Kyong Ihn

Hepatoblastoma (HB) is the most common primary liver malignancy in children, predominantly affecting those younger than 3 years of age. Although the overall survival rate has improved significantly over the past few decades, standardized clinical practice guidelines for HB in Korea have been lacking. This clinical practice guideline was developed to provide evidence-based recommendations for the diagnosis and treatment of HB in children. A multidisciplinary working group comprising pediatric hematologists/oncologists, radiologists, surgeons, pathologists, and methodologists systematically reviewed the literature published up to May 2023. For pretreatment imaging, both abdominal computed tomography and magnetic resonance imaging are useful for PRE-Treatment EXTent of disease (PRETEXT) staging, with each modality offering distinct advantages. PRETEXT staging at diagnosis is recommended as it provides valuable prognostic information. For standard-risk HB (PRETEXT I–III without metastasis and alpha-fetoprotein ≥100 ng/mL), cisplatinbased chemotherapy without doxorubicin is recommended over cisplatin-doxorubicin combination therapy, as it demonstrates equivalent survival outcomes with fewer adverse effects. In carefully selected patients who are eligible for complete resection at diagnosis, upfront surgery without neoadjuvant chemotherapy should be considered to spare unnecessary chemotherapy and its toxicities. For patients with residual pulmonary metastases after chemotherapy, pulmonary metastasectomy should be considered. Sodium thiosulfate administration following cisplatin should be considered to reduce ototoxicity, although the supporting evidence is derived predominantly from standard-risk patients and its availability in Korea remains limited. Regarding histological subtypes, well-differentiated fetal HB is associated with favorable outcomes and may be treated with surgery alone, whereas the small cell undifferentiated pattern is no longer classified as a HB subtype and requires immunohistochemical and molecular testing to distinguish it from malignant rhabdoid tumor and other small round cell tumors. This guideline represents the first evidence-based clinical practice guideline for pediatric HB in Korea. It aims to standardize the diagnostic and therapeutic approaches, ultimately improving outcomes for Korean children with HB. Given the rarity of this tumor, international collaboration and prospective data collection remain essential for future guideline updates.

Infection remains a leading cause of mortality among transplant recipients. Studies on vaccine-preventable diseases (VPDs) among transplant recipients in Asia are lacking. This study aimed to investigate the incidence and risk of VPD in transplant recipients in Korea. Individuals who underwent transplantation were retrospectively recruited using data from the Korea National Health Insurance system from 2008 to 2020. We selected four major VPDs-varicella, invasive pneumococcal disease (IPD), mumps, and pertussis-and evaluated the cumulative incidence rate and standardized incidence ratio (SIR), comparing it to that of the general population. A total of 55,363 transplant recipients were included. Among these, 1,503 cases of the four VPDs were identified. Multivariable analysis revealed a significantly higher risk of varicella in children and allogeneic hematopoietic stem cell transplantation recipients. Conversely, for IPD, the risk was higher among individuals aged ≥60 years and in solid organ transplantation recipients; however, the difference was not significant. The SIR for varicella was notably higher in adult recipients aged ≥ 20 years (SIR, 2.79; 95% confidence interval [CI], 2.53-3.06; SIR, 6.02; 95% CI, 4.89-7.33), while for IPD, the SIR for all ages was notably increased at 20.3 (95% CI, 18.72-21.96). For mumps, the SIR was significantly lower at 0.24 (95% CI, 0.19-0.30) across all age groups. Conversely, for pertussis, the SIR was 0.67 (95% CI, 0.32-1.24), but the difference was not significant. These findings underscore that transplant recipients are still at higher risk of getting VPDs than is the general population, emphasizing the importance of surveillance and vaccination efforts.

Background/Objectives: Foreign body (FB) ingestion is a common occurrence in children. We aimed to investigate the characteristics of patients hospitalized in the pediatric surgery department for FB ingestion and those requiring surgical treatment. Methods: We retrospectively analyzed pediatric patients admitted to the department of pediatric surgery at a single center for FB ingestion from June 2018 to September 2023. Overall, 35 patients were included. Results: The median age was 25.0 months (range: 7–204 months), with male predominance. Forty percent (14/35) of patients presented with symptoms at admission, including abdominal pain, vomiting, and fever; ingestion was unwitnessed in 42.9%. The median number of ingested FBs was 2.0 (range: 1–25), with magnets being the most common type (51.4%). Surgery was required in 51.4% of cases due to complications or impaction. These cases showed significantly higher symptomatic presentation rates (72.2% vs. 5.9%, p < 0.01), unwitnessed ingestion (66.7 vs. 17.6%, p = 0.006), and more ingested FBs (median 5.0 vs. 1.0, p = 0.001). Magnet ingestion was more frequent in these patients. Conclusions: FB ingestion predominantly affects young male children. Magnet ingestion frequently requires surgical intervention, underscoring the importance of preventive measures and safer environments. Surgical treatment should be considered in patients with symptomatic presentation, multiple ingested objects, or an uncertain ingestion history.

Background: Management of hepatic hemangioma (HH) in infancy ranges from close monitoring to surgical resection. We analyzed the clinical characteristics and outcomes of HH according to its treatment options, with particular focus on challenging cases. Methods: Data of patients diagnosed with HHs in their first year of life and followed up for at least 1 year were retrospectively reviewed and divided into treatment and observation groups. Serial imaging results, serum alpha-fetoprotein (AFP) levels, medications, and clinical outcomes were compared. The detailed clinical progress in the treatment group was reviewed separately. Results: A total of 87 patients (75 in the observation group and 12 in the treatment group) were included. The median HH size at the initial diagnosis and the maximum size were significantly larger in the treatment group than the observation group (2.2 [0.5–10.3] cm vs. 1.0 [0.4–4.0] cm and 2.1 [0.7–13.2] vs. 1.1 [0.4–4.0], respectively; all p < 0.05]. The median initial and last serum AFP levels were significantly higher in the treatment group than in the observation group (76,818.7 vs. 627.2 and 98.4 vs. 8.7, respectively; all p < 0.05). Serum AFP levels in both groups rapidly declined during the first 3 months of life and were almost undetectable after 6 months. Among the challenging cases, a large (14 × 10 × 6.5 cm sized) focal HH was successfully treated using stepwise medical-to-surgical treatment. Conclusions: Patients with large HH and mild symptoms can be treated using stepwise pharmacotherapy. More aggressive surgical treatment of tumors unresponsive to initial pharmacotherapy may help shorten the treatment period and improve outcomes.