• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.1
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• pp.8-11
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• 2001

Langerhans Cell Histiocytosis (LCH) is characterized by proliferation of Langerhans cells. The clinical manifestation varies from solitary bone lesion to multi-system, life threatening disorder. The younger the patient is and the more system is involved, the worse the prognosis is. The jaw is involved $10\sim20$ percent of all LCH and it is involved usually in early stage of LCH. In this case the patient is three years old girl who suffered from pain of whole mandibular body and histological examination confirmed the diagnosis LCH. She is referred to pediatrics and managed with combined chemotherapy. Due to the possibility of recurrence, we follow up the girl and she need orthodontic and prosthodontic treatment in the future because of the loss of lower left 2nd premolar. We report this case because early recognized LCH in dental hospital result in good prognosis.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.3
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• pp.249-253
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• 2009

Langerhans cell histiocytosis(LCH), previously known as histiocytosis X, is rare, proliferative disorder in which the accumulation of pathologic Langerhans cell leads to tissue infiltration and destruction. The jaw is involved 10~20% of all LCH and most common oral symptoms are jaw swelling or a palpation mass. Most of subjects are males and disease appears to start before the age of 10 years. An 11 years old girl was referred from pediatric department. Histological examination confirmed the diagnosis LCH. She was treated intra-lesional injection of corticosteroid. The overall outcome was excellent. After a follow-up period of 20 month, the patient present with no evidence of residual disease on Panoramic view and whole body bone scan. We report this early recognized LCH case treated by corticoid injection in good progress with literature review.

• Investigative Magnetic Resonance Imaging
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• v.24 no.1
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• pp.61-65
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• 2020

Langerhans cell histiocytosis (LCH) is generally considered a childhood disease that exhibits various nonspecific clinical and radiological manifestations that mimic infection or malignancy. Here, we present a case of LCH involving the rib in an adult patient. CT and MRI revealed an expansile lytic lesion with periosteal reaction on the left 8th rib, suggesting a malignant bone tumor. Surgical resection was performed and histopathological examination was consistent with LCH. Owing to its rare occurrence in adults and nonspecific aggressive features, LCH should be included in the differential diagnosis of an aggressive-appearing rib lesion in both adults and children.

• Korean Journal of Head & Neck Oncology
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• v.37 no.2
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• pp.67-69
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• 2021

Langerhans cell histiocytosis (LCH) is commonly characterized by abnormal function and differentiation or proliferation of monocytes. In LCH, granulomatous lesions, including langerine-positive histocytes and inflammatory infiltrates, can occur to all tissues, particularly well in the bones, skin, lungs, and pituitary gland. In case of external auditory canal LCH, conductive hearing loss may occur, and the most common symptom is otorrhea. Here we present a case that 49-year-old male with external auditory canal mass. Since no invasive findings were seen in radiologic study, endoscopic transcanal excision was performed and LCH was proven by pathologic report. We present this case of external auditory canal LCH with the review of literature.

• Journal of Chest Surgery
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• v.34 no.6
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• pp.506-510
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• 2001

Langerhans\` Cell Histiocytosis(LCH) is a disease of unknown origin, characterized by abnormal proliferation of Langerhans\` cells. Previously, it has been called histiocytosis X, which included eosinophilic granuloma, Hand-Sch ller-Christian\`s disease, and Letterer-Siwe disease. Any organs or tissues such as skin, lymph nodes, bone and bone marrow can be involved. However, LCH of chest wall is rarely reported in our country. We experienced a 18 month old male child, who had osteolytic lesion involving the rib with axillary lymph node metastasis. The tumor was confirmed as LCH after surgery.

• Journal of Korean Neurosurgical Society
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• v.47 no.6
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• pp.458-460
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• 2010

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.

• Journal of Korean Neurosurgical Society
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• v.59 no.2
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• pp.165-167
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• 2016

Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.45 no.3
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• pp.167-172
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• 2019

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of dendritic cells resulting in local or systemic symptoms. The clinical symptoms of patients with Langerhans cell histiocytosis depend on the site and the degree of involvement. This article describes two case histories of unifocal bony Langerhans cell histiocytosis with mandibular involvement and further discusses the appropriate management of such via a review of the literature.

• The Korean Journal of Cytopathology
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• v.18 no.1
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• pp.87-91
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• 2007

Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells (LCs) found most commonly in bone, lungs, mucocutaneous structures and endocrine organs. Among different sites, unifocal disease confined to a lymph node (LN) is rare. We report a case of LCH confined to a LN in a 38-year-old male who presented with a 2 cm-sized cervical mass. The fine needle aspiration (FNA) smears of cervical LN showed high cellularity having isolated LCs with contorted nuclei and nuclear grooves mixed in multinucleated giant cells, small lymphocytes and eosinophils. Charcot-Leyden crystals were also seen, as were a few dendritic-like cells and intranuclear inclusions. Confirmation of LCH was made by histopathologic studies, positive reactions for S-100 protein and CD1a immunohistochemical staining and by the demonstration of Birbeck granules on electron microscopy. The differentials to be considered include dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy, Hodgkin's lymphoma and malignant histiocytosis. The characteristic cytomorphologic pattern of LCH in a LN FNA smear plays an important role in suggesting the diagnosis of LCH.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.524-530
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• 2007

The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades, and is still controversial. Therapy can be reduced for low risk patients, and it is possible to discriminate early the non-responding patients with risk disease who might require more intensified treatment. Current therapy of LCH recommended by the Histiocyte Society (LCH-III protocol) is activated in 2001. Hemophaocytic histiocytosis (HLH) is fatal if diagnosis is delayed and appropriate therapy is not instituted rapidly. The diagnostic criteria for HLH is revised by the Histiocyte Society for the current treatment protocol (HLH-2004) which consists of dexamethasone, etoposide, and cyclosporin in combination with intathecal methotrexate. Hematopoietic stem cell transplantation is usually necessary for the primary HLH and recurrent secondary HLH.