• Tuberculosis and Respiratory Diseases
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• v.78 no.4
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• pp.463-468
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• 2015

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.

• 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.

• Journal of Korean Dental Science
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• v.9 no.1
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• pp.35-41
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• 2016

Langerhans cell histiocytosis (LCH) is characterized by proliferation of histiocyte-like cells (Langerhans cell histiocytes) with characteristic Birbeck granules, accompanied by other inflammatory cells. Treatments of LCH include surgery, chemotherapy, and radiotherapy. One of the representative forms of chemotherapy is intralesional injection of steroids. Surgical treatment in the form of simple excision, curettage, or even ostectomy can be performed depending on the extent of involvement. Radiotherapy is suggested in case of local recurrence, or a widespread lesion. This article shows the case of repetitively recurrent LCH of a 56-year-old man who had been through surgical excision and had to have marginal mandibulectomy and radiotherapy when the disease recurred. After the first recurrence occurred, lesions involved the extensive part of the mandible causing pathologic fracture, so partial mandibular bone resection was performed from the right molar area to the left molar area followed by the excision of the surrounding infected soft tissues. The resected mandibular bone was reconstructed with a segment of fibula osteomyocutaneous free flap and overdenture prosthesis supported by osseointegrated implants.

• 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.

• 대한세포병리학회:학술대회논문집
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• 1991.06a
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• pp.13.1-13.1
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• 1991

• 대한세포병리학회:학술대회논문집
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• 1993.06a
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• pp.25-26
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• 1993

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.31 no.3
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• pp.279-282
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• 2005

Hitiocytosis X, recently termed Langergans cell histiocytosis, is a rare disease histologically characterized by an abnormal proliferation of histiocytes, and of which the pathophysiology is still ambiguous. The clinical presentation is highly varied. Typically, there is a solitary or multiple bony involvements, and less frequently, lesions are found in other organs such as the lungs, liver, lymph nodes, skin, and mucosae. Interestingly, so far, there has been no report which shows multiple developments in both soft and hard tissues. In this report, we present a case of hitiocytosis X in both mandible and lung. This may provide some information for understanding the pathophysilogy of the histiocytosis X.

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.346-353
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• 2001

Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

• The Journal of the Korean bone and joint tumor society
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• v.19 no.1
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• pp.28-32
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• 2013

Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.

• Journal of the Korean Society of Radiology
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• v.82 no.3
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• pp.756-763
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• 2021

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, multi-systemic disease primarily affecting young male adults with a history of smoking. The two patients with PLCH in our report showed relatively early and atypical radiologic presentations at initial evaluation. On chest CT, PLCH presents variable radiologic features depending on the evolutional stage of the disease. Atypical CT features of PLCH may render precise radiologic diagnosis difficult and usually require lung biopsy for a confirmation of the diagnosis. Our case review is aimed at raising the awareness of radiologists on the atypical CT features of PLCH, to help make accurate radiologic diagnosis and prevent unnecessary and invasive diagnostic procedures.