years after completing treatment. She was treated with multiple surgery and radiotherapy. The authors aimed to emphasize the need for concern about second cancers of the parotid gland in children treated for ALL.

Purpose In this study, it has been compared the Planning Target Volume (PTV) and critical organs dose in Stereotactic Body Radiotherapy (SBRT) plans created by CyberKnife (CK) and volumetric arc radiotherapy (VMAT) techniques for patients with prostate cancer. Methods The computed tomography images of 8 patients with low or intermediate risk prostate cancer were acquired in 1 mm thickness and transferred to the MultiPlan v4.5.3 treatment planning system (TPS). The PTV and critical structures were contoured. The Clinical Target Volume (CTV) was generated by encompassing the prostate plus the 1 cm proximal part of seminal vesicles. To create the PTV, a margin of 3 mm was added in the posterior direction and 5 mm in the other directions to the CTV. The treatment plans were created with CK technique. To create the SBRT plans with the VMAT technique, the volumes were transferred to the Aria v15.1 system. The double arc VMAT plans were prepared. The prescribed dose for PTV was 33.50 Gy/5fr in both techniques. Conformity Index (CI), Homogeneity Index (HI) of PTV, critical organs doses, the integral dose (ID) to the patient body and monitor unit (MU) values were assessed using the Wilcoxon test. Results For plans created with CK and VMAT technique, CI was 1.11 ± 0.06 and 1.04 ± 0.01, HI was 1.20 ± 0.04 and 1.08 ± 0.01, respectively; there was a statistically significant difference between them. The differences between all critical organ doses except D1cc of the rectum were significant statistically in favor of VMAT. The mean of the difference of the mean right testicular dose was 137.1 cGy. It was 113.8 cGy for left testicular. MU value and the ID were found significantly to be higher in the CK technique. Conclusions The VMAT technique compared with the CK technique provides better sparing of the critical structures, and the irradiation time is shorter as the MU value is less. The less irradiation time is to be advantageous in minimizing uncertainties due to patient movement. In conclusion, the VMAT technique may be preferred, especially in young patients. This work was supported by Scientific Research Projects Coordination Unit of Istanbul University (Project Number: I.U. BAP-23057).

10547Background: The aim of this study is to evaluate the demographic, clinical and therapeutic characteristics and long-term outcome in childhood nasopharyngeal carcinoma (NPC) in a single center....

melikoglu, mustafa/0000-0001-9646-3787; Aydin, Burca/0000-0002-6013-6271 WOS: 000278808800001 PubMed: 20367260 Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Radiation-induced fibrosis (RIF) has since long been considered as irreversible. Further understanding of its mechanisms has led to trials investigating RIF treatment and prevention. The effect of superoxide dismutase (SOD)-gliadin, an oral form of SOD that resists gastrointestinal inactivation, on RIF treatment was evaluated in this experimental study.A total of 36 Wistar albino mice were randomly distributed into four groups. According to group, 25 Gy radiation or sham-radiation were performed on day 0. Acute and late reactions were recorded. After 6 months, mice were treated with SOD-gliadin, 10,000 units per kg per day, or placebo. SOD-gliadin and placebo treatments were administered daily for 8 days by oral gavage. Later the mice were sacrificed, dissected and histopathologically analyzed. Accumulated hyaline and collagen at the dermis is an indicator of fibrosis. Therefore measurements of the dermal thickness were used to quantify the degree of RIF. Additionally, the morphological changes were analyzed, and the differences reported.The mean and standard deviation for dermal thickness were 0.45±0.09 mm in the sham-irradiated placebo-treated group, 0.51 mm±0.16 mm in the sham-irradiated SOD-gliadin-treated group, 0.92 mm±0.23 mm in the irradiated placebo-treated group and 0.71 mm±0.17 mm in the irradiated SOD-gliadin-treated group. The difference in mean dermal thickness between irradiated placebo-treated and irradiated SOD-gliadin-treated mice was statistically significant (p=0.002).Quality of life while prolonging survival has an increasing importance in patients with cancer. RIF can be a crucial problem after all radiotherapy modalities. SOD-gliadin has advantageous effects on conditions that call for an increased expression of antioxidant enzymes. The results of our study suggest that oral SOD-gliadin may prevent or ameliorate RIF and patients can benefit from the positive effects of SOD.

e21014 Background: Prognosis of diffuse intrinsic pontine gliomas (DIPG) is dismal. We previously published results of our center 1990-2012. Patients recieving temozolamide or other chemotherapy as...

BACKGROUND Older age and axial location of Ewing's sarcoma have been reported as unfavorable prognostic factors. METHODS The records of patients older than 15 years with the Ewing's family of tumors were reviewed retrospectively. After the induction chemotherapy consisting of alternating vincristine, adriablastin, cyclophosphamide (VAC) and etoposide, ifosfamide with mesna protection (IE), a local treatment modality was chosen based on tumor and patient characteristics. RESULTS Twenty-five patients with a median age of 19 years were evaluated. Median follow-up was 26 months (range 4-58). Seventeen patients (68%) had died. In univariate analysis, factors predictive of shorter survival were the patients presenting with metastatic disease, with the primary tumor located at the pelvis, those who never achieved complete response to chemotherapy and those who had chemotherapy for

Purpose To report the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit. Design Interventional case report and review of the literature. Methods Ophthalmologic examination and computed tomography scan of the orbit were performed. Histologic and histochemical examination and immunohistochemistry of the biopsy specimen were evaluated. Main outcome measures Ocular and systemic disease control. Results A 5-month-old girl was seen with a papillomatous lesion in the right lower tarsal conjunctiva present from birth. Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. The patient was treated with combined chemotherapy (ifosfamide, epirubicin, and cisplatin), radiotherapy, and surgery (exenteration). She is alive with no evidence of disease after 4 years of follow-up. Facial asymmetry and dental malformations have developed as late effects. Further reconstructive surgery is planned for the malformations. Conclusions Extraskeletal mesenchymal chondrosarcoma in the orbit is extremely rare. This study reports the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit (also the youngest patient) both in skeletal and extraskeletal sites. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.

We wanted to investigate the effects of gamma radiation on DNA single-strand breaks (SSB) and glutathione (GSH) levels in mononuclear blood cells (MNC) of radiotherapy technicians. DNA SSB in MNC of radiotherapy technicians who use (60)Co-gamma source in their works were detected by alkaline filter elution and compared to control subjects. In addition, GSH levels were measured using the enzymatic method in MNC. Blood samples were collected from radiotherapy technicians on Monday and Friday. DNA SSB levels were found to be significantly higher in smoking controls compared to non-smoking controls. Significant increases of 36% and 49% in DNA SSB were detected from Monday to Friday for non-smoking and smoking radiotherapy technicians, respectively. GSH levels were found to be decreased significantly from Monday to Friday. Gamma-radiation resulted in increased DNA SSB levels of MNC in radiotherapy technicians throughout the working week and these breaks have been observed to be repaired at the weekend. Smoking habit caused an additional increase in the SSBs observed in radiotherapy technicians.

The primary objective of this study is to review the clinical characteristics of 25 patients in the adult and late adolescent age group, diagnosed and treated with small round cell tumors involving soft tissues (extraosseous Ewing sarcoma, rhabdo-myosarcoma, primitive neuroectodermal tumor, and undiffer-entiated small round cell tumors). Additionally, survival and prognostic factors influencing the outcome with multimodality treatment are evaluated. There were 19 males (76%) and 6 females (24%). The median age was 26 years (range: 15-56 years). In 9 patients (36%), the tumor was located at an extremity, whereas 16 patients (64%) had central localizations. Tumor size was larger than 10 cm in 7 patients (29.2%). Six patients (24%) had metastatic disease. Twelve patients (48%) received radiation and 16 patients (64%) underwent surgery. Among the resected tumors, 2 were resected with contaminated margins (12.5%), whereas 2 were radically resected and 12 (75%) were resected with wide margins. All patients were given a median of 4 cycles of multiagent chemotherapy (1-14 cycles). With preoperative chemotherapy, complete regression (CR) of the tumor was achieved in 6 patients (24%). In 4 patients (16%), a partial response was obtained. After the completion of multimodality treatment, 12 patients (48%) had a CR. Progression-free (PFS) and overall survival (OS) for the entire group was 25.0 +/- 10.8% at 1 year and 30.5 +/- 15.5% at 3 years, respectively. Nonmetastatic disease, wide and radical resection, and presence of CR to multimodality treatment were associated with a significantly longer PFS and OS by univariate analysis. By multivariate analysis, CR to multimodality treat-ment was the only independent predictive factor for a longer OS (p: 0.0036, relative risk [RR]: 23.6, 95% CI: 2.8; 198.7) and metastatic presentation was the only independent factor predic-tive for a shorter PFS (p: 0.017, RR. 15, 95% CI: 1.6; 141.2). Large-scale, multicenter studies are required for a better eval-uation of the nonpediatric age group with small round cell tumors.

e22009 Background: Bilateral retinoblastomas (BRBS) comprise 25 % of all RBS. Treatment decision depends on tumor burden, potential for vision, status of the contralateral eye. As the survival rate in retinoblastoma has increased, ocular salvage and late effects has become an important issue. The aim of the study is to evaluate the demographic features, treatment modalities, and late effects in BRBS. Methods: BRBS treated in Istanbul University, Oncology Institute and Opthalmology Department between 1990-2016 were retrospectively evaluated. All patients were multidisciplinarily evaluated for chemotherapy (chemoreduction /adjuvant), local opthalmologic therapies (transpupillary thermotherapy, cryotherapy, plaque), radiotherapy, enucleation. The chemotherapy (CT) protocol used had vincristine, cisplatin, etoposide, cyclophosphamide until 2009, and vincristine, etoposide, carboplatin since then. Since 2011, intraarterial, intravitreal CT was also used. Results: 114 BRBS (228 eyes) (56 male, 58 female) with a median age of 9 months (20days-42 mo.) were evaluated. Three had extraocular disease, two trilateral RBS. Seventeen had history of retinoblastoma in their families. According to ICRB classification, there were 67 eyes in group E, 43 group D, 27 group C, 45 group B, and 19 group A. Enucleation was done in 68 (30%) eyes, mostly group E. During 1990-2000, 23/26 patients underwent enucleation, whereas 45/88 underwent enucleation after 2000. Radiotherapy was used for 30 eyes, most before 2000. Bone and soft tissue deformities and cataracts were observed in irradiated patients. Five patients had a second cancer (4 sarcomas, 1 meningioma) at a median of 11 years, four in irradiated sites. The 5 yr survival was 93.5%, 9 patients died, 4 due to second cancer. Conclusions: As the survival rate in intraocular BRBS has increased, ocular salvage and late effects have gained importance. Chemoreduction (systemic, intraarterial) and local ophtalmic therapies enable preservation of vision in most group A, B, C tumors and some D tumors. Most group E tumors require enucleation. Radiotherapy is not used in most RBS in the last decade. Intra-arterial chemotherapy is promising in maintaining ocular salvage.

e21012 Background: The aim of this study was to evaluate the demographic, clinical and therapeutic characteristics and long-term outcome in childhood and adolescent nasopharyngeal carcinoma (NPC) r...

Decrease in serum alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) levels is considered as a response during chemotherapy of non-seminomatous germ cell testicular tumors, but data on the prognostic significance of marker half-life remains inconclusive. Serum marker half-life was evaluated in 34 patients with elevated markers, receiving chemotherapy (CT). Marker half-life was calculated from the natural logarithm of the sequential AFP or HCG concentrations. The correlation between event-free (EFS) and overall survival (OS) with unfavorable half-lives of AFP and HCG was evaluated. Median actual half-life (AHL) AFP was 3.9 days (range, 1.4-21.5) and median AHL HCG was 4.4 days (range, 1.4-21.0); 82% of the patients had a satisfactory initial decline in AFP, and 71% had a satisfactory initial decline in HCG. There was a significant difference in EFS and OS between the two groups of patients with an AFP half-life7 days and7 days. HCG half-life did not adversely affect EFS and OS. The correlation of better EFS and OS with appropriate AFP marker half-life during chemotherapy could provide a dynamic method, which could complement the standard baseline prognostic factors, for the prediction of prognosis.

Background Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and painful periosteal new bone formation of the tubular bones. The association of malignant disorders with HOA is more common in adults than in children. Purpose In this paper, a 12-year-old boy with intrathoracic Hodgkin disease and HOA is presented and four other children with HOA and Hodgkin disease in the literature reviewed. Discussion The presence of HOA has been thought to be a bad prognostic sign, but complete remission of Hodgkin disease and regression of clinical signs and symptoms of HOA were attained in our patient after chemotherapy and radiotherapy, and continue during 9 months post-therapy follow-up. Conclusions HOA accompanying a malignant tumor in children is very rare. Only 5 cases have been associated with Hodgkin disease, including the present boy. It is important that patients with symptoms of HOA and an intrathoracic mass be examined carefully to rule out a malignancy. Med. Pediatr. Oncol. 29:578–581, 1997. © 1997 Wiley-Liss, Inc.

Reconstructive surgery in previously irradiated areas is more difficult than in non-irradiated cases. A retrospective analysis of the outcome of 200 previously irradiated patients who had skin graft or flap reconstruction performed by the same surgeon is presented, and the most suitable surgical technique in irradiated areas is discussed. One hundred and fifty-six patients had skin and oral cavity cancer, and were operated on after local recurrence. Twenty patients had breast cancer; 15 were operated on for local recurrence and five for breast reconstruction. Twenty-four patients had soft tissue sarcomas. Eighty-five patients had a skin graft (group 1), 35 had a skin flap (group 2), 10 had a fascia/muscle flap plus skin graft and 70 had a myocutaneous flap (group 3). Analysis of complications revealed statistically significant differences in terms of incomplete graft/flap necrosis between group 1 and 2 (P0.001) and groups 1 and 3 (P0.001), and in terms of infection between groups 1 and 3 (P0.01). We conclude that the method of reconstruction is determined by the characteristics of the defect such as size and localization; the quality, fractionation, total dose, and energy of radiation used; skin and subcutaneous tissue changes due to radiation; and operation time. However, it is reasonable to choose fascia/muscle or myocutaneous flaps for reconstruction in previously irradiated areas. These methods are more resistant to bacterial inoculation, more prone to clean residual infection, and provide better vascularized tissue and volume replacement for contour defects.

We investigated the prognostic clinicopathologic factors associated with overall survival (os) and progression-free survival (pfs) in the once-daily continuous administration of first-line sunitinib in a consecutive cohort of Turkish patients with metastatic renal cell carcinoma (rcc). The study enrolled 77 Turkish patients with metastatic rcc who received sunitinib in a continuous once-daily dosing regimen between April 2006 and April 2011. Univariate analyses were performed using the log-rank test. Median follow-up was 18.5 months. In univariate analyses, poor pfs and os were associated with 4 of the 5 factors in the Memorial Sloan&ndash, Kettering Cancer Center (mskcc) score: Eastern Cooperative Oncology Group performance status of 2 or higher, low hemoglobin, high corrected serum calcium, and high lactate dehydrogenase. In addition to those factors, hypoalbuminemia, more than 2 metastatic sites, liver metastasis, non&ndash, clear cell histology, and the presence of sarcomatoid features on pathology were also associated with poor pfs, and male sex, hypoalbuminemia, prior radiotherapy, more than 2 metastatic sites, lung metastasis, nuclear grade of 3 or 4 for the primary tumour, and the presence of sarcomatoid features were also associated with poorer os. The application of the mskcc model distinctly separated the pfs and os curves (p < 0.001). Our study identified prognostic factors for pfs and os with the use sunitinib as first-line metastatic rcc therapy and confirmed that the mskcc model still appears to be valid for predicting survival in metastatic rcc in the era of molecular targeted therapy.

Purpose: Topotecan has recently been used in the treatment of pediatric cancer. We evaluated our experience with the modified combination of vincristine, topotecan, and cyclophosphamide (VTC) given in 3 days, in children with recurrent Ewing sarcoma. Method: Children received vincristine (1.5 mg/m(2)/1st day), cyclophosphamide (600 mg/m(2)/day x 2 days) + mesna, and topotecan (1 mg/m(2)/day x 3 days) every 21 days. Result: A total of 118 courses of VTC were given to 13 patients. One patient received VTC both at first and at second relapse. Thus, 14 relapse episodes in 13 patients were evaluated. After three courses of VTC chemotherapy (CT), two achieved complete response (CR), five achieved partial response, thus an objective response was attained in 7/14 (50%) episodes. Two patients had stable disease and two patients progressed. In three episodes, CR was achieved by surgery before CT. One of them had a second relapse and attained CR with VTC. Median time from diagnosis to relapse was 23 months (5-45 months). Site of relapse was local in four patients, and metastatic in 10 episodes of nine patients. Seven patients are alive, three with no evidence of disease and four alive with disease; six have died of disease. Local treatment was used in 11 episodes. The toxicity of the VTC combination was limited mainly to the hematopoietic system. Conclusion: In conclusion, the modified VTC protocol in 3 days every 3 weeks seems to be effective and tolerable in children and adolescents with recurrent/progressive Ewing sarcoma.

The prognosis of children with diffuse intrinsic pontine gliomas (DIPG) is dismal. This study aims to evaluate the characteristics and treatment outcome of children with DIPG in a single center.We reviewed the outcome of children with DIPG treated at the Oncology Institute of Istanbul University from February 1999 to May 2012.Fifty children (26 female, 24 male) with the median age of 7 years were analyzed. The median duration of symptoms was 30 days. All patients received radiotherapy (RT). Before the year 2000, 12 patients received only RT. Thirty-eight had concomitant and/or adjuvant chemotherapy with RT. Between 2000 and 2004, 17 patients received cis-platinum or vincristine as sensitizers during RT and CCNU + vincristine combination after RT. Since 2004, 21 patients received temozolomide (TMZ) concomitantly during RT and as adjuvant chemotherapy after RT. The median survival time of all patients was 13 months (1-160 months). Patients receiving RT + TMZ had a significantly higher overall survival than patients with only RT (p = 0.018). Patients receiving RT + chemotherapy other than TMZ also had a significantly higher overall survival than patients receiving only RT (p = 0.013). Patients receiving RT + TMZ + and chemotherapy other than TMZ had a significantly higher survival than patients receiving only RT (p = 0.005).In our series, patients receiving RT + TMZ and also patients receiving RT + chemotherapy other than TMZ had a significantly higher overall survival than patients treated with only RT. Hence, administering chemotherapy during and after RT seems to prolong survival in some DIPG patients.

IntroductionThe survival of children with Hodgkin lymphoma have increased significantly raising the issue of decreasing late effects by using risk adapted treatment. Hodgkin lymphoma has different epidemiologic features in developed and developing countries. In this study the epidemiologic, clinical characteristics and outcome of children with Hodgkin disease treated with a risk adapted treatment over a 25 year period are evaluated. MethodsThis retrospective study evaluates the clinical characteristics and outcome of 122 children treated with the same institutional risk-adapted protocol in the Istanbul University, Oncology Institute between 1991-2016. Clinical staging was done according to Ann-Arbor staging, all patients had biopsy confirmation and the WHO histopathological classification was used. Imaging (ultrasound, CT/MRI and/or PET-CT since 2004) was done in all patients. Bone marrow aspiration and biopsy was done for all with B symptoms or risk group 2 and 3. Risk group 1 (clinical group IA/B and IIA) recieved 2 courses of ABVD (adriamycin 25 mg/m2, bleomycin 10 U/m2, vinblastin 6 mg/m2 and dacarbazine 375 mg/m2 , day 1 and 15) chemotherapy, risk group 2 (stage IIB and IIIA) 4 courses of ABVD, risk group 3 patients (IIIB,IVA/B) 6 courses of COPP/ABV (cyclophosphamide 600 mg/m2, vincristine 1.4 mg/m2 on day 1, procarbazine 100 mg/m2 day1-7, prednisolone 40 mg/m2 day1-14; adriamycine 35 mg/m2, bleomycin 10 U/m2, vinblastine 6 mg/m2 day 8) chemotherapy, each course administered every 28 days. All patients recieved involved field radiotherapy 15-25 Gy adjusted to age (15 Gy for < 5, 20 Gy for 5-10, 25 Gy for >10 years old), + 5 Gy for bulky disease and/or partial response to chemotherapy. Results There were 83 males and 39 females (M/F: 2.1) with a median age of 10 (2-18) years. The most frequent histological subtypes were mixed cellularity (41%) and nodular sclerosing (41%). The most common involved site was the neck (cervical and supraclavicular lymph nodes) (%85). The median follow up period was 6 1/12 years (1-25 years). The 5 year event free survival and survival were 82% and 97% for all patients; they were 86% and 97% for risk 1 (48 patients), 80% and 96% for risk 2 (29 patients), 79% and 97% for risk 3 (45 patients) . B symptoms were present in 46%; %54 were staged as I-II; 46% as stage III-IV. When classified according to two time periods: before and after 2000, the median age increased [9 (2-17) vs 11 (3-18) years], M/F ratio decreased [2.7 (36/13) vs 1.8 (47/26)] and the most common histological subtype were mixed cellularity (51%) vs nodular sclerosing (49%) respectively. The 5 year event free survival and survival were 79% and 95% before 2000 and 83,5% and 98% after 2000 respectively. Ebstein Barr Virus-Latent membrane Protein (EBV-LMP1) was found to be positive by immunohistochemistry in all tumor samples of 21 patients analyzed, all were treated before 2000. During follow-up no clinically evident cardiotoxicity or pulmonary toxicity has been observed. Three patients developed secondary tumors (Langerhans cell histiocytosis, schwannoma, non-Hodgkin's lymphoma). ConclusionThe epidemiologic features of HL is related to socioeconomic status. In our cohort, the oberved change in epidemiologic features within 25 years, such as the increase in median age, decrease in the M/F ratio and increase in nodular sclerosing subtype, is thought to be related to the socioeconomic development. A high survival rate has been achieved with the institutional risk-adapted protocol for all risk groups. The use of risk adapted protocols providing efficient and least toxic treatment is very important in pediatric Hodgkin lymphoma. Disclosures No relevant conflicts of interest to declare.

Ten children, 3 to 15 years of age with high risk primary brain tumors were treated with postradiation 'eight in one' chemotherapy; vincristine, lomustine, procarbazine, hydroxyurea, cisplatin, cytosine arabinoside, cyclophosphamide and methylprednisolone. The tumors comprised of three medulloblastomas, two primitive neuroectodermal tumors, one ependymoblastoma and four anaplastic ependymomas. Treatment involved surgery (two total resection, six subtotal and two biopsy only) followed by conventional radiotherapy (primary tumor: 50-54 Gy, whole brain: 30-45 Gy, and spinal axis: 25-36 Gy). Objective tumor response with radiotherapy was achieved in 7 of 9 patients (78%) (6/8 patients with residual tumor and one patient with complete resection but positive cerebrospinal fluid cytology). Complete response was attained in 4 of 9 patients (44%). 'Eight in one' chemotherapy was initiated four weeks after radiation and repeated at 4 weekly intervals for 5-8 courses. Postradiation 'eight in one' failed to show any additional effect on tumor responses. Median survival was 34 months (range 9-48 months) with five of ten patients alive: four in complete and one in partial remission. All the five survivors were among the patients who had achieved response to initial treatment. This result suggested that degree of response to initial treatment might determine subsequent outcome and thus the choice of modality for initial therapy might be important.

The cellular and humoral immunological parameters (leucocyte, granulocyte, lymphocyte, total T, T4, T8 lymphocyte counts, lymphoproliferative response to PHA [LP-PHA), natural killer cell activity [NKCA], IgG, IgM and Ig A levels) of 20 pediatric brain tumor patients were investigated before and after chemo-(CT) and radiotherapy (RT) administered according to the UIOI-PBT-91 protocol. The T4 and T8 cell percentages and the LP-PHA values before therapy were found to be significantly diminished in comparison to values obtained from 12 healthy children (p < 0.05). In patients receiving postoperative CT, all cellular immunity parameters except T8 cell number and NKCA; IgG and IgA levels were significantly decreased after two courses of CT (p < 0.05). In 7 patients given postoperative RT, a depression in all cellular immunity parameters was observed (p < 0.05). In 6 patients treated with 2 courses of postoperative CT followed by RT administered concomitantly with low dose CDDP, there was a decrease in all cellular and humoral immunity parameters, which was not found to be significant. In 5/18 patients infectious episodes in mild to moderate severity were observed, none causing mortality. It was concluded that the UIOI-PBT-91 protocol caused cellular immunosuppression both after CT and after RT and some humoral immunosuppression after CT, but was found to be tolerable in regard to acute immunological side effects.

e22011Background: The 20 year cumulative risk of second malignancies (SM) in childhood cancer survivors is reported as 3.2 %. The aim of this study is to assess the incidence and outcome of SM in p...

(1995). Renal Cell Carcinoma in Childhood: A Case Report. Pediatric Hematology and Oncology: Vol. 12, No. 3, pp. 313-316.

Effective cancer biomarkers for early detection, prognosis, or therapy response prediction are urgently need in metastatic RCC. M30 and M65 are released during apoptotic cell death and precisely reflect epithelial tumor cell death. The aim of this study was to determine the prognostic value of plasma M30 and M65 levels in predicting survival rates for patients with metastatic RCC.Thirty-nine patients with metastatic RCC and 39 healthy control subjects were included in this study. Serum M30 and M65 levels were measured by ELISA.The median ages of the patients and control subjects were 60 and 58 years, respectively. No difference was detected in the median serum M30 level between the patients and control subjects (53.7 vs. 49.1 U/L; P = .31). The median serum M65 level was significantly higher in patients than in control subjects (334.0 vs. 179.1 U/L; P.001). Receiver operating characteristic analysis revealed that the best cutoff value for serum M65 level for predicting progression-free survival (PFS) was 313.6 U/L. The median PFS of patients whose M65 levels were ≤ 313.6 U/L was better than that of patients whose M65 levels were313.6 U/L (P = .03).To the best of our knowledge, this is the first study to evaluate serum M30 and M65 levels in patients with RCC. Serum M65 levels were significantly elevated in patients with metastatic RCC compared with healthy individuals. In addition, the serum M65 level could be predictive of PFS in patients with RCC.

A 4 1/2-year-old girl with a primary osteosarcoma of the seventh cervical vertebra, treated by surgery and chemotherapy, and who is off therapy for 24 months with no evidence of disease is presented and the literature reviewed. © 1994 Wiley-Liss, Inc.

Objective: Therapy targeted against the vascular endothelial growth factor pathway is a standard of care for patients with metastatic renal cell carcinoma. This study assessed the response rates and toxicity profiles of sunitinib on a continuous once-daily dosing regimen in Turkish patients with metastatic renal cell carcinoma. Methods: Between April 2006 and August 2010, 74 patients with metastatic renal cell carcinoma who received sunitinib on a continuous, once-daily dosing regimen were included. Sunitinib was administered daily at a dose of either 37.5 mg (94% of the patients) or 25 mg (6% of the patients), without interruption, either as a second-line treatment after interferon-a or as a first-line treatment. Response, toxicity, progression-free survival and overall survival were evaluated. Results: Of the 74 patients, 65 (88%) were diagnosed with clear cell renal cell carcinoma. The median treatment duration was 10 months (range, 2‐42 months). The most common treatment-related adverse events were fatigue (75%), stomatitis (51%) and hypertension (50%). The most common Grade 3 or 4 adverse events were anemia (10%) and hand‐foot syndrome (7%). Dose reductions were required in 50% of the patients, and early treatment discontinuation was necessary in 16% of the patients. Cardiovascular events were the most common adverse events that resulted in drug discontinuation. The objective response rate and the disease control rate were 30 and 78%, respectively. The median progression-free survival and overall survival were 13 and 25 months, respectively. Conclusions: Continuous, once-daily administration of sunitinib was generally well tolerated in Turkish patients with advanced renal cell carcinoma in a daily practice setting. This study’s response rates were comparable to those in previous randomized trials.

Background We evaluated the utility of post-treatment plasma levels of the circulating bone-morphogenetic protein-6-specific mRNA (cBMP6 mRNA), cell-free DNA (cf-DNA), apoptotic nucleosomes and Histone H3 lysine 27 trimethylation (H3K27me3), in discriminating metastatic prostate cancer (PCa) from organ confined, locally controlled disease. Methods Peripheral blood was taken from the patients at the end of therapy, and quantitative PCR was performed to amplify cBMP6 mRNA or cf-DNA from plasma while apoptotic nucleosomes and H3K27me3 were determined by ELISA-based approaches. Following blinded measurements, the markers were compared between the patients with local (n = 22), local advanced (n = 11) or metastatic disease (n = 28). Results Of the four markers investigated, the cBMP6 mRNA and H3K27me3 levels revealed significant differences between the three subgroups. We found higher levels of cBMP6 mRNA in the patients with metastases than in those with localized (p = 0.001) or local advanced disease (p = 0.05). When compared to cBMP6, H3K27me3 displayed an inverse distribution and was significantly lower in the patients with metastatic disease than in those with localized (p = 0.05) or local advanced disease (p = 0.024). There was no correlation between the different markers and total PSA levels or Gleason score at diagnosis. Conclusion Our study provides evidence that post-treatment analysis of cBMP6 mRNA and H3K27me3 may be used to distinguish metastatic PCa from organ confined, locally controlled disease.

Rhabdomyosarcoma (RMS) is rare disease in adults (age >or= 16 years). The data from randomized prospective trials are scarce; the clinical outcome of these patients seems poor with the currently available treatment strategies. In this study, we report a single institution's experience in the treatment of adult RMS. We reviewed the medical records of patients with RMS who were >or= 16 years and have been treated in our institution between 1988 and 2003 retrospectively. We analyzed the survival outcome of these patients and the prognostic impact of clinical/pathological factors on their survival. In total, 23 patients with RMS were identified. Median age was 26 years (range, 16-72 years). Majority of patients were male (n: 17, 73.9%), and had large tumors (>or= 5 cm, n: 13, 56.5%), localized disease (N0, M0, n: 12, 52.2%), and embryonal histology (n: 10, 43.5%). Median overall survival was 31.3 months, and the 3-year progression-free survival and overall survival rates were 19.9% and 34.94%, respectively. Patients with smaller tumors (< 5 cm) (p < 0.04), local disease (p < 0.01), and normal lactic dehydrogenase (LDH) level (p < 0.01) at the time of diagnosis were found to have better survival outcome. The tumor size, serum LDH level, and metastatic disease at the time of diagnosis are potential predictors of outcome in patients with adult RMS. Adult RMS is an aggressive disease with poor survival despite treatment. The data from prospective, randomized multicenter trials are necessary in order to improve the clinical outcome of adult RMS patients.

Diffuse infiltrating retinoblastoma is a rare subtype, occurring in 1% of all patients with retinoblastoma. It usually presents with pseudoinflammatory response in the anterior chamber and the vitreous, masquerading as endophthalmitis or uveitis. This report describes a 12-year-old boy with multiple free-floating intraocular pseudocysts as a unique finding in diffuse infiltrating retinoblastoma. These pseudocysts represent necrotic seeds without epithelial lining. Invasive surgical procedures should be avoided in children presenting with atypical, chronic, unilateral intraocular inflammation of unknown cause until retinoblastoma is excluded, [J Pediatr ophthalmol Strabismus 2009;46:312-316.]

Summary Background Thirty adult patients with head and neck soft tissue sarcoma (HNSTS) treated between 1987 and 2000 were retrospectively analysed. Patients and methods The most frequent histopathological subtypes were chondrosarcomas (27%) and malignant fibrous histiocytoma (20%). The surgical resection was performed in 25 of the 30 patients (83%). Twenty-three patients in the surgical resection arm received postoperative radiotherapy. Results Five-year local control rates for patients with negative surgical margins (n = 9), microscopically positive disease (n = 10), gross residual disease (n = 6) and inoperable cases (n = 5) were 64, 70, 20 and 0%, respectively. However, there was no significant difference in local control between patients with negative or microscopically positive disease who received postoperative radiotherapy (71 vs. 70%). The patients who received doses ≥60 Gy had significantly higher local control rates than the ones who received doses lower than 60 Gy (p = 0.048). The local control rates were lower in patients with grade 2–3 tumours when compared with grade 1 tumours (44 vs. 83%). The median overall survival of whole group was 31 months. Median survivals of patients receiving both surgery and radiotherapy with negative and microscopically positive margins were significantly better than patients who were not treated with surgery (34.8 and 36 vs. 13.3 months). Conclusion Our results confirm that the optimal treatment of HNSTSs is complete surgical excision, and that postoperative adjuvant radiotherapy clearly improves local control.