• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.866-869
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• 2005

Langerhans Cell Histiocytosis (LCH) is a pathologic proliferation and infiltration of various organs by Langerhans' cells of unknown cause. Incidence rate of one million parties 3$\∼$4 is seen in young child but the incidence is not sure in adult. Organ systems involved by LCH may include skin, ear, bone marrow, liver, spleen, lung, pituitary gland - hypothalamus and Gl tracts. In case pituitary-hypothalamus axis are involved, diabetes insipidus happened. Primary Pulmonary Langerhans Cell Histiocytosis(PLCH) with uninvolvement of other organs is rare and accompanied diabetes insipidus is more rare. There are many cases of LCH with diabetes insipidus involve such as central nervous system except lung. PLCH accompany central diabetes insipidus is only 1 case. We report a case of PLCH that accompany central diabetes insipidus with literature investigation in Department of Thoracic and Cardiovascular Surgery, Hallym University.

• Brain Tumor Research and Treatment
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• v.6 no.2
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• pp.73-77
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• 2018

Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.

• The Korean Journal of Cytopathology
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• v.2 no.2
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• pp.153-159
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• 1991

Langerhans' cell histiocytosis (LCH), known for histiocytosis X, is a clinicopathologic entity characterized by proliferation of Langerhans' cells (LCs) throughout the body including the reticuloendothelial system, bone, and skin. LCs is currently considered as a distinct type of histlocytic cells, not primarily phagocytic in nature. Recently, we could make the diagnosis on cytologic specimen in a 3 month-old-boy and a 3 year-old-boy. The cases were diagnosed on scraping smear from the skin and fine needle aspiration cytology from the lymph node, respectively. The characteristic cytologic features of Langerhans' cells were noted in the nuclei, namely eccentric, indented, elongated, and grooved nuclei. The cells also had abundant and acidophilic cytoplasm. The cytologic diagnoses were confirmed on the biopsies from the skin and lymph node, respectively.

• Journal of the Korean Society of Radiology
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• v.84 no.2
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• pp.472-476
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• 2023

Nasal bone involvement of Langerhans cell histiocytosis is rarely reported. Here we present a case of a 13-year-old boy with a palpable nasal mass. Ultrasonography revealed a hypoechoic mass on the left side of the nose. Both CT scanning and MRI showed an osteolytic mass. The lesion seen on MRI was well-defined mass with homogeneous enhancement. Histopathological examination of the resected specimen confirmed the diagnosis of LCH.

• Journal of Chest Surgery
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• v.38 no.11 s.256
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• pp.799-802
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• 2005

Langerhans cell histiocytosis (LCH) involves disorders previously referred as 'histiocytosis X' (including eosinophilic granuloma, Letterer-Siwe, and Hand-Schuller syndrome). Its clinical patterns are various and it is a basically benign tumoral condition but with a strong tendency toward dissemination and destruction. Its natural history is unpredictable. But, in solitary bone lesion, wide resection with tumor-free margin is required in order to provide the best chance for a cure. In the majority of patients LCH is a osteolytic lesion with a predilection for calvarium and is rarely seen in the skull base and the femur. LCM of rib, especially if solitary, is relatively rare. We report two rare cases of solitary LCH developed in the rib, which were successfully treated by surgical resection.

• Investigative Magnetic Resonance Imaging
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• v.13 no.1
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• pp.74-80
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• 2009

Purpose : To evaluate the characteristic MR imaging findings of Langerhans cell histiocytosis (LCH) in the skull and to compare them with those of plain radiography and computed tomography. Materials and Methods : A total of 10 lesions in 9 patients (Age range; 5-42 years, Mean age; 18, all women) with Langerhans cell histiocytosis in the skull were included in our study. Nine lesions in nine patients were histologically confirmed by surgery or fine needle aspiration biopsy. All patients performed with MRI, and plain radiography and CT scan were done in 7 patients (8 lesions). Two experienced neuroradiologists reviewed the radiological examinations independently with attention to location, size, shape and nature of the lesions in the skull and compared the extent and extension of the lesions to adjacent structures. Results : The lesions were distributed in all of the skulls without predilection site. On MRI, the masses were shown as well-enhancing soft tissue masses (10/10) mainly in diploic spaces (8/10) with extension to scalp (9/10) and dura mater (7/10). Dural enhancement (7/10) and thickening (4/10) were seen. The largest diameter of the soft tissue masses ranged 1.1 cm to 6.8 cm, shaped as round (5/10) or oval (5/10). On CT scans, the lesions were presented as soft tissue masses involving diploic space (6/8) and scalp extension (7/8) were also well visualized. Although bony erosion or destruction was more clearly seen on CT rather than those of MRI, enhancement of soft tissue masses and dura were not well visualized on CT. In contrast, all of the lesions in LCH were seen as punched out (4/8), beveled-edge appearance (4/8) osteolytic masses in plain radiography, but scalp and dural extension could not be seen. Conclusion : Characteristic MR findings in patients with LCH are soft tissue mass in diploic space with extension to dura and scalp, and MRI would be better imaging modality than plain radiography or CT.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.14-21
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• 2014

Purpose: To analyze the clinical features and treatment outcome of Langerhans' cell histocytosis. Materials and Methods: From August 1996 to June 2013, 28 patients who histologically proven with LCH were analyzed of medical records, radiography, pathologic character retrospectively. Results: A total of 28 cases of LCH including 22 child has been reported. Onset age was 0.6 to 51 years old, occurred in the average age was 14.8 years. Follow-up period was 6 months to 134 months average was 44.6 months. The M:F ratio was 2.5:1. The initial symptoms was pain in 18 cases, 5 cases of pathologic fracture, 3 case of palpable mass, 1 case of discovered by accident in radiography, 1 case of torticollis. In radiological examination osteolysis was seen all cases, 7 cases showed a periosteal reaction, 1 case showed soft tissue extension. Clinical type of all cases were eosinophilic granuloma. 25 cases were classified as unifocal disease and 3 cases were multifocal single systemic diseases. In all cases, incisional biopsy was performed. After histologic confirmed, 14 cases was treated with curettage or surgical excision of the lesion and the other 14 cases were followed up without treatment. There is no death during follow up period. 11 cases has no radiological improvement after 3-6 months observation, intralesional steroid injection was performed. Conclusion: Patients with LCH who has rapid systemic onset is very rare, so if you meet the young children who suspected LCH, you shoulder avoid the examination which cause excessive radiation exposure to the young patient. In order to confirm the diagnosis of disease, biopsy is needed. Close observation after confirmed by histological method will bring the satisfactory results. But the patients who had pathologic fracture or wide bone destruction already may need curettage and bone grafting to lesion or internal fixation. The lesion which has no radiological improvement after 3-6 months observation or appear with pain interferes daily life may need local steroid injection as a good treatment.