Clinical signs and genetic evaluation of patients with neurofibromatosis type 1 with and without optic pathway gliomas in a center in Turkey.

PURPOSE: Optic pathway gliomas (OPGs) occur in 15% of patients with neurofibromatosis type 1 (NF1). Their location renders biopsy or surgical resection difficult because of the risk of vision loss. Therefore, only a few NF1-OPGs have been used for tissue diagnosis, and only a few analyses have been published on the molecular changes that drive tumorigenesis. METHODS: Due to this reason, we evaluated 305 NF1 patients, 34 with OPG and 271 without OPG for germ line mutations. All subjects underwent clinical examination and DNA analysis of NF1, confirming the diagnosis of NF1. RESULTS: Clinically, the group with OPG had a significantly higher incidence of bone dysplasia (P < 0.001) and more café-au-lait spots (P = 0.001) compared to those in the group without OPG. The frequency of Lisch nodules was on the borderline of statistical significance (P = 0.058), whereas the frequency of neurofibromas did not differ significantly (cutaneous, P = 0.64; plexiform, P = 0.44). Individuals with OPG mostly had mutations in the first one-third of the NF1 gene compared with that in patients who did not have OPG. Some identical mutations were detected in unrelated families with NF1-OPG. CONCLUSION: The observation of certain phenotypic features and the correlation between genotype and phenotype might help to determine the risk of developing OPG with NF1.

Assessment of Ecotropic Viral Integration Site 2B (EVI2B) Gene in Juvenile Myelomonocytic Leukemia and Neurofibromatosis Type 1 NF1 Tumors.

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease that affects the development and growth of various tissues. NF1 is a major risk factor for the development of malignancies, particularly malignant peripheral nerve sheath tumors, optic gliomas, and leukemia. NF1 encodes a neurofibromin. Three genes, EVI2A, EVI2B, and OMGP, are embedded within intron 27b of NF1. However, the function of these genes remains unclear. EVI2A and EVI2B encode for putative transmembrane proteins. Mouse homologs are associated with viral insertions involved in leukemia in mice. Mouse Evi2b has been identified as a direct target gene of C/EBPα, a transcription factor critical for myeloid differentiation. Also possible is that these genes are related to the leukemia observed in patients with NF1. These genes might act as modifiers of NF1 phenotypic variations. Therefore, we investigated the EVI2B gene in leukemia and NF1 tumors. We analyzed DNA from 10, 20, and 3 patients with NF1, leukemia, and NF1-leukemia, respectively, and six NF1 tumor tissues. DNA sequencing analysis was used to identify the viral integration sequence, and the protein amounts and EVI2B gene expression were analyzed by flow cytometry and quantitative real-time PCR techniques. The EVI2B gene expression was increased in cutaneous neurofibroma compared with the control both at the level of protein and mRNA. However, its expression in plexiform neurofibroma was decreased significantly at protein level and increased at mRNA level compare to control. Moreover, integration of 455 bases near the 3' end of the exon was detected. When this integrated sequence was blasted into the NCBI retroviral genome database, an 87% match with the HIV-1 virus envelope gene was obtained. These preliminary results show that EVI2B might be important in NF1 tumorigenesis and leukemia.

Ganglioneuromas in Childhood: Hacettepe Experience With 70 Cases.

BACKGROUND: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. METHODS: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. RESULTS: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6-15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. CONCLUSIONS: Ganglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. LEVEL OF EVIDENCE: Case series, IV.

Primary ovarian tumors in children: a single center experience of 124 patients.

BACKGROUND: Primary ovarian tumors are rare in the pediatric age group. We reviewed our 40-year experience with ovarian tumors to evalute the clinical features and treatment results in a single institution. METHODS: Between January 1975 and October 2015, 124 girls with primary ovarian tumor were diagnosed and treated in our center. Tumors were identified with biopsy or total resection and/or serum markers. Seventy four children were included in the treatment analysis. RESULTS: Median age for 124 children was 11.0 years (0.73-17.63). The main complaint was abdominal pain in 85 patients (68.5%). One hundred and five patients (84.6%) had total one-sided salpingo-oophorectomy and five patients had bilateral salpingo-oophorectomy. Amongst 124 cases, 29 patients had mature teratoma, which was the most common tumor in this study. Dysgerminoma (n=21) was the most common malignant histopathologic type. Stage I disease was diagnosed in 57.2% and stage IV in 6.6% of the patients. Five year overall survival (OS) and event-free survival (EFS) for 124 children were 82.5% and 76.3% respectively. For 74 children who received treatment, 5-year OS and EFS were 75.2% and 67.1%, respectively. Age (p < 0.017), histopathological subgroup (p < 0.001), stage (p =0.003) and chemotherapy protocols (p =0.049) were significant prognostic factors for OS. CONCLUSIONS: The survival rates in children with ovarian tumors were comparable with studies in the literature. Although patients treated with platin based regimens had better survival rates, prognosis was still poor for the patients in advanced stages. This should be the focus for further studies and improvements.

Precursor B-cell Lymphoblastic Lymphoma in Children: Hacettepe Experience.

The purpose of the study was to review the clinical and pathologic characteristics and treatment results of children with precursor B-cell lymphoblastic lymphoma. Of 530 children diagnosed with non-Hodgkin lymphomas between 2000 and 2021, 39 (7.4%) were identified as having precursor B-cell lymphoblastic lymphoma. Clinical characteristics, pathologic, radiologic, laboratory data, treatments, responses, and overall outcomes were recorded from hospital files and analyzed. The median age of 39 patients (males/females, 23/16) was 8.3 years (range 1.3 to 16.1). The most common sites of involvement were the lymph nodes. At a median follow-up of 55.8 months, 14 patients (35%) had a recurrence of disease (11 stage IV, 3 stage III); 4 were in complete remission with salvage therapies, 9 died of progressive disease and one died due to febrile neutropenia. Five-year event-free survival and overall survival rates were 65.4% and 78.3% for all cases, respectively. Survival rates were higher in patients with a complete remission at the end of induction therapies. The survival rates were lower in our study compared with other studies, which could be explained by the high relapse rate and higher incidence of advanced-stage disease due to bone marrow involvement. We demonstrated a prognostic impact of treatment response at the end of the induction phase. Cases with a disease relapse have poor prognosis.

Giant cell tumor arising from the anterior arc of the rib: an extremely rare site in an adolescent girl.

BACKGROUND: Giant cell tumor is a rare and locally aggressive neoplasm of the long bones in children. Rib is the least frequently affected site, seen in less than 1% of all cases and most of them occur at the posterior arc. CASE: A 12-year-old girl presented with swelling and slight pain on the left inferior-anterior chest wall for two years. Physical examination revealed a giant, hard and fixed mass on the left chest wall. Hematological and biochemical test results were in normal limits but slight elevation of alkaline phosphatase level. Computed tomography of the chest showed a large expansive mass and lytic lesion with internal calcification arising from the anterior part of the 7th rib. En-bloc resection was performed including the 6th-8th ribs and a small part of the diaphragm. The pathological evaluation revealed giant cell tumor of bone. CONCLUSIONS: Herein, we aim to emphasize that giant cell tumor should be considered in the differential diagnosis of chest wall tumors in childhood whereby en-bloc resection and close follow up would be paramount.

Early postoperative complications in pediatric abdominal solid tumor surgery according to Clavian-Dindo classification.

PURPOSE: Postoperative period after abdominal solid tumor surgery is critical regarding complications. This study aimed to detect incidence and treatment of complications. METHODS: Single center retrospective study including years 2010-2019 for early postoperative complications were documented and graded according to Clavian-Dindo classification. RESULTS: The overall complication rate was 10% and they were chylous leak (n = 8, 2%), collection in surgical field (n = 8, 2%), acute renal failure (n = 7, 1.7%), hemorrhage (n = 5, 1.3%), intestinal obstruction (n = 4, 1%), surgical site infection (n = 3, 0.7%), thromboembolism (n = 3, 0.7%) and peripheric neuronal event (n = 2, 0.5%). Distribution of complications according to Clavian-Dindo classification was as follows: 2 grade I, 23 grade II, 9 grade IIIb, 5 grade IVa and 1 grade V. While age and percentage of benign or malignant disease were not different in groups with or without complications (p = 0.11, p = 0.24), males had more complications than female patients (p = 0.008). Having more than one surgery aiming tumor resection was associated with increased postoperative complications (p = 0.002). Incidence of complications were highest after extragonadal germ cell tumor (35%) and hepatic tumors (29%). CONCLUSIONS: Postoperative complications of abdominal tumor surgery are diverse in children. They are related with prolonged hospital stay and need for medical or surgical interventions. Number of surgeries, organ of origin and tumor type have an influence on risk of complications.

A Fatal Complication of Liver Transplant: Posttransplant Lymphoproliferative Disease.

Immunosuppressive therapy is a double-edged sword and causes a risk for some complications, such as opportunistic infections and posttransplant lymphoproliferative disease. The most likely risk factors for posttransplant lymphoproliferative disease are Epstein-Barr virus serology mismatch, prolonged and high viral load for Epstein-Barr virus, higher doses of immunosuppressive therapy, and cytomegalovirus infection. Transplant recipients who are seropositive for Epstein-Barr virus show a lower risk for posttransplant lymphoproliferative disease than seronegative recipients. Here, we present a 3.5-year-old boy who was seropositive for Epstein-Barr virus and developed posttransplant lymphoproliferative disease 18 months after liver transplant with a previous history of cytomegalovirus- related pneumatosis intestinalis.

Dual ophthalmic arteries: a new case.

We report one retinoblastoma patient with dual ophthalmic arteries (also known as "double ophthalmic arteries") arising from the internal carotid artery which fuse with each other inside the orbit. The caudal ophthalmic artery with larger caliber originated from the cavernous segment of internal carotid artery and passed through the superior orbital fissure. The cranial and tiny one arose more cranially from the supraclinoid internal carotid artery and passed through the optic canal. After superselective catheterization, frank anastomosis was shown between two ophthalmic arteries and the central retinal artery arose from the cranial one. To the best of our knowledge, this case is the first observation of a dual ophthalmic artery with cavernous/supracavernous origins fusing with each other proven by superselective ophthalmic angiography. Whether there is an anastomosis between two ophthalmic arteries is important for deciding which artery to be selected for intraarterial treatment. In cases of retinoblastoma, detailed angiographic analysis may be essential to examine the orbital arterial system and to avoid unexpected complications during intraarterial chemotherapy.

Salvage Liver Transplant in the Treatment of a Child With Local Recurrence of Hepatoblastoma.

Hepatoblastoma is the most common primary liver cancer of childhood, accounting for two-thirds of primary malignant hepatic neoplasms. Radical surgical removal combined with efficient chemotherapy is essential for cure. Despite a complete tumor resection, hepatoblastoma may recur as isolated local disease. Intrahepatic recurrence of hepatoblastoma after liver resection is among the indications for liver transplant. Here, we present a patient who underwent salvage liver transplant for the treatment of local recurrence of hepatoblastoma. A 13-year-old boy who was diagnosed with hepatocellular carcinoma arising from the left liver lobe and who had been treated with surgical resection was admitted to our outpatient oncology clinic for further evaluation because alpha-fetoprotein levels had started to increase after surgery. Histopathological reexamination of hemihepatectomy material showed a histological aspect of an epithelial hepatoblastoma. Magnetic resonance imaging revealed multifocal lesions in the right liver lobe compatible with local recurrence. Despite a favorable initial response to chemotherapy, the tumor showed progression with increased alpha-fetoprotein levels. The patient was deemed a viable candidate for an urgent liver transplant and underwent right lobe living donor liver transplant. He had excellent graft function without any complications or signs of malignancy in the last follow-up visit at 7 months posttransplant. Salvage liver transplant is a lifesaving and sometimes the only treatment option for patients with local recurrence of hepatoblastoma. Although transplant in the salvage setting has been associated with worse outcomes than primary transplant, recent data have indicated more favorable and acceptable outcomes. Further studies are warranted to better understand the role of salvage liver transplant in the treatment of hepatoblastoma. Early consultation with the liver transplant team is critical in children who are most likely to need extreme resection or liver transplant.

Paediatric appendiceal neuroendocrine tumours: a review of 33 cases from a single center.

BACKGROUND: Appendiceal neurendocrine tumours (NETs) are rare neoplasms and diagnosis is commonly incidental following appendectomy. We aimed to review our experience with appendiceal NETs. METHODS: Records of children with appendiceal NETs were reviewed and data concerning demographic characteristics, clinical findings, surgical procedures, histopathological findings, management and outcomes were recorded. RESULTS: Between 1985 and 2021, 33 cases with appendiceal NETs (median age 11.8 years, range 7.8-16; male/female = 10/23) were identified. All but one patients presented with abdominal pain, six had vomiting, four had fever and they underwent appendectomies with presumed diagnosis of acute appendicitis. Abscess drainage and appendectomy was performed in a 16-year-old girl for suspected right ovarian mass, and tumour was positive in the omentum. Histopathological diagnosis was classical carcinoid tumour (NET) in all cases. Median tumour size was 0.9 cm (n = 26, range, 0.1-3.5 cm); tumours were ≤1 cm in 19 cases, 2 cm in one, 3.5 cm in another case. Tumours were located in the tip (n = 11), body (n = 6) and base of appendix (n = 1) (in others data unavailable). In 28 patients with data, tumour extended to submucosa in five, to tunica muscularis in seven, to subserosa in six, to serosa in six, to mesoappendix in three, to periappendiceal fat in one. Three cases were lost to follow-up, 31 cases were alive (median follow-up 53 months). CONCLUSIONS: Paediatric appendiceal NETs do not behave aggressively and appendectomy alone is sufficient for tumours ≤2 cm regardless of local invasion. The need for further extensive surgery in tumours >2 cm also remains controversial.

Pediatric Neuroblastoma with Brain Metastasis: The Prognostic Role of Surgery.

AIM: To analyze the prognostic factors and treatment outcomes of neuroblastoma patients with brain metastasis. MATERIAL AND METHODS: The medical records of 7 patients with brain metastasis among 120 neuroblastoma patients treated with radiotherapy between June 2002 and December 2018 at our department were collected retrospectively. Survival time and prognostic factors were evaluated. RESULTS: The prevalence of brain metastasis was 7/120 (5.8%). The median age was 35 months (25-64). Three (43%) patients had single brain metastasis. Two (29%) patients with solitary metastases underwent gross total resection and 1 (14%) patient with two lesions underwent subtotal resection for brain metastasis. All patients received chemotherapy and radiotherapy. The median time interval between the initial diagnosis and the brain metastasis was 14 months (0-28 months) in all patients; 12 months (0-28 months) in deceased patients and 23 months (19-26 months) in patients who were still alive at the time of this analysis (p=0.245). The median overall survival time after the treatment of brain metastasis was 14 months (7-22 months). Five patients died from progressive disease and 2 patients who had gross total resection were alive for 164 and 187 months, respectively. While the 5-year overall survival rate was 67% in patients with isolated brain metastasis, the 5-year overall survival rate was 0% in the presence of other metastases (p=0.221). CONCLUSION: Isolated brain metastasis and gross total resection of the brain metastasis seems to be favorable prognostic factors. Patients with solitary brain metastasis should be consulted for neurological surgery.

Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.

Relapsed or refractory central nervous system (CNS) tumors still have poor prognosis, and, therefore, new treatment options are required. We retrospectively researched treatment results of patients with CNS tumors treated with nimotuzumab from 2010 to 2015. The study included nine patients with the diffuse intrinsic pontine glioma; eight with medulloblastoma; three each with anaplastic ependymoma, glioblastoma multiforme, and central nervous system primitive neuroectodermal tumor (CNS PNET); two patients with gliomatosis cerebri; and one patient each with other tumor types, including atypical teratoid rhabdoid tumor, thalamic astrocytoma, low-grade glial tumor, high-grade glial tumor, and cribriform neuroepithelial tumor. An objective response was observed in 10 of 33 patients with four patients showing a complete response, three a partial response, and three patients had stable disease. The 2-year overall survival (OS) and progression-free survival (PFS) rates were 35 ±9% and 19 ±8%, respectively. Due to the objective response in medulloblastoma, CNS PNET, and anaplastic ependymoma (MED group), survival rates of this group were analyzed. The 2-year OS and PFS for the MED group were 71 ±12% and 30 ±13%, respectively. The treatment was well tolerated. The treatment responses for medulloblastoma, CNS PNET, and anaplastic ependymoma have been promising. Likewise, some patients with relapsed or progressive CNS tumors may benefit through nimotuzumab-containing regimen.

Primary Paraspinal and Spinal Epidural Non-Hodgkin Lymphoma in Childhood.

Primary paraspinal/spinal epidural lymphoma (PPSEL) is rare in childhood. Here, we retrospectively evaluated patients with PPSEL treated in our department. We also reviewed the cases reported in the literature. Fifteen of 1354 non-Hodgkin lymphoma cases diagnosed over a 38-year period were PPSEPL. There were 11 male individuals and 4 female individuals with a median age of 13 years. Most common symptoms were pain and limb weakness. Physical examination revealed spinal cord compression in 80% of patients. The most common tumor location was the lumbar region. Histopathologic subtypes were lymphoblastic lymphoma in 6 and Burkitt lymphom in 5 patients. Subtotal or near-total excision of the tumor with laminectomy was performed in 6 patients. Thirteen and 9 patients received chemotherapy and radiotherapy, respectively. Neurologic recovery was observed in 70% of patients. Seven patients were alive without disease at a median of 88 months. Overall and event-free survival rates were 61.7% and 50.1%, respectively. We reviewed clinical features, treatment, and outcome of 69 PPSEL cases reported in the literature. Neurologic recovery and long-term survival was achieved in 66.7% of them. Heterogeneity in diagnostic methods and treatment have made it difficult to establish the prognostic indicators for neurologic outcome and survival. Multicenter prospective studies with more cases are necessary to determine the prognostic factors.

Primary intracranial germ cell tumors in children 36-year experience of a single center.

PURPOSE: Intracranial germ cell tumors (ICGCTs) comprise approximately 0.4%-3% of all brain tumors. In this study, we aim to evaluate clinical characteristics, treatment and outcomes of patients with ICGCT. PATIENTS AND METHODS: All patients with ICGCT diagnosed in Hacettepe University's Pediatric Oncology Department between January 1980 and January 2016 were evaluated, retrospectively. RESULTS: We identified 52 patients (male/female: 2.46) diagnosed with ICGT. Median age was 140 months. The median duration of symptoms was 3 months. Patients with endocrine symptoms were diagnosed later than others (P = 0.028). The primary site was pineal region in 20 patients, nonpineal region in 32 which included six bifocal involvements. Pineal location was more common in boys than girls (P = 0.02). Histopathological diagnosis was germinoma in 28 patients, nongerminomatous malignant germ cell tumors in 14 and immature teratoma in 4. The mean age for germinoma was higher than that of nongerminomatous tumors (P = 0.032). Patients treated with surgery and radiotherapy and chemotherapy. Median follow-up time was 52.5 months. Thirty-six patients were alive for 12-228 months. Relapsed/progressive disease was observed in 11 patients. Nongerminomatous tumors more frequently showed relapse/progression than germinoma (P = 0.06). Five-year overall and event-free survival rates for the whole group were 72.6% and 57.2%, respectively. Overall and event-free survival rates for germinoma were better than malignant nongerminomatous tumors. CONCLUSION: Although the ratio of ICGCTs to central nervous system tumors in our series was similar to western countries, some clinical features such as tumor location were similar to cases from East Asian countries. Although similar protocols were used survival rates lower than developed western and eastern developed countries.

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.

Primary gastric lymphoma: A report of 16 pediatric cases treated at a single institute and review of the literature.

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.

Comparison of intravitreal melphalan with and without topotecan in the management of vitreous disease in retinoblastoma.

PURPOSE: To evaluate clinical outcomes and enucleation rates after intravitreal melphalan (IVM) alone and after IVM combined with intravitreal topotecan (IVT) for the treatment of vitreous disease, and to a lesser extent subretinal and retrohyaloid seeds, in patients with retinoblastoma. STUDY DESIGN: A retrospective analysis of 77 eyes of 72 consecutive patients. METHODS: Demographic data, classification of tumors, seed type (dust, sphere or cloud) before injection and at the end of follow-up, injection type (IVM or IVM+IVT), doses of IVM and IVT, number of injections, follow-up time, enucleation status and side effects were recorded. Cox regression analysis and log-rank test for Kaplan-Meier curves were performed. RESULTS: Of 77 eyes, 40 received IVM alone (group 1) and 37 received IVM+IVT (group 2). Enucleation rates were 62.5% (n=25) in group 1 and 10.8% (n=4) in group 2 (p=0.001). Median eye survival was 23.6 months in group 1 and 25.6 months in group 2. Mantel-Cox test revealed statistically significant differences between Kaplan-Meier curves of group 1 and 2 (p=0.022). Multiple Cox regression analysis showed a significantly elevated enucleation rate associated with: IVM only treatment group (p=0.019) and pre-injection cloud type of seeding (p=0.014). CONCLUSION: The combined use of intravitreal melphalan and topotecan provides significantly better results in terms of avoiding enucleation and vitreal and subretinal seed control.

Assessment of the visual pathways in patients with neurofibromatosis-1 by 3S-space technique with 3-Tesla MRI

Background/aim/AIM: We aimed to evaluate the size/tortuosity of the optic nerve (ON) and the dilatation of the ON sheath (ONS) in neurofibromatosis type 1 (NF-1) patients with 3T-MRI, and to assess the usefulness of 3D-SPACE in imaging the optic pathway, ON, and ONS in NF-1 patients. Materials and methods: Twenty consecutive NF-1 patients without optic pathway glioma (OPG) (Group 1), 16 consecutive NF-1 patients with OPG (Group 2), and 19 controls were included in this study. The thickness and tortuosity of the ON and the diameter of the ONS were measured on STIR and 3D-SPACE images. Results: The thickness of the ON was similar in all groups on STIR images (P>0.05). The mean ONS diameter was higher in Group 2 with this sequence (P=0.009). Controls had significantly lower grades of ON tortuosity than Groups 1 and 2 (P=0.001), and Group 1 had significantly lower ON tortuosity compared to Group 2 (P=0.001). Severe tortuosity was only detected in Group 2. Conclusion: ON tortuosity and ONS diameter were increased in NF-1 patients in the presence of OPG. High-resolution cranium imaging with the 3D-SPACE technique using 3T-MRI seems to be helpful for detection of the optic pathway morphology and pathologies in NF-1 patients.

Desenlace de 102 pacientes con linfoma de Hodgkin menores de 5 años / Outcome of 102 patients under 5 years of age with Hodgkin lymphoma

Antecedentes. El linfoma de Hodgkin (LH) es uno de los tipos de cáncer pediátrico con más posibilidades de curación. Sin embargo, es raro entre los niños menores de 5 años, y aún se desconocen los factores pronósticos de la supervivencia debido a su baja frecuencia en este grupo etario. Objetivos. El objetivo de este estudio fue evaluar las características clínicas, los esquemas de tratamiento y el desenlace de los pacientes con LH menores de 5 años. Métodos: se realizó una evaluación retrospectiva de los pacientes con diagnóstico de LH entre 1972 y 2013. Todos los pacientes recibieron quimioterapia, con o sin radioterapia. Resultados. Participaron 102 pacientes; la mediana de edad fue de 4 años (intervalo: 2-4,9). La mediana de seguimiento fue de 13 años. Veintitrés pacientes tenían síntomas B, 15 pacientes tenían neoplasia maligna con gran masa tumoral, y los estadios I y II fueron los más frecuentes. Las tasas de supervivencia global (SG) fueron significativamente diferentes según el estadio del cáncer (p = 0,008). Si bien no se observaron diferencias estadísticamente significativas, la presencia de neoplasia maligna con gran masa tumoral y de síntomas B estuvo asociada con un mal pronóstico. Conclusión. En este estudio unicéntrico se incluyó la mayor cantidad de pacientes con LH menores de 5 años. El estadio fue el principal factor de predicción de SG; por otro lado, la presencia de síntomas B y neoplasia maligna con gran masa tumoral también afectó el pronóstico.

Background. Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group. Objectives. The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL. Methods. Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy. Results. There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median followup time was 13 years. Twenty-three patients had B symptoms, 15 patients had 'bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of 'bulky disease' and B symptoms were associated with poor prognosis. Conclusion. Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.