Thymic carcinoma is a rare malignant neoplasm of the thymic epithelium, distinguished from benign or invasive thymoma by the presence of malignant cytology and a greater incidence of local invasion and embolic metastases. The true incidence of this neoplasm is unknown. Nearly three fourth of their patients had symptoms of an anterior mediastinal mass, including cough, chest pain, and superior vena cava syndrome. These patients rarely have myasthenia gravis or other thymoma-associated paraneoplastic syndromes. The treatment of thymic carcinoma remains a controversial matter. We report eight cases of thymic carcinoma treated in our institution from 1990 to 1997.
Chest Pain ; Cough ; Epithelium ; Humans ; Incidence ; Mediastinum ; Myasthenia Gravis ; Neoplasm Metastasis ; Paraneoplastic Syndromes ; Superior Vena Cava Syndrome ; Thymoma*

PURPOSE: To report a case of progressive outer retinal necrosis treated by combined intravitreal foscarnet and ganciclovir. CASE SUMMARY: A 11-year-old male with a history of chemotherapy and cord blood transplantation due to precursor T-cell leukemia developed Herpes zoster lesion on his forehead and rapidly progressing peripheral retinal necrosis without vasculitis in the right eye. Varicella-Zoster virus was confirmed in his cerebrospinal fluid using polymerase chain reaction (PCR); and the patient was diagnosed with progressive outer retinal necrosis. Despite combined treatment with intravenous acyclovir and foscarnet and intravitreal foscarnet, retinal necrosis progressed to retinal detachment and total retinal necrosis. During follow-up, new retinal necrosis was observed in his left eye. The patient was started on combined intravenous and intravitreal foscarnet and ganciclovir; retinal necrosis in the left eye regressed and posterior pole was spared. With subsequent oral valganciclovir and intravitreal foscarnet and ganciclovir, the remaining retina was preserved with maintained vision.
Acyclovir ; Cerebrospinal Fluid ; Child ; Drug Therapy ; Fetal Blood ; Follow-Up Studies ; Forehead ; Foscarnet* ; Ganciclovir* ; Herpes Zoster ; Herpesvirus 3, Human ; Humans ; Leukemia, T-Cell ; Male ; Necrosis* ; Polymerase Chain Reaction ; Retina ; Retinal Detachment ; Retinaldehyde* ; Vasculitis

Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

Purpose: Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. Materials and Methods: From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. Results: Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). Conclusion The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.