Chemotherapy in Retinoblastoma: Current Approaches

Retinoblastoma (RB) is the most common childhood malignant intraocular tumor. Although enucleation and external beam radiotherapy have been historically used, today the most commonly used eye-sparing approach is chemotherapy. Chemotherapy can be used in both intraocular and extraocular RB cases. Chemotherapeutic agents may be applied in different ways, including systemic, subconjunctival, intra-arterial and intravitreal routes. The main purposes of application of systemic therapy are to reduce the tumor size for local treatment (chemoreduction), or to reduce the risk of metastasis after enucleation surgery (adjuvant therapy). Intra-arterial chemotherapy with the current name “super-selective intra-arterial infusion therapy” could be applied as primary therapy in tumors confined to the retina or as a secondary method in tumor recurrence. The most important advantage of intra-arterial therapy is the prevention of systemic chemotherapy complications. Intravitreal chemotherapy is administered in the presence of persistent or recurrent vitreous seeding. The term “extraocular RB” includes orbital invasion and metastatic disease. Current treatment for orbital invasion is neoadjuvant chemotherapy followed by surgical enucleation and adjuvant chemotherapy and radiotherapy after surgery. In metastatic disease, regional lymph node involvement, distant metastases, and/or central nervous system (CNS) involvement may occur. Among them, CNS involvement has the worst prognosis, remaining at almost 100% mortality. In metastatic disease, high-dose salvage chemotherapy and autologous hematopoietic stem cell rescue therapy are the possible treatment options; radiotherapy could also be added to the protocol according to the side of involvement

Prognostic Factors and a New Prognostic Index Model for Children and Adolescents with Hodgkin's Lymphoma Who Underwent Autologous Hematopoietic Stem Cell Transplantation: A Multicenter Study of the Turkish Pediatric Bone Marrow Transplantation Study Group

transplantation, posttransplant white blood cell count over 10x103/µL, posttransplant positron emission tomography positivity at day 100, and serum albumin of ;lt;2.5 g/dL at diagnosis were correlated with progression-free survival. No remission at the time of transplantation, bone marrow positivity at diagnosis, and relapse after AHSCT were significant parameters for overall survival. Conclusion: the major factors affecting the progression-free and overall survival were clearly demonstrated. A CIPI that uses a lactate dehydrogenase level of 500 IU/L worked well for estimating the prognosis. We recommend AHSCT at first complete remission for relapsed cases, and it should also be taken into consideration for patients with high prognostic scores at diagnosis.Amaç: Relaps/refrakter Hodgkin lenfomanın (HL) otolog hematopoetik kök hücre nakli (OHKHN) sonrasındaki prognozunu gösterecek belirteçler ve çocukluk çağında yeni bir prognostik skorlama araştırıldı. Gereç ve Yöntemler: Bu çalışmada, Ocak 1990-Aralık 2014 tarihleri arasında OHKHN uygulanan 61 hastanın OHKHN sonrası prognozunu etkileyen faktörlerin sağkalım üzerine etkisi araştırıldı. Aynı zamanda Yaşa Göre Düzeltilmiş Uluslararası Prognostik İndeks ve Çocukluk Dönemi Uluslararası Prognostik İndeks'lerinin (ÇDUPİ) prognoz üzerindeki etkisi değerlendirildi. Bulgular: Altmış bir hastanın ortanca yaşı OHKHN sırasında 14,8 yıl (5-20 yıl) idi. Hastalardan, 28 olguda bir relaps, 8 olguda >=2 relaps ve 25 olguda refrakter hastalık vardı. Kemosensitivite/kemoterapiye dirençlilik oranı 36/25 idi. Nakil öncesi radyoterapi almamak, nakil öncesi remisyonda olmamak, nakil sonrası beyaz kan hücresi sayısının 10x103/µL üzerinde olması, nakil sonrası 100. gün pozitron emisyon tomografisi pozitifliği, tanıda 2,5 g/dL'den düşük serum albümin düzeyi progresyonsuz sağkalım üzerinde etkili belirteçler iken transplantasyon zamanı remisyonda olmamak, tanıda kemik iliği pozitifliği ve OHKHN sonrası relaps ise genel sağkalım üzerinde etkili parametreler olarak bulundu. Sonuç: Relaps/refrakter HL'li çocuklarda progresyonsuz ve genel sağkalımı etkileyen faktörler açıkça gösterildi. Serum laktat dehidrogenaz üst sınırını 500 IU/L olarak kullanan ÇDUPİ prognozu göstermede etkili bulundu. Relaps hastalarda ilk tam remisyonda OHKHN yapılmasını ve tanı anında yüksek prognostik skoru olan olguların da OHKHN açısından değerlendirmeye alınmasını önermekteyiz

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

C-MOPP ve Radyoterapi Alan Hodgkin Lenfomalı Çocuklarda İkincil Neoplaziler: Dört Olgu Sunumu

Patients who survive Hodgkin lymphoma (HL) are at increased risk of secondary neoplasms (SNs). A wide variety of SNs have been reported, including leukemias, non-Hodgkin’s lymphomas, and solid tumors, specifically breast and thyroid cancers. Herein we report subsequent neoplasms in four patients with HL receiving chemoradiotherapy. It is interesting that three SNs, fibrosarcoma, thyroid carcinoma, and retrobulbar meningioma, were observed in the radiation area in one of our patients. A hypopharyngeal epithelioid malignant peripheral nerve sheath tumor as an unusual secondary malignant neoplasm developed in another patient, while a benign thyroid nodule and invasive ductal breast carcinoma were observed at different times in the female patient. Follicular adenoma of the thyroid gland developed in one of our patients.Yaşayan Hodgkin lenfomalı olgularda ikincil neoplazilerin gelişme riski yüksektir. Lösemiler, Hodgkin-dışı malign lenfomalar ve solid tümörler özellikle meme ve tiroid kanserlerini içeren çok çeşitli ikincil malign neoplaziler raporlanmıştır. Burada kemoradyoterapi alan Hodgkin lenfomalı dört olguda gelişen ikincil neoplaziler sunulmuştur. İlginç olarak hastalarımızdan birinde ışın alanında üç ikincil neoplazm; fibrosarkom, tiroid karsinom ve retrobulber meningiom, diğer bir hastamızda sıradışı ikincil malign neoplazm olarak hipofaringeal epitelyal malign periferik sinir kılıfı tümörü gelişirken kız hastada farklı zamanlarda benign tiroid nodülü ve invaziv duktal meme kanseri saptandı. Bir hastamızda ise, tiroidde folliküler adenoma gelişti

Hematopoietic stem cell transplantation in relapsed or refractory extracranial primitive neuroectodermal tumor of children and adolescents: A multicenter survey study

WOS: 000361247200483

Prognostic factors and a new prognostic index model for children and adolescents with Hodgkin’s lymphoma who underwent autologous hematopoietic stem cell transplantation: A multicenter study of the turkish pediatric bone marrow transplantation study group

Objective: The prognostic factors and a new childhood prognostic index after autologous hematopoietic stem cell transplantation (AHSCT) in patients with relapsed/refractory Hodgkin’s lymphoma (HL) were evaluated. Materials and Methods: The prognostic factors of 61 patients who underwent AHSCT between January 1990 and December 2014 were evaluated. In addition, the Age-Adjusted International Prognostic Index and the Childhood International Prognostic Index (CIPI) were evaluated for their impact on prognosis. Results: The median age of the 61 patients was 14.8 years (minimummaximum: 5-20 years) at the time of AHSCT. There were single relapses in 28 patients, ≥2 relapses in eight patients, and refractory disease in 25 patients. The chemosensitivity/chemorefractory ratio was 36/25. No pretransplant radiotherapy, no remission at the time of transplantation, posttransplant white blood cell count over 10x103/ μL, posttransplant positron emission tomography positivity at day 100, and serum albumin of <2.5 g/dL at diagnosis were correlated with progression-free survival. No remission at the time of transplantation, bone marrow positivity at diagnosis, and relapse after AHSCT were significant parameters for overall survival. Conclusion: The major factors affecting the progression-free and overall survival were clearly demonstrated. A CIPI that uses a lactate dehydrogenase level of 500 IU/L worked well for estimating the prognosis. We recommend AHSCT at first complete remission for relapsed cases, and it should also be taken into consideration for patients with high prognostic scores at diagnosis

Hematopoietic stem cell transplantation in refractory or recurrent lymphomas of children and adolescents: a multicenter survey of Turkish pediatric BTM study group

...European Group for Blood and Marrow Transplantatio