RESUMO
Rare neurovascular diseases (RNVDs) have not been well-recognized in Korea. They involve the central nervous system and greatly affect the patients’ lives. However, these diseases are difficult to diagnose and treat due to their rarity and incurability. We established a list of RNVDs by referring to the previous literature and databases worldwide to better understand the diseases and their current management status. We categorized 68 RNVDs based on their pathophysiology and clinical manifestations and estimated the prevalence of each disease in Korea. Recent advances in genetic, molecular, and developmental research have enabled further understanding of these RNVDs. Herein, we review each disease, while considering its classification based on updated pathologic mechanisms, and discuss the management status of RNVD in Korea.
RESUMO
Rare neurovascular diseases (RNVDs) have not been well-recognized in Korea. They involve the central nervous system and greatly affect the patients’ lives. However, these diseases are difficult to diagnose and treat due to their rarity and incurability. We established a list of RNVDs by referring to the previous literature and databases worldwide to better understand the diseases and their current management status. We categorized 68 RNVDs based on their pathophysiology and clinical manifestations and estimated the prevalence of each disease in Korea. Recent advances in genetic, molecular, and developmental research have enabled further understanding of these RNVDs. Herein, we review each disease, while considering its classification based on updated pathologic mechanisms, and discuss the management status of RNVD in Korea.
RESUMO
Small cell carcinoma of the ovary, hypercalcemic type is a rare ovarian tumor. This tumor has three distinctive clinical features: i) an occurrence in young women, ii) the presence of hypercalcemia in 62% of cases, and iii) a high degree of malignancy. The radiologic findings of the tumors are usually large and a predominantly solid mass with internal necrosis, hemorrhage and cystic degeneration. We report on the CT and MRI findings of an ovarian small cell carcinoma, hypercalcemic type in a 17-year-old female who presented 1 month abdominal discomfort with weight loss.
RESUMO
PURPOSE@#To prospectively assess the diagnostic potential of computed tomography urography (CTU) as a prior examination in the detection of bladder cancer.@*MATERIALS AND METHODS@#A total of 3280 CTU examinations were assessed in 3050 consecutive patients. Patients who were over 35 years of age, with gross hematuria, persistent microhematuria, or a history of urothelial tumor, were included in our study. Our study investigated the diagnostic capability of CTU over the course of two prior examinations. After the first examination, patients with a definite lesion observed by CTU were referred directly for rigid cystoscopy (RC) and patients with negative or probable lesion were referred for flexible cystoscopy (FC). After the second examination, patients with a definite lesion observed by CTU were referred directly for RC, patients with probable lesion were referred for FC, and patients with negative lesion were referred for clinical follow-up. Performance characteristics for the two prior examinations were determined by using pathologic findings or clinical follow-up as the reference standard.@*RESULTS@#The overall sensitivity, specificity, accuracy, positive predictive value, and negative predictive value for detecting bladder cancer were 95.2%, 95.4%, 95.4%, 69.1%, and 99.2%, respectively, for the first prior examination, and 93.4%, 93.3%, 93.3%, 61.1%, and 98.4%, respectively, for the second prior examination.@*CONCLUSION@#CTU as a prior examination is accurate for the early detection of bladder cancer. Notably, when used as a second prior examination, CTU could help to avoid the unnecessary use of FC in patients with negative lesions.