Orbital histiocytosis and fibrohistiocytosis: the clinicopathological characteristics of 117 patients, over a decade of experience.

PURPOSE: To describe the clinicopathological features of a large cohort of patients with orbital histiocytoses and fibrohistiocytosis, such as Langerhans cell histiocytosis (LCH) and non-LCH disorders, and correlate patients' clinical characteristics with their pathological diagnosis. METHODS: In this retrospective study, medical records of patients presenting to Farabi Eye Hospital, a tertiary eye care center in Tehran, Iran, from 2010 until 2022, were reviewed. Patients' demographics, chief complaint, location and laterality of the tumor, best-corrected visual acuity, presence of bone erosion on imaging, and their pathological diagnosis were retrieved. Excisional biopsy was performed and evaluated through light microscopy and immunohistochemistry study for their respective markers, including CD1a, CD68, CD207, and S100. RESULTS: A total of 117 patients with 11 pathological subtypes of histiocytoses and fibrohistiocyosis were identified, with 56.4% male and 43.6% female patients. The mean age at presentation was 23.4 years (range 1.5 months-73 years). Swelling and palpable mass were the most common chief complaints. LCH was the most common pathology (32.5%), followed by juvenile xanthogranuloma (26.5%) and adult xanthogranuloma (21.4%). Age, lesion location, and bone erosion had a statistically significant difference among the various diagnosed subtypes. CONCLUSIONS: Histiocytoses and fibrohistiocytosis are diverse and rare disorders potentially involving multiple organ systems. Ophthalmic manifestations of these diseases are even more uncommon. We reviewed their orbital presentation along with their respective histopathological findings. Our results also suggested that an orbital CT scan can be of diagnostic value to discriminate LCH from other histiocytic pathologies.

Epidemiological aspect of retinoblastoma in the world: a review of recent advance studies.

AIM: To collect and present updated evidence about epidemiological aspects of retinoblastoma (Rb) in the world. METHODS: A comprehensive search without the time and language restrictions was conducted in international databases, including MEDLINE, Scopus, Web of Science, and PubMed. The search keywords were "retinoblastoma" OR "retinal Neuroblastoma" OR "retinal glioma" OR "retinoblastoma eye cancer" OR "retinal glioblastoma". RESULTS: The worldwide incidence of Rb is 1 in 16 000-28 000 live births, but was higher in developing compared to developed countries. Several attempts for improving early detection and treatment had increased the Rb survival rate from 5% to 90% in developed countries over the past decade, but its survival was lower in developing countries (about 40% in low-income countries) and the majority of mortalities occurred in developing countries. The etiology of Rb could be viewed as genetics in the heritable form and environmental and lifestyle factors in the sporadic form. Some environmental risk factors such as in vitro fertilization; insect sprays; father's occupational exposure to oil mists in metal working, and poor living conditions might play a role in the occurrence of the disease. Although ethnicity might affect Rb incidence, sex has no documented effect and the best treatment approaches were now ophthalmic artery chemosurgery and intravitreal chemotherapy. CONCLUSION: Determining the role of genetics and environmental factors helps to accurately predict the prognosis and identify the mechanism of the disease, which can reduce the risk of tumor development.

Ocular Manifestations of Newly Diagnosed Acute Leukemia Patients.

Purpose: To present primary ocular manifestations in acute leukemia. Methods: This cross-sectional descriptive hospital-based study evaluated all newly diagnosed leukemia patients of three referral hospitals of Tehran University of Medical Sciences in 2015-2016 and Mahak Hospital in Tehran in 2017. Exclusion criteria included the patients with the previous history of chemotherapy, cases of relapsing disease, and the patients with a history of ocular disease or other systemic conditions with ophthalmic manifestations. Results: A total of 85 patients (170 eyes) were evaluated in our study, including 29 children (34.1%) and 43 females (50.6%). The mean patient age was 37.84 ± 11.91 years in the adult group and 6.28 ± 4.70 years in the pediatric category. Ophthalmic involvement was seen in 27 patients (31.8%), including 6 pediatric patients (20.7%) and 21 adult patients (37.5%). Two patients (2.3%) had direct infiltration by leukemic cells and 76 patients (89.41%) of patients were asymptomatic. There was a correlation between ophthalmic involvement and platelet count and hemoglobin level. In patients with ocular signs, higher mortality rates were observed. Conclusions: At the time of diagnosis in acute leukemia patients, complete ophthalmic evaluation including dilated fundus examination is suggested as ocular involvement in these patients is common and sometimes asymptomatic. Ophthalmic involvement in leukemic patients should be identified in a timely manner, particularly in individuals with low platelet counts and hemoglobin levels, due to the potential prognostic relevance.

Psycho-art-drama: development and testing a new integrated complementary method of psychiatric treatments for hospitalised children with cancer (a case study).

Objective: Art therapy as a psychotherapy method using art, and psychodrama as a therapeutic method using role play, have different roots, principles, techniques and different effect mechanisms and advantages. Integrated psychotherapies are aimed to maximise each of the component advantages, as well as minimising disadvantages. Therefore, the main objective of this study was to develop a new integrated psychotherapy method, named psycho-art-drama (PAD) for hospitalised children with cancer and testing its effectiveness.Methods: After designing the PAD protocol, five hospitalised children with cancer accompanied with their mothers selected based on inclusion and exclusion criteria to participate in this single-subject study.Results: The results showed that PAD was significantly effective in reducing the bio-psycho-social expressions of incompatibility in the hospital (BPSEIH) in all of the five children.Conclusions: Findings of this study give preliminary support using the PAD model in similar conditions.

A Single-Center Experience With Hematopoietic Stem Cell Transplantation for Pediatric Acute Lymphoblastic Leukemia: A Modest Pitch for Non-Total Body Irradiation Conditioning Regimens.

OBJECTIVES: Allogeneic hematopoietic stem cell transplantation has been used for several decades to treat patients with acute lymphoblastic leukemia. Total body irradiation has been promoted as an important component of conditioning regimens for this process; however, recent reports of chemotherapy-based conditioning regimens have shown comparable outcomes. MATERIALS AND METHODS: We report our experience with radiation-free conditioning using busulfan and cyclophosphamide in 127 pediatric patients with acute lymphoblastic leukemia who were treated between 1997 and 2014. The median age was 11 years (range, < 1 to 15 y), 70% of patients were male, 81.1% received transplants from HLA-matched siblings, 83% received peripheral blood stem cells, 41% were in second complete remission at the time of transplant, and 83% had B-lineage immunophenotype. RESULTS: In patients who were in complete remission at the time of transplant, 5-year overall survival, leukemia-free survival, and relapse rates were 62.48% (95% confidence interval, 52.29-71.09%), 49.43% (95% confidence interval, 39.57-58.53%), and 45.64% (95% confidence interval, 35.85-54.88%), respectively. We observed significant differences between outcomes in patients by time of transplant, presence of chronic graft-versus-host disease, and remission status. CONCLUSIONS: Our relapse rates were comparable to those shown in recent studies, although the transplant-related mortality rate was lower. The results of our study showed that a busulfan/cyclophosphamide conditioning regimen has acceptable outcomes without the undesirable adverse effects of total body irradiation, particularly in pediatric patients. Large multicenter studies are needed to assess less toxic conditioning regimens with fewer adverse effects in these patients.

Acute Lymphoblastic Leukemia with Infective Endocarditis Presented with Unusual Intracardiac Mass.

Acute lymphoblastic leukemia (ALL) is a systemic disease that is presented with different symptoms and signs. Cardiac manifestation is rare in ALL, but it is very important and needs appropriate management. It usually presents as leukemic myocardial infiltration and in the presence of cardiac mass comprehensive evaluation for other etiologies is mandatory. We reported on a 6-year-old boy in remission phase of ALL and large cardiac mass in the right atrium with obscure early symptoms and signs, in whom infective endocarditis (IE) was diagnosed and appropriate medical treatment was performed. Because the mass was sustained, surgical resection was considered for the patient.

Assessment of immune response following immunization with DTP/Td and MMR vaccines in children treated for acute lymphoblastic leukemia.

BACKGROUND: This study was performed to determine the antibody titers against diphtheria-tetanus-pertussis, and measles-mumps-rubella before and after re-vaccination in different periods of time in patients with acute lymphoblastic leukemia (ALL) to provide a suitable vaccination schedule. METHODS: Ninety patients with ALL were studied in three stages, including newly diagnosed patients, patients on maintenance therapy, and 1 to 12 months after cessation of therapy. Baseline antibody titers were assessed in all participants. Postimmunization antibody titers to DTP/Td and MMR vaccines were evaluated 3 weeks after vaccination. RESULTS: Patients on maintenance therapy had considerable decreased in seropositivity rate; they achieved seroconversion rate of 100% for diphtheria and tetanus and of 71.4% for pertussis following DTP/Td vaccination. In patients after one month off therapy, the seroconversion rates for diphtheria, tetanus, and pertussis were 71.4%, 80%, and 100%, respectively. The amounts of seroconversion in patients after 3 months off therapy were 66.5%, 80%, and 66.5% for measles, mumps, and rubella, respectively. In patients after 6 months off therapy, the seroconversion rates were 85.7% for diphtheria, 100% for pertussis and tetanus, 83.3% for mumps, 50% for rubella, and 41.6% for measles. Patients after 12 months off therapy achieved seroconversion rate of 100% for diphtheria, tetanus, pertussis, and mumps, of 71.5% for rubella, and of 63.5% for measles. CONCLUSION: Administration of one dosage of DTP/Td vaccine during maintenance therapy and after cessation of therapy could be recommended, while one dosage of MMR vaccine at least three months after completion of therapy could be safe and beneficial.

Expression and prognostic significance of Oct4 and Nanog in neuroblastoma.

Neuroblastoma is the most common extracranial solid tumor of children, accounting for an estimated 15% cancer-related deaths in this period. It has been hypothesized that drug resistance of cancer stem cells may be responsible for chemotherapy failure, sustained tumor growth, and recurrence in many solid tumors. In this study, we investigated the expression of Octamer-binding transcription factor 4 (Oct4) and Nanog, two stem cell markers, in 47 neuroblastic tumors by immunohistochemistry and correlated their expression by other prognostic factors especially with NMYC amplification using both fluorescent and chromogenic in situ hybridization methods. Twenty three cases (48.9%) showed Oct4 signals and eight cases (17%) showed Nanog expression. All Nanog positive tumors showed Oct4 expression. Seven cases (14.1%) had NMYC amplification. There was also no association between positive Oct4 and Nanog reactivity and tumor morphology, age, mitosis-karyorrhexis index, NMYC amplification, favorable or unfavorable histology, and risk groups (p > 0.05). Cancer stem cells hypothesis is a challenging issue and controversies exist about their significance. Although our study did not show strong association between prognostic factors and expression of stem cell markers, performing of further large-scale studies of various neuroblastic tumors with various stages is suggested.