Search

Objective: The use of ophthalmic artery chemotherapy (OAC) as a front-line and salvage therapy for retinoblastoma has grown. However, the risk of metastatic death in these patients remains unclear. Materials and Methods: This study of metastatic deaths in OAC may benefit physicians managing retinoblastoma patients. A literature search of Medline, Scopus, Science Direct, and CINAHL was conducted from conception until November 2023. The primary outcome was metastatic death in patients treated with OAC. Results: From the 219 evaluated articles, nine met the inclusion criteria. A total of 596 (635 eyes) patients were treated with OAC; and 20 cases resulted in death due to metastasis of the retinoblastoma. The metastatic mortality rate was 2.5% (95% confidence interval: 0.8%-4.2%) which was statistically significant (p < 0.05). The central nervous system was the most common site of metastasis, followed by multiple sites of metastasis. Conclusion: OAC treatment is associated with the risk of metastatic death, but it is lower than the overall mortality rate of retinoblastoma. Further studies to identify the risk of metastasis are needed.

Objective: To compare the prevalence of HS and quality of life in non-transfusion dependent thalassemia (NTDT) and Transfusion dependent thalassemia (TDT) patients and to identify risk factors associated with HS in pediatric thalassemia. Materials and Methods: We conducted a cross-sectional study of pediatric thalassemic patients aged from 6 months - 18 years between January 2020 and October 2020, at Thammasat University Hospital, Thailand. Results: There were 141 thalassemia patients (35 TDT and 106 NTDT), aged 7 months-18 years, 73 (51.8%) were male. Sixty-eight patients (48.2%) reported snoring; 28 patients (19.9%) had HS; the remaining 40 patients (28.4%) had simple snoring. The prevalence of HS was not significantly different between TDT and NTDT group (6 (17.1%) VS 22 (20.8%); P= 0.527). Quality of life assessed by OSA-18 score was not significant difference between TDT and NTDT groups (51.3 ± 18.8 VS 45.7 ± 11.4; P=0.141). The associating risk factors for the development of HS after multivariate logistic analysis were nasal congestion, and male gender, with an adjusted OR of 5.3 and 3.0, respectively. Conclusion: Prevalence of HS was increased in children with thalassemia. Factors such as nasal congestion and male gender were strongly associated with HS in this population. The quality of life assessment using the OSA-18 questionnaire indicated that thalassemia children generally exhibited a good quality of life. Additionally, our study observed relatively low serum ferritin levels in comparison to previous studies. The standard care provided for TDT patients, includes regular blood transfusion and effective iron chelation, may contribute to slowing down the degree of nasopharyngeal narrowing in thalassemia patients.

Objective: To determine the clinical features and treatment outcomes of pediatric extracranial germ cell tumor (EGCT) in Thailand. Materials and methods: A retrospective chart review of children under 15 years old with newly diagnosed EGCT who were treated at Faculty of Medicine Siriraj Hospital from January, 2004 to December, 2013 was conducted. Results: Forty-four patients were included in the study. The median age at diagnosis was 1.74 years (1 day-14.7 years) with the median follow up time of 6.9 years (14 days-15.2 years). Twenty-eight patients (64%) had extragonadal tumor. The most common primary tumor location was the sacrococcygeal area. Majority of the patients (61%) had malignant EGCT; yolk sac tumor was the most common diagnosis. Six patients (14%) had stage IV disease. Forty patients (91%) underwent surgery; 27 patients (61%) received chemotherapy. Thirty-eight patients (86%) achieved remission; 3 patients (7%) subsequently relapsed at a median time of 1 year. Eight patients (18%) died, mostly from tumor progression. The 5-year event-free survival (EFS) and overall survival (OS) rate were 78.3% and 81.1%, respectively. Patients achieving total tumor removal had significantly better 5-year EFS and OS. Cox regression analysis revealed that the adequacy of surgery was the only prognostic factor for survival. Conclusion: The survival rate of pediatric EGCT in our study was relatively favorable, but still inferior to that of developed countries. Novel therapy may be warranted for those patients who are unresponsive to the current treatment.

Objective: Although the outcomes of treatment of Kasabach-Merritt syndrome (KMS) have improved over time, some patients succumb to uncontrollable hemorrhage. Additionally, there is no consensus guideline for the management of KMS. Clinical research on KMS might benefit physicians who treat such patients. Methods: A retrospective chart review of patients diagnosed as KMS by Siriraj Hospital from 2006 to 2016 was conducted. Results: Ten patients were diagnosed with KMS. Four patients underwent surgical intervention and obtained pathological results; 3 of them had kaposiform hemangioendothelioma (KHE), while the fourth had infantile hemangioma (IH). The combination of propranolol and prednisolone, with or without vincristine, was the most common first-line regimen, with a complete response of 37.5%. A combination of vincristine, aspirin, and ticlopidine (VAT) was prescribed as the second-line therapy for 5 patients, but there were no responses in this cohort. Another 2 patients attained hematological remission with embolization and prednisolone monotherapy. A further 2 patients with KHE who were refractory to other treatments responded well to sirolimus, while the tenth patient died of abdominal hemorrhage. Conclusion: The combination of propranolol and prednisolone seems to be effective for KMS. Sirolimus may be considered for salvage therapy for those whose disease is recalcitrant to standard treatment, especially in cases of KMS secondary to KHE. However, research on a larger cohort should be conducted to substantiate the efficacy of such treatments.

Background: Malignancy-associated hemophagocytic syndrome (MAHS), a secondary form of hemophagocytic lymphohistiocytosis (HLH), can be found with several types of malignancy. It can be manifested either before or after the diagnosis of the underlying malignancy. However, mediastinal germinoma associated HLH has never been reported in previous literatures. Case report: A 13-year-old boy presented with prolonged fever for 10 days with marked hepatosplenomegaly and progressive bicytopenia. Additional investigations demonstrated hyperferritinemia and increased hemophagocytic activity in the bone marrow without evidence of malignancy, compatible with the diagnosis of HLH. He responded well to the HLH-treatment with intravenous immunoglobulins and dexamethasone, but the HLH recrudesced 5 days later. Further investigation revealed anterior mediastinal mass. He quickly deteriorated afterwards and developed pulmonary hemorrhage leading to respiratory failure and died on the following day. Result of the post-mortem tumor biopsy was consistent with mediastinal germinoma. Conclusion: MAHS should be considered in HLH patients who do not respond well or develop recurrence after the appropriate HLH-immunochemotherapy. HLH associated with mediastinal germinoma is rare and fatal. Making diagnosis of the underlying mediastinal germinoma is complicated and challenging. Early diagnosis and prompt treatment of HLH along with the appropriate treatment of germinoma might be the important key for the treatment success.