Langerhans Cell Histiocytosis of the Orbit: Five Clinicopathologic Cases and Review of the Literature

Herwig, Martina C.; Wojno, Ted H.; Zhang, Qing; Grossniklaus, Hans E.

doi:10.1016/j.survophthal.2012.09.004

Cited by 79 publications

(93 citation statements)

References 68 publications

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“…Pathologically, LCH is characterized by multinucleated Langerhans' cells, histiocytes and eosinophils, despite the hallmark cell of LCH being Langerhans' cell histiocyte (16). The gold standard for the diagnosis of the disease requires the presence of Birbeck granules on an electron microscopic examination; however, Birbeck granules are not exclusively observed in this disease, since they have already been identified in other inflammatory conditions of the lymph nodes (16). However, in certain cases, immunohistochemistry can be fundamental in estab lishing a diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Langerhans' cell histiocytosis of the temporal bone: A case report

Yang

2017

Experimental and Therapeutic Medicine

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Abstract. Langerhansmporal histiocytosis (LCH) refers to a group of diseases that are characterized by the primary pathogenesis of an abnormal polyclonal proliferation of Langerhans cells that affect different structures of the human body, including the temporal bone. Thus far, the etiology of LCH remains unclear. Diagnosis of LCH is based on a synthetic analysis of the clinical presen tations, as well as on features of imaging and histopathology. The clinical features, diagnosis, treatment and prognosis of LCH remain obscure, and temporal LCH is often confused with ear inflammatory lesions and malignant tumors. There are several therapeutic modalities for temporal bone LCH that include surgery, chemotherapy, radiotherapy and steroidal injections. The present study reports the case of an infant presenting a 1-month history of worsening left facial paralysis and a slowly enlarging post auricular mass followed by a 1-month history of postauricular swelling in the left ear. Computed tomography demonstrated a large mass of organized tissue. Moreover, the diagnosis of LCH was confirmed by histopathological and immunohistochemical examinations. The patient also suffered from multiple organ failure, including the liver, kidney, lymphatic system, skin, hematopoietic system and lungs. Following surgical intervention with mastoidotympanectomy, the parents of the patient refused further chemotherapy, and the patient succumbed to the disease ~6 months later.

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Section: Discussionmentioning

confidence: 99%

Langerhans' cell histiocytosis of the temporal bone: A case report

Yang

2017

Experimental and Therapeutic Medicine

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“…It manifests as a unisystem or multisystem disease, and pathogenesis may be related to immune dysfunction. 9,10 Bones, skin, and lymph nodes are most commonly affected. 9,11 The clinical symptoms of T. marneffei infection in HIV-negative patients (enlarged lymph nodes, osteolytic lesions, and pulmonary lesions) and pathological findings (suppuration and granuloma formation) are similar to those of LCH, tuberculosis or nontuberculous mycobacterial infection, histoplasmosis, and allergic granulomatous vasculitis, leading to a risk of missed diagnosis or misdiagnosis, especially by inexperienced clinicians, when both diseases coexist.…”

Section: Discussionmentioning

confidence: 99%

“…LCH should be immunohistochemically confirmed by characteristic Birbeck granules (X bodies) and positivity for S100 protein and CD1a. 10 Talaromyces marneffei associated with pleural effusion has been reported in HIV-negative individuals; however, microbial or pathological evidence for its causative role was lacking, and multiple nodules on the pleura caused by T. marneffei have not been reported. The most common causes for pleural effusion in adults are heart failure, malignancy, pneumonia, tuberculosis, and pulmonary embolism.…”

Section: Discussionmentioning

confidence: 99%

“…Recommended treatments for adult LCH include surgical curettage or resection, irradiation, local or systemic (e.g., prednisone) chemotherapy, which may have deleterious effects on immune function. 10,14,15 However, systemic chemotherapy may lead to spreading of the infection, hampering its control. 8 The use of chemotherapy thus remains controversial.…”

Section: Discussionmentioning

confidence: 99%

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Two Unusual Cases of Human Immunodeficiency Virus–Negative Patients with Talaromyces marneffei Infection

Qiu

Pan

Zhang

et al. 2016

The American Society of Tropical Medicine and Hygiene

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Abstract. Talaromyces marneffei (formerly known as Penicillium marneffei) is a dimorphic fungus endemic in south and southeast Asia. It is not only commonly found in human immunodeficiency virus (HIV)-infected patients, but also among HIV-negative immunocompromised patients. The infection caused by this pathogen can disseminate hematogenously to other locations. Herein, we report for the first time two cases complicated with a rare disease or involving a rare site: in the first case, T. marneffei infection was complicated by Langerhans cell histiocytosis, whereas the second case showed clear etiological evidence of pleural nodules and pleural effusion caused by T. marneffei and diagnosed by thoracoscopic pleural biopsy.

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“…Although the etiology of LCH has not been fully elucidated, the gold standard for the diagnosis of LCH is the presence of Birbeck granules, and positivity for CD1a, S-100 and/or CD45 on pathological examination (1,2). LCH may occur at any age, although it is more common in children, and has various clinical manifestations, depending on the type and number of systems or organs involved throughout the body (3).…”

Section: Introductionmentioning

confidence: 99%

Bone Langerhans cell histiocytosis with pulmonary involvement in an adult non-smoker: A case report and brief review of the literature

Shen

Feng

2016

Molecular and Clinical Oncology

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Abstract. Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause, which encompasses a set of disorders of multiple organs with various clinical presentations that share the common characteristic of sizeable Langerhans cell infiltration. The clinical spectrum of LCH ranges from solitary bone lesions to involvement of other organs. Lung involvement in LCH may be part of a multisystem disease, which almost exclusively occurs in adult smokers, while it is overlooked or misdiagnosed in the majority of non-smokers. High-resolution computed tomography (HRCT) of the chest is crucial for diagnosis; however, the treatment and prognosis of this disease have not been clearly determined. We herein present the case of a non-smoking adult patient who presented with lower limb pain and was diagnosed via biopsy with LCH with multisystem involvement, including the bone and lungs. Lytic lesions in the corpus of the sacroiliac joint, sacrum, acetabulum and femoral head by a soft tissue mass were observed on diagnostic CT. In addition, chest HRCT revealed multiple cysts in the bilateral lungs, predominantly in the upper lobes. The final diagnosis of LCH was confirmed by histopathological examination and immunohistochemical staining for CD1a and S-100. Corticosteroid treatment alleviated lower limb pain and improved the patient's quality of life; thus, corticosteroids may be considered as a potential treatment option for patients with LCH. IntroductionLangerhans cell histiocytosis (LCH) is a rare disease of unknown etiology characterized by mixed cellular infiltration. Although the etiology of LCH has not been fully elucidated, the gold standard for the diagnosis of LCH is the presence of Birbeck granules, and positivity for CD1a, S-100 and/or CD45 on pathological examination (1,2). LCH may occur at any age, although it is more common in children, and has various clinical manifestations, depending on the type and number of systems or organs involved throughout the body (3).Specific acute symptoms may include local pain, weight loss, fatigue, fever, skin rash and neurological changes, while the bone and lung are the most commonly involved organs (4). It has been reported that the proportion of LCH with lung involvement in adults is higher compared with that in children (4). Furthermore, the disease is self-limited in the majority of pediatric patients, which is not the case in adults. A variety of factors have been implicated in the prognosis of LCH, such as patient age and extent of the disease. Several therapeutic approaches may be considered, including surgery, radiotherapy and chemotherapy; however, there is currently no standard treatment for LCH patients with multisystem involvement (MS-LCH). Case reportA 38-year-old male patient with a 2-year history of left leg pain, involving numbness extending from the left thigh to the knee, was referred to Nanjing Chest Hospital, Medical School of Southeast University. The pain and numbness in the left thigh were aggravated by exertion, such as walking or running, and they ...

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Langerhans Cell Histiocytosis of the Orbit: Five Clinicopathologic Cases and Review of the Literature

Cited by 79 publications

References 68 publications

Langerhans' cell histiocytosis of the temporal bone: A case report

Langerhans' cell histiocytosis of the temporal bone: A case report

Two Unusual Cases of Human Immunodeficiency Virus–Negative Patients with Talaromyces marneffei Infection

Bone Langerhans cell histiocytosis with pulmonary involvement in an adult non-smoker: A case report and brief review of the literature

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