Solitary fibrous tumors (SFT) are rare fibroblastic mesenchymal neoplasms which are originally described as neoplasms of the pleura originating from the spindle cells. Although it can originate from extrapleural sites including the head and neck, it is exceedingly rare in the sinonasal tract. There has been no reported cases of SFT involving the paranasal sinuses in Korea; however, there was case of a 34-year-old man who presented with persistent left nasal obstruction and watering of the left eye. Imaging by CT and MRI revealed a large, highly vascular tumor occupying the maxilloethmoidal sinus cavities associated with bony wall destruction, masquerading as maxillary sinus cancer. The tumor mass occupying sinus cavities was removed through endoscopic and Caldwell-Luc approach. Histopathological examination of the tumor was consistent with SFT. We report this case to further insights regarding the diagnosis and management of this rare tumor.

OBJECTIVE: To evaluate engraftment by visualizing the location of human bone marrow-derived mesenchymal stem cells (hBM-MSCs) three-dimensionally in photothrombotic cerebral infarction (PTCI) models of rats. MATERIALS AND METHODS: Magnetic resonance imaging (MRI) of an agarose block containing superparamagnetic iron oxide (SPIO)-labeled hBM-MSCs was performed using a 3.0-T MRI, T2-(T2WI), T2*-(T2*WI), and susceptibility-weighted images (SWI). PTCI was induced in 6 rats, and 2.5 x 10(5) SPIO-labeled hBM-MSCs were infused through the ipsilateral internal carotid artery (ICA group) or tail vein (IV group). MRI was performed on days 1, 3, 7, and 14 after stem cell injection. Dark signal regions were confirmed using histology. Three-dimensional MRI reconstruction was performed using the clinical workflow solution to evaluate the engraftment of hBM-MSCs. Volumetric analysis of the engraftment was also performed. RESULTS: The volumes of SPIO-labeled hBM-MSCs in the phantom MRI were 129.3, 68.4, and 25.9 microL using SWI, T2*WI, and T2WI, respectively. SPIO-labeled hBM-MSCs appeared on day 1 after injection, encircling the cerebral infarction from the ventral side. Dark signal regions matched iron positive cells and human origin (positive) cells. The volume of the engraftment was larger in the ICA group on days 1, 3, and 7, after stem cell injection (p < 0.05 on SWI). SWI was the most sensitive MRI pulse sequence (p < 0.05). The volume of infarction decreased until day 14. CONCLUSION: The engraftment of SPIO-labeled hBM-MSCs can be visualized and evaluated three-dimensionally in PTCI models of rats. The engraftment volume was larger in the ICA group than IV group on early stage within one week.
Animals ; Cerebral Infarction/pathology/*radiography ; Contrast Media ; Dextrans ; Humans ; Imaging, Three-Dimensional/methods ; Magnetic Resonance Imaging/*methods ; Magnetite Nanoparticles ; Male ; *Mesenchymal Stem Cell Transplantation ; Mesenchymal Stromal Cells/radiography ; Nanoparticles ; Neuroimaging/*methods ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Tomography, X-Ray Computed

Background and Objectives: Carotid body paraganglioma is the common type of carotid body tumor for which angiography, carotid artery balloon occlusion test (BOT) and tumor embolization could be considered before the surgery. We analyzed cases in a single institute and reviewed related literature to investigate the necessity of these preoperative examinations.Subjects and Method Medical records of patients who were diagnosed with paraganglioma were retrospectively analyzed from 2000 to 2019. Results: Sixteen patients were identified. Of the total, 14 patients underwent surgery at this institute, and 13 underwent angiography. Of the 13 patients who underwent angiography, 6 patients underwent carotid artery BOT, and 12 patients underwent tumor embolization. The average tumor size of 6 patients who underwent carotid artery BOT was 28.7 mm, and 8 patients who did not undergo carotid artery BOT was 30.1 mm. The average tumor size of 12 patients who underwent tumor embolization was 29.4 mm. Two patients did not undergo tumor embolization, and their average tumor size was 30 mm. In 1 patient, both preoperative angiography and carotid artery BOT were performed, but tumor embolization was not performed due to spasm of tumor vessels. Conclusion Preoperative carotid artery BOT can be performed to reduce side effects in patients with the potential for carotid resection. In addition, tumor embolization is performed regardless of tumor size. By reducing the amount of bleeding during surgery and reducing the size of the tumor, it is possible to secure an appropriate surgical field of view to facilitate operation during surgery; however, its effectiveness needs to be clearly identified.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.