• Journal of Korean Neurosurgical Society
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• v.53 no.3
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• pp.187-189
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• 2013

We report an unusual case of cerebral aneurysmal subarachnoid hemorrage (SAH) with Fabry's disease. A 42-year-old woman presented with aneurysmal SAH originated from a saccular aneurysm of the right posterior communicating artery. The patient was treated by an endovascular coil embolization of aneurysm. Postoperatively the patient recovered favorably without any neurological deficit. During her admission, the patient had a sign of proteinuria in urine analysis. The pathologic findings of kidney needle biopsy implied nephrosialidosis (mucolipidosis of lysosomal stroage disease), which is consistent with a Fabry's disease. It is uncommon that Fabry's disease is presented with aneurysmal SAH, especially in middle-aged patients, but could be a clinical concern. Further investigations are needed to reveal risk factors, vascular anatomy, and causative mechanisms of Fabry's disease with aneurysmal SAH.

• Journal of Korean Neurosurgical Society
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• v.40 no.5
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• pp.391-393
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• 2006

Dermoid cysts of the spinal canal are rare benign congenital tumors, accounting for $1{\sim}2%$ of all intraspinal tumors. We report a case of lumbar extramedullary cyst, combined with congenital sacral meningocele. The clinical features, characteristics on MRI, pathologic findings, and surgical treatment of such a rare extramedullary benign tumor is discussed with the relevant literature.

• Annals of Clinical Neurophysiology
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• v.25 no.2
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• pp.106-109
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• 2023

Inclusion body myositis (IBM) is a late-onset myopathy that manifests as distinct muscle weakness in the quadriceps, finger flexors, and ankle dorsiflexors. T-cell large granular lymphocyte (T-LGL) leukemia is a late-onset clonal disorder of CD8+ cytotoxic T-cells that is often accompanied by autoimmune diseases. To date, the association between IBM and T-LGL leukemia has been infrequently reported. Here, we report a case of a patient with T-LGL leukemia who developed IBM, along with in-depth laboratory, electrophysiological, and pathologic findings.

• Investigative Magnetic Resonance Imaging
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• v.20 no.2
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• pp.114-119
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• 2016

We report a case of vegetation in a 4-year-old female with infective endocarditis, diagnosed by late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) imaging. The patient had a history of primary closure for ventricular septal defect and presented with mild febrile sensation. No remarkable clinical symptoms or laboratory findings were noted; however, transthoracic echocardiography demonstrated a 14 mm highly mobile homogeneous mass in the right ventricle. On LGE CMR imaging, the mass showed marginal rim enhancement, which suggested the diagnosis of vegetation rather than thrombus. The extracellular volume fraction (${\geq}42%$) of the lesion was higher than that of normal myocardium. Based on the patient's clinical history of congenital heart disease and pathologic confirmation of the lesion, a diagnosis of infective endocarditis with vegetation was made.

• Investigative Magnetic Resonance Imaging
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• v.12 no.2
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• pp.188-196
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• 2008

Purpose : To describe the clinical, MR imaging, and pathologic findings of pediatric meningiomas. Materials and Methods : The authors retrospectively reviewed the medical records and MR images of 16 pediatric patients with pathologically proven meningioma. Mean patient age at diagnosis was 14 years (range, 3-18). MR images were reviewed for details of lesion sizes, locations, signal intensity (SI), marginal characteristics, internal architectures, enhancements, and dural and parenchymal changes. The findings of other imaging modalities and of pathological examinations were also analyzed. Results : Mean tumor size was 5.24 cm (range, 1.3-18.1 cm) and locations were supratentorial in 12 and infratentorial in 4. SI of masses were variable, that is, high in 9, iso in 4, and low in 3 on T2 weighted images (T2WI), and low (n=11), iso (n=4), or high (n=1) on T1WI images. All lesions were visualized as well-demarcated enhancing masses. Five of the tumors were heterogeneous with cystic or necrotic components. Dural attachment was observed in 11 patients and adjacent brain edema in 10. Tumors exhibited hyperdense (n=6) or isodense (n=4) on non-enhanced CT scans, and 3 of the 7 angiograms demonstrated blood supply from the internal carotid artery. Pathologic examinations revealed the following subtypes; transitional cell (n=4), meningotheliomatous (n=4), chordoid (n=2), fibrous (n=2), clear cell (n=1), hyalinized (n=1), rhabdoid papillary (n=1), and atypical (n=1). Conclusion : Pediatric meningiomas occur usually in teenagers, have diverse pathological types, and may produce atypical imaging findings, such as, a heterogeneous internal content or findings suggestive of intraaxial tumors.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1107-1111
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• 2003

Purpose : Neuroblastoma is a malignant neoplasm which arises from primitive sympathetic neuroblasts, and occasionally can matured from a malignant neuroblastoma into a benign ganglioneuroma. It has the highest rate of spontaneous regression of any pediatric tumor. We performed a retrospective study of pathologic features after combination therapy in advanced neuroblastoma. Prognostic effects of the individual morphologic feature and prognostic groupings according to modified Shimada classification systems were analyzed. Methods : The treatment results for six patients with neuroblastoma seen at Keimyung University from Jan. 1991 to June 2000 were analyzed. Patients were treated with a combination of chemotherapy, radiation therapy, and surgery, and classified by two major prognostic criteria based on morphological features of neuroblastoma, such as modified Shimada classification and histologic grading. Results : Three cases were classified to a good histologic group; among them, two cases survived, but one case was lost in follow-up. There were three cases classified in a poor histologic group. All of these patients expired due to sepsis and hemorrhagic pancreatitis. Conclusion : Prognostic classification due to pathologic findings had significant value in evaluating the survival rate of neuroblastoma patients.

• Tuberculosis and Respiratory Diseases
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• v.82 no.4
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• pp.277-284
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• 2019

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.

• The Journal of the Korean bone and joint tumor society
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• v.2 no.1
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• pp.60-64
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• 1996

Giant cell tumor of tendon sheath is a slow-growing, unilateral and solitary lesion that is most commonly seen in the digits of the hand and knee, but occasionally occurs in the hips, ankles, toes and wrists. The lesion is asymptomatic or mildly painful, especially if it is diffuse and located in a major joint. The concepts concerning about the pathogenesis of these lesion have undergone constant revision, which include neoplastic process, inflammation and lipid metabolism. Authors analysized 20 patients with giant cell tumor of tendon sheath about the etiology, clinical findings, pathologic findings and treatment results. The results were as follows: 1. 13 cases were females (65%) and 7 cases were males (35%), and the range of age was from 9 years old to 60 years old. 2. The hand was most frequently involved site in which 14 cases (70%) were included, and the foot was involved in 5 cases (25%). 3. Solitary lesions were 15 cases and multiple lesions were 5 cases. 4. Radiographically, in 3 cases bony erosion was seen. 5. All cases were treated by surgical excision and presented no recurrence. In conclusion, the giant cell tumor of tendon sheath, which has been considered to be benign tumorous conditions appeared to be necessary for complete surgical excision to prevent recurrence.

• The Journal of the Korean bone and joint tumor society
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• v.4 no.2
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• pp.88-93
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• 1998

Neurilemoma usually discovered incidentally, is a benign nerve-sheath tumor which has been described as a painless mass. In most cases, the size of the mass was smaller than 5cm. However, it was reported that there were masses, sometimes associated with local tenderness and pain, whose size was over 6cm. Then, we have found there is a relationship between mass size and neurologic symptoms, as well as with, postoperative complications. It is important to diagnose early and to treat it. Twenty neurilemoma patients, who were treated at Keimyung University Dongsan Medical Center were analyzed using their clinical symptoms, pathologic findings, radiologic findings and complications. There was no sexual difference in tumor incidence. The anatomical locations of tumors were as follows. ; upper extremities in 15 cases(69%), axilla in 1 case(4%) and lower extremities in 6 cases(27%). Symptoms were palpable mass in 22 cases, local tenderness in 8 cases(36%), radiating pain in 6 cases(27%) and paresthesia in 6 cases(27%), Median nerve was involved most frequently(33%). There were 2 patients(10%) with multiple symptoms. Tumor enucleation was done in all cases. The size of tumors in longest axis was smaller than 2cm in 2 cases, between 2 and 4cm in 11 cases and more than 5cm in 9 cases. There was no case of malignant transformation or recurrence. In conclusion, incidence of clinical symptom and postoperative complications are increased with the size of the tumor especially over the 5cm. Preoperative MRI finding was the most accurate method of diagnosis and most helpful in determining surgical resection margin.

• Endocrinology and Metabolism
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• v.32 no.3
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• pp.353-359
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• 2017

Background: Cushing syndrome is characterized by glucose intolerance, cardiovascular disease, and an enhanced systemic inflammatory response caused by chronic exposure to excess cortisol. Eosinopenia is frequently observed in patients with adrenal Cushing syndrome, but the relationship between the eosinophil count in peripheral blood and indicators of glucose level in patients with adrenal Cushing syndrome has not been determined. Methods: A retrospective study was undertaken of the clinical and laboratory findings of 40 patients diagnosed with adrenal Cushing syndrome at Chungnam National University Hospital from January 2006 to December 2016. Clinical characteristics, complete blood cell counts with white blood cell differential, measures of their endocrine function, description of imaging studies, and pathologic findings were obtained from their medical records. Results: Eosinophil composition and count were restored by surgical treatment of all of the patients with adrenal Cushing disease. The eosinophil count was inversely correlated with serum and urine cortisol, glycated hemoglobin, and inflammatory markers in the patients with adrenal Cushing syndrome. Conclusion: Smaller eosinophil populations in patients with adrenal Cushing syndrome tend to be correlated with higher levels of blood sugar and glycated hemoglobin. This study suggests that peripheral blood eosinophil composition or count may be associated with serum glucose levels in patients with adrenal Cushing syndrome.