• Archives of Craniofacial Surgery
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• v.10 no.1
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• pp.37-39
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• 2009

Purpose: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includes eosinophilic granuloma, Letterer-Siwe diseases, and Hand-Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area. Methods: A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a $33{\times}25mm$ sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof. Results: Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment. Conclusion: The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.

• Tuberculosis and Respiratory Diseases
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• v.66 no.2
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• pp.98-103
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• 2009

Background: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. Methods: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. Results: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18-67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1-180 months) and only two patients died during this period. Conclusion: This study provides a national survey of the patients with PLCH during a long follow-up period.

• Journal of the Korean Society of Radiology
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• v.85 no.3
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• pp.531-548
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• 2024

Developmental dysplasia of the hip is a condition characterized by hip joint instability due to acetabular dysplasia in infancy, necessitating precise ultrasound examination. Legg-Calvé-Perthes disease is caused by a temporary disruption in blood flow to the femoral head during childhood, progressing through avascular, fragmentation, re-ossification, and residual stages. Slipped capital femoral epiphysis is a condition where the femoral head shifts medially along the epiphyseal line during adolescence due to stress, such as weight-bearing. Differentiating between transient hip synovitis and septic arthritis may require joint fluid aspiration. Osteomyelitis can be associated with soft tissue edema and osteolysis. When multiple lesions are present, it is essential to distinguish between Langerhans cell histiocytosis and metastatic neuroblastoma. This review will introduce imaging techniques and typical findings for these conditions.

• Nuclear Medicine and Molecular Imaging
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• v.41 no.6
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• pp.553-560
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• 2007

Purpose: We evaluated the standard uptake value (SUV) of F-18 FDG at PET/CT for differentiation of benign from malignant tumor in primary musculoskeletal tumors. Materials and Methods: Forty-six tumors (11 benign and 12 malignant soft tissue tumors, 9 benign and 14 malignant bone tumors) were examined with F-18 FDG PET/CT (Discovery ST, GE) prior to tissue diagnosis. The maxSUV(maximum value of SUV) were calculated and compared between benign and malignant lesions. The lesion analysis was based on the transverse whole body image. The maxSUV with cutoff of 4.1 was used in distinguishing benign from malignant soft tissue tumor and 3.05 was used in bone tumor by ROC curve. Results: There was a statistically significant difference in maxSUV between benign (n=11; maxSUV $3.4{\pm}3.2$) and malignant (n=12; maxSUV $14.8{\pm}12.2$) lesions in soft tissue tumor (p=0.001). Between benign bone tumor (n=9; maxSUV $5.4{\pm}4.0$) and malignant bone tumor (n=14; maxSUV $7.3{\pm}3.2$), there was not a significant difference in maxSUV. The sensitivity and specificity for differentiating malignant from benign soft tissue tumor was 83% and 91%, respectively. There were four false positive malignant bone tumor cases to include fibrous dysplasia, Langerhans-cell histiocytosis (n=2) and osteoid osteoma. Also, one false positive case of malignant soft tissue tumor was nodular fasciitis. Conclusion: The maxSUV was useful for differentiation of benign from malignant lesion in primary soft tissue tumors. In bone tumor, the low maxSUV correlated well with benign lesions but high maxSUV did not always mean malignancy.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.26 no.1
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• pp.58-62
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• 2015

Xanthoma disseminatum (XD) is a benign, non-Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions with face, flexures, and mucosa. Most of XD develops in mucocutaneous lesions including skin, oral cavity and pharynx, however laryngeal involvement is uncommon. While the natural course of XD is usually benign and often self-limiting, but XD develop in critical anatomical locations may result in morbidity and mortality. Localized mucous lesions in oropharynx and larynx lead to dysphagia, dyspnea and air way obstruction. The diagnosis of XD was based on clinical, histological and immunohistochemical findings. The treatment is complex and non-consensual. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results. Systemic medication with peroxisome proliferator-activated gamma receptors, statins, fenofibrate, chlorodeoxyadenosine, cyclophosphamide, doxycycline, and cyclosporine have also been reported, but none have proven particularly successful. A 59-year-old man presented with respiratory symptoms because of laryngeal involvement of XD. We had to remove the obstructive lesion for relieving the symptoms. We experienced XD in Larynx that was rare in otorhinolaryngology. So we report this case with review of literatures.

• Archives of Craniofacial Surgery
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• v.14 no.1
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• pp.69-72
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• 2013

Lateral eyebrow mass with primary skull lesion are rare in pediatric population. Although epidermoid cyst and dermoid cyst are the most commonly encountered skull lesions in pediatric population, Langerhans cell histiocytosis (LCH) is rarely reported. We report a case of LCH arising from the lateral eyebrow with osteolytic lesion involving the frontal bone. A 5-year-old boy was presented with a hard, fixed mass in his lateral eyebrow. Contrast magnetic resonance imaging revealed inhomogeneous enhancement of the mass with direct invasion of the frontal bone and adjacent dura mater. Under general anesthesia, linear incision at the lateral eyebrow region was made. Intraoperative evaluation revealed hard, fixed and well-defined soft tissue mass. The final extirpated mass was $2.5{\times}2.4cm$ in size, and was accompanied by a $1{\times}1cm$ sized defect on the frontal bone with intact dura mater. The surgical wound was closed primarily by a layer-by-layer fashion. Histologic examination was later performed for definite diagnosis. The histologic examination revealed abnormal proliferation of Langerhans cell with granuloma formation. Radionuclide bone scan and positron emission tomography was taken and revealed free of multi-organ involvement. At 3 months after surgery, natural looking contour at the lateral eyebrow region was observed with no tumor recurrence. Differential diagnosis of the hard and fixed mass at the lateral eyebrow region affecting the primary skull lesion from pediatric population includes epidermoid cyst, dermoid cyst and LCH. Generally, brief physical examination with plain X-ray view can be performed for clinical evaluation, but for a definite diagnosis, contrast MRI may be helpful.

• Journal of Yeungnam Medical Science
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• v.22 no.2
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• pp.259-265
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• 2005

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain, polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased from 109 mOsmol/kg to 327 mOsmol/kg (300%). Brain MRI showed a thickened pituitary stalk and air bubble like lesions sized with 5cm, 7cm was shown on fifth L-spine and right hip bone at hip bone CT. CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes. The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1007-1015
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• 2002

Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes were classified into a few, some, and many according to the number of histiocytes comparing with the white cells. Results : The age of thirty patients ranged from two months to 15 years. The median age was 5.6 years with male predominance(2.3 : 1). The most frequent clinical manifestation was fever; others were respiratory symptom, hepatosplenomegaly, lymphadenopathy and skin rash(in order of frequency). Common laboratory findings were leukopenia, thrombocytopenia, anemia and abnormal liver function test. Infection was present in 30 patients; causative organisms were documented in 15 patients, and bacterial infection was more common. Epstein Barr virus was the cause of infection in four patients. Bone marrow examination showed a few(20.0%), some(75.0%) and many(30.0%) hemophagocytic histiocytes. Combination immunochemotherapy including immunoglobulin, steroids and cyclosporine were helpful in 22 out of 30 patients. The mortality rate was high in young patients who showed some to many hemophagocytic histiocytes. Conclusion : Bone marrow examinations and early detection of histiocytes will be helpful in children who have fever, hepatosplenomegaly and abnormal liver function test. Immunochemotherapy were helpful and further investigation will be needed for the detection of the relationship between the bone marrow findings and prognosis.

• Tuberculosis and Respiratory Diseases
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• v.42 no.1
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• pp.58-66
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• 1995

Background: Acute interstitial pneumonia is a relatively rare form of interstitial pneumonia, since the vast majority of interstitial pneumonia have a more chronic course. It corresponds to the lesion described by Hamman and Rich, as Hamman-Rich disease in 1944. Another name in the clinical literature is accelerated interstitial pneumonia, idiopathic acute respiratory distress syndrome (idiopathic ARDS), and the organizing stage of diffuse alveolar damage. Acute interstitial pneumonia differs from chronic interstitial pneumonia by clinical and pathologic features. Clinically, this disease is characterized by a sudden onset and a rapid course, and reversible disease. Method and Purpose: Five cases of pathologically proven acute interstitial pneumonia were retrospectively studied to define the clinical, radiologic, and pathologic features. Results: 1) The five cases ranged in age from 31 to 77 years old. The onset of illness was acute in all patients, it began with viral-like prodrome 6~40 days prior to shortness of breath, and respiratory failure eventually developed in all patients. In 2 cases, generalized skin rash was accompanied with flu-like symptoms. Etiologic agent could not be identified in any case. 2) All patients had leukocytosis and severe hypoxemia. Pulmonary function test of 3 available cases shows restrictive ventilatory defect, and one survived patient(case 5) has a complete improvement of pulmonary function after dismissal. 3) Diffuse bilateral chest infiltrates were present radiologically. Theses were the ground-glass, consolidation, and reticular densities without honeycomb fibrosis in all patients. The pathologic abnormalities were the presence of increased numbers of macrophages and the formation of hyaline membranes within alveolar spaces. There was also interstitial thickening with edema, proliferation of immature fibroblast, and hyperplasia of type II pneumocyte. In the survived patient(case5), pathologic findings were relatively early stage of acute interstitial pneumonia, such as hyaline membrane with mild interstitial fibrosis. 4) Of the 5 patients, four patients died of respiratory failure 14~90 days after onset of first symptom, and one survived and recovered in symptoms, chest X ray, and pulmonary function test Conclusion: These results emphasize that acute interstitial pneumonia is clinically, radiologically, and pathologically distinct form of interstitial pneumonia and should be separated from the group of chronic interstitial pneumonia. Further studies will be needed to evaluate the pathogenesis and the treatment of acute interstitial pneumonia.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.2
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• pp.53-60
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• 2000

Purpose : The purpose of the current study is to evaluate the functional and oncologic results of the limb salvage surgery with intramedullary nailing and cementization in malignant bone tumors of the proximal humerus. Materials and Methods : We reviewed 18 cases of limb salvage surgery of resection and reconstruction with an intramedullary nail and cement-molded humeral head for the malignant bone tumors of the proximal humerus, which performed between August, 1992 through the April, 1998. The diagnoses included the osteosarcoma in 7 patients, chondrosarcoma in 3, the recurrent giant cell tumor in 3, metastatic tumor in 3, multiple myeloma in one patient and the one patient with malignant fibrous histiocytoma. The mean age at the time of surgery was 38 years(range, 15-73 years). The mean follow-up period was 26 months(range, 6-67 months). Results : Average functional score by ISOLS evaluation system was 21.1(70.3%). There were 3 local recurrences and 5 distant metastases. There were one case of shoulder instability and one case of deep infection. The seven patients are continuously disease free state and seven patients are alive with disease. Four patients died from the disease. Conclusion : The limb salvage surgery with intramedullary nailing and cementization in the bone tumors of the proximal humerus may be considered an option for the malignant bone tumors of the proximal humerus in selected patients.