• Korean Journal of Head & Neck Oncology
• /
• v.29 no.1
• /
• pp.18-21
• /
• 2013

랑게르한스 세포 조직구증은 골수에서 유래하는 랑게르한스 세포 조직구의 이상 증식에 의해 발병하는 희귀한 질병으로 알려져 있다. 비록 모든 장기에서 발생 할 수 있으나 갑상선을 침범하는 경우는 매우 드물다. 18세 남자가 5달전부터 점점 커지는 갑상선 종괴를 주소로 내원하여 세침흡인 세포검사, 총샘검, 경부 전산화단층촬영을 시행하였다. 세침흡인 세포검사에서 악성신생물이 의심되었고, 총생검에서 랑게르한스 세포 조직구증으로 나타났다. 경부 전산화단층촬영에서는 우측 갑상선에서 윤곽이 잘 구분되는 저음영의 종괴와 우측 기관 주위 림프절의 종대가 관찰되었다. 갑상선 전절제술과 우측 중앙 선택적 경부 림프절 청소술이 시행되었다. 랑게르한스 세포 조직구증이 갑상선을 침범하는 경우는 드물지만 갑상선 비대가 있는 환자가 뇌하수체 기능부전의 증상이나 뼈와 폐의 침범과 관련된 증상을 호소한다면 갑상선의 랑게르한스 세포 조직구증 침범을 고려해야 한다. 또한, 다른 장기의 랑게르한스 세포 조직구증을 치료한 과거력이 있는 경우는 갑상선 종괴를 감별 진단하는데 있어 랑게르한스 세포 조직구증을 고려해야 한다.

• Pediatric Infection and Vaccine
• /
• v.16 no.2
• /
• pp.220-223
• /
• 2009

Langerhans cell histiocytosis is a rare disease in children. However, Langerhans cell histiocytosis encompasses a wide spectrum of clinical presentations and mimics other conditions. A 1-year-old boy presented with signs of periorbital cellulitis that initially responded to antibiotics, but remained as a same-sized mass with serial orbital computed tomography. The lesion was partially excised. Histopathology and immunohistochemical staining confirmed the diagnosis of Langerhans cell histiocytosis. This case demonstrates that in patients with periorbital cellulitis which has relapsed or responded inadequately to antibiotics, further investigation should initiated to rule out other inflammatory causes.

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

• The Journal of the Korean bone and joint tumor society
• /
• v.19 no.2
• /
• pp.78-82
• /
• 2013

Langerhans cell histiocytosis is known as one of the diseases related to excessive proliferation of normal monocytes and has the variety of clinical courses and treatment. Especially, in cases with the spine, it shows a feature of single or multiple osteolysis. According to the location, disease progression and concomitant symptom, variety of treatments (observation, radiotherapy, chemotherapy, surgery, etc.) have been attempted, however, appropriate treatment has not been established yet. The authors introduce the case of single system Langerhans cell histiocytosis which involves cervical and lumbar vertebrae simultaneously with bone marrow destruction and pathologic fracture.

• Journal of the Korean Society of Radiology
• /
• v.84 no.2
• /
• pp.472-476
• /
• 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 the Korean Society of Radiology
• /
• v.82 no.4
• /
• pp.936-942
• /
• 2021

Langerhans cell histiocytosis (LCH) is a rare condition that usually occurs in children and commonly affects the skeletal system. It is extremely rare in adults, especially in the clavicles. In this report, we describe a pathologically confirmed case of LCH in the clavicle of a 50-year-old male. We report various radiological findings, such as plain radiography, CT, MR, and PET-CT, along with a review of the literature.

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

• Korean Journal of Head & Neck Oncology
• /
• v.33 no.2
• /
• pp.81-84
• /
• 2017

Langerhans cell histiocytosis (LCH) is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal Langerhans cells. It is a rare entity that may involve various organ levels such as the skeletal, pulmonary, hematopoietic and lympho-vascular systems. The patient was a 1-year-old female presented with fever associated with otorrhea and palpable cervical lymph node for 4 days. Neck ultrasonography and Computed tomography imaging revealed multiple enlarged lymph nodes suggesting suspicious malignant morphology. Lymph node biopsy was performed under general anesthesia. Histological and immunophenotypic examination showed the lymph node to be consistent with LCH. The patient was given chemotherapy.

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

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.4 no.1
• /
• pp.120-124
• /
• 2001

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.