Current understanding of angiosarcoma: disease biology and evolving treatment

Angiosarcoma is a very rare soft tissue sarcoma that originates from endothelial cells and typically has a poor prognosis. It is most commonly found in elderly white men and can occur anywhere in the body, particularly in the head, neck, and scalp. Patients who have undergone previous radiation treatment or who have chronic lymphedema also face an elevated risk of this condition. Various genetic changes are suspected to contribute to the development of angiosarcoma, and these changes have been identified as potential targets for treatment. For localized disease, wide surgical resection is often the prudent course of action. A multidisciplinary approach, which may include surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes. In this review, we discuss the general understanding of angiosarcoma and its management, with a particular focus on the current evolving treatments for the disease.

Clinical Characteristics and Treatment Outcomes of Childhood Acute Promyelocytic Leukemia in Korea: A Nationwide Multicenter Retrospective Study by Korean Pediatric Oncology Study Group / Journal of the Korean Cancer Association, 대한암학회지

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.

Facial injury patterns associated with stand-up electric scooters in unhelmeted riders

Background@#In recent years, the number of people riding stand-up electric scooters in South Korea has increased rapidly. This study analyzed the facial injury patterns associated with stand-up electric scooter-related trauma in patients who visited our emergency center. @*Methods@#In this retrospective review, we collected data from patients who had visited our emergency center for stand-up electric scooter-related trauma between January 1, 2018 and December 31, 2020. @*Results@#The average age of the patients was 26.7 years. Of the 73 patients, 47 were male and 26 were female, highlighting a male predominance. Seven patients were injured after alcohol consumption. None of the patients had worn helmets. Additionally, upon comparing the frequency of facial injuries according to the quarter of the year, the facial injury rate was higher in the second and third quarters than in the first and fourth quarters. Facial soft tissue injuries were classified into facial cosmetic subunits and analyzed according to their location. The highest number of injuries was on the chin (n=18, 20.9%). Of the 73 patients with stand-up electric scooter-related facial injuries, 16 had acute facial osseous fractures. The commonest acute facial osseous fractures were those of the orbit (n=7, 26.9%). @*Conclusions@#The patterns of facial trauma reported here can be helpful for quickly evaluating and treating patients with facial trauma related to stand-up electric scooters. Additionally, people riding stand-up electric scooters should be aware of the risks associated with facial trauma and the importance of wearing protective devices, such as helmets and masks.

Current concepts of Kimura disease: pathophysiology and evolution of treatment

Kimura disease (KD) is a chronic inflammatory disorder that frequently involves the subcutaneous tissue of the head and neck regions. It often manifests as regional lymphadenopathy or salivary gland enlargement in the affected area. The histologic architecture of the lymph nodes in KD patients is preserved, while the affected tissues show follicular hyperplasia and eosinophilic infiltration. No single modality has been adopted as an optimal treatment for KD. This article concentrates on the fundamental features of KD and reviews current approaches to its treatment.

Clinical efficacy of adjuvant chemotherapy in stage IB (< 4 cm) non-small cell lung cancer patients with high-risk factors

Background/Aims@#Adjuvant chemotherapy is the standard of care for resected stage II-IIIA non-small cell lung cancer (NCSLC). The efficacy of adjuvant chemotherapy in stage IB (< 4 cm) NSCLC with high-risk factors is controversial. @*Methods@#This retrospective multicenter study included 285 stage IB NSCLC patients with high-risk factors according to the 8th edition tumor, node, metastasis (TNM) classification from four academic hospitals. High-risk factors included visceral pleural invasion, vascular invasion, lymphatic invasion, lung neuroendocrine tumors, and micropapillary histology patterns. @*Results@#Of the 285 patients, 127 (44.6%) were included in the adjuvant chemotherapy group and 158 (55.4%) were included in the non-adjuvant chemotherapy group. The median follow-up was 41.5 months. Patients in the adjuvant chemotherapy group had a significantly reduced recurrence rate and risk of mortality than those in the non-adjuvant chemotherapy group (hazards ratio, 0.408; 95% confidence interval, 0.221 to 0.754; p = 0.004 and hazards ratio, 0.176; 95% confidence interval, 0.057 to 0.546; p = 0.003, respectively). Adjuvant chemotherapy should be particularly considered for the high-risk factors such as visceral pleural involvement or vascular invasion. Based on the subgroup analysis, adjuvant chemotherapy should be considered when visceral pleural involvement is present, even if the tumor size is < 3 cm. @*Conclusions@#Adjuvant chemotherapy may be useful for patients with stage IB NSCLC with high-risk factors and is more relevant for patients with visceral pleural involvement or vascular invasion.

Development of the Parental Questionnaire for Cerebral Visual Impairment in Children Younger than 72 Months

Background@#and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. @*Methods@#The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. @*Results@#The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. @*Conclusions@#The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

Korean Clinical Imaging Guidelines for the Appropriate Use of Chest MRI

MRI has the advantages of having excellent soft-tissue contrast and providing functional information without any harmful ionizing radiation. Although previous technical limitations restricted the use of chest MRI, recent technological advances and expansion of insurance coverage are increasing the demand for chest MRI. Recognizing the need for guidelines on appropriate use of chest MRI in Korean clinical settings, the Korean Society of Radiology has composed a development committee, working committee, and advisory committee to develop Korean chest MRI justification guidelines. Five key questions were selected and recommendations have been made with the evidence-based clinical imaging guideline adaptation methodology.Recommendations are as follows. Chest MRI can be considered in the following circumstances:for patients with incidentally found anterior mediastinal masses to exclude non-neoplastic conditions, for pneumoconiosis patients with lung masses to differentiate progressive massive fibrosis from lung cancer, and when invasion of the chest wall, vertebrae, diaphragm, or major vessels by malignant pleural mesothelioma or non-small cell lung cancer is suspected. Chest MRI without contrast enhancement or with minimal dose low-risk contrast media can be considered for pregnant women with suspected pulmonary embolism. Lastly, chest MRI is recommended for patients with pancoast tumors planned for radical surgery.

Development of the Parental Questionnaire for Cerebral Visual Impairment in Children Younger than 72 Months

Background@#and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. @*Methods@#The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. @*Results@#The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. @*Conclusions@#The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

Removal of intraosseous hemangioma in frontal bone under direct vision through a small incision

Complete surgical excision within a margin of normal healthy bone is the treatment of choice for intraosseous hemangioma. A 56-year-old man visited with complaints of a firm, mildly tender, immovable, and palpable mass on the right forehead (size: 1.5× 1.5 cm). Non-contrast brain computed tomography performed preoperatively revealed a 1.5 cm heterogenous osteolytic lesion with suspected internal trabeculation in the right frontal bone. Under general anesthesia, a 2 cm transverse incision was made on the forehead skin rather than bicoronal incision. Full-thickness en bloc resection of the frontal bone including the mass was performed. The frontal bone was removed with care taken not to damage the frontal sinus mucosa. The frontal sinus was sealed with a collagen patch (Tachocomb) and a cranioplasty was performed using bone cement. At 6 months postoperative, a clean wound was confirmed without any complications, and there was no local recurrence. Surgical excision of intraosseous hemangioma in the frontal sinus bone can be performed via direct incision or the bicoronal approach. In this case, the direct incision approach was used to achieve smaller scars and faster recovery than the bicoronal approach.

Korean Clinical Imaging Guidelines for the Appropriate Use of Chest MRI

MRI has the advantages of having excellent soft-tissue contrast and providing functional information without any harmful ionizing radiation. Although previous technical limitations restricted the use of chest MRI, recent technological advances and expansion of insurance coverage are increasing the demand for chest MRI. Recognizing the need for guidelines on appropriate use of chest MRI in Korean clinical settings, the Korean Society of Radiology has composed a development committee, working committee, and advisory committee to develop Korean chest MRI justification guidelines. Five key questions were selected and recommendations have been made with the evidence-based clinical imaging guideline adaptation methodology.Recommendations are as follows. Chest MRI can be considered in the following circumstances:for patients with incidentally found anterior mediastinal masses to exclude non-neoplastic conditions, for pneumoconiosis patients with lung masses to differentiate progressive massive fibrosis from lung cancer, and when invasion of the chest wall, vertebrae, diaphragm, or major vessels by malignant pleural mesothelioma or non-small cell lung cancer is suspected. Chest MRI without contrast enhancement or with minimal dose low-risk contrast media can be considered for pregnant women with suspected pulmonary embolism. Lastly, chest MRI is recommended for patients with pancoast tumors planned for radical surgery.

A forehead hematoma as the initial clinical sign of lung cancer

Primary lung cancer commonly metastasizes to the brain, bones, liver, and adrenal glands. In some cases, bone metastasis serves as the first presenting sign of lung cancer with bone pain and headache, but it is not common. The incidence of skull metastasis in lung squamous cell carcinoma (SCC) is low, and there have been only a few cases of skull metastases serving as the first sign of malignancy with skull mass and epidural bleeding; however, no similar cases have been reported regarding that of hematoma. We report a case of an 84-year-old man who first presented with a simple forehead hematoma and was eventually diagnosed with SCC of the lung.

Extensive and aggressive growth of adenoid cysticcarcinoma in the lacrimal gland

Adenoid cystic carcinoma (ACC) in the lacrimal gland is a very rare disease with poor overall prognosis.Its primary treatment is surgical excision, including orbital exenteration and radical orbitectomy,which is combined with radiotherapy and chemotherapy. Age, histopathologic type, boneinvasion, and tumor extent are known factors that affect the prognosis of ACC. Furthermore, perineuralinvasion is highly associated with local tumor recurrence and tumor base invasion. Here,we report a rare case of ACC in the lacrimal gland with superior sagittal sinus invasion that repeatedlyrecurred after the surgical excision.

Business Cycle and Occupational Accidents in Korea

Background@#Occupational accidents occur for a variety of reasons, such as unsafe behaviors of workers and insufficient safety equipment at the workplace, but there are also various economic and social factors that can impact working conditions and working environment. This study analyzed the relationship between changes in economic factors and the occurrence of occupational accidents in Korea. @*Methods@#Multilinear regression analysis was used as the analysis model. The general to specific method was also used, which consecutively removes statistically insignificant variables from a general model that includes dependent variables and lagged variables of dependent variables. @*Results@#The frequency of occupational accidents was found to have a statistically significant relationship to economic indicators. The monthly number of cases of occupational injury and disease and fatal occupational injuries were found to be closely related to manufacturing capacity utilization, differences in the production index in the services sector, and commencements of building construction. The increase in equipment investment indicators was found to reduce fatal occupational injuries. @*Conclusion@#The results of this study may be used to develop occupational accident trends or leading indicators, which in turn can be used by organizations that manage and monitor occupational accidents toward taking administrative action designed to reduce occupational accidents. The results also imply that short-term and mid- to long-term economic and social changes that can impact workers, workplaces and working conditions, and workplace organizations must be taken into account if more effective government policies are to be established and implemented toward further prevention of occupational accidents.

Fluorescence Image-Based Evaluation of Gastric Tube Perfusion during Esophagogastrostomy

During esophagectomy and esophagogastrostomy, the prediction of anastomotic leakagerelies on the operating surgeon’s tactile or visual diagnosis. Therefore, anastomoticleaks are relatively unpredictable, and new intraoperative evaluation methods or tools areessential. A fluorescence imaging system enables visualization over a wide region of interest,and provides intuitive information on perfusion intraoperatively. Surgeons can choosethe best anastomotic site of the gastric tube based on fluorescence images in real timeduring surgery. This technology provides better surgical outcomes when used with anoptimal injection dose and timing of indocyanine green.

Seroprevalence of Hepatitis A Virus in Pediatric Patients with Hematologic Malignancies after Chemotherapy and Hematopoietic Cell Transplantation / 감염과화학요법

This retrospective study was performed to determine the seroprevalence of hepatitis A virus (HAV) in children and adolescents with hematologic malignancies after the completion of chemotherapy and hematopoietic cell transplantation (HCT). Of 97 enrolled patients, 60 (61.9%) were seropositive for HAV. The seroprevalences in patients undergoing chemotherapy and HCT were 60.3% (41/68) and 65.5% (19/29), respectively (P = 0.628). No significant factors associated with seropositivity for HAV after chemotherapy and HCT were identified. Anti-HAV tests and HAV re-vaccinations can be considered in children and adolescents with underlying hematologic malignancies after chemotherapy and HCT based on the anti-HAV results.

Intracranial abscess from facial cellulitis

Cellulitis, one of most common diseases of everyday life, is often overlooked for its significance. Although cellulitis does not cause or lead to serious problems usually, its possibility to cause life-threatening problem should be known. In present case, a patient who had received acupuncture treatment a week earlier presented to the clinic with symptoms of facial cellulitis. The disease resolved within few weeks under empirical antibiotic treatment but recurred after 3 months. Under close history review of the patient, we found out that the patient had received craniectomy 20 years ago. The patient had blunt headache with no other neurological symptoms that could suspect cranial infection, but considering the risk originating from the patient’s surgical history, brain computed tomography (CT) was taken. CT images revealed abscess formation in the subgaleal and epidural spaces. Craniotomy with abscess evacuation was done promptly. With additional antibiotic treatment postoperatively, the disease resolved, and the 1-month postoperative follow-up brain CT showed no signs of abscess formation.

Analysis of subbrow upper blepharoplasty by measuring the lid-to-brow distance

BACKGROUND: Conventional upper blepharoplasty through a supratarsal incision is commonly performed for periorbital rejuvenation. However, the drawbacks of this technique include insufficient removal of lateral hooding and an unnatural postoperative appearance. Subbrow upper blepharoplasty with submuscular fascia fixation was developed to avoid these shortcomings. Herein, we report our experiences with this technique and present an objective analysis of the results. METHODS: Subbrow upper blepharoplasty was performed in 30 patients between June 2016 and October 2017. All patients had preoperative frontal view photographs, and the distance from the upper lid margin to the lower edge of the eyebrow was measured at the lateral limbus (LBD-L). RESULTS: Between preoperative and postoperative measurements, the LBD-L was shortened by an average of 0.800 and 0.833 mm on the right and left, respectively (P=0.047 and P=0.070). Most patients were satisfied with their aesthetic appearance and the improvement of their visual field. CONCLUSIONS: The lid-to-brow distance was minimally changed by the procedure, but the results were aesthetically favorable. This seemingly paradoxical finding is explained by the characteristics of the surgical procedure and the related anatomy. Among the various possible blepharoplasty techniques, subbrow upper blepharoplasty with submuscular fascia fixation has definite value for Asians.

Multiple foreign bodies causing an orocutaneous fistula of the cheek

Foreign bodies impacted in the maxillofacial region are often a diagnostic challenge. They can be a source of chronic inflammatory reactions and infections leading to the formation of an orocutaneous fistula. Such orocutaneous fistulas cause significant morbidity in most patients, eventually requiring surgery. Recently, we encountered a very rare case of an orocutaneous fistula caused by multiple foreign bodies in the cheek. Precise removal of the foreign bodies was required, and a double-sided anterolateral thigh free flap was used to reconstruct the defect. Surgeons should be aware of the complications of multiple foreign bodies and should be able to diagnose these on careful clinical examination.

Correction of Cicatricial Upper Eyelid Ectropion Using a Dermofat Graft and an Anti-Adhesive Agent

Ectropion is characterized by eversion of the eyelid margin and exposure of the cornea and conjunctiva. This leaves the inner eyelid surface exposed and prone to irritation, and in severe cases, poor eye closure. Ectropion can occur for several reasons including congenital malformations, trauma, burns, anti-cancer medications, allergies, and inappropriate eyelid surgery. Surgical treatments usually include local flaps or skin grafts, depending on the severity of the defect. We present a case of successful cicatricial upper eyelid ectropion correction using a dermofat graft and an anti-adhesive agent (Guardix-Sol) after the wide release of tethering scar tissue.

A Case of Tuberculous Peritonitis Presenting as Small Bowel Obstruction / 대한소화기학회지

The peritoneum is one of the common extrapulmonary sites of tuberculosis infection. Patients with underlying end-stage renal or liver disease are frequently complicated by tuberculous peritonitis; however, the diagnosis of the tuberculous peritonitis is difficult due to its insidious nature, well as its variability in presentation and limitation of available diagnostic tests. Once diagnosed, the preferred treatment is usually antituberculous therapy in uncomplicated cases. However, surgical treatment may also be required for complicated cases, such as small bowel obstruction or perforation. An 85-year-old woman was referred our hospital for abdominal pain with ileus. Despite medical therapy, prolonged ileus and progression to sepsis were shown, she underwent surgery to confirm the diagnosis and relief of mechanical ileus. Intraoperative peritoneal biopsy and macroscopic findings confirmed tuberculous peritonitis. Therefore, physicians should consider the possibility of tuberculous peritonitis in patients with unexplained small bowel obstruction.