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Sample records for cell histiocytosis treatment

  1. Langerhans cell histiocytosis (histiocytosis X).

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    Lam, K Y

    1997-01-01

    There has been a renewed interest in Langerhans cell histiocytosis in recent years due both to advances in basic research and to improvements in diagnostic and treatment approaches. In this article, we review the various aspects of the disease and the potential implications of these recent scientific researches for our understanding and management of the disorder.

  2. Histiocytosis

    Science.gov (United States)

    Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease

  3. Pulmonary langerhans cell histiocytosis: PET/CT for initial workup and treatment response evaluation.

    Science.gov (United States)

    Hansen, Neil J; Hankins, Jordan H

    2015-02-01

    A 40-year-old man underwent pan-endoscopy owing to abdominal pain. Biopsies of the gastrointestinal tract demonstrated diffuse Langerhans cell histiocytosis. PET/CT was done, with CT demonstrating classic pulmonary manifestations of Langerhans cell histiocytosis that had association with intense FDG uptake on PET. Bowel appeared normal. Treatment was initiated with smoking cessation and 6 cycles of cytarabine. Follow-up PET/CT after initial treatment demonstrated improvement of parenchymal abnormalities seen on CT, with resolution of hypermetabolic activity. Maintenance chemotherapy was initiated. PET/CT is increasingly being used for initial staging and treatment response assessment in this rare disorder. PMID:24999688

  4. Langerhans cell histiocytosis

    OpenAIRE

    Xiao-ling YAN

    2014-01-01

    A case report and literature review. We present a boy with a multisystemic presentation of Langerhans cell histiocytosis and severe pulmonary lesion.Skin lesion helped to suspect and confirm diagnosis by scalpbiopsy. Chemotherapy was important for favorable result.

  5. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement

    International Nuclear Information System (INIS)

    Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a ''button sequestrum.'' Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement. (orig.)

  6. Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review

    OpenAIRE

    Strehl, Johanna D; Stachel, Klaus-Daniel; Hartmann, Arndt; Agaimy, Abbas

    2012-01-01

    The synchronous or metachronous development of Langerhans cell histiocytosis and non-Langerhans cell histiocytosis in the same patient is rare. To date, only seven cases of xanthogranulomas developing in young patients with a history of Langerhans cell histiocytosis and systemic therapy have been reported in the literature. As of yet, the pathogenesis and the clinical significance of this phenomenon are unclear. We report the case of a 3 year old boy who developed juvenile Xanthogranulomas on...

  7. Langerhans cell histiocytosis

    OpenAIRE

    Aruna, D. R.; G Pushpalatha; Sushma Galgali; Prashanthy,

    2011-01-01

    Langerhans cell histiocytosis (LCH) is a group of rare disorders histologically characterized by the proliferation of Langerhans cells. Multiple organs and systems may be involved by the disease. Typically, there is bone involvement and, less frequently, lesions may be found in the lungs, liver, lymph nodes, skin, and mucosa. Oral soft tissue lesions without bone involvement are rare. We present a case of oral lesions associated with LCH in a young woman.

  8. Pulmonary langerhans cell histiocytosis

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    Suri Harpreet S; Yi Eunhee S; Nowakowski Gregorz S; Vassallo Robert

    2012-01-01

    Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events r...

  9. Langerhans Cell Histiocytosis in Otorhinolaryngology

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    Fernandes, Humberto de Barros

    2009-12-01

    Full Text Available Introduction: The Langerhans cell histiocytosis is an inflammatory cells proliferative disorder of unknown etiology. It is uncommon disease in children. Objective: To proceed with a literature review on Langerhans cell histiocytosis, with focus on the otorhinolaryngological complications. Materials and methods: The methodology used was advised based on online data from MEDLINE, between 1966 and 2008, with research of terms related to Langerhans cell histiocytosis, temporal bones and otorhinolaryngology. Literature Review: The manifestations in the head and neck are the most common ones and their diagnosis becomes difficult once it mimetizing other more common diseases the otorhinolaryngologist sees as external ear eczema, acute mastoiditis and gingivitis. The temporal bone disease manifests as recurrent otorrhea and external auditory meatus and retroauricular granulomas. The radiological evaluation confirms lytic lesions especially in the cranial cap, jaw, temporal bones and spines. The definitive diagnosis is made by biopsy through the histopathological discoveries and immunohistochemistry detection of the CD1a antigen. The main form of treatment is by chemotherapy and, in a lower scale, radiotherapy or surgery. Conclusions: he otorhinolaryngological manifestations must be suspected for recurrent otological symptoms and the presence of retroauricular granulation tissue or and the external auditory meatus. The biopsy with characteristic histological discoveries and immunohistochemistry positive for CD1a were diagnostic. The chemotherapy may be the initial treatment in most cases or secondary in refractory or recurrent forms.

  10. [Pulmonary Langerhans cell histiocytosis].

    Science.gov (United States)

    Popper, H H

    2015-09-01

    Pulmonary Langerhans cell histiocytosis is regarded as a reactive proliferation of the dendritic Langerhans cell population stimulated by chronic tobacco-derived plant proteins due to incomplete combustion but can also occur in childhood as a tumor-like systemic disease. Currently, both these forms cannot be morphologically distinguished. In the lungs a nodular proliferation of Langerhans cells occurs in the bronchial mucosa and also peripherally in the alveolar septa with an accompanying infiltration by eosinophilic granulocytes and destruction of the bronchial wall. Langerhans cells can be selectively detected with antibodies against CD1a and langerin. In the reactive isolated pulmonary form, abstinence from tobacco smoking in most patients leads to regression of infiltration and improvement of symptoms. In high-resolution computed tomography (HRCT) the small star-like scars can still be detected even after complete cessation of tobacco smoking. PMID:26289803

  11. Adult Onset Langerhans’ Cell Histiocytosis

    OpenAIRE

    Rahime İnci; Hamide Sayar; Mehmet Fatih İnci; Perihan Öztürk

    2014-01-01

    Langerhans’ cell histiocytosis (LCH) is a group of diseases of unknown cause resulting from abnormal proliferation of bone marrow-originated dendritic cells called histiocytes. The incidence is between 0.5-5.4 per million. More common in childhood, it is extremely rare in adults. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease. In this article, the clinical, radiological and histopathological findings of a 51-year-old male patient...

  12. Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Arpita J Nishal

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which Langerhans′s cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry findings suggested diagnosis of LCH.

  13. Clinical characteristics and recent progress in the diagnosis and treatment for langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: To discuss the clinical characteristics, reactivation, diagnosis, treatment and prognosis of Langerhans cell histiocytosis (LCH) based on the new progress. Methods: Fifty-five such patients treated from Jan. 1974 to May 1998 were retrospectively analyzed. The patients aged from 2 to 67 years at the time of diagnosis, with a median age of 31 years, male 47, female 8. Forty patients had single system disease and the other 15 with multisystem disease. Partial or total excision and/or local radiotherapy were the main treatments. The involved irradiation field was given, with a median dose of 30 Gy. Results: The soft tissue of head and neck was the most frequently involved (63.6%), followed by the bone (23.6%). Patients ≤15 years had more bony involvement (66.7%) compared to those aged > 15 years (11.6%) ( P 15 years were 58.3% and 74.4%, respectively, (P=0.830); whereas it was 75.0% for single system disease and 60.0% for multisystem disease (P=0.130). The total reactivation for all patients was 43.6%. Two thirds of these patients developed recurrence within 5 years after treatment with 75% developing new foci. At the time of the last follow-up, 74.5% of the patients have survived without disease, while 27.6% of survivors still had active disease. Conclusions: Langerhans cell histiocytosis, not being confined to children, involves the head and neck area most commonly. The frequency of bony invasion is associated with age. Reactivation is very frequent. Patients without risk organs involvement usually carry a favorable prognosis. (authors)

  14. CASE REPORT OF LANGERHANS CELL HISTIOCYTOSIS

    OpenAIRE

    Umesh Reddy; Somaiah; Ravikanth; Navneeth Reddy; Gowthami

    2014-01-01

    : Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  15. Pulmonary Langerhans cell Histiocytosis.

    Science.gov (United States)

    Harari, S; Comel, A

    2001-10-01

    Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocytes, and their accumulation in the distal bronchioles; these cells express on their surface the CD1a and CD1c antigens, and the B7 molecule, essential for activating quiescent T lymphocytes. In its evolution the granuloma is characterized by the progressive reduction in the LC number, with the increase of fibrosis, surrounding and destroying the bronchiolar lumen; the remaining of the lumen, or the traction exerted by fibrous tissue on the adjacent alveolar spaces leads to the cyst development. Vascular involvement occurs frequently, and may explain the onset of pulmonary hypertension in advanced cases of the disease. The disease may be asymptomatic, or it may present with aspecific respiratory signs and symptoms, and has characteristic radiological findings, being included in the group of cysticaerial parenchymal alterations. The diagnosis could be suggested by the finding of a number of LC in BAL greater than 5%. Different therapies have been proposed, but it seems that the most important measure is smoking cessation. PMID:11587096

  16. Indeterminate cell histiocytosis: report of a case.

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    Alireza Ghanadan

    2014-10-01

    Full Text Available Indeterminate cell histiocytosis is a very rare disorder of histiocytes proliferation. It has both Langerhans and non-Langerhans cell histiocytosis immunophenotypic features. We described a 45-year-old man with a 2 years history of multiple yellow-brown papules and a few red nodules on his trunk and extremities. No internal involvement was detected first and after 8 months. As his lesions were asymptomatic and sparse, he didn't receive any treatment. He will be followed at regular intervals.

  17. Genetics Home Reference: Langerhans cell histiocytosis

    Science.gov (United States)

    ... Association Histio UK Histiocytosis Association of America National Organization for Rare Disorders Genetic Testing Registry (1 link) Langerhans cell histiocytosis, multifocal Scientific articles on PubMed (1 link) PubMed OMIM (1 link) ...

  18. Pulmonary langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Suri Harpreet S

    2012-03-01

    Full Text Available Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

  19. Langerhans' cell histiocytosis in an adult.

    Science.gov (United States)

    Lindelöf, B; Forslind, B; Hilliges, M; Johansson, O; Aström, L

    1991-01-01

    A woman with typical skin lesions of histiocytosis X is reported. Electron microscopic and immunohistochemical investigations revealed a large number of markedly long Birbeck Langerhans' cell granulae. During treatment with Interferon alpha -2b, the patient developed infarctus cerebri and died. PMID:1675535

  20. Adult Onset Langerhans’ Cell Histiocytosis

    Directory of Open Access Journals (Sweden)

    Rahime İnci

    2014-12-01

    Full Text Available Langerhans’ cell histiocytosis (LCH is a group of diseases of unknown cause resulting from abnormal proliferation of bone marrow-originated dendritic cells called histiocytes. The incidence is between 0.5-5.4 per million. More common in childhood, it is extremely rare in adults. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease. In this article, the clinical, radiological and histopathological findings of a 51-year-old male patient with both skin, bone and pulmonary involvement were presented and discussed with recent literature.

  1. Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis

    OpenAIRE

    Abdul Aziz, Dayang Anita; Abdul Rahman, Nur Afdzillah; Tang, Swee Fong; Abdul Latif, Hasniah; Mohd Zaki, Faizah; Mohd Annuar, Zulfiqar; Alias, Hamidah; Abdul Latiff, Zarina

    2011-01-01

    Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleuro...

  2. [Pulmonary manifestations of Langerhans cell histiocytosis].

    Science.gov (United States)

    Obert, J; Tazi, A

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies. PMID:26003197

  3. CASE REPORT OF LANGERHANS CELL HISTIOCYTOSIS

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    Umesh Reddy

    2014-08-01

    Full Text Available : Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which pathological Langerhans cells (LCs accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  4. Langerhans cell histiocytosis with multiple spinal involvement

    OpenAIRE

    Jiang, Liang; Liu, Xiao Guang; Zhong, Wo Quan; Ma, Qing Jun; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting; Liu, Zhong Jun

    2010-01-01

    To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four sof...

  5. Langerhans Cell Histiocytosis in childhood

    OpenAIRE

    MULLER, Judit

    2010-01-01

    Histiocytoses are rare and heterogeneous group of disorders in childhood. The clinical presentation of Langerhans cell histiocytosis (LCH) is highly variable, from asymptomatic to clinically significant symptoms and consequences. As it can involve nearly every organ of the body, the clinical manifestations depend on the site of the lesions, on the organs and systems involved and whether their function is affected. The most common sites of involvement in LCH are bone, skin, lymph nodes, lung, ...

  6. The histopathology of Langerhans cell histiocytosis.

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    Favara, B. E.; Jaffe, R.

    1994-01-01

    Selected aspects of the histopathology of Langerhans cell histiocytosis representing diagnostic difficulty and/or controversy are presented with emphasis on the composition of pathological lesions. Lesional cell phenotypes and the factors influencing variations are noted. Features of several skin-based histiocytic disorders, dermatopathic lymphadenopathy and Rosai-Dorfman disease are compared. Associations between Langerhans cell histiocytosis and juvenile xanthogranuloma and malignant disord...

  7. Multisystem Langerhans' cell histiocytosis with pancreatic involvement.

    OpenAIRE

    Yu, R C; Attra, A; Quinn, C M; Krausz, T; Chu, A C

    1993-01-01

    Langerhans' cell histiocytosis, a rare disorder of unknown cause affecting both children and adults, can affect many different organs and present to a wide range of medical specialties. An infant with fatal multisystem Langerhans' cell histiocytosis in whom the pancreas and the intestine were extensively affected is reported. The direct pancreatic involvement by this disease has not previously been described.

  8. Paediatric neuroradiological aspects of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Demaerel, Philippe [University Hospital K.U.Leuven, Department of Radiology, Leuven (Belgium); Gool, Stefaan van [University Hospital K.U.Leuven, Paediatric Haemato-oncology/Neuro-oncology, Leuven (Belgium)

    2008-01-15

    Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse. (orig.)

  9. Paediatric neuroradiological aspects of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse. (orig.)

  10. Localized Langerhans' cell histiocytosis of bone: treatment and follow-up in children

    International Nuclear Information System (INIS)

    We report a case of Langerhans' cell histiocytosis (LCH) involving the right scapula in a 4-year-old child. Because of progressive shoulder pain and immobility methylprednisolone was injected directly into the lesion under computed tomography (CT) guidance. Follow-up studies with magnetic resonance imaging (MRI) and ultrasound (US) revealed continuous ossification of the osteolytic lesion with healing after 6 months. Plain films and CT confirmed the healing process. We consider intralesional corticoid instillation a safe method when performed under CT guidance. For initial evaluation and follow-up, MRI and US yielded reliable results in comparison to plain films and CT, thus helping to reduce the radiation dosage in children. (orig.)

  11. Localized Langerhans` cell histiocytosis of bone: treatment and follow-up in children

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    Libicher, M. [Dept. of Diagnostic Radiology, Univ. Heidelberg (Germany); Roeren, T. [Dept. of Diagnostic Radiology, Univ. Heidelberg (Germany); Troeger, J. [Dept. of Pediatric Radiology, Univ. Heidelberg (Germany)

    1995-11-01

    We report a case of Langerhans` cell histiocytosis (LCH) involving the right scapula in a 4-year-old child. Because of progressive shoulder pain and immobility methylprednisolone was injected directly into the lesion under computed tomography (CT) guidance. Follow-up studies with magnetic resonance imaging (MRI) and ultrasound (US) revealed continuous ossification of the osteolytic lesion with healing after 6 months. Plain films and CT confirmed the healing process. We consider intralesional corticoid instillation a safe method when performed under CT guidance. For initial evaluation and follow-up, MRI and US yielded reliable results in comparison to plain films and CT, thus helping to reduce the radiation dosage in children. (orig.)

  12. Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

    International Nuclear Information System (INIS)

    Pulmonary Langerhans' cell histiocytosis is an uncommon granulomatous disorder of unknown cause. The authors retrospectively evaluated radiography and computed tomographic findings of five patients with biopsy proven pulmonary Langerhans' cell histiocytosis. The main structural abnormalities consisted of small nodules and cystic air spaces, but one cases showed only pneumothorax due to bullae rupture. Its distribution has been known predominantly in the upper lung fields, but in our cases, the lung lesions were distributed in the entire lung fields or predominently in the lower lung fields. We propose that pulmonary Langerhans' cell histiocytosis is extremely variable of its structural abnormalities and distribution

  13. [Pulmonary Langerhans cell histiocytosis in adults].

    Science.gov (United States)

    Feuillet, Séverine; Giroux-Leprieur, Bénédicte; Tazi, Abdellatif

    2010-01-01

    Pulmonary Langerhans-cell histiocytosis in adults is a rare condition of unknown etiology characterized by the accumulation of Langerhans cells organized in granulomas involving the distal bronchioles and destroying their walls. It occurs in young subjects who smoke, with frequency peaking between 20 and 40 years. High-resolution thoracic CT is essential for diagnosis; in typical forms it shows a combination of nodules, cavitary nodules, thick-walled cysts, and thin-walled cysts. Diagnostic certainty requires a surgical lung biopsy, by videothoracoscopy, but only if a specialist considers it indicated. It is difficult to predict the disease course for any given patient. A prospective multicenter cohort study currently underway should provide more information about the natural history of this disease. Management is empirical, for efficacy has not been proved for any treatment. Stopping smoking is especially important to prevent the added development of chronic obstructive pulmonary disease (COPD), cardiovascular complications, or the onset of bronchopulmonary cancer, the frequency of which appears elevated in these patients. Oral corticosteroids are used to treat disease progression, especially in the symptomatic mainly nodular forms, but their efficacy for respiratory function has not been shown. Vinblastine, the reference treatment for multisystem forms of Langerhans-cell histiocytosis, is not indicated for pulmonary involvement in adults. Better knowledge of the pathogenic mechanisms involved in this condition should eventually make it possible to develop innovative treatment strategies. The creation of the national reference center for Langerhans-cell histiocytosis has given new momentum to clinical and pathophysiologic research on this orphan disease. PMID:19959324

  14. Cerebral and brain stem Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Two patients with central nervous system manifestations of Langerhans cell histiocytosis, both with brain stem involvement, are reported. The onset of symptoms was at an age when the diagnosis might not have been considered. (orig.)

  15. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement

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    Azouz, E. Michel [University of Miami, Pediatric Radiology Section, Department of Radiology, Miami, FL (United States); Saigal, Gaurav [McGill University, Department of Medical Imaging, Quebec (Canada); Rodriguez, Maria M. [University of Miami, Department of Pathology, Miami, FL (United States); Podda, Antonello [University of Miami, Division of Pediatric Hematology/Oncology, Miami, FL (United States)

    2005-02-01

    Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a ''button sequestrum.'' Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement. (orig.)

  16. Blood Patch Pleurodesis in the Treatment of Persistant Air Leakage in Langerhans Cell Histiocytosis; A Case Report

    Directory of Open Access Journals (Sweden)

    Ayse Baccioglu

    2013-08-01

    Full Text Available Pulmonary langerhans cell histiocytosis is a rare reactive disorder with unclear pathogenesis. 16-year-old male patient complained about shortness of breath induced with exercise, non-productive cough, and intermittent chest pain for one year. Pulmonary function tests were in restrictive nature. There were multiple air cysts in lung parenchyma smaller than one cm and minimal pneumothorax on the left hemithorax in high resolution computed tomography of thorax. There was no hypoxemia in arterial blood gas analysis, and no pulmonary hypertension in echocardiography. Pulmonary langerhans cell histiocytosis was diagnosed with clinical features and typical radiographic appearance. Existence of multisystem langerhans cell histiocytosis was excluded thorough a detailed history, comprehensive physical examination, and baseline radiographic, blood and urine tests. He was recommended to quit smoking and close follow-up was planned. A tube thoracostomy was performed for left sided total pneumothorax one month later, and %u201Cblood patch%u201D pleurodesis was done due to persistent air leakage with a successful outcome. As far as we know this is the first case report of pulmonary langerhans cell histiocytosis who was treated with autologous blood pleurodesis in the literature.

  17. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids.

    Science.gov (United States)

    Leveson, James; Bourque, Jean-Marc; Lukovic, Jelena; Dar, A Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  18. Recurrence of recipient Langerhans' cell histiocytosis following bilateral lung transplantation

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    Habib, S.; Congleton, J; Carr, D; Partridge, J; Corrin, B.; Geddes, D; Banner, N.; Yacoub, M; Burke, M.

    1998-01-01

    Langerhans' cell histiocytosis may cause irreversible respiratory failure due to progressive destruction of lung parenchyma and widespread cystic change. Transplantation offers a therapeutic option. A case is described of recurrence of Langerhans' cell histiocytosis which was associated with deterioration in lung function four years following bilateral lung transplantation. Patients transplanted for Langerhans' cell histiocytosis should be followed up with this complication in min...

  19. Langerhans Cell Histiocytosis Involving Maxilla and Mandible

    Directory of Open Access Journals (Sweden)

    M. Guna Shekhar

    2009-06-01

    Full Text Available Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults. Jaws are involved in less than 10% of children with the disease while mandibular involvement in young children is uncommon and bilateral affection is very rare. The purpose of this report is to describe a unique and very rare case of simultaneous and bilateral occurrence of Langerhans cell histiocytosis in both the jaws of a four-year-old boy.

  20. Langerhans cell histiocytosis followed by Hodgkin's lymphoma.

    Science.gov (United States)

    Park, Ik Soo; Park, In Keun; Kim, Eun Kyoung; Kim, Shin; Jeon, Sang Ryong; Huh, Joo Ryung; Suh, Cheol Won

    2012-12-01

    A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma. PMID:23269889

  1. Blood Patch Pleurodesis in the Treatment of Persistant Air Leakage in Langerhans Cell Histiocytosis; A Case Report

    OpenAIRE

    Ayse Baccioglu

    2013-01-01

    Pulmonary langerhans cell histiocytosis is a rare reactive disorder with unclear pathogenesis. 16-year-old male patient complained about shortness of breath induced with exercise, non-productive cough, and intermittent chest pain for one year. Pulmonary function tests were in restrictive nature. There were multiple air cysts in lung parenchyma smaller than one cm and minimal pneumothorax on the left hemithorax in high resolution computed tomography of thorax. There was no hypoxemia in arteria...

  2. [Langerhans cell histiocytosis causing cervical myelopathy].

    Science.gov (United States)

    Doléagbénou, A K; Mukengeshay Ntalaja, J; Derraz, S; El Ouahabi, A; El Khamlichi, A

    2012-08-01

    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 corporectomy and fusion. An 8-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying upper and lower limbs motor weakness. CT scans revealed destruction of C5 body and magnetic resonance imaging showed a tumoral process at C5 with cord compression. Interbody fusion using anterior cervical plate 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 limbs. 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. PMID:22552159

  3. Langerhans cell histiocytosis of the sacrum

    OpenAIRE

    Hatem, M.A.

    2014-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  4. Langerhans cell histiocytosis of the sacrum

    Directory of Open Access Journals (Sweden)

    M.A. Hatem

    2014-01-01

    Full Text Available Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  5. Langerhans Cell Histiocytosis and Frontal Headache

    OpenAIRE

    J Gordon Millichap

    2002-01-01

    A most recent case record of the Massachusetts General Hospital involved a clinicopathological presentation of a 15-year-old boy admitted with frontal headache, right retro-orbital pain, and a retro-orbital mass that proved to be a Langerhans’ cell histiocytosis involving the right sphenoid sinus and orbit.

  6. Langerhans cell histiocytosis of the sacrum

    OpenAIRE

    Hatem, M.A.

    2015-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  7. Langerhans Cell Histiocytosis of Thyroid—A Diagnostic Dilemma

    OpenAIRE

    Pandyaraj, R. A.; Sathik Mohamed Masoodu, K.; Maniselvi, S.; S Savitha; Divya Devi, H.

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease of antigen presenting cells and involvement of thyroid is really uncommon. The thyroid if involved is usually seen in multisystem disease but isolated thyroid involvement is very rare. The diagnosis of Langerhans cell histiocytosis of the thyroid is very challenging due to its rarity and is usually misdiagnosed as benign goiters, undifferentiated carcinoma, lymphoma, etc. Management of Langerhans cell histiocytosis of the thyroid also rema...

  8. Langerhans Cell Histiocytosis in 2 Cases of Cholestasis

    OpenAIRE

    Erkan, Tülay; Kutlu, Tufan; Çokuğraş, Fügen; Ceyhan, İpek; Yıldız, İnci; Özbay, Gülşen; T.Tümay, Güngör

    1995-01-01

    Two cases of Langerhans cell histiocytosis who had multiorgan involvement with obstructive jaundice anemia and hepatosplenomegaly painful ulceration purulent exudation squamous and maculopapular rash in the skin were presented The diagnosis of Langerhans cell histiocytosis was made by skin biopsies that showed the presence of S 100 staining cells We suggest that the diagnosis of Langerhans cell histiocytosis should be considered in patients presenting with clinical and biochemical evidence of...

  9. Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Perić Predrag

    2016-01-01

    Full Text Available Introduction. Erdheim-Chester disease (ECD is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family with characteristic bilateral symmetrical long bone osteosclerosis, associated with xanthogranulomatous extras-keletal organ involvement. In ECD, central nervous system (CNS and orbital lesions are frequent, and more than half of ECD patients carry the V600E mutation of the proto-oncogene BRAF. The synchronous or metachronous development of ECD and Langerhans cell histiocytosis (LCH in the same patients is rare, and the possible connection between them is still obscure. Cladribine is a purine substrate analogue that is toxic to lymphocytes and monocytes with good hematoencephalic penetration. Case report. We presented a 23-year-old man successfully treated with cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and CNS involvement. ECD developed metachronously, 6 years after chemotherapy for multisystem LCH with complete disease remission and remaining central diabetes insipidus. During ECD treatment, the patient received 5 single-agent chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4 weeks, with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to severe neutropenia and thoracic dermatomal herpes zoster infection following the fourth course. Radiologic signs of systemic and CNS disease started to resolve 3 months after the end of chemotherapy, and CNS lesions completely resolved within 2 years after the treatment. After 12-year follow-up, there was no recurrence or appearance of new systemic or CNS xanthogranu-lomatous lesions or second malignancies. Conclusion. In accordance with our findings and recommendations provided by other authors, cladribine can be considered an effective alternative treatment for ECD, especially with CNS involvement and BRAF V600E-mutation-negative status, when interferon-α as the first-line therapy fails.

  10. Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

    OpenAIRE

    Rawlins, Frederic A.; Hull, James E.; Morgan, Julia A.; Morris, Michael J.

    2013-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but...

  11. Bronchoscopic Diagnosis of Langerhans Cell Histiocytosis and Lymphangioleiomyomatosis

    OpenAIRE

    Harari, Sergio; Torre, Olga; Cassandro, Roberto; Taveira-DaSilva, Angelo M.; Moss, Joel

    2012-01-01

    Limited data are available regarding the role of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBB) as diagnostic tools in pulmonary Langerhans’ Cell Histiocytosis (LCH) and lymphangioleiomyomatosis (LAM).

  12. Langerhans cell histiocytosis of the sternum

    OpenAIRE

    Tsuchie, Hiroyuki; Okada, Kyoji; Nagasawa, Hiroyuki; Yano, Michihiro; Nanjyo, Hiroshi; SHIMADA Yoichi

    2009-01-01

    We report a rare case of Langerhans cell histiocytosis involving the sternum. The patient was a 12-year-old girl presenting with anterior chest pain and swelling. Radiographs and computed tomography showed an osteolytic lesion in the sternum. Technetium bone scintigraphy revealed increased uptakes in the sternum, the greater trochanter of the right femur, and the right distal tibia. Incisional biopsy for the sternum lesion was performed, and the histopathologic diagnosis was Langerhans cell h...

  13. Multisystem langerhans cell histiocytosis in adult

    OpenAIRE

    Anubhav Garg; Pramod Kumar

    2012-01-01

    Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy sh...

  14. Langerhans cell histiocytosis in sequential discordant lymphoma

    OpenAIRE

    Adu-Poku, K; Thomas, D. W.; Khan, M. K.; Holgate, C S; Smith, M E F

    2005-01-01

    B cell non-Hodgkin lymphoma of the follicular subtype (grade 3/3) affecting the nasopharynx and breast, and containing foci of Langerhans cell histiocytosis, was diagnosed in a 56 year old white woman who was a longstanding heavy smoker. Four years before this she had developed stage 1a mixed cellularity Hodgkin lymphoma affecting the right inguinal region, which was treated by irradiation and chemotherapy without recurrence. Review of the original Hodgkin lymphoma histology demonstrated a sm...

  15. Langerhans Cell Histiocytosis Followed by Hodgkin's Lymphoma

    OpenAIRE

    Park, IK Soo; Park, In Keun; Kim, Eun Kyoung; Kim, Shin; Jeon, Sang Ryong; Huh, Joo Ryung; Suh, Cheol Won

    2012-01-01

    A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurren...

  16. Indeterminate cell histiocytosis successfully treated with phototherapy.

    Science.gov (United States)

    Zerbini, Maria Claudia Nogueira; Sotto, Mirian Nacagami; de Campos, Fernando Peixoto Ferraz; Abdo, Andre Neder Ramires; Pereira, Juliana; Sanches, José Antônio; Martins, Jade Cury

    2016-01-01

    First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome. PMID:27547741

  17. Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

    Directory of Open Access Journals (Sweden)

    Frederic A. Rawlins

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.

  18. Photodynamic therapy for multi-resistant cutaneous Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Arjen F. Nikkels

    2010-06-01

    Full Text Available Langerhans cell histiocytosis is a rare group of proliferative disorders. Beside cutaneous involvement, other internal organs can be affected. The treatment of cutaneous lesions is difficult and relies on topical corticosteroids, carmustine, nitrogen mustard, and photochemotherapy. Systemic steroids and vinblastine are used for recalcitrant skin lesions. However, some cases fail to respond. An 18-month old boy presented a CD1a+, S100a+ Langerhans cell histocytosis with cutaneous and severe scalp involvement. Topical corticosteroids and nitrogen mustard failed to improve the skin lesions. Systemic corticosteroids and vinblastine improved the truncal involvement but had no effect on the scalp lesions. Methyl-aminolevulinate (MAL based photodynamic therapy (PDT resulted in a significant regression of the scalp lesions. Control histology revealed an almost complete clearance of the tumor infiltrate. Clinical follow-up after six months showed no recurrence. Although spontaneous regression of cutaneous Langerhans cell histiocytosis is observed, the rapid effect of photodynamic therapy after several failures of other treatment suggests that photodynamic therapy was successful. As far as we know this is the first report of photodynamic therapy for refractory skin lesions. Larger series are needed to determine whether photodynamic therapy deserves a place in the treatment of multiresistant cutaneous Langerhans cell histiocytosis.

  19. Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

    OpenAIRE

    Juvet, Stephen C; David Hwang; Downey, Gregory P.

    2010-01-01

    Pulmonary Langerhans’ cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert f...

  20. An unusual radiological presentation of pulmonary Langerhans’ cell histiocytosis

    OpenAIRE

    Burr, Lucy; Fanning, Steven; Wong, David; Bowler, Simon

    2012-01-01

    Langerhans’ cell histiocytosis is a proliferative disease of the dendritic cell lineage. It usually has a characteristic radiological presentation of apical nodules and small cysts occurring predominantly in young adult smokers. Here we report a case of multisystem Langerhans cell histiocytosis in a 75-year-old woman with unusual chest radiology.

  1. Langerhans cell histiocytosis of the atlas in an adult.

    Science.gov (United States)

    Zhong, Wo Quan; Jiang, Liang; Ma, Qing Jun; Liu, Zhong Jun; Liu, Xiao Guang; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting

    2010-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a rare disorder (approximately 1:1,500,000 inhabitants) characterized by clonal proliferation and excess accumulation of pathologic Langerhans cells causing local or systemic effects. The exact etiology of LCH is still unknown. LCH could affect patients of any age, although most present when they are children. The most frequent sites of the bony lesions are the skull, femur, mandible, pelvis and spine. A variety of treatment modalities has been reported, but there was no evidence suggesting that any one treatment was more advantageous than another. We present an adult with LCH of the atlas. A 26-year-old young man presented with a 2-month history of neck pain and stiffness. CT revealed osteolytic lesion in the left lateral mass of atlas with compression fracture. Histopathological diagnosis was Langerhans cell histiocytosis by percutaneous needle biopsy under CT guidance. The patient underwent conservative treatment, including Halo-vest immobilization and radiotherapy. At 7-year follow-up, the patient was asymptomatic except for mild motion restriction of the neck. CT revealed a significant reconstruction of the C1 lateral mass. PMID:19844749

  2. Langerhans' cell histiocytosis: possible association with malignant germ cell tumour.

    OpenAIRE

    Ng, W K; Lam, K Y; Ng, I O

    1995-01-01

    A rare case of adult onset Langerhans' cell histiocytosis associated with dysgerminoma in a 35 year old Chinese woman is reported. The patient had a history of dysgerminoma of left ovary 15 years previously and had undergone surgery followed by radiotherapy and an uneventful recovery. She presented again in March 1994, this time with a left clavicular mass, which was shown histologically to be Langerhans' cell histiocytosis. The report illustrates the probable association between the two lesi...

  3. CT findings of orbital langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Gina; Yoon, Hye Kyung; Han, Bokyung Kim; Kim, Kyeong Ah; Choo, In Wook [Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul (Korea, Republic of)

    2000-05-01

    To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n=3D5) and MR (n=3D1) imaging findings were also reviewed. Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units (HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatosplenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolved, with bone remodeling and reossification. A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification. (author)

  4. CT findings of orbital langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n=3D5) and MR (n=3D1) imaging findings were also reviewed. Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units (HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatosplenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolved, with bone remodeling and reossification. A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification. (author)

  5. Langerhans cell histiocytosis of the temporal bone in a child

    OpenAIRE

    Chakravarti, A; Rajeev Gupta; Sahni, J. K.

    2011-01-01

    Langerhans cell histiocytosis of temporal bone is a benign tumor like condition with variable clinical course. This rare clinical entity may mimic common ENT clinical condition like otitis media , mastoiditis and otitis externa . A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  6. Pulmonary histiocytosis

    Directory of Open Access Journals (Sweden)

    Milenković Branislava

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, and bone marrow. Pulmonary histiocytosis. Adult pulmonary Langerhans cell histiocytosis is a rare disorder of unknown etiology. It occurs predominantly in male smokers, with an incidence peak between 20 and 40 years of age. High-resolution computed tomography of the chest can show nodules, cavitated nodules, and thickand thin-walled cysts. The definite diagnosis of pulmonary Langerhans cell histiocytosis requires identification of Langerhans’ cell granulomas infiltrating and destroying distal bronchioles, which is usually achieved by lung biopsy at a site selected by chest high-resolution computed tomography. Treatment. Treatment options for adults have never been clarified by a clinical trial. The published literature provides minimal data on the comparative efficacy of various treatment options which include surgery/curettage, steroids, radiation, and various chemotherapy regimens. The improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans cell histiocytosis should help in the development of specific therapeutic strategies and effective treatment.

  7. Langerhans Cell Histiocytosis of the Temporal Bone.

    Science.gov (United States)

    Ginat, Daniel Thomas; Johnson, Daniel N; Cipriani, Nicole A

    2016-06-01

    Langerhans cell histiocytosis involving the temporal bone region is uncommon and can resemble malignant neoplasms on imaging due to high cellularity. Although recognizing the presence of sharp margins with beveled-edges can be helpful, tissue sampling is often necessary for confirming the diagnosis. Cytology classically demonstrates kidney-bean shaped nuclei within the Langerhans cells and immunohistochemical staining is positive for S-100, peanut agglutinin (PNA), MHC class II, CD1a, and Langerin (CD 207). These features are exemplified in this sine qua non radiology-pathology correlation article. PMID:25903273

  8. Langerhans cell histiocytosis with multiple spinal involvement.

    Science.gov (United States)

    Jiang, Liang; Liu, Xiao Guang; Zhong, Wo Quan; Ma, Qing Jun; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting; Liu, Zhong Jun

    2011-11-01

    To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four soft tissue involvement and three had posterior arch extension. Compiling data from the eight largest case series of the spinal LCH reveals that 27.2% multiple vertebrae lesions. In these 55 cases, there were 26 female and 29 male with the mean age of 7.4 years (range 0.2-37). A total of 182 vertebrae were involved including 28.0% in the cervical spine, 47.8% in thoracic and 24.2% in the lumbar spine. Extraspinal LCH lesion was documented in 54.2% cases, visceral involvement in 31.1% and vertebra plana in 50% cases. Paravertebral and epidural extension were not documented in most cases. Pathological diagnosis was achieved in 47 cases including 8 open spine biopsy. The treatment strategy varied depending on different hospitals. One patient died, two had recurrence and the others had no evidence of the disease with an average of 7.2 years (range 1-21) of follow-up. Asymptomatic spinal lesions could be simply observed with or without bracing and chemotherapy is justified for multiple lesions. Surgical decompression should be reserved for the uncommon cases in which neurologic compromise does not respond to radiotherapy or progresses too rapidly for radiotherapy. PMID:20496040

  9. Liver involvement in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. (orig.)

  10. Liver involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Wong, Adelaine; Ortiz-Neira, Clara L.; Abou Reslan, Walid; Kaura, Deepak [Alberta Children' s Hospital, Department of Diagnostic Imaging, Calgary, Alberta (Canada); Sharon, Raphael; Anderson, Ronald [Alberta Children' s Hospital, Department of Oncology, Calgary, AB (Canada); Pinto-Rojas, Alfredo [Alberta Children' s Hospital, Department of Pathology, Calgary, AB (Canada)

    2006-10-15

    Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. (orig.)

  11. Langerhans cell histiocytosis of bone: MR imaging

    International Nuclear Information System (INIS)

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies. (orig.)

  12. Rapid resolution of pulmonary Langerhans cell histiocytosis

    OpenAIRE

    Chong, Sy Giin; Samaha, Mario; Samaha, Georges; Casserly, Brian

    2013-01-01

    A 38-year-old woman with a long-term history of smoking presented to the lung cancer clinic with weight loss and recurrent lower respiratory tract infections. A diagnosis of Langerhans cell histiocytosis was made by specimen samples taken from video-assisted thoracoscopic surgery.  She was advised to stop smoking, which she adhered to. She was followed up in the respiratory clinic with pulmonary function tests every 6 months, which were all normal. Follow-up chest x-ray showed complete resolu...

  13. Langerhans cell histiocytosis in the adult.

    Science.gov (United States)

    Malpas, J S; Norton, A J

    1996-12-01

    A study of 47 well-documented patients with Langerhans cell histiocytosis (LCH) showed a slight female preponderance, with onset as late as the ninth decade. The skin was the commonest site of presentation, but pulmonary and bone involvement was frequent. Patients with single-site disease did best. The worst prognosis was seen in the elderly or those with organ dysfunction. A high incidence of associated malignant disease was seen, which could precede, be coincidental with, or occur after a diagnosis of LCH. PMID:8888814

  14. Langerhans cell histiocytosis on the penis: a case report

    OpenAIRE

    Kuroda Isao; Ashimine Satoshi; Ueno Munehisa; Hagiuda Jun; Ishizawa Keisuke; Deguchi Nobuhiro

    2006-01-01

    Abstract Background Langerhans cell histiocytosis affects mainly young children and features an accumulation of CD1a+ dendritic Langerhans cells in the bone, skin, and other organs. A few cases of Langerhans cell histiocytosis on the penis have been reported in the literature. We present a case of Langerhans cell histiocytosis on the penis and review the similar cases in the literature. Case presentation The patient was a 13-year-old boy who had a history of lymph node, femur bone, and pituit...

  15. Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jong Sung; Bang, Duk Ja; Rhim, Hyun Chul; Jeon, Seok Chol; Lee, Seung Ro; Hahm, Chang Kok [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1993-09-15

    Pulmonary Langerhans' cell histiocytosis is an uncommon granulomatous disorder of unknown cause. The authors retrospectively evaluated radiography and computed tomographic findings of five patients with biopsy proven pulmonary Langerhans' cell histiocytosis. The main structural abnormalities consisted of small nodules and cystic air spaces, but one cases showed only pneumothorax due to bullae rupture. Its distribution has been known predominantly in the upper lung fields, but in our cases, the lung lesions were distributed in the entire lung fields or predominently in the lower lung fields. We propose that pulmonary Langerhans' cell histiocytosis is extremely variable of its structural abnormalities and distribution.

  16. The Variable Presentation and Natural History of Langerhans Cell Histiocytosis

    OpenAIRE

    Buckwalter, Joseph A.; Brandser, Eric; Robinson, Robert A.

    1999-01-01

    Langerhans cell histiocytosis is not a well defined or predictable disease. Instead, it is a spectrum of disorders of unknown etiology that vary widely in presentation and natural history, but have in common the proliferation of histiocytic cells and infiltration of these cells into normal tissues. Although the lesions of Langerhans cell histiocytosis consist primarily of histiocytes, eosinophils are a prominent feature in some lesions. Lesions may develop in any tissue, but bone, skin and ly...

  17. Concomitant nodal involvement by Langerhans Cell Histiocytosis and Hodgkin Lymphoma

    OpenAIRE

    Geurten, Claire; Thiry, Albert; Jamblin, Paul; Demarche, Martine; Hoyoux, Claire

    2015-01-01

    Introduction : Langerhans cell histiocytosis is defined as a clonal neoplastic proliferation of myeloid dendritic cells that upon activation migrate from the mucosal to lymph nodes. Definitive diagnosis is made by anatomo-pathological and immunohistochemical analysis. Langerhans cell histiocytosis is rarely, yet not exceptionally, found coexisting with other malignant neoplasms, suggesting it might arise in reaction to the cytokinic secretion of malignant cells. Case : We report the case o...

  18. Langerhans cell histiocytosis: An uncommon presentation, successfully treated by thalidomide

    Directory of Open Access Journals (Sweden)

    Mohammad Shahidi-Dadras

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease and generally affects children under 15 years of age. Adult onset form and cutaneous features at presentation are uncommon. There are some options for treatment of the skin lesions of LCH such as topical and intralesional corticosteroid, nitrogen mustard, etc., which are not completely curative. Herein, we report a case of perianal LCH in a 20-year-old man with one-year history of recalcitrant well-demarcated, erythematous, and ulcerated plaque surrounding the anal orifice, with pain and difficulty in defecation that was successfully treated with thalidomide.

  19. Conventional radiology in the bony compromise of Langerhans cells Histiocytosis

    International Nuclear Information System (INIS)

    We present a descriptive study of 47 patients who attended the National Cancer Institute in Bogota, Colombia with pathological diagnosis of Langerhans cell histiocytosis. We reviewed the most frequent conventional x-ray findings

  20. Langerhans cell histiocytosis of the clavicle: a case report

    International Nuclear Information System (INIS)

    A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion. (orig.) (orig.)

  1. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    International Nuclear Information System (INIS)

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  2. Langerhans cell histiocytosis : A clinical and immunological study

    OpenAIRE

    Bernstrand, Cecilia

    2003-01-01

    Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, eosinophilic granuloma, Hand-Schüller-Christian or Letterer-Siwe disease, is a rare disease with a reported incidence in childhood of 5.4 cases per million children per year. The disease can present at any age but young children are most often affected. It is characterized by an accumulation of abnormal and clonal Langerhans cells in various organs such as the skin, bone, lymph nodes, lungs, liver, spl...

  3. Concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma.

    Science.gov (United States)

    Geurten, Claire; Thiry, Albert; Jamblin, Paul; Demarche, Martine; Hoyoux, Claire

    2015-12-01

    A 10-year-old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process. PMID:26556799

  4. Langerhans Cell Histiocytosis in Bilateral Mastoid Cavity

    Directory of Open Access Journals (Sweden)

    Kazım Bozdemir

    2013-01-01

    Full Text Available A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH. In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.

  5. An unusual presentation of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Palak Agarwal

    2014-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a relatively rare and unique disease. An incidence of 7.9% in the jaws is reported. We report a case of 9-year-old male child referred to us from dental outpatient department, who presented with a firm swelling in right lower jaw along with bilateral submandibular lymphadenopathy for 1-month. Fine-needle aspiration was done from lytic lesion in the body of mandible and multiple smears were prepared. On the basis of the clinical and cytomorphological findings, a diagnosis of LCH was suggested. The diagnosis was confirmed on histology. Thus, a high possibility of LCH should be considered in children presenting with lytic lesions in head and neck region.

  6. Defective alloantigen-presenting capacity of 'Langerhans cell histiocytosis cells'.

    OpenAIRE

    Yu, R C; Morris, J F; Pritchard, J.; Chu, T C

    1992-01-01

    The functional activity of skin cells derived from an infant who died of multisystem Langerhans cell histiocytosis (LCH) was examined. Involved and non-involved skin was obtained at postmortem examination within three hours of death; normal epidermal Langerhans cells and 'LCH cells' were separated by means of dispase digestion. The functional activity of different populations of CD1a positive cells was assessed using the conventional six day allogeneic mixed cell reaction. Compared with Lange...

  7. Pulmonary histiocytosis X after successful treatment of ovarian cancer lung metastases

    International Nuclear Information System (INIS)

    We report a case of a woman who developed a pulmonary histiocytosis X after treatment of an ovarian cancer with intrapulmonary metastases. Development of histiocytosis X seems to be coincidental and not to be therapy induced. Inspired by this case radiological findings of pulmonary histiocytosis X are presented. (orig.)

  8. Langerhans cell histiocytosis of the mandible in a six-year-old child

    Directory of Open Access Journals (Sweden)

    Ramani Pratibha

    2007-01-01

    Full Text Available Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis - solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.

  9. Multifocal Langerhans cell histiocytosis of bone: Indications for radiotherapy

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100, 000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology. (authors)

  10. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    Science.gov (United States)

    Khoddami, Maliheh; Nadji, Seyed-Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad-Reza

    2016-01-01

    Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR) method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66%) out of 30 patients and in 1 (3.3%) of 30 control samples with a P value of 1 (1.00 > 0.05) using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. PMID:27307972

  11. Langerhans cell histiocytosis with presentation as orbital disease

    Directory of Open Access Journals (Sweden)

    Ashok B Bhanage

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration with acute inflammation (1-week duration giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009 but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

  12. Diagnosis of Primary Langerhans Cell Histiocytosis of the Vulva in a Postmenopausal Woman

    OpenAIRE

    Sefa Kurt; Mehmet Tunc Canda; Aycan Kopuz; Dudu Solakoglu Kahraman; Abdullah Tasyurt

    2013-01-01

    Langerhans cell histiocytosis (LCH) is a very rare disease of female genital tract, most commonly seen in vulva and unusual in postmenopausal period. Herein, we report the 8th case of pure vulvar LCH in a postmenopausal woman. We pay attention to the differential diagnosis in postmenopausal state, features of pathologic diagnosis, and treatment options.

  13. Effects of treatment with a combination of cardiac rehabilitation and bosentan in patients with pulmonary Langerhans cell histiocytosis associated with pulmonary hypertension.

    Science.gov (United States)

    Fukuda, Yusuke; Miura, Shin-ichiro; Fujimi, Kanta; Yano, Masaya; Nishikawa, Hiroaki; Yanagisawa, Jun; Hiratsuka, Masafumi; Shiraishi, Takeshi; Iwasaki, Akinori; Saku, Keijiro

    2014-12-01

    Pulmonary hypertension (PH), which is classified as group 5 in the clinical classification of PH, is sometimes a complication of Pulmonary langerhans cell histiocytosis (PLCH), and is associated with high mortality. A 36-year-old woman had suffered from severe dyspnea 9 years previously and was diagnosed with PLCH and was on a waiting list for a lung transplant. Right heart failure had been observed and the mean pulmonary artery pressure was over 40 mmHg. The patient was diagnosed as PLCH with PH. After combined treatment with exercise rehabilitation and bosentan for 6 months, the cardiothoracic ratio, brain natriuretic peptide, and bodyweight were significantly decreased (cardiothoracic ratio from 43 to 38%, brain natriuretic peptide from 284 to10 pg/ml and bodyweight from 63 to 58 kg). Six-minute walk test also improved from 214 to 275 meters and the SF36 score for screening of depressive and anxiety disorders was improved. This is the report demonstrating the efficacy and safety of cardiac rehabilitation in combination with bosentan in a single patient with PLCH associated with PH. PMID:23897898

  14. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    Directory of Open Access Journals (Sweden)

    Bruno Araujo

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.

  15. Adult langerhans cell histiocytosis with hepatic and pulmonary involvement.

    Science.gov (United States)

    Araujo, Bruno; Costa, Francisco; Lopes, Joanne; Castro, Ricardo

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment. PMID:25977828

  16. CT manifestations of pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: To analyzes the CT manifestations of pulmonary Langerhans cell histiocytosis (PLCH). Methods: CT features of 11 patients with PLCH proved pathologically were analyzed retrospectively. Results: The main findings in 11 PLCHs were cysts and nodules. Two cases only had cysts, and 1 only had nodules, which most had cavitations. The other 8 cases showed cysts and nodules with 4 cases mainly manifested with cysts and nine mainly manifested with nodules. Two cases had pulmonary interstitial changes. One case only had cysts in the left upper lung field and 10 cases had lesions not only in the upper and middle but in the lower lung field, which 2 cases had more lesions in the lower and costo-phrenic angle field and 8 cases had less lesions in the costo-phrenic angle field. Three of these 8 cases had more lesions in the superior lobe and apical segment of lower lobe. Conclusion: CT manifestations of PLCH are helpful for the early diagnosis. (authors)

  17. Radiological signs of childhood Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    To describe the most prominent radiological sings of Langerhans cell histiocytosis (LCH) in childhood and review the general aspects of greatest interest to the general radiologist. The clinical and radiological histories of 40 children diagnosed on the basis of biopsy and/or cytology as having LCH in our center over a 16-year period were reviewed. Bone involvement was observed in 95% of patients; flat bones were affected in 95% of the cases, most frequently the cranium. Radiological changes in temporal bone were viewed in 15% of cases. There were long-bone lesions in 21% of the patients, involving, metaphyseal-diaphyseal bones in every case; multiple epiphyseal dysplasia was found in one and a purely cortical lesion in another. Vertebral involvement was detected in 18% of patients. Clinical evidence of diabetes insipidus (DI) was present in 22.5% of cases, while there were clinical or radiological signs of lung involvement in 15%. Bone involvement is the most common association reported in childhood LCH and is usually the reason for medical consultation. The flat bones, especially the cranium, are those most often affected. Diagnosis and follow-up studies are mainly based on plain radiography. DI due to hypothalamic infiltration in usually associated with normal CT scan. Lung involvement, uncommon in pediatric patients, is the major clinical and radiological difference with respect to the adult form. (Author) 33 refs

  18. Histiocytosis

    Science.gov (United States)

    ... or surgery may also be used to treat bone lesions. Other treatments may include: Antibiotics to fight infections ... caused by spreading of the tumors to the bone marrow Diabetes insipidus (a rare form of diabetes) Lung ...

  19. Sudden death of a patient with pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Nakhla, Hassan; Jumbelic, Mary I

    2005-06-01

    We report a case of sudden death due to bilateral pneumothorax in a previously healthy 16-year-old adolescent white girl. She presented with sudden onset of shortness of breath followed by loss of consciousness. Postmortem chest radiograph showed bilateral pneumothoraces. Autopsy confirmed the bilateral pneumothorax and additionally showed emphysematous changes and bullae throughout the lung tissue. Microscopic sections of the lungs showed Langerhans cell histiocytosis. To the best of our knowledge, this is the first reported case of fatal presentation of pulmonary Langerhans cell histiocytosis. PMID:15913433

  20. Complete pathological resolution of pulmonary Langerhans cell histiocytosis

    OpenAIRE

    Ninaber, Maarten; Dik, Hans; Peters, Elke

    2014-01-01

    Cigarette smoking is a major risk factor for pulmonary Langerhans cell histiocytosis (pLCH) and lung cancer. Resolution of pLCH may occur spontaneously, after smoking cessation or other interventions. However, despite clinicoradiological resolution, residual pulmonary Langerhans cells may be present and may lead to recurrent disease. We report the first case of pLCH with a complete histological resolution.

  1. Langerhans cell histiocytosis causing cervical myelopathy in a child.

    Science.gov (United States)

    Jang, Kun Soo; Jung, Youn Young; Kim, Seok Won

    2010-06-01

    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. PMID:20617093

  2. Recurrent adult-onset hypophyseal Langerhans cell histiocytosis after radiotherapy: a case report

    Directory of Open Access Journals (Sweden)

    Funk Ryan K

    2012-10-01

    Full Text Available Abstract Introduction Langerhans cell histiocytosis is a rare disease within the adult population, with very few cases reported as solitary hypophyseal lesions in adults. Of the reported cases, most have been treated successfully with surgery, radiotherapy, and/or chemotherapy. Radiotherapy has been thought to be curative at the relatively low dose of 20Gy. Here we report a case of recurrent hypophyseal Langerhans cell histiocytosis 9 months after radiotherapy with an interval period of symptomatic and radiographic response to therapy. Case presentation A 50-year-old Caucasian woman who had headaches, memory difficulties, and diabetes insipidus was found to have a 2.5cm suprasellar mass. Langerhans cell histiocytosis was diagnosed following stereotactic brain biopsy. Further workup revealed no other lesions. Initial radiation treatment succeeded in shrinking the tumor and relieving clinical symptoms temporarily; however, growth and recurrence of clinical symptoms was noted at 9 months. Re-irradiation was well tolerated and the patient had no acute side effects. Conclusion Isolated hypophyseal involvement by Langerhans cell histiocytosis in adults is a unique presentation of a rare disease. Although radiotherapy doses as low as 20Gy have been reported to offer control, this case demonstrates that higher doses may be warranted to ensure tumor control. With modern imaging and radiotherapy techniques higher doses should offer little increased more durable risk to surrounding critical structures.

  3. Langerhans cell histiocytosis with involvement of the pons: case report

    International Nuclear Information System (INIS)

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.)

  4. Langerhans cell histiocytosis of the clavicle: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Verbist, B.; Geusens, E.; Brys, P.; Verslegers, I.; Baert, A.L. [Department of Radiology, University Hospitals, Leuven (Belgium); Samson, I. [Department of Orthopedic Surgery, University Hospitals, Leuven (Belgium); Sciot, R. [Department of Pathology II, University Hospitals, Leuven (Belgium)

    1998-10-01

    A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion. (orig.) (orig.) With 3 figs., 7 refs.

  5. Thallium-201 uptake in Langerhans` cell histiocytosis of bone

    Energy Technology Data Exchange (ETDEWEB)

    Bar-Sever, Z. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Connolly, L.P. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Jaramillo, D. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Treves, S.T. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States)

    1996-10-01

    A case in which there was thallium-201 ({sup 201}Tl) uptake in Langerhans` cell histiocytosis (LCH) of the left femoral diaphysis is presented. The authors propose that {sup 201}Tl scintigraphy is potentially useful in the diagnosis and follow-up of patients with LCH. (orig.). With 4 figs.

  6. Langerhans cell histiocytosis with multifocal sites. A case report

    International Nuclear Information System (INIS)

    The authors report a case of Langerhans cell histiocytosis in which the patient, at the time of the diagnosis, had lesions at the central nervous system, mediastinum and long bones, all detectable in simple roentgenogram. The recent reclassification of this disease is discussed together with it's radiological findings. (author)

  7. Thallium-201 uptake in Langerhans' cell histiocytosis of bone

    International Nuclear Information System (INIS)

    A case in which there was thallium-201 (201Tl) uptake in Langerhans' cell histiocytosis (LCH) of the left femoral diaphysis is presented. The authors propose that 201Tl scintigraphy is potentially useful in the diagnosis and follow-up of patients with LCH. (orig.). With 4 figs

  8. Langerhans cell histiocytosis with involvement of the pons: case report

    Energy Technology Data Exchange (ETDEWEB)

    Vourtsi, A. [Xatzopoulou, Athens (Greece)]|[Department of Radiology, University of Athens Medical School, Athens (Greece); Papadopoulos, A.; Moulopoulos, L.A.; Vlahos, L. [Department of Radiology, University of Athens Medical School, Athens (Greece); Xenellis, J. [Department of Otorhinolaryngology, University of Athens Medical School, Athens (Greece)

    1998-03-01

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.) With 2 figs., 17 refs.

  9. Oral manifestations of chronic disseminated langerhans cell histiocytosis: a case report

    OpenAIRE

    Namdar Pekiner, Filiz; BORAHAN, M.Oğuz; ÖZBAYRAK, Semih; Alatlı, Canan; Kızılyel, Gizem

    2012-01-01

    The term histiocytosis X was introduced as a collective designation for a spectrum of clinopathologic disorders characterized by proliferation of histiocyte-like cells. The distinctive histiocytic cells present in this lesion have been identified as Langerhans cells, and the condition is now designated as Langerhans cell histiocytosis. Chronic disseminated langerhans cell histiocytosis is a disease involving bone, skin, and viscera (Hand-Schüller-Christian disease). The often-cited Chronic di...

  10. Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective.

    Science.gov (United States)

    Roden, Anja C; Yi, Eunhee S

    2016-03-01

    Context .- Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway-targeted treatment might be useful for therapy-resistant patients. Objective . -To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources .- Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions . -Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases. PMID:26927717

  11. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

    OpenAIRE

    Saede Atarbashi Moghadam; Ali Lotfi; Batool Piroozhashemi; Sepideh Mokhtari

    2015-01-01

    Statement of the Problem: Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose: The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Meth...

  12. Langerhans Cell Histiocytosis Presenting as Brown Lichenoid Patches

    OpenAIRE

    Kwon, Hyuk; Lee, Jang Hyun; Kim, Sung Keun; Park, Young Lip; Lee, Jong Suk; Cho, Moon Kyun

    2009-01-01

    Langerhans cell histiocytosis (LCH) is related diseases characterized by proliferation of Langerhans cell with involvement of bone, skin, lung and other organs. LCH usually occurs in childhood and are presented as multiple small papules or eczematoid lesion mostly. We report a 50-year-old man with 3 brown lichenoid patches on left dorsal foot. He was diagnosed pulmonary LCH 5 years ago. Typical LC cells on skin lesion and CD1 complex positive staining confirm the diagnosis of LCH. We consider...

  13. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    Directory of Open Access Journals (Sweden)

    Anupam Patra

    2015-01-01

    Full Text Available Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a indicated a diagnosis of Langerhans cell histiocytosis. Clinicians should consider pulmonary Langerhans cell histiocytosis in differential diagnoses in dealing such a case.

  14. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation

    OpenAIRE

    K S Divya

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The progn...

  15. Langerhans cell histiocytosis presenting with complicated pneumonia, a case report

    OpenAIRE

    Ottink, Mark; Feijen, Simone; Rosias, Philippe; Robben, Simon; Granzen, Bernd; Heynens, Jan; Jöbsis, Rijn

    2013-01-01

    We describe a 2 ½ year old boy presenting with fever, abdominal pain and splinter haemorrhages of the nails. On further examination there were signs of pneumonia with pleural effusion. This was treated with mini-thoracotomy, drainage and intravenous antibiotics. Further diagnostic workup for underlying causes showed diffuse cystic lung disease, suggestive of Langerhans cell histiocytosis. This was confirmed on pathology specimens, which showed Langerhans cells in lung tissue, nail bed and ski...

  16. Proteome analysis of bronchoalveolar lavage in pulmonary langerhans cell histiocytosis

    OpenAIRE

    2011-01-01

    Background Pulmonary Langerhans-cell histiocytosis (PLCH) is a rare interstitial lung disease characterized by clusters of Langerhans cells, organized in granulomas, in the walls of distal bronchioles. It is a diffuse lung disease related to tobacco smoking but otherwise of unknown etiopathogenesis. Methods In this study we used a proteomic approach to analyze BAL protein composition of patients with PLCH and of healthy smoker and non-smoker controls to obtain insights into the pathogenetic m...

  17. Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

    OpenAIRE

    Kapoor, Rohit; Loizides, Anthony M; Sachdeva, Soumya; Paul, Premila

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely describe...

  18. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    OpenAIRE

    Bruno Araujo; Francisco Costa; Joanne Lopes; Ricardo Castro

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocyt...

  19. Langerhans cell histiocytosis: Current concepts in dentistry and case report

    Science.gov (United States)

    Ramos-Gutiérrez, Efraín; Alejo-González, Francisco; Ruiz-Rodríguez, Socorro; Garrocho-Rangel, José-Arturo

    2016-01-01

    Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopathological features, to describe the bucodental management provided, and to discuss special dental considerations of this disease. Key words:Children, dental management, histiocytosis, Langerhans cells. PMID:26855698

  20. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Saatci, I.; Baskan, O.; Haliloglu, M.; Aydingoz, U. [Department of Radiology, Hacettepe University Hospital, Sihhiye 06100, Ankara (Turkey)

    1999-06-01

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.) With 5 figs., 22 refs.

  1. Disseminated langerhans cell histiocytosis presenting as cholestatic jaundice.

    Science.gov (United States)

    Kapoor, Rohit; Loizides, Anthony M; Sachdeva, Soumya; Paul, Premila

    2015-02-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta. PMID:25859497

  2. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.)

  3. Langerhans cell histiocytosis involving central nervous system: a case report

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI

  4. Langerhans cell histiocytosis involving central nervous system: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Won Jin; Park, Dong Woo; Lee, Seung Ro; Hahm, Chang Kok; Ju, Kyung Bin [Hanyang University College of Medicine, Seoul (Korea, Republic of); Kim, Sung Tae [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    1997-01-01

    Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI.

  5. Langerhans Cell Histiocytosis Causing Cervical Myelopathy in a Child

    OpenAIRE

    Jang, Kun Soo; Jung, Youn Young; Kim, Seok Won

    2010-01-01

    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 tumora...

  6. Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

    OpenAIRE

    WEI, PING; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-01-01

    Abstract Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature. A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = ...

  7. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    OpenAIRE

    Mozhgan Rezaei Kanavi; Fatemeh Javadi; Mohammad-Ali Javadi; Amir Faramarzi

    2012-01-01

    Purpose: To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH) simulating a limbal papilloma. Case report: A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of "acute bullous inflammation with granulation tissue" was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral ...

  8. Langerhan′s cell histiocytosis: A single institutional experience

    OpenAIRE

    Singh Tejinder; Satheesh C; Appaji L; Aruna Kumari B; Mamatha H; Giri G; Rao Clementina

    2010-01-01

    Background: Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. Aim: We analyzed our single institutional experience of managing children with LCH. Settings and Design: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated ret...

  9. Langerhan’s cell histiocytosis: A single institutional experience

    OpenAIRE

    Singh, Tejinder; C T Satheesh; Appaji, L.; B S Aruna Kumari; Mamatha, H. S.; G V Giri; Rama Rao, Clementina

    2010-01-01

    Background: Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. Aim: We analyzed our single institutional experience of managing children with LCH. Settings and Design: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated ret...

  10. Langerhans cell histiocytosis case with dense metaphyseal band sign.

    Science.gov (United States)

    Kikkawa, Ichiro; Aihara, Toshinori; Morimoto, Akira; Watanabe, Hideaki; Furukawa, Rieko

    2013-02-01

    Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10-year-old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X-ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10-year-old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X-ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now. PMID:23409985

  11. Langerhan′s cell histiocytosis: A single institutional experience

    Directory of Open Access Journals (Sweden)

    Singh Tejinder

    2010-01-01

    Full Text Available Background: Langerhans cell histiocytosis (LCH is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. Aim: We analyzed our single institutional experience of managing children with LCH. Settings and Design: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated retrospectively. Materials and Methods: Clinicopathological features, laboratory findings, treatment modalities and long-term outcome were analyzed. Results: Children were aged between 2 months and 12 years, with a mean of 3 years. Majority of the children were below 5 years of age. Group B constituted a bulk of children. Disseminated cases were less (five patients. Liver function dysfunction was seen in four (10% children. Pulmonary interstitial infiltrates were seen in two (5% cases. Diabetes insipidus manifested in three patients. There was one death. Conclusion: A better understanding of the etiology and pathogenesis of LCH will result in more directed and efficacious treatment regimens.

  12. MR and CT findings of temporal bone langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    To describe the MRI and CT findings of temperal bone langerhans cell histiocytosis. The MRI (n=8) and CT (n=7) findings of nine lesions of temporal bone Langerhans cell histiocytosis in six children were retrospectively reviewed. Eight lesions were pathologically confirmed and one was clinically diagnosed. The findings were analyzed for bilaterality, location, lesion extent, signal intensity, the attenuation of soft tissue lesions seen at MRI or precontrast CT, enhancement pattern at MRI or CT, and the pattern of bony destruction at CT. Bilateral involvement was present in three of six patients (50%). Lesions were most frequently located in the mastoid (n=8, 89%), followed by the petrous ridge (n=6, 67%), and the squamous portion (n=3, 33%). Seven (78%) lesions extended to the ipsilateral cavernous sinus (n=3), sphenoid bone (n=3), orbit (n=2), or epidural space (n=2). The signals of the soft tissue lesions were isointense in five cases (63%) on T1-weighted images and hyperintense in six (75%) at MRI, and homogeneous in five (71%) at CT. All lesions demonstrated bony destruction without periosteal reaction and five (71%) showed ill-defined destruction, with crossing sutures. Familiarity with findings of predominant mastoid involvement, isointense or isodense soft tissue lesions seen on T1-weighted images or at precontrast CT, with relatively homogeneous enhancement at CT, and irregular bony destruction with crossing sutures may be helpful in narrowing the diagnosis of temporal bone langerhans cell histiocytosis

  13. MR and CT findings of temporal bone langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Jae Ig; Lee, Hee Jung; Kim, Heung Sik [Keimyung Univ. School of Medicine, Dongsan Medical Center, Taegu (Korea, Republic of)

    2001-11-01

    To describe the MRI and CT findings of temperal bone langerhans cell histiocytosis. The MRI (n=8) and CT (n=7) findings of nine lesions of temporal bone Langerhans cell histiocytosis in six children were retrospectively reviewed. Eight lesions were pathologically confirmed and one was clinically diagnosed. The findings were analyzed for bilaterality, location, lesion extent, signal intensity, the attenuation of soft tissue lesions seen at MRI or precontrast CT, enhancement pattern at MRI or CT, and the pattern of bony destruction at CT. Bilateral involvement was present in three of six patients (50%). Lesions were most frequently located in the mastoid (n=8, 89%), followed by the petrous ridge (n=6, 67%), and the squamous portion (n=3, 33%). Seven (78%) lesions extended to the ipsilateral cavernous sinus (n=3), sphenoid bone (n=3), orbit (n=2), or epidural space (n=2). The signals of the soft tissue lesions were isointense in five cases (63%) on T1-weighted images and hyperintense in six (75%) at MRI, and homogeneous in five (71%) at CT. All lesions demonstrated bony destruction without periosteal reaction and five (71%) showed ill-defined destruction, with crossing sutures. Familiarity with findings of predominant mastoid involvement, isointense or isodense soft tissue lesions seen on T1-weighted images or at precontrast CT, with relatively homogeneous enhancement at CT, and irregular bony destruction with crossing sutures may be helpful in narrowing the diagnosis of temporal bone langerhans cell histiocytosis.

  14. Langerhans cell histiocytosis involving the liver of a male smoker: a case report

    OpenAIRE

    Savva-Bordalo Joana; Freitas-Silva Margarida

    2008-01-01

    Abstract Introduction Langerhans' cell histiocytosis is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. Case presentation The authors report a case of Langerhans' cell histiocytosis in a 48-year-old man with multisystemic disease presentation, including liver involvement. Conclusion Hepatic involvement is an uncommon feature in this rare disease and there is no consensus on the most effective therapeutic approach.

  15. CT and MRI preliminary study of langerhans' cell histiocytosis in temporal bone

    International Nuclear Information System (INIS)

    Objective: To study the CT and MRI features of Langerhans' cell histiocytosis affecting the temporal bone. Methods: Ten cases of Langerhans' cell histiocytosis of the temporal bone proved by clinical materials and pathology were retrospectively analyzed. CT was performed in all the 10 cases. MRI was also performed in 5 cases. Results: Temporal bone involvement presented as an isolated manifestation in 5 cases and was associated with lesions of other organs in the remaining 5 cases. External auditory canal and mastoid process were involved in 10 cases, squamous and petrous parts in 8 cases, and middle ear in 7 cases on CT scans. Destruction of ossicles and bony labyrinth were detected in 2 cases, respectively. CT showed massive lytic destruction in the affected region, associated with large soft tissue masses. The lesions had irregular border and well-defined margin without osteosclerosis in 8 cases. On MR imaging, external auditory canal and mastoid process were involved in 5 cases, squamous and petrous parts in 4 cases, middle ear in 3 cases, and membranous labyrinth in 1 case. The lesions were isointense or hypointense compared to brain on T1WI and hyperintense on T2WI in 5 cases. Postcontrast CT and MR imaging scans demonstrated marked enhancement. Conclusion: CT may clearly depict the bony lesions of the temporal bone in Langerhans' cell histiocytosis, and enhance the diagnostic accuracy to a great degree. MRI may effectively define the extent in the temporal bone, especially the intracranial invasion, and aid the selection of the optimal treatment procedure. Together with the two imaging modalities, the diagnosis, treatment, and follow-up would be greatly improved in patients with the temporal bone Langerhans' cell histiocytosis

  16. Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.

    Directory of Open Access Journals (Sweden)

    Maria A Cantu

    Full Text Available BACKGROUND: There is little data on treatment of Langerhans cell histiocytosis (LCH in adults. Available data is on small numbers of patients with short follow-up times and no comparison of results from different treatment regimens. We analyzed the responses of adult LCH patients with bone lesions to three primary chemotherapy treatments to define the optimal one. METHODS AND FINDINGS: Fifty-eight adult patients with bone lesions, either as a solitary site or as a component of multisystem disease, were analyzed for disease location and response to surgery, curettage, steroids, radiation, vinblastine/prednisone, 2-Chlorodeoxyadenosine (2-CdA, or cytosine arabinoside (ARA-C. The mean age of patients was 32 years, with equal gender distribution. Twenty-nine patients had 1 lesion; 16, 2 lesions; 5, 3 lesions; and 8 had 4 or more. Most bone lesions were in the skull, spine, or jaw. Chemotherapy, surgery, curettage, or radiation, but not steroids alone, achieved improvement or resolution of lesions in a majority of patients. Comparison of the three chemotherapy regimens revealed 84% of patients treated with vinblastine/prednisone either did not respond or relapsed within a year, whereas 59% of patients treated with 2-CdA and 21% treated with ARA-C failed. Toxicity was worse with the vinblastine/prednisone group as 75% had grade 3-4 neuropathy. Grade 3-4 cytopenias occurred in 37% of the 2-CdA -treated patients and 20% of the ARA-C-treated patients. The major limitation of this study is it is retrospective and not a clinical trial. CONCLUSIONS: ARA-C is an effective and minimally toxic treatment for LCH bone lesions in adults. In contrast, vinblastine/prednisone results in poor overall responses and excessive toxicity.

  17. The role of bone scintigraphy in Langerhans' cell histiocytosis; a case report

    International Nuclear Information System (INIS)

    We present one case of bone-Langerhans cell histiocytosis in a three-year-old male child presenting osseous lesions in the skull and the femur, which are very frequent localizations in histiocytosis. Bone scintigraphy is useful for both initial staging and follow-up associated with other imaging modalities. (authors)

  18. BISPHOSPHONATES IN LANGERHANS CELL HISTIOCYTOSIS: AN INTERNATIONAL RETROSPECTIVE CASE SERIES

    Directory of Open Access Journals (Sweden)

    Deepak Chellapandian

    2016-07-01

    Full Text Available Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH, which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77% had a single system bone disease, and 3 (23% had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years. Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve  (92% achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years. One patient did not respond. No major adverse effects were reported in this series.  Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.

  19. Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Mourah, Samia; How-Kit, Alexandre; Meignin, Véronique; Gossot, Dominique; Lorillon, Gwenaël; Bugnet, Emmanuelle; Mauger, Florence; Lebbe, Celeste; Chevret, Sylvie; Tost, Jörg; Tazi, Abdellatif

    2016-06-01

    The mitogen-activated protein kinase (MAPK) pathway is constantly activated in Langerhans cell histiocytosis (LCH). Mutations of the downstream kinases BRAF and MAP2K1 mediate this activation in a subset of LCH lesions. In this study, we attempted to identify other mutations which may explain the MAPK activation in nonmutated BRAF and MAP2K1 LCH lesions.We analysed 26 pulmonary and 37 nonpulmonary LCH lesions for the presence of BRAF, MAP2K1, NRAS and KRAS mutations. Grossly normal lung tissue from 10 smoker patients was used as control. Patient spontaneous outcomes were concurrently assessed.BRAF(V600E) mutations were observed in 50% and 38% of the pulmonary and nonpulmonary LCH lesions, respectively. 40% of pulmonary LCH lesions harboured NRAS(Q61K) (/R) mutations, whereas no NRAS mutations were identified in nonpulmonary LCH biopsies or in lung tissue control. In seven out of 11 NRAS(Q61K) (/R)-mutated pulmonary LCH lesions, BRAF(V600) (E) mutations were also present. Separately genotyping each CD1a-positive area from the same pulmonary LCH lesion demonstrated that these concurrent BRAF and NRAS mutations were carried by different cell clones. NRAS(Q61K) (/R) mutations activated both the MAPK and AKT (protein kinase B) pathways. In the univariate analysis, the presence of concurrent BRAF(V600E) and NRAS(Q61K) (/R) mutations was significantly associated with patient outcome.These findings highlight the importance of NRAS genotyping of pulmonary LCH lesions because the use of BRAF inhibitors in this context may lead to paradoxical disease progression. These patients might benefit from MAPK kinase inhibitor-based treatments. PMID:27076591

  20. Extra-osseous involvement of Langerhans' cell histiocytosis in children

    International Nuclear Information System (INIS)

    The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis. (orig.)

  1. Pulmonary Langerhans' Cell Histiocytosis: Report of Six Cases

    OpenAIRE

    SEZGİ, Cengizhan; Abakay, Abdurrahman; DALLI, Ayşe; EREN, Şevval

    2013-01-01

    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare pulmonary interstitial disease. In this report between January 2004 and December 2009 treated by the Dicle University Faculty of Medicine PLCH patients records were retrospectively studied. We found six PLCH patients. Two of six were female and four male. Mean age was 28,2± 7,3. There were only lung invoment in three, lungs and the pituitary involvement in two, lung,and skin involvement in one. Diagnosis of PLCH was established by vide...

  2. A rare case of "switch on and off" multi-system Langerhans cell histiocytosis in an adult patient

    Directory of Open Access Journals (Sweden)

    Froudarakis Marios E

    2011-07-01

    Full Text Available Abstract Introduction We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a "switch on and off" mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. Case presentation The patient had first presented at the age of 20 years with a solitary lesion of the humerus which spontaneously regressed. At that time, no therapy or smoking cessation was indicated. Four years later she presented with bilateral pneumothorax and pulmonary lesions of Langerhans cell histiocytosis. She had pleurodesis for this disease-related complication, and no further systemic treatment was applied, except with regard to smoking cessation. During the follow-up period, her pulmonary lesions regressed without recurrence six years after the initial lung involvement. Conclusion This uncommon case of remission of multi-system Langerhans cell histiocytosis indicates the unpredictable evolution of the disease, raising the question of conservative management in such a patient.

  3. Langerhans cell histiocytosis: a cytokine/chemokine-mediated disorder?

    Science.gov (United States)

    Garabedian, Lara; Struyf, Sofie; Opdenakker, Ghislain; Sozzani, Silvano; Van Damme, Jo; Laureys, Geneviève

    2011-09-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by an abnormal accumulation and/or proliferation of cells with a Langerhans cell phenotype. Although no clear cause of LCH has been identified, it has been postulated that LCH might be the consequence of an immune dysregulation, causing Langerhans cells to migrate to and accumulate at various sites. Production of cytokines and chemokines is a central feature of immune regulation. Cytokines are abundantly present within LCH lesions. We review here the potential role of cytokines and chemokines in the pathogenesis of LCH. The type, distribution, and number of different cytokines released within lesions can provide clues to the possible aetiology of LCH and, ultimately, might offer therapeutic possibilities using recombinant cytokines or antagonists for this disorder. PMID:22001902

  4. Langerhans Cell Histiocytosis of the Clavicle in a 13-Year-Old Boy

    OpenAIRE

    Parikh, Shital N.; Desai, Vishal R.; Anita Gupta; Anton, Christopher G.

    2014-01-01

    Langerhans Cell Histiocytosis (LCH) is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient’s lesion was treated by curettage, bone grafting, and internal fixa...

  5. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    OpenAIRE

    Routy, B.; J. Hoang; Gruber, J

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette ...

  6. Langerhans cell histiocytosis revisited: Case report with review

    Directory of Open Access Journals (Sweden)

    Y Pavan Kumar

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist.

  7. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    Science.gov (United States)

    Kim, Sung Hyun; Choi, Moon Young

    2016-01-01

    Single-site, single-system Langerhans cell histiocytosis (LCH) of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed. PMID:26933424

  8. Langerhans cell histiocytosis of the cervical spine in an adult: a case report.

    Science.gov (United States)

    Sayhan, Salih; Altinel, Deniz; Erguden, Cenk; Kizmazoglu, Ceren; Guray, Merih; Acar, Umit

    2010-07-01

    We present a case of a 47-year-old-woman with a complaint of cervical pain with paresthetic appearance on her left arm. She was treated with analgetics. Further radiological evaluation because of the persistent pain revealed an osteolytic destruction of the fourth cervical vertebra. The patient underwent anterior cervical corpectomy with total excision of the tumor. Stabilization of the cervical spine was performed. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH) of the cervical spine. This case report presents the histopathological evaluation, diagnostic work-up and the treatment procedures because of rarity of cervical spinal LCH cases in the literature. PMID:20669118

  9. Imaging findings in Langerhans' cell histiocytosis of the liver and the spleen in an adult

    International Nuclear Information System (INIS)

    We present a case of Langerhans' cell histiocytosis (LCH) of the liver and spleen in an adult. The imaging features are different from those in the few previously reported cases of individual organ involvement by LCH. (orig.) (orig.)

  10. Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying B | Division of Cancer Prevention

    Science.gov (United States)

    DESCRIPTION (provided by applicant): Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying Biomarkers for Early Detection and Risk Assessment. This application addresses Program Announcement PA-09-197: Biomarkers for Early Detection of Hematopoietic Malignancies (R01). The overall aim of this project is to identify novel biomarkers that may be used to diagnose and treat patients with Langerhans Cell Histiocytosis (LCH). LCH occurs with similar frequency as other rare malignancies including Hodgkin's lymphoma and AML. |

  11. Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

    OpenAIRE

    Anil Gungadeen; Peter Kullar; Philip Yates

    2013-01-01

    Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis...

  12. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    OpenAIRE

    Anupam Patra; Sujit K Bhattacharyay; Arnab Maji; Abhijit Mandal

    2015-01-01

    Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a i...

  13. Langerhans cell histiocytosis in monozygotic twins with central diabetes insipidus and hypophyseal masses

    OpenAIRE

    Wei, Sung-Tai; Chen, Der-Cherng; Cho, Der-Yang; Lin, Hung-Lin

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a systemic disease mainly affecting children and young adults. It can manifest as single system disorder or multi-system involvement. When the central nervous system is involved, the hypothalamic–pituitary axis is the most common location affected. Herein we report a rare case of Langerhans cell histiocytosis in monozygotic twins both with central diabetes and hypophyseal masses. This is the first report about LCH in monozygotic twins with hypophyseal le...

  14. Unusual location of central nervous system langerhans cell histiocytosis: case report

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis

  15. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation

    Directory of Open Access Journals (Sweden)

    K S Divya

    2014-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.

  16. Intracranial manifestations of Langerhans cell histiocytosis. MR imaging

    International Nuclear Information System (INIS)

    We report on 3 patients with intracranial manifestations on Langerhans cell histiocytosis (LH). The results are correlated with histological, clinical and radiological reports on some 70 patients described in the literature as suffering from intracranial LH. Two different morphological pictures can be differentiated. First, typical infiltrates can be seen by microscopy; these can be located in every part of the brain, but are seen mainly in the region of te hypothalamus. These infiltrations are shown as space-occupying lesions with Gd-DTPA enhancement. The other manifestation is a demyelinized lesion with a sparse infiltration of Langerhans cells. These lesions, which are located mainly in the region of the nucleus dentatus of the cerebellum and the brain stem, show increased signal intensity in the T2 sequence and no Gd-DTPA enhancement on MRI. (orig.)

  17. Cytological diagnosis of Langerhans cell histiocytosis with cutaneous involvement

    Directory of Open Access Journals (Sweden)

    Sushama A Chandekar

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease affecting predominantly children. The course of the disease varies, from spontaneous resolution to a progressive multisystem disorder with organ dysfunction and potential life-threatening complications. Diagnosis of LCH is often difficult and may be delayed because of its rarity and especially so if it occurs with unusual presentation. Fine needle aspiration cytology of a 4 year old male child, a case of LCH is presented with a purpose of highlighting the characteristic cytological features. A high index of suspicion, awareness of characteristic cytological features of LCH and its differential diagnoses is necessary. This can obviate the need of biopsy and electron microscopy. Immunohistochemistry if available can be performed on cytology smear and cell block.

  18. Congenital histiocytosis X

    International Nuclear Information System (INIS)

    Congenital histiocytosis X involving multiple organs is a rare disease that causes rapid mortality in intrauterine and neanatal life. The diagnosis of histiocytosis X (Litterer-Siwe disease should be considered in a neonate with vesiculated crusting skin lesions. We present clinical, radiographic and histopathological findings in a neonate with congenital histiocytosis who died of respiratory failure due to diffuse infilteration of lungs with histiocytic cells. Congenital histiocytosis X is a rare form of Langerhans cell histiocytosis. We report on an infant with congenital histiocytosis X who died within 10 days of birth due to diffuse infiltration of multiple organ systems with Langerhans histiocytic cells. To our knowledge, this is the first case of the radiographic illustration of progressive lung involvement in an infant with histiocytosis X. (orig.)

  19. General Information about Langerhans Cell Histiocytosis (LCH)

    Science.gov (United States)

    ... such as the abdomen , the drugs mainly affect cancer cells in those areas ( regional chemotherapy ). Chemotherapy agents given by injection or by mouth are used to treat LCH. Chemotherapy agents include vinblastine , cytarabine , cladribine , and methotrexate . Nitrogen mustard is a drug that is put ...

  20. Radiotherapy of Langerhans' cell histiocytosis. Results and Implications of a national patterns-of-care study

    International Nuclear Information System (INIS)

    Purpose: This patterns-of-care study was performed to define the current clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany and to define open questions resulting from this study. Material and Methods: A standardized questionnaire was sent to 198 German radiotherapy institutions. Data about patient characteristics, stage of disease, practice and fractionation of radiotherapy, outcome of therapy, etc. were systematically evaluated. 123 of 198 institutions answered the complete questionnaire (62.1%). Results: Only 23 of the 123 institutions (18.7%) reported experience with radiotherapy of Langerhans' cell histiocytosis of adults. 18 institutions with 98 patients were evaluable. The majority of patients (72 of 98) was treated on a linear accelerator. The median single dose of radiotherapy was 2 Gy, while the median total dose was 24 Gy. 81 of 89 evaluable patients (91%) reached a local control of the treated lesion(s), 69 of those had a complete remission. Eight of 89 patients (9%) developed an in-field recurrence. 87.8% of patients experienced no acute and 97% of patients no late side effects of radiotherapy. Conclusion: Clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany is still very limited. Nevertheless, the clinical results - with high remission and local control rates - confirm the effectiveness of radiotherapy in the multidisciplinary treatment of this disease. Due to the small number of patients in this study despite higher incidence rates, the knowledge of this disease has to be multiplied in Germany. Future patients should be systematically included into a prospective radiotherapy registry. (orig.)

  1. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    Directory of Open Access Journals (Sweden)

    Mozhgan Rezaei Kanavi

    2012-01-01

    Full Text Available Purpose: To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH simulating a limbal papilloma. Case report: A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of "acute bullous inflammation with granulation tissue" was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral patch grafting. Histopathological studies of the excised lesion led to a final diagnosis of LCH. Conclusion: To the best of our knowledge, this is the second report of a rare presentation of LCH in the limbus which recurred after excision of the primary mass. The recurrent lesion was diagnosed based on histopathology and managed accordingly.

  2. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    Science.gov (United States)

    Rezaei Kanavi, Mozhgan; Javadi, Fatemeh; Javadi, Mohammad-Ali; Faramarzi, Amir

    2012-01-01

    Purpose To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH) simulating a limbal papilloma. Case report A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of “acute bullous inflammation with granulation tissue” was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral patch grafting. Histopathological studies of the excised lesion led to a final diagnosis of LCH. Conclusion To the best of our knowledge, this is the second report of a rare presentation of LCH in the limbus which recurred after excision of the primary mass. The recurrent lesion was diagnosed based on histopathology and managed accordingly. PMID:23264867

  3. Current status of gene transfer into haemopoietic progenitor cells: application to Langerhans cell histiocytosis.

    OpenAIRE

    M. Brenner

    1994-01-01

    A number of recent studies have shown that it is possible to obtain significant levels of gene transfer and expression in marrow progenitor cells and their progeny by using retroviral vectors. The data obtained from these studies and the possible applications to Langerhans cell histiocytosis (LCH) are reviewed.

  4. Pulmonary histiocytosis

    OpenAIRE

    Milenković Branislava; Bogdanović Andrija; Cvok Tijana; Mitić Javorka; Stojšić Jelena; Dudvarski-Ilić Aleksandra

    2013-01-01

    Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, ...

  5. Radiotherapy for management of Langerhans' cell histiocytosis. Two case reports and a literature review

    International Nuclear Information System (INIS)

    The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7x1.8 Gy and 7x2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required. (orig.)

  6. A Gluteal Mass Of Langerhans Cell Histiocytosis Mimicking Malignancy In A Two-Year-Old Boy: A Case Report

    OpenAIRE

    Ibrahim, Zainal Abidin; Lung, Wong Siong; Long, Pan Kok

    2009-01-01

    Langerhans cell histiocytosis is a disease primarily affects the bone. More than 50 percent of the disease occurs between the age of 1 and 15. We reported a case of a 2 year old boy who presented with a gluteal mass. Radiographic imaging showed an osteolytic lesion suspicious of malignancy. However, the histological diagnosis was Langerhans cell histiocytosis.

  7. Langerhans Cell Histiocytosis in Childhood: Review, Symptoms in the Oral Cavity, Differential Diagnosis and Report of One Case

    Directory of Open Access Journals (Sweden)

    Mohammad Shooriabi

    2016-08-01

    Full Text Available Background Langerhans cell histiocytosis (LCH is a rare disease in which monoclonal migration and proliferation of specific dendritic cells is seen. The disease primarily affects the bones and skin, but there is a possibility that involves other organs or appears as a multi-systemic disease. Case Report In oral examination of a nine-month girl, two deep wounds with a yellow membrane with approximate size of 1 × 1 cm on both sides of mandibular alveolar ridge were seen. The edges of the wounds were swollen and proliferated and redder than the surrounding mucosa. At the touch the edges of the wound were not indurated. The wound were created from the third-month and the size of wounds had become slightly larger within 6 months. According to the chronic wound and being non-responsive to various systemic and local treatments, incisional biopsy was taken from the wounds. Langerhans cell histiocytosis was confirmedhistologically and immune histochemically. Conclusion Mouth ulcers may be the only symptoms of Langerhans cell histiocytosis. Therefore, the role of dentist could be important in diagnosis of this disease.

  8. Langerhans cell histiocytosis of the cervical spine: a single institution experience in four patients.

    Science.gov (United States)

    Tan, Grace; Samson, Ignace; De Wever, Ivo; Goffin, Jan; Demaerel, Philippe; Van Gool, Stefaan W

    2004-03-01

    When Langerhans cell histiocytosis (LCH) occurs at critical sites, such as in the cervical spine, there is a substantial risk for morbidity. Therefore, reports on clinical experiences with those patients remain important. We summarize the history of four patients with unifocal LCH at the cervical spine. All four patients received a biopsy to prove the histopathological diagnosis of LCH by demonstration of CD1a+cells. They were treated with oral prednisolone. All patients recovered completely and kept a normal function of the cervical spine. No reactivation of the disease occurred with an observation time of 3.4-7.3 years. This report contributes to the clinical experience for the treatment of LCH at critical sites. PMID:15076592

  9. Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

    Directory of Open Access Journals (Sweden)

    Santos-Machado T.M.

    1999-01-01

    Full Text Available Symptomatic involvement of the gastrointestinal (GI tract as a prominent symptom in Langerhans' cell histiocytosis (LCH is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%, protein-losing enteropathy (33.3% and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.

  10. Pituitary and pulmonary abnormalities in an adult patient with Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Pulmonary histiocytosis is an uncommon interstitial lung disease, which is infrequent in adults and is part of a spectrum of disorders characterized by monoclonal proliferation and infiltration of organs by Langerhans cells. Histiocytosis X affects only the lungs in more than 85% of the cases. We report a case of a 21-years-old male patient with Diabetes Insipidus (DI) and respiratory symptoms due to a spontaneous pneumothorax with favorable clinical evolution. High-resolution CT demonstrated bilateral nodular and cystic pulmonary lesions. MRI revealed a nodular widening of the pituitary stalk. An endoscopic trans bronchial biopsy was negative for Langerhans cells. After thoracoscopy with surgical biopsy of the lung the diagnosis of Langerhans' cell histiocytosis was confirmed. (author)

  11. IL-17A is not expressed by CD207+ cells in Langerhans Cell Histiocytosis lesions

    OpenAIRE

    Allen, Carl E.; McClain, Kenneth L.

    2009-01-01

    Interleukin-17 (IL-17A) is a pro-inflammatory cytokine that has recently been implicated in pathogenesis of Langerhans Cell Histiocytosis (LCH), a potentially fatal disease characterized by lesions including CD207+ (langerin +) histiocytes. However, in this study we were unable to identify IL-17A gene expression in Langerhans cell lesions, and plasma levels of IL-17A did not correlate with disease activity. Therefore, this study does not support a central role for IL-17A in LCH pathogenesis.

  12. Unusual location of central nervous system langerhans cell histiocytosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, E. Yup; Lee, Jae Kyu; Kim, Chan Kyo; Lee, Chang Hyun; Kang, Chang Ho; Chung, Phil Wook [Armed Forces Capital Hospital, Seongnam (Korea, Republic of)

    2003-03-01

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis.

  13. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, K.-W. [Department of Pediatrics, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); McLeary, M.S. [Div. of Pediatric Radiology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Zuppan, C.W. [Dept. of Pathology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Won, D.J. [Div. of Pediatric Neurosurgery, Loma Linda University Children' s Hospital, Loma Linda, CA (United States)

    2000-05-01

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  14. 18F-Fluorodeoxyglucose PET/CT in Langerhans cell histiocytosis: spectrum of manifestations.

    Science.gov (United States)

    Agarwal, Krishan Kant; Seth, Rachna; Behra, Abhishek; Jana, Manisha; Kumar, Rakesh

    2016-04-01

    The objective of this article is to provide an illustrative tutorial highlighting the utility of 18F-FDG PET/CT imaging to detect the spectrum of manifestations in patients with Langerhans cell histiocytosis. FDG PET/CT is a powerful tool for making an early diagnosis; it allows higher diagnostic confidence with regard to lesions, measuring the extent of disease (staging) and assessing disease activity, and is consequently useful for evaluating the response to therapy in patients with Langerhans cell histiocytosis. PMID:26759026

  15. [Anesthetic Management of a Patient with Langerhans Cell Histiocytosis during Cesarean section].

    Science.gov (United States)

    Ishii, Erika; Takaenoki, Yumiko; Shizukuishi, Masaaki; Fukuda, Isao; Kazama, Tomiei

    2015-04-01

    Langerhans cell histiocytosis is a rare disease, associated with histiocyte increases, and granuloma, in various organs. About 160 patients are reported in Japan. A pregnant patient with a pulmonary Langerhans cell histiocytosis underwent cesarean section under spinal anesthesia. She had repeated pneumothorax with bilateral pulmonary cysts rapidly becoming worse during pregnancy. She was treated with continuous oxygen after 28 weeks of the pregnancy. On 34 weeks of the pregnancy, spinal anesthesia with 0.5% hyperbaric bupivacaine (2 ml) and fentanyl (25 μg) for cesarean section was performed, and provided excellent analgesia without any side-effects. PMID:26419115

  16. Radiotherapy in langerhans cell histiocytosis - a rare indication in a rare disease

    International Nuclear Information System (INIS)

    Langerhans Cell Histiocytosis (LCH) represents a rare benign disorder, previously designated as “Histiocytosis X”, “Type II Histiocytosis” or “Langerhans Cell Granulomatosis”. Clinical presentation includes osteolysis, ulcerations of skin and soft tissues but also involvement of the CNS is described. Because treatment concepts are not well defined the German Cooperative Group on Radiotherapy for Benign Diseases performed a retrospective analysis. Eight closely cooperating centres collected patients’ data of the past 45 years. As study endpoints disease free survival, recurrent disease, death and therapy related side effects were defined. A total of 80 patients with histologically proven LCH were irradiated within the past 45 years. According to the LCH classification of Greenberger et al. 37 patients had stage Ia, 21 patients stage Ib, 13 patients stage II and 9 patients stage IIIb and the median age was 29 years. The median Follow up was 54 months (range 9–134 months). A total of 39 patients had a surgical intervention and 23 patients a chemotherapy regimen. Radiation treatment was carried out with a median total dose of 15 Gy (range 3–50.4 Gy). The median single fraction was 2 Gy (range 1.8-3 Gy). Overall, 77% patients achieved a complete remission and 12.5% achieved a partial remission. The long-term control rate reached 80%. Within an actuarial overall 5-year survival of 90% no radiogenic side and late effects ≥EORTC/RTOG II° were observed. In the present study a large collective of irradiated patients was analysed. Radiotherapy (RT) is a very effective and safe treatment option and even low RT doses show sufficient local control

  17. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Jeong Hyun [Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States); Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Rivera, Andreana [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Naeini, Ramin M.; Yedururi, Sireesha; Megahead, Hatem [Baylor College of Medicine, Radiology Department, Houston, TX (United States); Bayindir, Petek [Ege Universitesi, Radiology Department, Lojmanlari (Turkey); Fuller, Gregory N. [MD Anderson Cancer Center, Pathology Department, Houston, TX (United States); Suh, Jeong Soo [Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Adesina, Adekunle M. [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Texas Children' s Cancer Center, Houston, TX (United States); Hunter, Jill V. [Texas Children' s Cancer Center, Houston, TX (United States); Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States)

    2008-05-15

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  18. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  19. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    OpenAIRE

    S. Alavi; N. Hemmati; M.T. Arzanian; Ashena, Z

    2005-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  20. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    Directory of Open Access Journals (Sweden)

    K. Earlam

    2016-01-01

    Full Text Available Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

  1. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    Science.gov (United States)

    Earlam, K.; Souza, C. A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

  2. Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Tazi, A; Montcelly, L; Bergeron, A; Valeyre, D; Battesti, J P; Hance, A J

    1998-06-01

    In most patients with pulmonary Langerhans cell histiocytosis (LCH), clinical and radiological abnormalities initially either stabilize or regress, often without treatment. Little information is available, however, concerning the subsequent evolution of disease in patients who initially follow a benign course. We describe four patients with biopsy-confirmed pulmonary LCH whose initial course was characterized by regression of parenchymal nodular lesions, but who subsequently developed one or more episodes of active disease 7 mo to 7.5 yr after their initial presentation. In each case, the subsequent episodes of active disease were characterized by the reappearance or marked increase in nodular radiographic abnormalities, whose presence was confirmed by high-resolution computed tomography (HRCT). Thus, initial regression of nodular lesions in pulmonary LCH does not preclude the reappearance of one or more episodes of active disease, and may have important consequences on the long-term prognosis of these patients. PMID:9620939

  3. Adult Langerhans cell histiocytosis presenting as metachronous colonic polyps

    Directory of Open Access Journals (Sweden)

    Aloísio Felipe-Silva

    2013-03-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease characterized by proliferation of Langerhans-type cells that express CD1a, Langerin (CD207 and S100 protein. Birbeck granules are a hallmark by ultrastructural examination. LCH presents with a wide clinical spectrum, ranging from solitary lesions of a single site (usually bone or skin to multiple or disseminated multisystemic lesions, which can lead to severe organ dysfunction. Most cases occur in children. Gastrointestinal tract involvement is rare and has been associated with systemic illness and poor prognosis especially in children under the age of 2 years. Adult gastrointestinal LCH is very rare. We report a case of a previously healthy, nonsmoking 48-year-old male who was referred for routine screening colonoscopy. Two sessile, smooth, firm and yellowish LCH polyps measuring 0.2 cm and 0.3 cm were detected in the sigmoid colon. Fifteen months later a second colonoscopy found two histologically confirmed hyperplastic polyps at the sigmoid colon. No other LCH lesions were seen. A third colonoscopy after 28 months of follow-up found a submucosal 0.5 cm infiltrated and ulcerated LCH polyp in the cecum, close to the ostium of the appendix. The patient had been asymptomatic for all this period. Imaging investigation for systemic or multiorgan disease did not find any sign of extracolonic involvement. On histology all lesions showed typical LCH features and immunohistochemical analysis showed strong and diffuse staining for CD1a and CD207. This case illustrates two distinct clinicopathologic features not previously reported in this particular clinical setting: metachronous colonic involvement and positivity for CD207.

  4. Pulmonary langerhans cell histiocytosis: case series and literature review.

    Science.gov (United States)

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-11-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature.A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH.Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking.The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  5. The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis.

    Science.gov (United States)

    Le Guennec, Loïc; Decaix, Caroline; Donadieu, Jean; Santiago-Ribeiro, Maria; Martin-Duverneuil, Nadine; Levy, Richard; Delgadillo, Daniel; Kas, Aurélie; Drier, Aurélie; Magy, Laurent; Bayen, Eleonore; Hoang-Xuan, Khe; Idbaih, Ahmed

    2014-08-01

    Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25-75; range 20-33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis. PMID:24848633

  6. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis.

    Directory of Open Access Journals (Sweden)

    Elena Sieni

    Full Text Available Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy.We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI (group 1 or specific risk factors for (diabetes insipidus, craniofacial bone lesions, but no evidence of, neurodegenerative MRI changes (group 2. All patients underwent clinical, neurophysiological and MRI studies.Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs, and five had abnormal brainstem auditory evoked potentials (BAEPs. MR spectroscopy (MRS showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV and negative predictive value (NPV for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%, 100% (69.2%-100%, 100% (73.5%-100%, and 66.7% (38.4%-88.2%, respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient.A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future therapeutic trials.

  7. Langerhans cell histiocytosis - eosinophilic granuloma in the maxillo-facial region

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) includes three entities with unknown etiology, which have common histological features where the affected tissues are infiltrated with Langerhans cells. LCH (previously known as Histiocytosis X) comprises rare diseases: Eosinophilic granuloma, Hand-Schueller-Christian syndrome, Letterer-Siwe syndrome, Hashimoto-Pritzke syndrome ect. where the accumulation of pathological Langerhans cells lead to destruction of tissues. Therefore the clinical manifestation of Langerhans cell histiocytosis varies from monoosal or polyosal disorders with unifocal or multifocal bone alteration to dessiminative forms affecting many organs and systems. Eosinophilic granuloma - the localized form of Langerhans cell histiocytosis may flow with single or multiple changes in the skeleton, without involving the internal organs. Eosinophilic granuloma in maxillo-facial region can affect each of the bones, but most often found in the mandible (approx. 70%). First clinical symptoms of EG in maxillo-facial region usually take place in the oral cavity and the bone changes are twice as frequent as oral soft tissue's changes. Sometimes oral manifestation - single or multiple bone and oral soft tissue nonspecific lesions could be the first or the only sign of this disease. That's way early symptoms of Eosinophilic granuloma in maxillo-facial region manifested in jaw area can be first recognized by dental professionals. (authors)

  8. Persistent pulmonary interstitial emphysema in a case of Langerhans cell histiocytosis

    OpenAIRE

    Pooja Abbey; Mahender K Narula; Rama Anand; Jagdish Chandra

    2014-01-01

    We present the case of a 10-month-old boy with multisystem Langerhans cell histiocytosis showing thin-walled lung cysts along with computed tomography (CT) evidence of persistent pulmonary interstitial emphysema (PPIE), in the absence of pneumothorax or pneumomediastinum. Follow-up CT performed after 6 months demonstrated complete resolution of interstitial emphysema.

  9. Langerhans cell histiocytosis forming an asymptomatic solitary nodule in the spleen.

    OpenAIRE

    Lam, K Y; Chan, A C; Wat, M. S.

    1996-01-01

    A case of solitary Langerhans cell histiocytosis (LCH) in the spleen of a 29 year old Chinese man, discovered incidentally at necropsy, is reported. This is the first documented case of LCH confined to the spleen and suggests that LCH should be included in the differential diagnosis of space occupying lesions in the spleen.

  10. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings

    Energy Technology Data Exchange (ETDEWEB)

    Kilborn, T.N.; Teh, J.; Goodman, T.R

    2003-04-01

    Langerhans cell histiocytosis is a rare disease in children. However, its ability to present in many ways, to mimic other conditions, and to manifest itself in many organs makes it a fascinating disease for radiologists. This article reviews the history of the disease, the features that are most useful in determining prognosis, and the various radiological findings seen in paediatric patients.

  11. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location

    International Nuclear Information System (INIS)

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. (orig.)

  12. Orbital manifestations of Langerhans Cell Histiocytosis: A report of three cases

    OpenAIRE

    Das, Jayanta K.; Ronel Soibam; B K Tiwary; Magdalene, D.; S B Paul; Cida Bhuyan

    2009-01-01

    Langerhans Cell Histiocytosis (LCH) is a spectrum of disorders characterized by accumulation of histiocytes in various tissues. It is rarely encountered in ophthalmic practice and has an affinity for the orbit. We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

  13. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location

    Energy Technology Data Exchange (ETDEWEB)

    Geusens, E.; Brys, P.; Ghekiere, J.; Baert, A.L. [Department of Radiology, University Hospital Gasthuisberg KU Leuven (Belgium); Samson, I. [Department of Orthopedic Surgery, University Hospitals, Leuven (Belgium); Sciot, R. [Department of Pathology II, University Hospitals, Leuven (Belgium); Brock, P. [Department of Pediatrics, University Hospitals, Leuven (Belgium)

    1998-09-01

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. (orig.) With 3 figs., 7 refs.

  14. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a rare disease in children. However, its ability to present in many ways, to mimic other conditions, and to manifest itself in many organs makes it a fascinating disease for radiologists. This article reviews the history of the disease, the features that are most useful in determining prognosis, and the various radiological findings seen in paediatric patients

  15. Imaging findings in Langerhans` cell histiocytosis of the liver and the spleen in an adult

    Energy Technology Data Exchange (ETDEWEB)

    Mampaey, S. [Dept. of Radiology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium)]|[Dept. of Radiology, University of Antwerp (Belgium); Warson, F. [Dept. of Anatomopathology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium); Hedent, E. van [Dept. of Radiology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium); Schepper, A.M. de [Dept. of Radiology, University of Antwerp (Belgium)

    1999-02-01

    We present a case of Langerhans` cell histiocytosis (LCH) of the liver and spleen in an adult. The imaging features are different from those in the few previously reported cases of individual organ involvement by LCH. (orig.) (orig.) With 2 figs., 5 refs.

  16. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location.

    Science.gov (United States)

    Geusens, E; Brys, P; Ghekiere, J; Samson, I; Sciot, R; Brock, P; Baert, A L

    1998-01-01

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. PMID:9724427

  17. Persistent pulmonary interstitial emphysema in a case of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Pooja Abbey

    2014-01-01

    Full Text Available We present the case of a 10-month-old boy with multisystem Langerhans cell histiocytosis showing thin-walled lung cysts along with computed tomography (CT evidence of persistent pulmonary interstitial emphysema (PPIE, in the absence of pneumothorax or pneumomediastinum. Follow-up CT performed after 6 months demonstrated complete resolution of interstitial emphysema.

  18. Role of Fine Needle Aspiration in the Diagnosis of the Rare Disease of Langerhans Cell Histiocytosis in a Child

    OpenAIRE

    Seema Lale; Daniel Soto; Patricia G. Wasserman

    2014-01-01

    Langerhan’s cell histiocytosis (LCH) results from the proliferation of immunophenotypically and functionally immature, morphologically rounded Langerhan’s cells along with eosinophils, macrophages, lymphocytes, and, commonly, multinucleated giant cells. Here we report a case in a 6-year-old boy of differential diagnoses including dermatopathic lymphadenitis (DL), parasitic infection, Kimura’s disease, hypersensitivity reactions, cat-scratch disease, sinus histiocytosis with massive lymphad...

  19. Immunocytochemical characterization of pulmonary histiocytosis X cells in lung biopsies.

    OpenAIRE

    Soler, P; S. Chollet(Ecole Polytechnique, IN2P3-CNRS); Jacque, C.; Y. Fukuda(Miyagi University of Education); Ferrans, V J; Basset, F.

    1985-01-01

    Morphologic and immunohistochemical studies were made of open lung biopsies from 9 patients with pulmonary histiocytosis X (HX) and 12 patients with other conditions, and of skin biopsies from patients with cutaneous sarcoidosis, Chester-Erdheim disease, and eruptive histiocytoma. The monoclonal antibody OKT6 was detected with the use of goat anti-mouse IgG labeled with fluorescein (FITC) for light microscopy, and sheep antimouse Fab'2 fragment of IgG labeled with horseradish peroxidase (HRP)...

  20. Langerhans Cell Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report

    OpenAIRE

    Lee, Jong-Sil; Ko, Gyung Hyuck; Kim, Ho Cheol; Jang, In Seok; Jeon, Kyung-Nyeo; Lee, Jeong-Hee

    2006-01-01

    Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomograph...

  1. Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis.

    Science.gov (United States)

    Sacco, Oliviero; Moscatelli, Andrea; Conte, Massimo; Grasso, Chiara; Magnano, Gian Michele; Sementa, Angela Rita; Martelli, Alberto; Rossi, Giovanni A

    2016-05-01

    Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem-LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long-term ECMO are discussed. PMID:26840616

  2. A rare case of "switch on and off" multi-system Langerhans cell histiocytosis in an adult patient

    OpenAIRE

    Froudarakis Marios E; Koutsopoulos Anastasios; Karpathiou Georgia

    2011-01-01

    Abstract Introduction We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a "switch on and off" mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. Case presentation The patient had first presented at the age of 20 years with a solitary lesion of the humerus which spontaneously regressed. At that time, no therapy ...

  3. Radiotherapy of Langerhans' cell histiocytosis. Results and Implications of a national patterns-of-care study

    Energy Technology Data Exchange (ETDEWEB)

    Seegenschmiedt, M.H. [Alfried Krupp Krankenhaus, Essen (Germany). Dept. of Radiation Oncology and Radiotherapy; Olschewski, T.

    2006-11-15

    Purpose: This patterns-of-care study was performed to define the current clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany and to define open questions resulting from this study. Material and Methods: A standardized questionnaire was sent to 198 German radiotherapy institutions. Data about patient characteristics, stage of disease, practice and fractionation of radiotherapy, outcome of therapy, etc. were systematically evaluated. 123 of 198 institutions answered the complete questionnaire (62.1%). Results: Only 23 of the 123 institutions (18.7%) reported experience with radiotherapy of Langerhans' cell histiocytosis of adults. 18 institutions with 98 patients were evaluable. The majority of patients (72 of 98) was treated on a linear accelerator. The median single dose of radiotherapy was 2 Gy, while the median total dose was 24 Gy. 81 of 89 evaluable patients (91%) reached a local control of the treated lesion(s), 69 of those had a complete remission. Eight of 89 patients (9%) developed an in-field recurrence. 87.8% of patients experienced no acute and 97% of patients no late side effects of radiotherapy. Conclusion: Clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany is still very limited. Nevertheless, the clinical results - with high remission and local control rates - confirm the effectiveness of radiotherapy in the multidisciplinary treatment of this disease. Due to the small number of patients in this study despite higher incidence rates, the knowledge of this disease has to be multiplied in Germany. Future patients should be systematically included into a prospective radiotherapy registry. (orig.)

  4. Role of multidisciplinary approach in a case of Langerhans cell histiocytosis with initial periodontal manifestations

    OpenAIRE

    A. Cisternino; F. Asa'Ad; FUSCO, N.; Ferrero, S; Rasperini, G

    2015-01-01

    Introduction: Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia of unknown etiology occurring in both children and adults. This condition is characterized by an abnormal proliferation of Langerhans cells that may virtually affect all sites in the human body. Oral manifestations of LCH could be the first clinical sign of disease and its periodontal localization could be easily mistaken for other more common entities, such as chronic periodontitis, aggressive periodon...

  5. Biliary wall calcification in Langerhans cell histiocytosis: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Caruso, Settimo; Miraglia, Roberto; Maruzzelli, Luigi; Luca, Angelo; Gridelli, Bruno [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IsMeTT), Department of Diagnostic and Interventional Radiology, Palermo (Italy)

    2008-07-15

    Langerhans cell histiocytosis (LCH) is a disorder of unknown pathogenesis affecting one or more organs (unifocal or disseminated form) due to clonal proliferation of Langerhans cells. Liver involvement is more frequent in the disseminated form and the radiological findings of end-stage liver disease due to LCH are similar to those of sclerosing cholangitis. We present the multidetector CT findings in two children with LCH liver involvement and the unique finding of calcification of the biliary wall. (orig.)

  6. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    Directory of Open Access Journals (Sweden)

    S. Alavi

    2005-07-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  7. Biliary wall calcification in Langerhans cell histiocytosis: report of two cases

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a disorder of unknown pathogenesis affecting one or more organs (unifocal or disseminated form) due to clonal proliferation of Langerhans cells. Liver involvement is more frequent in the disseminated form and the radiological findings of end-stage liver disease due to LCH are similar to those of sclerosing cholangitis. We present the multidetector CT findings in two children with LCH liver involvement and the unique finding of calcification of the biliary wall. (orig.)

  8. Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

    OpenAIRE

    Lovrenski Aleksandra; Đurić Mirna; Klem Ištvan; Eri Živka; Panjković Milana; Tegeltija Dragana; Považan Đorđe

    2013-01-01

    Introduction. Langerhans cell histiocytosis (LCH) is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal le...

  9. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    OpenAIRE

    Adam May; Garvan Kane; Eunhee Yi; Robert Frantz; Robert Vassallo

    2015-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement...

  10. Magnetic resonance imaging of thickened pituitary stalk proceeding to langerhans cell histiocytosis in a child

    International Nuclear Information System (INIS)

    Magnetic resonance imaging has shown isolated pituitary stalk thickening in certain cases of idiopathic or secondary central diabetes insipidus (Dl) due to infiltrative processes. We present a 4-year-old boy who was initially diagnosed as having central Dl. The MRI showed isolated pituitary stalk thickening with prominent homogeneous contrast enhancement. The remaining findings on MRI were within normal limits. The patient's personal and family history and laboratory and clinical findings were unremarkable; therefore, he was initially diagnosed as having idiopathic Dl. Since central Dl and isolated pituitary stalk thickening may be considered to be the first manifestations of Langerhans cell histiocytosis, we decided to follow up the patient. After 5 months, following the initial diagnosis, on skeletal X-ray survey, the patient did indeed develop multiple lytic skull lesions which, on biopsy, were histologically typical bone lesions of Langerhans cell histiocytosis. Copyright (2006) Blackwell Science Pty Ltd

  11. Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion

    International Nuclear Information System (INIS)

    Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic biopsy was performed and histological examination showed diffuse infiltrate of macrophages with foamy cytoplasm. Four months later there was recurrence of seizure activity despite anti-epileptic medication and a repeat MR scan showed a persistent enhancing lesion in the left frontal lobe. Histological examination of the resection specimen resembled juvenile xanthogranuloma (JXG) involving the central nervous system. In the absence of skin lesions a diagnosis of non-Langerhans cell histiocytosis was made. The child made a full recovery following surgery with resolution of his symptoms. (orig.)

  12. Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion

    Energy Technology Data Exchange (ETDEWEB)

    Rajaram, Smitha; Shackley, Fiona; Raghavan, Ashok [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); Wharton, Stephen B. [University of Sheffield, Department of Neurosciences, Sheffield (United Kingdom); Connolly, Daniel J.A. [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); University of Sheffield, Academic Radiology, Sheffield (United Kingdom)

    2010-12-15

    Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic biopsy was performed and histological examination showed diffuse infiltrate of macrophages with foamy cytoplasm. Four months later there was recurrence of seizure activity despite anti-epileptic medication and a repeat MR scan showed a persistent enhancing lesion in the left frontal lobe. Histological examination of the resection specimen resembled juvenile xanthogranuloma (JXG) involving the central nervous system. In the absence of skin lesions a diagnosis of non-Langerhans cell histiocytosis was made. The child made a full recovery following surgery with resolution of his symptoms. (orig.)

  13. HISTIOCYTOSIS X: CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    E. Y. Ponomareva

    2014-07-01

    Full Text Available Two clinical cases of pulmonary Langerhans cell histiocytosis X have been analyzed demonstrating lung and other inner organ pathology, common clinical and X-ray features but different life prognosis.

  14. HISTIOCYTOSIS X: CLINICAL OBSERVATIONS

    OpenAIRE

    E. Y. Ponomareva; A. P. Rebrov; E. E. Archangelskaja; A. A. Roshchina; R. N. Steshenko; A. S. Bukia; N. V. Dvornikova; N. N. Moskaleva

    2014-01-01

    Two clinical cases of pulmonary Langerhans cell histiocytosis X have been analyzed demonstrating lung and other inner organ pathology, common clinical and X-ray features but different life prognosis.

  15. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

    International Nuclear Information System (INIS)

    We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity. (orig.)

  16. Congenital solid neck mass: a unique presentation of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) presenting in the neonatal period is very rare. In most cases, a self-limited cutaneous disease is the exclusive manifestation. We report an unusual case of neonatal LCH presenting with a large congenital solid neck mass without skin lesions. LCH should be considered in the differential diagnosis of solid masses in neonates and prompt physicians to search for visceral organ involvement. (orig.)

  17. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Ernemann, U.; Skalej, M.; Voigt, K. [Department of Neuroradiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Hermisson, M.; Platten, M. [Department of Neurology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Jaffe, R. [Pathology Department, Children' s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213 (United States)

    2002-09-01

    We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity. (orig.)

  18. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    OpenAIRE

    Hesam Jahandideh; Yasser Nasoori; Sara Rostami; Mahdi Safdarian

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

  19. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    Science.gov (United States)

    Jahandideh, Hesam; Nasoori, Yasser; Rostami, Sara; Safdarian, Mahdi

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease. PMID:27127670

  20. Langerhans cell histiocytosis in adults: Contribution of PET-CT with 18F-FDG

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a rare disease whose clinical presentation is highly variable, and with unpredictable outcome. Once the diagnosis is established, evaluation of the extent of the disease is required for therapeutic purposes and prognosis. PET-CT with 18F-FDG can detect multi systemic involvement, demonstrating metabolically active lesions. We present a case report showing the utility of PET-CT in staging and therapy response evaluation

  1. Congenital solid neck mass: a unique presentation of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Tantiwongkosi, Bundhit [Northeastern Ohio Universities College of Medicine and Canton Affiliated Hospitals, Department of Radiology, Canton, OH (United States); Goske, Marilyn J. [Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Steele, Mark [Akron Children' s Hospital, Department of Pathology, Akron, OH (United States)

    2008-05-15

    Langerhans cell histiocytosis (LCH) presenting in the neonatal period is very rare. In most cases, a self-limited cutaneous disease is the exclusive manifestation. We report an unusual case of neonatal LCH presenting with a large congenital solid neck mass without skin lesions. LCH should be considered in the differential diagnosis of solid masses in neonates and prompt physicians to search for visceral organ involvement. (orig.)

  2. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

    OpenAIRE

    M. Vaziri; A. Pazooki L. Zahedi

    2008-01-01

    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was ...

  3. Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis.

    OpenAIRE

    Grobost V.; Khouatra C.; Lazor R; Cordier J.F.; Cottin V

    2014-01-01

    BackgroundPulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte proli...

  4. Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

    OpenAIRE

    Castoldi, M. C.; A. Verrioli; De Juli, E.; Vanzulli, A.

    2014-01-01

    Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are i...

  5. Intriguing bronchoalveolar lavage proteome in a case of pulmonary langerhans cell histiocytosis

    OpenAIRE

    Ghafouri, Bijar; Persson, H Lennart; Tagesson, Christer

    2013-01-01

    Background: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease associated with tobacco smoke exposure. New insights into its pathogenesis and how it differs from that of chronic obstructive pulmonary disease (COPD) may be provided by proteomic studies on bronchoalveolar lavage fluid (BALF). Case Report: We present the BALF proteome in a biopsy-proven case of PLCH and compare it with typical proteomes of COPD and of the healthy lung. The BALF proteins were separ...

  6. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    OpenAIRE

    Earlam, K.; Souza, C.A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are biz...

  7. Pulmonary Langerhans Cell Histiocytosis X Presented with Bilateral Pneumothorax: A Case Report

    OpenAIRE

    Majed Al-Mourgi

    2014-01-01

    Background: The report describes the case of a 20-year-old Saudi male patient with Pulmonary Langerhans cell histiocytosis X (PLCH). He presented with bilateral chest pain of few hours duration starting first on the right side. The patient had also dyspnea and non productive cough. The patient was heavy smoker for 6 years. General examination revealed that the patient had tachypnea but he was hemodynamically stable. Chest examination showed bilateral distant breath sounds but no hyperresonanc...

  8. Extrathoracic investigation in adult patients with isolated pulmonary langerhans cell histiocytosis

    OpenAIRE

    Tazi, Abdellatif; de Margerie-Mellon, Constance; Vercellino, Laetitia; Naccache, Jean Marc; Fry, Stéphanie; Dominique, Stéphane; Jouneau, Stéphane; Lorillon, Gwenaël; Bugnet, Emmanuelle; Chiron, Raphael; Wallaert, Benoit; Valeyre, Dominique; Chevret, Sylvie

    2016-01-01

    Background An important objective on diagnosis of patients with Langerhans cell histiocytosis (LCH) is to determine the extent of disease. However, whether systematic extrathoracic investigation is needed in adult patients with clinically isolated pulmonary LCH (PLCH) has not been evaluated. Methods In this prospective, multicentre study, 54 consecutive patients with newly diagnosed clinically isolated PLCH were systematically evaluated at inclusion by bone imaging and blood laboratory testin...

  9. Presentation of pulmonary Langerhans cell histiocytosis before the development of lung cysts

    OpenAIRE

    Martin, Irene; Ballester, Marta; Ruiz, Yolanda; Llatjós, Roger; Alarza, Fernando; Molina, Maria

    2013-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) affects mainly young, predominantly smoking adults with a peak at 20–40 years of age. Patients with PLCH often present with a nonproductive cough and/or dyspnea. High-resolution CT (HRCT) is the most important diagnostic modality in PLCH. The typical HRCT pattern combines small poorly limited nodules, cavitated nodules, and finally thick- and thin-walled cysts. In rare cases, HRCT enables PLCH to be diagnosed prior to the development of cysts.

  10. Modern concepts of pathogenesis and morphogenesis of pulmonary Langerhans’ cell histiocytosis of adults

    OpenAIRE

    Liskina, I. V.

    2013-01-01

    The article presents detailed information about modern pathogenetic mechanisms of pulmonary Langerhans’ cell histiocytosis of adults, as an isolated no neoplastic process, which  in many moments of its development is uncontrolled abnormal immune response to a number of antigens not fully qualified by nature. Morphogenesis of pathology is described in details, as well as all stages of the disease, with emphasis on diagnostic morphological signs. Characteristic diagnostic biopsy microphotograph...

  11. Clinical and Radiological Presentation of Pulmonary Langerhans Cell Histiocytosis: A Case Series of 20 Patients

    OpenAIRE

    A. Fakharian; H. Jamaati; M.O. Tahbaz; S. Khalilzadeh; Pour Abdollah, M.; M. Behkam Shadmehr

    2008-01-01

    Introduction & Objective: Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon (5% ILD) interstitial tissue disorder with significant importance. It occurs predominantly in adult smokers. The organs involved in LCH include skin, bone, pituitary gland, thyroid, lymph node, and lungs.Materials & Methods: In this descriptive study all patients admitted to the Masih Daneshvari Hospital in Tehran from 1996 to 2007 diagnosed with LCH (recognized by clinical features, laboratory investigati...

  12. Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis

    OpenAIRE

    Grobost, Vincent; Khouatra, Chahera; Lazor, Romain; Cordier, Jean-François

    2014-01-01

    BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte pro...

  13. The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study

    OpenAIRE

    Tazi, Abdellatif; de Margerie, Constance; Naccache, Jean Marc; Fry, Stéphanie; Dominique, Stéphane; Jouneau, Stéphane; Lorillon, Gwenaël; Bugnet, Emmanuelle; Chiron, Raphael; Wallaert, Benoit; Valeyre, Dominique; Chevret, Sylvie

    2015-01-01

    Background The natural history of pulmonary Langerhans cell histiocytosis (PLCH) has been unclear due to the absence of prospective studies. The rate of patients who experience an early progression of their disease is unknown. Additionally, conflicting effects of smoking cessation on the outcome of PLCH have been reported. Methods In this prospective, multicentre study, 58 consecutive patients with newly diagnosed PLCH were comprehensively evaluated over a two-year period. Our objectives were...

  14. Simultaneous Occurrence of Hodgkin's Lymphoma and Langerhans Cell Histiocytosis of the Spine : A Rare Combination

    OpenAIRE

    Hyun, Seung-Jae; Rhim, Seung-Chul; Huh, Jooryung

    2010-01-01

    Hodgkin's disease presenting with spinal cord compression owing to extradural and bone involvement is extremely unusual. A 48-year-old man presented with progressive lower extremity weakness resulting from spinal cord compression attributable to an epidural mass in the thoracic vertebrae. The patient underwent decompressive surgery, and was then treated with chemotherapy for Langerhans cell histiocytosis. However, the disease progressed, and we performed second decompressive surgery with stab...

  15. Polyclonal T-cells express CD1a in Langerhans cell histiocytosis (LCH lesions.

    Directory of Open Access Journals (Sweden)

    Jennifer A West

    Full Text Available Langerhans cell histiocytosis (LCH is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a(+/CD3(+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs. We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a(+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH.

  16. Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?

    Directory of Open Access Journals (Sweden)

    Andreas Meyer

    2012-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is a rare disease of unknown etiology with different clinical features. A standardised treatment has not been established so far. Case Report. We report a case of a 28-year-old patient who initially presented with hypesthesia of the fifth cranial nerve and pain of the left ear. Diagnosis showed a tumour localised in the cranial base with a maximum diameter of 4.1 cm. The diagnosis of LCH was confirmed histologically by biopsy. Diagnostic workup verified the cranial lesion as the sole manifestation of LCH. A total dose of 9 Gy (single dose 1.8 Gy was delivered. The symptoms dissolved completely within 6 months after radiation; repeated CT and MRI scans revealed a reduction in size of the lesion and a remineralisation of the bone. After a followup of 13 years the patient remains free of symptoms without relapse or any side effects from therapy. Discussion. Due to the indolent course of the disease with a high rate of spontaneous remissions the choice of treatment strongly depends on the individual clinical situation. In the presented case low-dose radiotherapy was sufficient to obtain long-term local control in a region with critical structures and tissues.

  17. Radiotherapy for bony manifestations of Langerhans Cell Histiocytosis. Review and proposal for an international registry

    International Nuclear Information System (INIS)

    Purpose: to examine the role of radiotherapy (RT) in adult Langerhans Cell Histiocytosis (LCH) for osseous manifestations, to define open questions regarding RT, and to develop recommendations for the clinical decision-making and problem-solving process. Material and methods: a literature review using different medical databases was conducted including the last 3 decades, and resulting questions regarding the use of ionizing radiation were systematically compiled. Results: the literature review revealed a local control rate of 96% (93% complete remissions) in patients with osseous single-system disease and of 92% (76% complete remissions) in patients with bony involvement in multi-system disease. To increase our knowledge, a prospective registry has been developed to allow a differentiated analysis of RT outcome and definition of potential prognostic factors. Conclusion: ionizing radiation can be successfully applied as a single treatment or in combination with other therapies for osseous manifestations of LCH. It leads to high remission and local control rates. Nevertheless, many open questions still exist. A prospective clinical registry is proposed to define the exact role of RT in this disease and to develop future interdisciplinary treatment guidelines. (orig.)

  18. Radiotherapy for bony manifestations of Langerhans Cell Histiocytosis. Review and proposal for an international registry

    Energy Technology Data Exchange (ETDEWEB)

    Olschewski, T.; Seegenschmiedt, M.H. [Dept. of Radiotherapy and Radiation Oncology, Alfried Krupp Krankenhaus, Essen (Germany)

    2006-02-01

    Purpose: to examine the role of radiotherapy (RT) in adult Langerhans Cell Histiocytosis (LCH) for osseous manifestations, to define open questions regarding RT, and to develop recommendations for the clinical decision-making and problem-solving process. Material and methods: a literature review using different medical databases was conducted including the last 3 decades, and resulting questions regarding the use of ionizing radiation were systematically compiled. Results: the literature review revealed a local control rate of 96% (93% complete remissions) in patients with osseous single-system disease and of 92% (76% complete remissions) in patients with bony involvement in multi-system disease. To increase our knowledge, a prospective registry has been developed to allow a differentiated analysis of RT outcome and definition of potential prognostic factors. Conclusion: ionizing radiation can be successfully applied as a single treatment or in combination with other therapies for osseous manifestations of LCH. It leads to high remission and local control rates. Nevertheless, many open questions still exist. A prospective clinical registry is proposed to define the exact role of RT in this disease and to develop future interdisciplinary treatment guidelines. (orig.)

  19. Multifocal Langerhans cell histiocytosis of bone: Indications for radiotherapy; Histiocytose langerhansienne osseuse multifocale: place de la radiotherapie

    Energy Technology Data Exchange (ETDEWEB)

    Gaundong Mbethe, G.L.; Dejean, C.; Henriques de Figueiredo, B.; Sargos, P.; Italiano, A.; Kantor, G. [Service de radiotherapie, centre regional de lutte contre le cancer, institut Bergonie, 33 - Bordeaux (France)

    2010-12-15

    Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100, 000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology. (authors)

  20. A Rare Case of Vulvar Langerhans Cell Histiocytosis and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Didem Didar Balcı

    2009-12-01

    Full Text Available Langerhans cell histiocytosis (LCH of the vulva is rare. A 32 year-old woman presented with a two year history of non-healing vulvar ulceration and a nine month history of ulcerative nodular lesion in the left inguinal region. Biopsy results of the vulva and inguinal lesions were consistent with LCH. Screening of the body systems revealed hepatosplenomegaly, iliac, inguinal, paraaortic lymph nodes and lung involvement. She was treated with systemic steroid, resulting in complete improvement of vulvar lesion. She also underwent a local excision of left inguinal lesion. Three months later, we found a lesion on her left inguinal region that was consistent with a recurrence and enlargement in the lymph nodes. Therefore, high dose systemic steroid therapy and then a 12-session radyotherapy were given. After the treatment, a complete improvement was obtained. A 1-year of systemic methotrexate therapy was recommended to prevent recurrence of the disease. In women with vulvar ulcer, LCH should be considered in the differential diagnosis.

  1. Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Yang, Dong Hyun [Asan Medical Center, Department of Radiology, University of Ulsan College of Medicine, Seoul (Korea); Ra, Young Shin [Asan Medical Center, Department of Neurosurgery, University of Ulsan College of Medicine, Seoul (Korea); Song, Joon Sup; Im, Ho Joon; Seo, Jong Jin; Ghim, Thad; Moon, Hyung Nam [Asan Medical Center, Department of Pediatric Oncology, University of Ulsan College of Medicine, Seoul (Korea)

    2006-10-15

    In Langerhans cell histiocytosis (LCH) evaluation of the extent of disease is one of the major predictors of patient outcome. Historically this is undertaken using plain radiography and bone scintigraphy. Recently, whole-body (WB) MRI has been reported to be useful in detecting skeletal and extraskeletal metastases in both adults and children. To evaluate the usefulness of WB MRI in patients with LCH in comparison with plain radiography and bone scintigraphy. In nine children (1-7 years of age; mean 3.3 years) who had a pathological diagnosis of LCH and had either plain radiography or bone scintigraphy for comparison, 43 WB MR examinations were performed. Skeletal and extraskeletal lesions of the disease on WB MRI were compared with those on plain radiography and bone scintigraphy. LCH showed unifocal single-system involvement in one patient, multifocal single-system involvement in three, and multifocal multisystem disease in five. WB MRI identified additional skeletal lesions in three (38%) of eight patients, compared with plain radiography, and in two (25%) of eight, compared with bone scintigraphy. WB MRI detected extraskeletal lesions of the disease in five (56%) of the nine patients exclusively, except for one patient whose lung lesions were also detected on plain radiography. In two patients, treatment was changed according to WB MRI findings. (orig.)

  2. Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy

    International Nuclear Information System (INIS)

    In Langerhans cell histiocytosis (LCH) evaluation of the extent of disease is one of the major predictors of patient outcome. Historically this is undertaken using plain radiography and bone scintigraphy. Recently, whole-body (WB) MRI has been reported to be useful in detecting skeletal and extraskeletal metastases in both adults and children. To evaluate the usefulness of WB MRI in patients with LCH in comparison with plain radiography and bone scintigraphy. In nine children (1-7 years of age; mean 3.3 years) who had a pathological diagnosis of LCH and had either plain radiography or bone scintigraphy for comparison, 43 WB MR examinations were performed. Skeletal and extraskeletal lesions of the disease on WB MRI were compared with those on plain radiography and bone scintigraphy. LCH showed unifocal single-system involvement in one patient, multifocal single-system involvement in three, and multifocal multisystem disease in five. WB MRI identified additional skeletal lesions in three (38%) of eight patients, compared with plain radiography, and in two (25%) of eight, compared with bone scintigraphy. WB MRI detected extraskeletal lesions of the disease in five (56%) of the nine patients exclusively, except for one patient whose lung lesions were also detected on plain radiography. In two patients, treatment was changed according to WB MRI findings. (orig.)

  3. Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings

    Energy Technology Data Exchange (ETDEWEB)

    Binkovitz, Larry A.; Adler, Brent H. [Department of Radiology, Columbus Children' s Hospital, OH (United States); Olshefski, Randal S. [Department of Hematology and Oncology, Columbus Children' s Hospital, OH (United States)

    2003-09-01

    Bone involvement in Langerhans' cell histiocytosis (LCH) is common. Both bone scintigraphy and plain films are used to identify osseous lesions, but lack specificity for disease activity and response to therapy. FDG-PET is a sensitive technique for identifying bone lesions when histiocytes are present. To describe the potential of coincidence FDG-PET (cFDG-PET) for identification of active bone lesions in LCH and to determine whether it can provide more specific information regarding lesional response to therapy than bone scintigraphy or radiography. The clinical data and imaging findings of three patients with osseous lesions of LCH were retrospectively reviewed. cFDG-PET identified all active LCH osseous lesions in these patients, differentiated active from healed lesions, and demonstrated normalization of uptake in a treated lesion earlier than bone scintigraphy and radiography. cFDG-PET appears to have greater specificity than bone scintigraphy and radiography for the identification of active osseous lesions in LCH. It also may predict response to treatment earlier than conventional techniques. Its use in the evaluation of LCH warrants further study. (orig.)

  4. Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings

    International Nuclear Information System (INIS)

    Bone involvement in Langerhans' cell histiocytosis (LCH) is common. Both bone scintigraphy and plain films are used to identify osseous lesions, but lack specificity for disease activity and response to therapy. FDG-PET is a sensitive technique for identifying bone lesions when histiocytes are present. To describe the potential of coincidence FDG-PET (cFDG-PET) for identification of active bone lesions in LCH and to determine whether it can provide more specific information regarding lesional response to therapy than bone scintigraphy or radiography. The clinical data and imaging findings of three patients with osseous lesions of LCH were retrospectively reviewed. cFDG-PET identified all active LCH osseous lesions in these patients, differentiated active from healed lesions, and demonstrated normalization of uptake in a treated lesion earlier than bone scintigraphy and radiography. cFDG-PET appears to have greater specificity than bone scintigraphy and radiography for the identification of active osseous lesions in LCH. It also may predict response to treatment earlier than conventional techniques. Its use in the evaluation of LCH warrants further study. (orig.)

  5. Pulmonary Langerhans' cell granulomatosis (histiocytosis X): clinical analysis of 8 cases.

    Science.gov (United States)

    Callebaut, W; Demedts, M; Verleden, G

    1998-10-01

    A detailed retrospective analysis of 8 cases has been set up to obtain an update of the clinical, radiological, diagnostic and therapeutical aspects of pulmonary Langerhans' cell granulomatosis (PLCG), previously called Histiocytosis X. This disease represents 2.8% of interstitial lung diseases (ILD) in a registration by pneumologists in Flanders. Seventy five% of our patients were active smokers. Cough, dyspnoe and constitutional symptoms were the main presenting symptoms. There was a 37.5% frequency of pneumothorax during the whole disease evolution and all these patients had to be treated with chemical or surgical pleurodesis. Spirometric pattern was variable but CO-transferfactor (TLCO) was significantly impaired in all our patients. Radiologically nodules and/or cystic lesions were found with preserved volumes. Open lung biopsy led to the diagnosis in 6 of the 8 cases. Transbronchial biopsies (TBB) were found to be not sensitive. Systemic staging in our group showed 50% of the patients to have a second focus of organ involvement. Treatment with corticosteroids and possibly immunosuppressives was without clear effect. Since 25% of our patients ultimately became candidate for lung transplantation extrapulmonary staging is prerogative and the high frequency of pleurodesis procedures in the past medical history of our patients has important implications since bilateral pleurodesis is still a contraindication for lung transplantation in some but not all institutions. PMID:9861760

  6. Button sequestrum in a case of localized Langerhans' cell histiocytosis of the ilium: case report

    International Nuclear Information System (INIS)

    Langerhans' cell histiocytosis (LCH) is characterized by a proliferation of cells exhibiting the same immunohistochemical and ultra-structural characteristics as Langerhans' cells of the epidermis. Eosinophilic granuloma, chronic polyostotic disease (Hand-Schuller-Christian disease) and multisystemic disease (Letterer-Siwe disease) all belong to the spectrum LCH. Osseous lesions are the most common findings. the radiological appearance of skeletal LCH depends on the site and on disease activity. Button sequestrum has been described as uncommon in LCH of the skull and exceedingly rare in LCH at other sites. We report a case of localized LCH of the ilium with a button sequestrum. (author)

  7. Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: A single institutional experience

    Directory of Open Access Journals (Sweden)

    Biswas G

    2007-01-01

    Full Text Available Background : Langerhans cell histiocytosis (LCH is a rare disorder characterized by clonal proliferation of immature and abnormal bone marrow derived langerhans cells. Treatment is usually multimodal. Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and its proven efficacy in many lymphoproliferative disorders has made 2-CDA a rational choice in treatment of LCH. Aim : To evaluate the efficacy and toxicity profile of 2-CDA in children with relapsed or refractory LCH. Setting and Design : This is a pilot study and we present the initial data of the first seven patients treated at our institution. Materials and Methods : Seven patients of relapsed and refractory LCH were enrolled from July 2000 to June 2004. The cohort of seven patients included six males and one female with a median age at initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years. Three patients had received one prior chemotherapy regimen while the rest were heavily pretreated. Cladribine was administered over two hours IV daily for five days and repeated every four weeks. Results : After a median of six courses of cladribine (range, 2 to 9, two (33% patients achieved PR and two (33% patients have SD on imaging but are clinically better. None experienced grade 3 or 4 hematologic toxicity. At a median follow-up of 19 months (range, 8 to 52 months, five patients remain alive and one patient has died. Conclusion : Our study shows that single agent 2-CDA is active and well-tolerated in children with relapsed or refractory LCH.

  8. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

    Energy Technology Data Exchange (ETDEWEB)

    Makras, P. [Athens General Hospital, Department of Endocrinology and Diabetes, Athens (Greece); Athens General Hospital, Department of Radiology, Athens (Greece); Samara, C.; Antoniou, M.; Nikolakopoulou, Z. [Athens Hospital, 9. Pulmonary Department, Athens (Greece); Zetos, A. [General Hospital, Department of Pathology, Athens (Greece); Papadogias, D.; Piaditis, G.; Kaltsas, G.A. [Athens General Hospital, Department of Endocrinology and Diabetes, Athens (Greece); Toloumis, G. [Athens General Hospital, Department of Radiology, Athens (Greece); Andreakos, E.; Kontogeorgos, G.

    2006-01-01

    Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. (orig.)

  9. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. (orig.)

  10. Langerhans cell histiocytosis in children: from the bench to bedside for an updated therapy.

    Science.gov (United States)

    Aricò, Maurizio

    2016-06-01

    Langerhans cell histiocytosis (LCH) is a rare disease, affecting subjects of any age, with extremely variable clinical manifestations. Although most patients with LCH have localized disease, requiring local or even no therapy, those patients with disseminated, 'multi-system' disease require specific therapy because they may be at risk for morbidity or even mortality. The current standard of care has developed empirically, based mainly on the experience of treating children with leukaemia and other haemo-proliferative disorders. At the time of writing, the combined use of vinblastine and prednisone remains the standard of care for children with multi-system LCH. The combination of cytarabine and cladribine is the current standard for second-line therapy of refractory cases with vital organ dysfunction. Recent advances in the knowledge of the pathogenesis of LCH may support a change in treatment strategy. Evidence of mutations that aberrantly activate RAF/MEK/ERK signalling in over two thirds of patients with LCH may direct a target therapy strategy. Vemurafenib, a small molecule widely used in the treatment of melanoma, is the main candidate for testing in prospective trials for patients with evidence of BRAF(V) (600E) mutation on lesional tissue. Additional molecules, including the recently approved trametinib, could follow. Identification of mutations in other genes in the remaining multisystem LCH cases could contribute to define a scenario in which target therapy becomes the main therapeutic choice in this intriguing disorder. However, because the long-term risks and benefits of these agents in children are unknown, and other effective treatments exist for many LCH patients, the optimal indications for administering a tyrosine kinase inhibitor to children is an open question. PMID:26913480

  11. Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b)

    International Nuclear Information System (INIS)

    We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before. (orig.)

  12. Langerhans Cell Histiocytosis Arising from the Mandible as Diagnosed by US-guided Core Biopsy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Soo Jin [Center of Thyroid Cancer, National Cancer Center, Goyang (Korea, Republic of); Kim, Eun Kyung [Research Institute of Radiological Science, Yonsei University Heath System, Seoul (Korea, Republic of); Lee, Min Kyung [Eulji University College of Medicine, Eulji University Hospital, Daejeon (Korea, Republic of)

    2010-09-15

    Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells. Although LCH is not considered a malignant disease, its appearance on radiographs may be similar to that of a malignant tumor. The diagnosis of LCH is usually made by a soft tissue biopsy, or by bone marrow aspiration or curettage. We present a patient with a mandibular mass confirmed to be LCH by US-guided core needle biopsy, and present a strategy for diagnosing localized LCH of the bone based on the usefulness and reliability of the percutaneous biopsy

  13. Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b)

    Energy Technology Data Exchange (ETDEWEB)

    Herman, M.; Demaerel, P.; Wilms, G. [University Hospitals Leuven, Department of Radiology, Leuven (Belgium); Gool, S. van [University Hospitals Leuven, Department of Pediactrics, Leuven (Belgium); Casteels, I. [University Hospitals Leuven, Department of Ophthalmology, Leuven (Belgium)

    2007-12-15

    We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before. (orig.)

  14. Langerhans Cell Histiocytosis Arising from the Mandible as Diagnosed by US-guided Core Biopsy

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells. Although LCH is not considered a malignant disease, its appearance on radiographs may be similar to that of a malignant tumor. The diagnosis of LCH is usually made by a soft tissue biopsy, or by bone marrow aspiration or curettage. We present a patient with a mandibular mass confirmed to be LCH by US-guided core needle biopsy, and present a strategy for diagnosing localized LCH of the bone based on the usefulness and reliability of the percutaneous biopsy

  15. Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study

    Directory of Open Access Journals (Sweden)

    Narula G

    2007-01-01

    Full Text Available Context: Langerhans cell histiocytosis (LCH is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject. Aim: To present the experience of management of LCH at a single institution. Settings and Design: This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002. Materials and Methods: Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded. Statistical Analysis Used: Statistical analyses were done using Student′s ′t′ test, test for proportion and survival estimates based on the Kaplan-Meier method. Results: The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80% required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae. Conclusions: The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrolment in clinical trials with innovative therapeutic strategies to improve outcome.

  16. Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

    Directory of Open Access Journals (Sweden)

    R. Saxena

    2009-09-01

    Full Text Available We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

  17. Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

    OpenAIRE

    R. Saxena; Pati, H. P.; Dutta, S.; Kar, R.; Sharma, P.

    2009-01-01

    We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

  18. Langerhans' cell histiocytosis of the clivus: case report and literature review

    International Nuclear Information System (INIS)

    This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus. (orig.)

  19. Langerhans` cell histiocytosis of the spine: use of MRI in guiding biopsy

    Energy Technology Data Exchange (ETDEWEB)

    Kaplan, G.R.; Saifuddin, A. [Department of Diagnostic Imaging, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom); Pringle, J.A.S. [Department of Morbid Anatomy, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom); Noordeen, M.H.; Mehta, M.H. [Department of Spinal Deformities, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom)

    1998-12-01

    The MRI features of two cases of spinal Langerhans` cell histiocytosis with multilevel involvement are presented in which MRI was of help in differentiating active from inactive healing lesions by the demonstration of signal changes in the vertebral body marrow of the active lesion, manifest as low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. This distinction could not be made by plain radiography or bone scintigraphy. In cases where biopsy is required for diagnosis, MRI is recommended to guide the biopsy towards levels suggestive of active involvement. (orig.) With 7 figs., 13 refs.

  20. Extra-osseous involvement of Langerhans' cell histiocytosis in children

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, Sabine; Gudinchet, Francois [Departments of Radiology and Interventional Radiology, University Hospital Centre - CHUV, Lausanne (Switzerland); Eich, Georg [Department of Paediatric Radiology, Children' s Hospital, Zuerich (Switzerland); Hanquinet, Sylviane [Department of Paediatric Radiology, Hopital Cantonal, Geneva (Switzerland); Tschaeppeler, Heinz [Department of Paediatric Radiology, Children' s Hospital, Bern (Switzerland); Waibel, Peter [Department of Paediatric Radiology, Children' s Hospital, St. Gallen (Switzerland)

    2004-04-01

    The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis. (orig.)

  1. A rare case of Langerhans cell histiocytosis of the skull in an adult: a systematic review

    Directory of Open Access Journals (Sweden)

    Corinna Chiong

    2013-07-01

    Full Text Available We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1 mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining.

  2. Langerhans' cell histiocytosis of the spine: use of MRI in guiding biopsy

    International Nuclear Information System (INIS)

    The MRI features of two cases of spinal Langerhans' cell histiocytosis with multilevel involvement are presented in which MRI was of help in differentiating active from inactive healing lesions by the demonstration of signal changes in the vertebral body marrow of the active lesion, manifest as low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. This distinction could not be made by plain radiography or bone scintigraphy. In cases where biopsy is required for diagnosis, MRI is recommended to guide the biopsy towards levels suggestive of active involvement. (orig.)

  3. BRAF V600E-Positive Multisite Langerhans Cell Histiocytosis in a Preterm Neonate

    Directory of Open Access Journals (Sweden)

    Sara V. Bates

    2013-10-01

    Full Text Available Hemorrhagic pustules with a “blueberry muffin” appearance accompanied by respiratory failure in a neonate present a challenging differential diagnosis that includes infections and neoplasms. We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH, positive for the oncogenic BRAF V600E mutation, in a preterm neonate. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. This case is unusual in that the newborn presented with severe multiorgan involvement. Due to the rare incidence, wide spectrum of clinical manifestations, and high mortality rate, clinicians must maintain a high index of suspicion for LCH.

  4. MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Koenig, C.W.; Pfannenberg, C.; Truebenbach, J.; Remy, C.; Claussen, C.D. [Tuebingen Univ. (Germany). Abt. fuer Radiologische Diagnostik; Boehmer, G.M. [Dept. of Gastroenterology, Univ. of Tuebingen (Germany); Ruck, P. [Inst. of Pathology, Univ. of Tuebingen (Germany)

    2001-12-01

    Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients. (orig.)

  5. The rapid evolution of CT findings in pulmonary langerhans cell histiocytosis: a case report

    International Nuclear Information System (INIS)

    Imaging findings of pulmonary Langerhans cell histiocytosis (PLCH) demonstrate evolving changes over time, and the radiological transitions shown by imaging tools may allow a prediction of histopathological activity in PLCH. However, there are no reports describing how rapidly CT findings change with time. We describe a case of PLCH that showed a rapid evolutional change of the pulmonary lesions in a 48-year-old man, in which the nodular lesions showed cystic changes within two-month follow-up periods on chest CT scans

  6. An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Furmanczyk, Paul S. [University of Washington Medical Center, Department of Pathology, 1959 NE Pacific, Room BB220, P.O. Box 356100, Seattle, WA (United States); Bruckner, James D. [University of Washington Medical Center, Department of Orthopaedics and Sports Medicine, Seattle, WA (United States); Gillespy, Thurman [Harborview Medical Center, Department of Radiology, Seattle, WA (United States); Rubin, Brian P. [The Cleveland Clinic, Department of Anatomic Pathology, Cleveland, OH (United States)

    2007-09-15

    Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are both exceedingly rare histiocytic proliferations that can involve the skeletal system. We report on a case of ECD with some features suggestive of LCH. Radiographs demonstrated a large lytic lesion in the left femur, with multiple lesions of sclerosis involving both distal femurs and tibias. Both the lytic lesion and a sclerotic lesion were biopsied and demonstrated distinctive histologic features characteristic of ECD in the tibia and features of LCH in the femur. The clinical/radiologic and pathologic features that distinguish ECD and LCH as distinct entities are reviewed, and the underlying biological connection between them is discussed. (orig.)

  7. MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients. (orig.)

  8. Langerhans' cell histiocytosis of the clivus: case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Hurley, M.E.; Hayes, R. [Dept. of Radiology, Our Lady' s Hospital for Sick Children, Crumlin, Dublin (Ireland); O' Meara, A. [Dept. of Oncology, Our Lady' s Hospital for Sick Children, Dublin (Ireland); Fogarty, E. [Dept. of Orthopaedics, Our Lady' s Hospital for Sick Children, Dublin (Ireland)

    2004-03-01

    This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus. (orig.)

  9. An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are both exceedingly rare histiocytic proliferations that can involve the skeletal system. We report on a case of ECD with some features suggestive of LCH. Radiographs demonstrated a large lytic lesion in the left femur, with multiple lesions of sclerosis involving both distal femurs and tibias. Both the lytic lesion and a sclerotic lesion were biopsied and demonstrated distinctive histologic features characteristic of ECD in the tibia and features of LCH in the femur. The clinical/radiologic and pathologic features that distinguish ECD and LCH as distinct entities are reviewed, and the underlying biological connection between them is discussed. (orig.)

  10. The rapid evolution of CT findings in pulmonary langerhans cell histiocytosis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Tae Wook; Lee, Kyung Soo; Cho, Eun Yoon [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2007-07-15

    Imaging findings of pulmonary Langerhans cell histiocytosis (PLCH) demonstrate evolving changes over time, and the radiological transitions shown by imaging tools may allow a prediction of histopathological activity in PLCH. However, there are no reports describing how rapidly CT findings change with time. We describe a case of PLCH that showed a rapid evolutional change of the pulmonary lesions in a 48-year-old man, in which the nodular lesions showed cystic changes within two-month follow-up periods on chest CT scans.

  11. [Symptomatic Langerhans-cell-histiocytosis of the cervical spine in a child: case report].

    Science.gov (United States)

    Schroers, C; Donauer, E; Laudan, M; Herbst, E W; Barz, H

    2000-01-01

    We report on a six year old female presented with a painful torticollis and a hemidysaesthesia caused by destruction of the third cervical vertebra and a paravertebral soft-tissue mass. At diagnostic routine finally a biopsy gives the diagnosis of Langerhans cell histiocytosis. In a second open approach the destructed vertebral body was replaced by a precisely adjusted autologous bone interponate and the patient was maintained in halo vest immobilisation. The outcome is described and an overview of the current literature is given. PMID:10916784

  12. Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation.

    Science.gov (United States)

    Kinoshita, Yoshiaki; Watanabe, Kentaro; Sakamoto, Atsuhiko; Hidaka, Kouko

    2016-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking. PMID:26935369

  13. A Rare Case of Langerhans Cell Histiocytosis of the Skull in an Adult: a Systematic Review

    Science.gov (United States)

    Chiong, Corinna; Jayachandra, Shruti; D. Eslick, Guy; Al-Khawaja, Darweesh; Casikar, Vidyasagar

    2013-01-01

    We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining. PMID:24179650

  14. Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

    Directory of Open Access Journals (Sweden)

    Lovrenski Aleksandra

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis. In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.

  15. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    Directory of Open Access Journals (Sweden)

    B. Routy

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8 vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented.

  16. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation.

    Science.gov (United States)

    Routy, B; Hoang, J; Gruber, J

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  17. Langerhans cell histiocytosis of skin: A clinicopathologic analysis of five cases

    Directory of Open Access Journals (Sweden)

    Punia Rajpal

    2006-01-01

    Full Text Available Background and Aims: Langerhans cell histiocytosis (LCH is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. Methods: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. Results: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. Conclusion: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.

  18. Langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying metastatic thyroid papillary carcinoma.

    Science.gov (United States)

    Ceyran, A Bahar; Senol, Serkan; Bayraktar, Barış; Ozkanlı, Seyma; Cinel, Z Leyla; Aydın, Abdullah

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. PMID:25349760

  19. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Shi, Yingyan; Qiao, Zhongwei; Gong, Ying; Yang, Haowei; Li, Guoping; Pa, Mier [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China); Xia, Chunmei [Shanghai Medical College of Fudan University, Physiology and Pathophysiology Department, Shanghai (China)

    2014-06-15

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  20. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  1. High-resolution computed tomography findings in pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: The present study was aimed at characterizing main lung changes observed in pulmonary Langerhans cell histiocytosis by means of high-resolution computed tomography. Materials and Methods: High-resolution computed tomography findings in eight patients with proven disease diagnosed by open lung biopsy, immunohistochemistry studies and/or extrapulmonary manifestations were retrospectively evaluated. Results: Small rounded, thin-walled cystic lesions were observed in the lung of all the patients. Nodules with predominantly peripheral distribution over the lung parenchyma were observed in 75% of the patients. The lesions were diffusely distributed, predominantly in the upper and middle lung fields in all of the cases, but involvement of costophrenic angles was observed in 25% of the patients. Conclusion: Comparative analysis of high-resolution computed tomography and chest radiography findings demonstrated that thinwalled cysts and small nodules cannot be satisfactorily evaluated by conventional radiography. Because of its capacity to detect and characterize lung cysts and nodules, high-resolution computed tomography increases the probability of diagnosing pulmonary Langerhans cell histiocytosis. (author)

  2. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases

    Energy Technology Data Exchange (ETDEWEB)

    Hindman, B.W.; Thomas, R.D.; Young, Lionel W. [Loma Linda University Medical Center, 11234 Anderson Street, Loma Linda, CA 92354 (United States); Yu, Leisure [Department of Orthopedic Surgery, Loma Linda University Medical Center, Loma Linda, CA 92354 (United States)

    1998-04-01

    Objective. Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. Design and patients. Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. Results. Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial `button` sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. Conclusions. Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis. (orig.) With 7 figs., 10 refs.

  3. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases

    International Nuclear Information System (INIS)

    Objective. Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. Design and patients. Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. Results. Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial 'button' sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. Conclusions. Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis. (orig.)

  4. High-resolution computed tomography findings in pulmonary Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Rodrigues, Rosana Souza [Universidade Federal do Rio de Janeiro (HUCFF/UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho. Unit of Radiology; Capone, Domenico; Ferreira Neto, Armando Leao [Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ (Brazil)

    2011-07-15

    Objective: The present study was aimed at characterizing main lung changes observed in pulmonary Langerhans cell histiocytosis by means of high-resolution computed tomography. Materials and Methods: High-resolution computed tomography findings in eight patients with proven disease diagnosed by open lung biopsy, immunohistochemistry studies and/or extrapulmonary manifestations were retrospectively evaluated. Results: Small rounded, thin-walled cystic lesions were observed in the lung of all the patients. Nodules with predominantly peripheral distribution over the lung parenchyma were observed in 75% of the patients. The lesions were diffusely distributed, predominantly in the upper and middle lung fields in all of the cases, but involvement of costophrenic angles was observed in 25% of the patients. Conclusion: Comparative analysis of high-resolution computed tomography and chest radiography findings demonstrated that thinwalled cysts and small nodules cannot be satisfactorily evaluated by conventional radiography. Because of its capacity to detect and characterize lung cysts and nodules, high-resolution computed tomography increases the probability of diagnosing pulmonary Langerhans cell histiocytosis. (author)

  5. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society.

    Science.gov (United States)

    Aricò, M; Girschikofsky, M; Généreau, T; Klersy, C; McClain, K; Grois, N; Emile, J-F; Lukina, E; De Juli, E; Danesino, C

    2003-11-01

    Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year

  6. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

    Directory of Open Access Journals (Sweden)

    Anjan Madasu

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.

  7. Pulmonary Langerhans Cell Histiocytosis in an Adult Diagnosed with Solitary Inguinal Lymphadenopathy.

    Science.gov (United States)

    Luo, Wei; Li, Ya-lun; Jia, Lian; Liu, Chun-tao

    2015-01-01

    We herein report an extremely rare case of pulmonary Langerhans cell histiocytosis with a solitary enlarged inguinal lymph node. A 19-year-old man presented with a non-productive cough lasting for over a five-month period and an enlarged left inguinal lymph node that had persisted for four months. A histopathological study of the lymph node specimens found Langerhans cells coupled with eosinophils. Positive immunohistochemical staining for langerin, Cluster of Differentiation 1a, S100 in the Langerhans cells confirmed the diagnosis, and a mildly impaired ventilation function in addition to multiple peripheral pulmonary cystic lesions were detected. The patient was managed with prednisone (0.5 mg/kg daily), with slow tapering over several months. PMID:26134198

  8. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT)

    Energy Technology Data Exchange (ETDEWEB)

    Turpin, Sophie [CHU Sainte-Justine, Nuclear Medicine, Montreal (Canada); Carret, Anne-Sophie [CHU Sainte-Justine, Hemato-Oncology, Montreal (Canada); Dubois, Josee [CHU Sainte-Justine, Radiology, Montreal (Canada); Buteau, Chantal [CHU Sainte-Justine, Infectious Diseases, Montreal (Canada); Patey, Natalie [CHU Sainte-Justine, Pathology, Montreal (Canada)

    2015-11-15

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis. (orig.)

  9. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT)

    International Nuclear Information System (INIS)

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis. (orig.)

  10. A case of coexisting Warthin tumor and langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction in intraparotid lymph nodes

    OpenAIRE

    Fredrik Petersson; Gangaraju Changal Raju; Char Loo Tan

    2011-01-01

    We present a patient (50-year-old male) with coexisting Warthin tumor and involvement of two intraparotid lymph nodes by Langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction. This is the second documented case of this unusual combination of histological changes in nodal Langerhans cell histiocytosis and the first case involving intraparotid lymph nodes occurring together with an ipsilateral Warthin tumor.

  11. Increased expression of growth factor genes for macrophages and fibroblasts in bronchoalveolar lavage cells of a patient with pulmonary histiocytosis X.

    OpenAIRE

    Barth, J.; Kreipe, H; Radzun, H. J.; Heidorn, K.; Petermann, W; Bewig, B; Parwaresch, M. R.

    1991-01-01

    Pulmonary histiocytosis X is the local manifestation of a systemic disorder of unknown cause characterised by infiltration of Langerhans cell like histiocytes and parenchymal fibrosis. In a male smoker with histologically proved histiocytosis X and functional impairment bronchoalveolar lavage showed an increase in CD-1/OKT-6 antigen positive histiocytes to 8%. Northern blot analysis of RNA from bronchoalveolar lavage cells showed an exaggerated expression of the M-CSF gene and of the c-fms ge...

  12. FNAC of Bacillus- Calmette- Guerin lymphadenitis masquerading as Langerhans cell histiocytosis.

    Directory of Open Access Journals (Sweden)

    Gupta Nalini

    2004-12-01

    Full Text Available Abstract Bacillus Calmette Guerin (BCG lymphadenitis is a well known entity. Disseminated BCG infection usually presents as generalized lymphadenopathy, skin rash and hepatosplenomegaly and at times, can pose a diagnostic challenge to clinicians. There are only a few published studies on the cytological findings of BCG lymphadenitis. In this letter we report the fine needle aspiration cytology (FNAC of BCG lymphadenitis clinically masquerading as Langerhans cell histiocytosis (LCH. FNA smears showed sheets of foamy macrophages and many polymorphs in a dirty necrotic background with many macrophages as well as polymorphs showing negatively stained rod like structures within their cytoplasm. Zeihl Neelson stain revealed that these cells were heavily loaded with acid fast bacilli (AFB. In the index case, AFB were also seen within the cytoplasm of polymorphs, which has not been documented earlier in the literature.

  13. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI.

    Science.gov (United States)

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang; Sun, Gang

    2016-03-01

    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. PMID:26962425

  14. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    Directory of Open Access Journals (Sweden)

    Adam May

    2015-01-01

    Full Text Available Pulmonary Langerhans cell histiocytosis (PLCH is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.

  15. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.

    Science.gov (United States)

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2015-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  16. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    Directory of Open Access Journals (Sweden)

    Shahzaib Nabi

    2015-01-01

    Full Text Available A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

  17. Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

    International Nuclear Information System (INIS)

    Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.

  18. The value of bone scintigraphy in the diagnosis and follow-up of Langerhans' cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: To investigate the clinical value of bone scintigraphy in the diagnosis and follow-up of Langerhans' cell histiocytosis (LCH). Methods: Whole-body bone scintigraphy was performed on 23 LCH patients. Results: Nineteen of 23 patients (82.6%) showed positive in their bone scan. Eight cases were with cranial abnormal uptake, clavicle, rib and pelvis involvements were seen and each was of 3 cases, respectively, upper limb, lower limb and spinal abnormal uptakes were seen in 2, 4 and 5 patients, respectively, and 1 child had photon deficient area in the lower part of the sternum. Among 19 positive scintigrams, solitary bone lesion was shown in 9 patients (47.4%). Conclusion: Characters of bone scintigraphy in LCH are useful in the diagnosis and follow-up of this disease. (authors)

  19. Brain MR in patients with Langerhans cell histiocytosis: Findings and enhancement with Gd-DTPA

    International Nuclear Information System (INIS)

    The brains of eight patients with Langerhans Cell Histiocytosis (LCH) were studied with magnetic Resonance Imaging (MRI). One scan was normal and a spectrum of abnormalities was seen in the others. Five patients had absence of the posterior pituitary bright spot, and four with evidence of pituitary dysfunction had a lesion in the region of the hypothalamus. An orbital extraconal mass was noted in one child with exopthalmus. This decreased in a follow-up study after Vinblastine therapy. The cerebellum was abnormal in two patients; the area of the dentate nuclei exhibited abnormal signal in one asymptomatic child and the MRI of another boy with a ten year history of progressive cerebellar dysfunction showed cerebral and cerebellar atrophy. Three of the five patients with deficiency of antidiuretic hormone (ADH) were studied both before and after the administration of intravenous gadolinium DTPA. In all three, after administration of the contrast agent, there was enhancement of involved areas. (orig.)

  20. Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Bano, Shahina, E-mail: dr_shahinaindia@yahoo.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Chaudhary, Vikas, E-mail: dr_vikaschaudhary@yahoo.com [Department of Radiodiagnosis, Employees’ State Insurance Corporation (ESIC) Model Hospital, Gurgaon 122001, Haryana (India); Narula, Mahender Kaur, E-mail: narulamk@gmail.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Anand, Rama, E-mail: rama_home@yahoo.co.in [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Venkatesan, Bhuvaneswari, E-mail: venkatesanbhuvana@yahoo.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Mandal, Shramana, E-mail: shramana@hotmail.co.in [Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002 (India); Majumdar, Kaushik, E-mail: drkaushik.m@gmail.com [Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002 (India)

    2014-01-15

    Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.

  1. Rare interstitial lung disease: Pulmonary Langerhans Cell Histiocytosis in a young non smoking Indian female.

    Science.gov (United States)

    Fernandes, Lalita; Vadala, Rohit; Mesquita, Anthony Menezes; Vaideeswar, Pradeep

    2015-01-01

    Adult Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare interstitial lung disease which occurs almost exclusively in smokers. A marked male predominance was initially reported, but recent studies show both men and women are equally affected due to the increasing smoking habits in women. The natural history is variable with 25% of patients having asymptomatic disease while 10-20% progress rapidly to respiratory insufficiency and death. The diagnosis is not easily recognized by clinicians or pathologists. Awareness of the clinical presentation and classical HRCT findings helps in early diagnosis and management of this disease. We report a rare case of severe PLCH in a young non smoking female with a short history who progressed rapidly to respiratory failure and died. PMID:25857566

  2. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

    Directory of Open Access Journals (Sweden)

    M. Vaziri

    2008-06-01

    Full Text Available Pulmonary Langerhans Cell Histiocytosis (PLCH is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

  3. A case of pulmonary Langerhans cell histiocytosis in a young woman with coeliac disease.

    Science.gov (United States)

    Mastruzzo, Claudio; Vancheri, Carlo; Li Mura, Dario; Poletti, Venerino; Failla, Marco; Crimi, Nunzio

    2007-06-01

    Pulmonary Langerhans cells histiocytosis (pulmonary LCH) is an idiopathic unusual lung disease and its association with other systemic diseases has been rarely observed. Here, we describe a young non-smoking woman with concomitant pulmonary LCH and coeliac disease that, despite therapy, suddenly deteriorated. To the best of the authors' knowledge, this is the first report in the medical literature describing an association of coeliac disease with pulmonary LCH. Considering the concomitant occurrence of both diseases in our patient and the severe course of pulmonary LCH observed, we hypothesise that coeliac disease and pulmonary LCH might be related by a common disturbance in immunity and the onset and/or the course of pulmonary LCH could be influenced or markedly worsened by the presence of coeliac disease. PMID:17629812

  4. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female

    OpenAIRE

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the u...

  5. Percutaneous vertebroplasty in osteoporosis, myeloma and Langerhans' cell histiocytosis.

    LENUS (Irish Health Repository)

    Kevane, B

    2012-01-09

    This review aims to assess the effectiveness of percutaneous vertebroplasty as a treatment for the severe refractory pain associated with vertebral fracture, in a group of patients with fractures secondary to either osteoporotic or neoplastic disease. A retrospective review of 20 patients treated with percutaneous vertebroplasty in Cork University Hospital up until March 2007 was carried out and a questionnaire was prepared and distributed. Prior to vertebroplasty, patients had been symptomatic with severe pain for a mean of 20.9 weeks. Of those thirteen whom replied to a postal questionnaire, 12 (92.3%) reported pain relief and this improvement occurred within 7 days in 9 (81.8%). This was associated with decreased analgesic requirements, as determined on chart review. Prior to the procedure only 5 (38.4%) were independently mobile and this figure rose to 10 (76.9%) afterwards, occurring within one week in the majority. Subjective outcomes were better in the group of patients with neoplasm-induced fractures.

  6. Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Nemoto, Kenji; Oh-Ishi, Shuji; Inui, Toshihide; Nakazawa, Mariko; Hyodo, Kentaro; Nakajima, Masayuki; Kanazawa, Jun; Miura, Yukiko; Takaku, Takio; Minami, Yuko; Hayashihara, Kenji; Saito, Takefumi; Kawabata, Yoshinori

    2016-01-01

    Pulmonary arterial hypertension (PAH) secondary to pulmonary Langerhans cell histiocytosis (PLCH) is known to be a relatively common complication and is associated with a poor prognosis. However, the optimal therapeutic approach for these cases remains to be established. A 57-year-old man visited our hospital because of a progressive dry cough. A thoracic computed tomography examination showed a combination of diffuse thick-walled cysts and reticulonodular shadows that were predominant in bilateral upper lobes of the lungs. He was diagnosed as having PLCH based on the results of video-assisted thoracoscopic lung biopsies. During a 3-year clinical course, his condition deteriorated despite smoking cessation. A systemic evaluation demonstrated precapillary PAH caused by PLCH (PAH-PLCH), and treatment with tadalafil, a phosphodiesterase-5 inhibitor, was started. During a 50-month period of treatment with tadalafil, improvements in his dyspnea, 6-min walking distance, and hemodynamics were maintained without either overt hypoxemia or pulmonary edema. We considered that tadalafil therapy may be a useful option in the treatment of patients with PAH-PLCH. PMID:27330952

  7. Congenital self-healing histiocytosis presenting as blueberry muffin baby: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Svetlana Popadic

    2012-01-01

    Full Text Available Congenital self-healing Langerhans cell histiocytosis (CSHLCH, also called as Hashimoto-Pritzker disease, is a rare, benign variant of histiocytosis. Despite the initial dramatic clinical presentation, affected infants are otherwise healthy and skin lesions disappear spontaneously within several weeks to months. We present a case of CSHLCH presenting as blueberry muffin baby. The lesions appeared in the first week of life and lasted 6 months. The follow-up period was 24 months, without any signs of relapse. At the pediatric dermatology unit of our clinic, during the last 20 years, we had 10 children with Langerhans cell histiocytosis and among them only one with CSHLCH. In the literature, we found only 5 newborns with Langerhans cell histiocytosis presenting as blueberry muffin baby, among them only 4 with self-healing CSHLCH. The early recognition of CSHLCH may spare children from redundant and potentially toxic systemic treatment.

  8. Histiocytosis mimicking a pineal gland tumour

    International Nuclear Information System (INIS)

    We report an unusual case of isolated Langerhans cell histiocytosis of the central nervous system. A 19-year-old man presented with an incomplete ocular palsy. MRI revealed a solitary mass in the pineal gland with marked contrast enhancement. Complete microsurgical excision was followed by local radiotherapy. Histological examination revealed histiocytosis. Unifocal brain involvement by histiocytosis X is rare with few cases in the literature; the most commonly involved areas are the hypothalamus and the pituitary gland. (orig.)

  9. Histiocytosis mimicking a pineal gland tumour

    Energy Technology Data Exchange (ETDEWEB)

    Gizewski, E.R.; Forsting, M. [Dept. of Neuroradiology, Univ. of Essen (Germany)

    2001-08-01

    We report an unusual case of isolated Langerhans cell histiocytosis of the central nervous system. A 19-year-old man presented with an incomplete ocular palsy. MRI revealed a solitary mass in the pineal gland with marked contrast enhancement. Complete microsurgical excision was followed by local radiotherapy. Histological examination revealed histiocytosis. Unifocal brain involvement by histiocytosis X is rare with few cases in the literature; the most commonly involved areas are the hypothalamus and the pituitary gland. (orig.)

  10. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma

    OpenAIRE

    A. Bahar Ceyran; Serkan Şenol; Barış Bayraktar; Şeyma Özkanlı; Z. Leyla Cinel; Abdullah Aydın

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac ar...

  11. Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Harpreet Singh

    2010-01-01

    Full Text Available The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature.

  12. Measuring diffuse metabolic activity on FDG-PET/CT: new method for evaluating Langerhans cell histiocytosis activity in pulmonary parenchyma

    OpenAIRE

    Szturz Petr; Řehák Zdeněk; Koukalová Renata; Adam Zdeněk; Krejčí Marta; Pour Luděk; Zahradová Lenka; Vaníček Jiří; Nebeský Tomáš; Hájek Roman; Mayer Jiří

    2012-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare cause of interstitial lung disease characterized by formation of nodules in the active phase of the disease that evolve into nonactive cystic lesions later on. To evaluate PLCH activity in patients, we developed a new method for measuring diffuse metabolic activity on fluorine-18-fluorodeoxyglucose positron emission tomography/ computed tomography (FDG-PET/CT) using a lung-to-liver activity ratio. Material and Methods: We retrospectivel...

  13. Intraluminal fibrosis and elastic fiber degradation lead to lung remodeling in pulmonary Langerhans cell granulomatosis (histiocytosis X).

    OpenAIRE

    Y. Fukuda(Miyagi University of Education); Basset, F.; Soler, P; Ferrans, V J; Masugi, Y.; Crystal, R G

    1990-01-01

    To evaluate the morphogenesis of lung remodeling in pulmonary Langerhans cell granulomatosis (LCG; previously called histiocytosis X or eosinophilic granuloma), lung tissues obtained by open biopsy from 62 patients with pulmonary LCG were studied by light and electron microscopy. Tissues from 20 patients were also studied by immunohistochemical methods for the detection of fibronectin, elastin, and S-100 protein, and samples from six patients were studied using OKT6 monoclonal antibody. In ea...

  14. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    OpenAIRE

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae; Kim, Sang-Ha

    2015-01-01

    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 insipi...

  15. Diagnosis and Intralesional Corticotherapy in Oral Ulcers Occurring as the Sole Manifestation of Langerhans Cell Histiocytosis. A Case Report

    Science.gov (United States)

    Gambirazi, Liane; Libório, Tatiana; Nunes, Fábio; Sugaya, Norberto; Migliari, Dante

    2016-01-01

    This article reports a case of oral mucosa lesions as the sole manifestation in Langerhans cell histiocytosis (LCH). This is a very uncommon manifestation of LCH since this disease preferably affects the bones with frequent involvement of the jaws. LCH may also involve other organs, particularly the lungs, liver, lymph nodes, and skin. The highlights of this report are the differential diagnosis, immunohistochemical analysis and, mostly, the therapeutic approach. PMID:27398106

  16. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report.

    Science.gov (United States)

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae; Kim, Sang-Ha

    2015-10-01

    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. PMID:26508947

  17. A rare case of langerhans cell histiocytosis of the gastrointestinal tract

    Institute of Scientific and Technical Information of China (English)

    Uday Shankar; Monika Prasad; Om P Chaurasia

    2012-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-year-old woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD-1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increasing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.

  18. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass.

    Science.gov (United States)

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung; Park, Hyeon Jin

    2016-04-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  19. A solitary pulmonary ground-glass nodule in adult systemic langerhans' cell histiocytosis.

    Science.gov (United States)

    Zhou, Ying; Li, Yunyuan; Fan, Li; Liu, Shiyuan

    2015-01-01

    During a thoracic computed tomography (CT) scan, a 36-year-old male was diagnosed with a solitary oval pulmonary mixed ground-glass nodule in the right upper lobe of the lung. The edge of the nodule was well-defined, and its largest axial size was approximately 1.1 × 0.9 cm(2). This nodule was slightly lobulated, but not obviously speculated. Solid components, micro-cystic lucency shadow, small high-density rings and tiny vascular branches were all visible in the nodule. During hospitalization, a technetium 99 m methylene diphosphonate (Tc-99 m MDP) bone scan was performed, which showed a skeletal foci with abnormal uptake in the left iliac. A pulmonary lobectomy of the right upper lobe of the lung by video-assisted thoracoscopy was performed. In post-operative pathological photomicrographs, proliferative Langerhans' cells, eosinophils and lymphocytes were found. Immunohistochemistry showed that the expression of S-100 protein, CD1a, and CD68 antigen all stained positive. Since Langerhans' cell histiocytosis (LCH) that is also associated with isolated mixed ground-glass nodules is relatively rare, such a multi-systemic LCH case as identified herein, is reported. PMID:26722574

  20. A solitary pulmonary ground-glass nodule in adult systemic langerhans’ cell histiocytosis

    Science.gov (United States)

    Zhou, Ying; Li, Yunyuan; Fan, Li; Liu, Shiyuan

    2015-01-01

    During a thoracic computed tomography (CT) scan, a 36-year-old male was diagnosed with a solitary oval pulmonary mixed ground-glass nodule in the right upper lobe of the lung. The edge of the nodule was well-defined, and its largest axial size was approximately 1.1×0.9 cm2. This nodule was slightly lobulated, but not obviously speculated. Solid components, micro-cystic lucency shadow, small high-density rings and tiny vascular branches were all visible in the nodule. During hospitalization, a technetium 99 m methylene diphosphonate (Tc-99 m MDP) bone scan was performed, which showed a skeletal foci with abnormal uptake in the left iliac. A pulmonary lobectomy of the right upper lobe of the lung by video-assisted thoracoscopy was performed. In post-operative pathological photomicrographs, proliferative Langerhans’ cells, eosinophils and lymphocytes were found. Immunohistochemistry showed that the expression of S-100 protein, CD1a, and CD68 antigen all stained positive. Since Langerhans’ cell histiocytosis (LCH) that is also associated with isolated mixed ground-glass nodules is relatively rare, such a multi-systemic LCH case as identified herein, is reported. PMID:26722574

  1. [Pulmonary Langerhans cell histiocytosis in a non-smoking Japanese woman].

    Science.gov (United States)

    Tadokoro, Akira; Ishii, Tomoya; Bandoh, Shuji; Yokomise, Hiroyasu; Haba, Reiji; Ishida, Toshihiko

    2011-03-01

    A 44-year-old Japanese woman was admitted to our hospital because of dry cough and dyspnea on exertion. She had never smoked. She had been passively exposed to smoking by her husband and co-workers from the age of 21 (1984) to 33 (1996). She had previously developed pneumothorax twice, in 1985. On admission, computed tomography (CT) of the chest showed reticulonodular opacities predominant in bilateral upper lung fields, and pulmonary function tests revealed a decrease in vital capacity. The differential diagnoses were sarcoidosis, idiopathic pulmonary fibrosis and pulmonary Langerhans cell histiocytosis (PLCH). Video-assisted thoracic surgery was performed to make a definitive diagnosis. A histological specimen revealed the presence of CD1a-positive Langerhans cells in bronchiolocentric nodular lesions, leading to a diagnosis of PLCH. She was given 0.5 mg/kg bodyweight/ day oral prednisolone. Her symptoms disappeared with steroid maintenance therapy, and her vital capacity on pulmonary function testing was prevented from further deterioration. Based on the pathogenesis of PLCH, this case suggested that not only active smoking, but also passive smoking, played an important role in the development of PLCH. PMID:21485154

  2. Button sequestrum in a case of localized Langerhans' cell histiocytosis of the ilium: case report

    Energy Technology Data Exchange (ETDEWEB)

    Tordeur, M. [Pitie-Salpetriere Teaching Hospital, Radiology Dept., Paris (France); Wybier, M. [Lariboisiere Teaching Hospital, Paris (France); Laporte, J.L. [Pitie-Salpetriere Teaching Hospital, Pathology Dept., Paris (France); Grenier, P. [Pitie-Salpetriere Teaching Hospital, Radiology Dept., Paris (France); Laredo, J.D. [Lariboisiere Teaching Hospital, Paris (France)

    2000-04-15

    Langerhans' cell histiocytosis (LCH) is characterized by a proliferation of cells exhibiting the same immunohistochemical and ultra-structural characteristics as Langerhans' cells of the epidermis. Eosinophilic granuloma, chronic polyostotic disease (Hand-Schuller-Christian disease) and multisystemic disease (Letterer-Siwe disease) all belong to the spectrum LCH. Osseous lesions are the most common findings. the radiological appearance of skeletal LCH depends on the site and on disease activity. Button sequestrum has been described as uncommon in LCH of the skull and exceedingly rare in LCH at other sites. We report a case of localized LCH of the ilium with a button sequestrum. (author)

  3. Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus.

    Science.gov (United States)

    Cooper, Stacy L; Arceci, Robert J; Gamper, Christopher J; Teachey, David T; Schafer, Eric S

    2016-02-01

    Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RDD), is a non-neoplastic, lymphoproliferative disorder that usually resolves spontaneously or with minimal conventional chemotherapy. Rarely, SHML can be associated with autoimmune findings. Such cases are often treatment resistant and have high rates of morbidity and mortality. We present a case of a patient with long-standing autoimmunity in the context of SHML, dependent on standard-treatment until he was transitioned to novel monotherapy with sirolimus. Sirolimus treatment resulted in a complete remission, now sustained after discontinuation of all treatments for over 23 months, with no observable long-term sequelae. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc. PMID:26452062

  4. Histiocytosis--an introduction.

    OpenAIRE

    Pritchard, J.; Broadbent, V

    1994-01-01

    The Histiocytoses are a group of rare and puzzling multisystem disorders, currently regarded as non-malignant but often treated with 'cancer chemotherapy'. In this article, the origins of histiocytes and of the Histiocyte Society's classification of the Histiocytoses are described with suggested minor modifications to the classification. The current nomenclature for the 2 principal diseases, now named 'Langerhans cell histiocytosis' and 'Haemophagocytic Lymphohistiocytosis', is less confusing...

  5. Clinicopathological pattern of cranial unifocal Langerhans cell histiocytosis: A study at medical college hospital

    Directory of Open Access Journals (Sweden)

    Salma Bhat

    2015-01-01

    Full Text Available Background: Eosinophilic granuloma (EG of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic or multiple sites of involvement (polyostotic. Materials and Methods: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014. They all had been preoperatively evaluated by skull X-ray and computed tomography. To rule out a multifocal disease scintigraphy was performed in all cases preoperatively. Surgical excision was performed, and EG was diagnosed on histopathology and immunohistochemistry. Results: There was a male predominance. Parietal bone was the most common affected bone. Total excision of the lesion was performed in all cases. No patient received postoperative radiotherapy. The follow-up period ranged from 6 months to 3 years. No tumor recurrence was noted. Conclusion: With an unknown etiology, nonspecific clinical and radiological findings with diagnosis possible only on histopathological examination, EG needs to be considered in the differential diagnosis as a skull mass, especially in children.

  6. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients

    Energy Technology Data Exchange (ETDEWEB)

    Song, You Seon; Lee, In Sook; Kim, Do Kyung [Pusan National University Hospital, Medical Research Institute, Department of Radiology, Busan (Korea, Republic of); Pusan National University, School of Medicine, Department of Radiology, Busan (Korea, Republic of); Yi, Jae Hyuck [Kyungpook National University Hospital, Department of Radiology, Daegu (Korea, Republic of); Cho, Kil Ho [Yeungnam University College of Medicine, Department of Radiology, Daegu (Korea, Republic of); Song, Jong Woon [Inje University Haeundae Paik Hospital, Department of Radiology, Busan (Korea, Republic of)

    2011-11-15

    The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a ''budding'' appearance on MRI; and the presence of sclerotic margins or septations on CT. The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults. (orig.)

  7. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients

    International Nuclear Information System (INIS)

    The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a ''budding'' appearance on MRI; and the presence of sclerotic margins or septations on CT. The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults. (orig.)

  8. Langerhans cell histiocytosis in children: CT manifestation (report of 13 cases)

    International Nuclear Information System (INIS)

    Objective: To study the value and limitation of CT in the damage to multiple organs in children with Langerhans cell histiocytosis (LCH). Methods: Analysis of CT manifestations was performed in 13 cases of LCH proved by clinical features, laboratory data, and pathologic changes. Localized LCH was found in 4 cases, and disseminated LCH in 9 cases. All cases were examined by plain scan in craniocerebral, chest, liver, and spleen, and four cases by contrast-enhanced CT scan. Results: The male outnumbered female in this group. Cranial bones were involved in the sequence of temporal bone (8 cases), parietal bone (3 cases), occipital bone (2 cases), and frontal bone (1 case). CT findings were very sensitive in showing the early destructions of the bones. The characteristic manifestation on CT was multiple lytic lesions of the flat bones at any sites, without reactive sclerosis and soft tissue mass accompanied with the bony lesion. Imaging findings presented later than the clinical manifestations for LCH involving the hypothalamus/pituitary axis. Involvement of the lung at various stages by LCH could be demonstrated by CT scan. Conclusion: Imaging manifestations had no characteristics in LCH involvement. The sites of predilection and related CT manifestations could be suggestive of the diagnosis. In addition, CT findings can be helpful in the classification and evaluation of the therapy and prognosis in LCH

  9. Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone

    International Nuclear Information System (INIS)

    Background. The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. Objective. To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. Materials and methods. Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). Results. Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/RBS+ abnormalities (n = 10) were most frequently insignificant. Conclusion. In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed. (orig.). With 2 figs., 4 tabs

  10. 18F-FDG PET in neuro-degenerative Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Introduction: The so called 'neuro-degenerative Langerhans cell histiocytosis' (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease. Objectives: To search for CNS metabolic changes in NDLCH. Methods: Seven patients suffering from ND-LCH were investigated by 18F-FDG PET in this prospective study and compared with 21 healthy controls. Results: ND-LCH patients demonstrated recurrent abnormalities including bilateral hypo-metabolism in the cerebellum, the basal ganglia (caudate nuclei), frontal cortex and, bilateral, a relatively increased metabolism in the amygdalae (p ≤ 0.001). Functional changes in these anatomical regions may be detected in the absence of any apparent lesion on MRI. Conclusions: ND-LCH demonstrates a recurrent 18F-FDG PET metabolic signature. Our results suggest that 18F-FDG PET might be a useful tool for an early diagnosis of ND-LCH before neuro-radiologic abnormalities appear. (authors)

  11. Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone

    Energy Technology Data Exchange (ETDEWEB)

    Nieuwenhuyse, J.P. van [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Clapuyt, P. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Malghem, J. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Everarts, P. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Melin, J. [Department of Nuclear Medicine, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Pauwels, S. [Department of Nuclear Medicine, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Brichard, B. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Ninane, J. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Vermylen, C. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Cornu, G. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium)

    1996-10-01

    Background. The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. Objective. To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. Materials and methods. Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). Results. Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/RBS+ abnormalities (n = 10) were most frequently insignificant. Conclusion. In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed. (orig.). With 2 figs., 4 tabs.

  12. Clinical and Radiological Presentation of Pulmonary Langerhans Cell Histiocytosis: A Case Series of 20 Patients

    Directory of Open Access Journals (Sweden)

    A. Fakharian

    2008-07-01

    Full Text Available Introduction & Objective: Pulmonary Langerhans Cell Histiocytosis (PLCH is an uncommon (5% ILD interstitial tissue disorder with significant importance. It occurs predominantly in adult smokers. The organs involved in LCH include skin, bone, pituitary gland, thyroid, lymph node, and lungs.Materials & Methods: In this descriptive study all patients admitted to the Masih Daneshvari Hospital in Tehran from 1996 to 2007 diagnosed with LCH (recognized by clinical features, laboratory investigations, radiological manifestations, BAL and biopsy were evaluated.Results: A total of 20 patients were studied; mean age was 27.8 yrs.; 40% were smokers; 80% had pulmonary involvement while in 20% other organs were affected. On spirometry, a mixed pattern (50% was the most common and on chest radiography (CXR 77% of lung cysts were visible. The most frequent finding on CT- scan was diffuse lung cysts (80%.Conclusion: It is notable that ground glass opacity and consolidation occur in the early stages of disease. Since most of the patients refer with features of advanced disease, it is essential for the physicians to consider the early signs during the diagnostic stage. Also bronchoscopy and bronchial lavage are recommended in selected cases.

  13. {sup 18}F-FDG PET in neuro-degenerative Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Ribeiro, M.Jo. [CEA, DSV, DRM, Serv Hosp Frederic Joliot, F-91406 Orsay (France); Idbaih, A.; Hoang-Xuan, K. [UPMC, Grp Hosp Pitie Salpetriere, ServNeurol Mazarin, AP-HP, Paris (France); Thomas, C. [CHU Nantes, Unite Hematol et Oncol Pediat, F-44035 Nantes 01 (France); Remy, P. [CEA, Serv Hosp Frederic Joliot, CNRS, URA 2210, F-91406 Orsay (France); Remy, P. [CHU Henri Mondor, Fac Med Paris 12, Dept Neurosci, AP-HP, F-94010 Creteil (France)

    2008-07-01

    Introduction: The so called 'neuro-degenerative Langerhans cell histiocytosis' (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease. Objectives: To search for CNS metabolic changes in NDLCH. Methods: Seven patients suffering from ND-LCH were investigated by {sup 18}F-FDG PET in this prospective study and compared with 21 healthy controls. Results: ND-LCH patients demonstrated recurrent abnormalities including bilateral hypo-metabolism in the cerebellum, the basal ganglia (caudate nuclei), frontal cortex and, bilateral, a relatively increased metabolism in the amygdalae (p {<=} 0.001). Functional changes in these anatomical regions may be detected in the absence of any apparent lesion on MRI. Conclusions: ND-LCH demonstrates a recurrent {sup 18}F-FDG PET metabolic signature. Our results suggest that {sup 18}F-FDG PET might be a useful tool for an early diagnosis of ND-LCH before neuro-radiologic abnormalities appear. (authors)

  14. The role of bone scintigraphy in Langerhans' cell histiocytosis; a case report; Interet de la scintigraphie osseuse dans l'histiocytose langerhansienne: a propos d'un cas

    Energy Technology Data Exchange (ETDEWEB)

    Razzouk, M.; Carrier, P.; Darcourt, J. [Centre TEP, Hopital de l' Archet-1, Service Universitaire de Medecine Nucleaire, 06 - Nice (France); Razzouk, M.; Carrier, P.; Darcourt, J. [Federation interhospitaliere, CHU, CAL universitaire de Nice, 06 - Nice (France); Deville, A. [Hopital de l' Archet, Service d' Hemato-Oncologie Pediatrique, 06 - Nice (France); Cadet, G. [Centre Hospitalier intercommunal de Grasse, Service de Pediatrie, 06 - Grasse (France)

    2008-09-15

    We present one case of bone-Langerhans cell histiocytosis in a three-year-old male child presenting osseous lesions in the skull and the femur, which are very frequent localizations in histiocytosis. Bone scintigraphy is useful for both initial staging and follow-up associated with other imaging modalities. (authors)

  15. 99mTc bone scan and fluorodeoxyglucose positron emission tomography in evaluation of disseminated langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder in which pathological langerhans cells accumulate in a variety of organs. Manifestations may include lung infiltrates, lymph node involvements, bone lesions, hepatic, hematopoietic and endocrine dysfunctions. In this case report we present fluorine-18 positron emission tomography (F-18 PET/CT) and bone scintigraphy findings of a 18-year-old male patient with disseminated LCH, mimicking multiple hypermetabolic metastatic lesions. Clinicians should be aware that LCH infiltrations can be seen as intense uptake and to differentiate infiltrations from other metastatic intense uptake with fluorodeoxyglucose PET/CT and bone scintigraphy, clinical and laboratory findings should be kept in mind. (author)

  16. BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases.

    Science.gov (United States)

    Alayed, Khaled; Medeiros, L Jeffrey; Patel, Keyur P; Zuo, Zhuang; Li, Shaoying; Verma, Shalini; Galbincea, John; Cason, R Craig; Luthra, Rajyalakshmi; Yin, C Cameron

    2016-06-01

    Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells, often associated with lymphocytes, eosinophils, macrophages, and giant cells. BRAF mutations, usually V600E, have been reported in 40%-70% of cases, and recently, MAP2K1 mutations have been reported in BRAF-negative cases. We assessed 50 cases of LCH for BRAF mutations and assessed a subset of cases for MAP2K1 mutations. The study group included 28 men and 22 women (median age, 36.5 years; range, 1-78 years). BRAF V600E mutation was detected in 8 (16%) cases including 3 (30%) skin, 2 (11%) bone, 1 (50%) colon, 1 (20%) lung, and 1 (33%) extradural, intracranial mass. MAP2K1 mutations were detected in 6 of 13 (46%) BRAF-negative cases including 2 (100%) lymph node, 2 (50%) bone, 1 (25%) skin, and 1 (100%) orbit. Patients with BRAF mutation were younger than patients with wild-type BRAF (median age, 28 versus 38 years; P = .026). The median age of MAP2K1-mutated patients was 34.5 years, similar to patients without MAP2K1 mutation (41 years; P = .368). In agreement with 2 recent studies, we showed a high frequency of MAP2K1 mutations in BRAF-negative LCH cases. Unlike other studies, the overall frequency of BRAF mutation in this cohort is substantially lower than what has been reported in pediatric patients, perhaps because most patients in this study were adults. Moreover, we showed a high concordance between mutational and immunohistochemical analysis for BRAF mutation. There was no statistically significant association between BRAF or MAP2K1 mutation and anatomic site, unifocal versus multifocal presentation, or clinical outcome. PMID:26980021

  17. Histiocytosis X in the lung.

    Science.gov (United States)

    Colby, T V; Lombard, C

    1983-10-01

    The histologic and clinical features of pulmonary histiocytosis X are discussed. The majority of patients are in the third and fourth decades of life. They present with nonspecific respiratory complaints and bilateral reticulonodular infiltrates that can be seen on chest radiographs. The histologic lesions of pulmonary histiocytosis X are scattered, discrete nodules that frequently center on small airways. The initially cellular nodules are replaced progressively by fibrous tissue. The cell infiltrates in active lesions contain variable numbers of eosinophils and histiocytes resembling Langerhans cells of the epidermis (Hx cells). In most patients the disease resolves or stabilizes, leaving few, if any, significant residues. A minority of patients develop progressive pulmonary disease that is ultimately fatal. The cause of pulmonary histiocytosis X is unknown, but two recent series suggest a relationship to cigarette smoking. PMID:6352456

  18. Primary Langerhans cell histiocytosis of the vulva: Report of a case and brief review of the literature

    Directory of Open Access Journals (Sweden)

    Pan Zenggang

    2009-01-01

    Full Text Available Primary Langerhans cell histiocytosis (LCH of the vulva is rare. Fifteen cases of primary cutaneous vulvar LCH have been reported in English literature. We report an additional case of LCH confined to the vulva. In this article, we describe the clinical presentation, histopathology and immunohistochemistry findings of vulvar LCH that are helpful to both gynecologists and pathologists in the diagnosis of this entity. We briefly discuss the pathogenesis of LCH. The debate whether LCH is a reactive or neoplastic entity is still ongoing.

  19. Detection of Epstein-Barr Virus DNA in Langerhans Cell Histiocytosis

    Science.gov (United States)

    Khoddami, Maliheh; Nadji, Seyed Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad Reza

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic proliferation of unknown etiology. It is characterized by granuloma-like proliferation of Langerhans-type dendritic cells and mainly affects young children. Although multiple investigators have suggested the possible role of viruses, such as Epstein-Barr virus (EBV), human herpesvirus-6 (HHV-6), Herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus (CMV) in the pathogenesis of LCH, it remains, however, debated. Objectives: The EBV infection is reported to be associated with LCH. Nevertheless, no report could be found about involved Iranian children in English medical literature. In this study, we investigated the presence of EBV in Iranian children with LCH. Patients and Methods: In this retrospective study, in which we investigated the prevalence of presence of EBV DNA in LCH, using paraffin-embedded tissue samples of 30 patients with LCH and 30 age and tissue-matched controls, who were operated for reasons other than infectious diseases (between the years 2002 and 2012), by real-time polymerase chain reaction (RT-PCR) method, in the department of pediatric pathology. No ethical issues arose in the study, because only the pathology reports were reviewed, retrospectively, and the patients were anonymous. Results: There was a significant difference in prevalence of EBV presence between patients and controls. The EBV was found by RT-PCR in 19 (63.33%) out of 30 patients and only in eight (26.7%) of 30 control samples. The P = 0.004, was calculated using chi-square test (OR: 4.75; 95% CI: 1.58 ‒ 14.25). Conclusions: Our study is the first investigation performed on patients with LCH and its possible association with EBV in Iran. Considering the P = 0.004, which is statistically significant, the findings do support the hypothesis of a possible role for EBV in the pathogenesis of LCH. These results are in accordance with several previous investigations, with positive findings. PMID:26870310

  20. Measuring diffuse metabolic activity on FDG-PET/CT: new method for evaluating Langerhans cell histiocytosis activity in pulmonary parenchyma

    International Nuclear Information System (INIS)

    Introduction: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare cause of interstitial lung disease characterized by formation of nodules in the active phase of the disease that evolve into nonactive cystic lesions later on. To evaluate PLCH activity in patients, we developed a new method for measuring diffuse metabolic activity on fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) using a lung-to-liver activity ratio. Material and Methods: We retrospectively studied a series of 4 FDG-PET and 23 FDG-PET/CT scans from 7 patients with PLCH and analyzed a sample of 100 randomly chosen FDG-PET/CT studies free from any known lung or hepatic diseases. Maximum standardized uptake value (SUVmax) in a spherical volume (6–8 cm in diameter) in the right lung was put into relation with SUVmax in a spherical volume (9–10 cm in diameter) in the reference liver parenchyma to set up the SUVmaxPULMO/SUVmaxHEPAR index. The index values were compared to the disease course in each patient. Results: In patients with PLCH, a close correlation between the index value and the disease course was found in all seven subjects, where the increasing index values indicated disease activity, while decreasing index values were observed after therapy administration. In the group of 100 healthy control subjects, we found index values lower than 0.3 in 80% and lower than 0.4 in 96% [range: 0.14–0.43; 0.24±0.07 (100)]. Conclusion: Measuring SUVmaxPULMO/SUVmaxHEPAR values and their time-trend monitoring represent simple, noninvasive screening tools allowing an early diagnosis and treatment response follow-up assessment in patients with PLCH.

  1. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    International Nuclear Information System (INIS)

    The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials

  2. Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    In Langerhans cell histiocytosis (LCH), FDG PET demonstrates active disease in bone. Other imaging modalities show the effects of bone destruction by LCH. To evaluate a selective CT method for reducing effective dose from FDG PET/CT in LCH, using whole-body modified attenuation correction CT at extremely low exposure settings, with repeat selective limited-volume CT at typical localization settings. Fifty-one PET/CT scans were performed in 23 LCH patients, median patient age 8.5 years (range: 1-25 years). Thirty-four were performed with modified attenuation correction CT settings, with bed positions (excluding head and neck) repeated at localization CT settings in regions with abnormal or difficult to interpret PET findings. Of 34 modified attenuation correction PET/CT scans, 10 required repeat localization CT of 1 to 3 bed positions (total: 17 bed positions). Lytic bone lesions were easily recognized at modified attenuation correction settings. Calculated average effective dose for the 34 whole-body CT scans at modified attenuation correction settings was 1.65 mSv. Average effective dose per patient for repeat imaging of 17 bed positions at localization settings was 1.19 mSv. Average total effective dose from CT for all 34 scans performed at the modified attenuation correction CT settings, including the 10 repeat localization CT scans, was 2.0 mSv. High-quality PET scans were consistently obtained with reduced FDG-administered activities of 3.7 MBq/kg (0.10 mCi/kg). In active LCH, abnormal FDG uptake was seen in all lytic bone lesions ≥9 mm, including cranial vault lesions. Substantial reduction in effective dose is possible using selective CT techniques for FDG PET/CT. (orig.)

  3. The value of bone scintigraphy in diagnosing of langerhans cells histiocytosis

    International Nuclear Information System (INIS)

    Purpose: To investigate the clinical value of bone scintigraphy in the diagnosis of Langerhans cells histiocytosis (LCH). Methods: Whole-body bone scintigraphy was performed in 23 patients, 16 males and 7 females, average age was 4.5+1.4, with histologic diagnosis of LCH. The clinical presentation varied from local mass (10 cases), tenderness (12 cases), othemorrhea (1 case), limb fatigue (3 cases), restriction of spinal activity (2 cases) to irregular fever, cough and rash (4 cases). 99Tcm-MDP was injected intravenously , the dose modified as age, according to the formula of [ adult dosage x [(age+l)/(age+7)] ] , and sedation was given to the young children who could not cooperate well. After 3 hours of administration whole-body bone scan was performed. Images were acquired using GE Millennium SPR, with a high-resolution low-energy collimator, 8 min/m sweep speed, matrix 1024 x 256, zoom 1. Results: 19 of 23 patients (82.6%) showed positive in their bone scan. 8 cases with cranial abnormal uptake, some of them exhibited peripheral high and central low uptake; clavicle, rib and pelvis involvements were 3 respectively; 2 cases in upper limb, 4 in lower limb, most lesions of limb were located in the diaphysis; spinal abnormal uptakes were seen in 5 patients, and 1 child had photon deficient area in the lower of sternium. Among 19 positive scintigram, single bone lesion was shown in 9 patients (47.3%), the most common lesion located in cranium (5 cases). Conclusion: Some characters of bone scintigraphy in LCH are useful in the diagnosis of this disease, and accompanied with comprehensive sensation of clinical manifestation of LCH, the diagnostic accuracy will be promoted. (authors)

  4. Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Gelfand, Michael J.; Sharp, Susan E. [Cincinnati Children' s Hospital Medical Center, Section of Nuclear Medicine, Department of Radiology, Cincinnati, OH (United States); Palumbo, Joseph S. [Cincinnati Children' s Hospital Medical Center, Cancer and Blood Diseases Institute, Department of Pediatrics, Cincinnati, OH (United States)

    2014-08-23

    In Langerhans cell histiocytosis (LCH), FDG PET demonstrates active disease in bone. Other imaging modalities show the effects of bone destruction by LCH. To evaluate a selective CT method for reducing effective dose from FDG PET/CT in LCH, using whole-body modified attenuation correction CT at extremely low exposure settings, with repeat selective limited-volume CT at typical localization settings. Fifty-one PET/CT scans were performed in 23 LCH patients, median patient age 8.5 years (range: 1-25 years). Thirty-four were performed with modified attenuation correction CT settings, with bed positions (excluding head and neck) repeated at localization CT settings in regions with abnormal or difficult to interpret PET findings. Of 34 modified attenuation correction PET/CT scans, 10 required repeat localization CT of 1 to 3 bed positions (total: 17 bed positions). Lytic bone lesions were easily recognized at modified attenuation correction settings. Calculated average effective dose for the 34 whole-body CT scans at modified attenuation correction settings was 1.65 mSv. Average effective dose per patient for repeat imaging of 17 bed positions at localization settings was 1.19 mSv. Average total effective dose from CT for all 34 scans performed at the modified attenuation correction CT settings, including the 10 repeat localization CT scans, was 2.0 mSv. High-quality PET scans were consistently obtained with reduced FDG-administered activities of 3.7 MBq/kg (0.10 mCi/kg). In active LCH, abnormal FDG uptake was seen in all lytic bone lesions ≥9 mm, including cranial vault lesions. Substantial reduction in effective dose is possible using selective CT techniques for FDG PET/CT. (orig.)

  5. B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease.

    Directory of Open Access Journals (Sweden)

    Takeshi Satoh

    Full Text Available BACKGROUND: Langerhans cell histiocytosis (LCH features inflammatory granuloma characterised by the presence of CD1a+ dendritic cells or 'LCH cells'. Badalian-Very et al. recently reported the presence of a canonical (V600EB-RAF mutation in 57% of paraffin-embedded biopsies from LCH granuloma. Here we confirm their findings and report the identification of two novel B-RAF mutations detected in LCH patients. METHODS AND RESULTS: Mutations of B-RAF were observed in granuloma samples from 11 out of 16 patients using 'next generation' pyrosequencing. In 9 cases the mutation identified was (V600EB-RAF. In 2 cases novel polymorphisms were identified. A somatic (600DLATB-RAF insertion mimicked the structural and functional consequences of the (V600EB-RAF mutant. It destabilized the inactive conformation of the B-RAF kinase and resulted in increased ERK activation in 293 T cells. The (600DLATB-RAF and (V600EB-RAF mutations were found enriched in DNA and mRNA from the CD1a+ fraction of granuloma. They were absent from the blood and monocytes of 58 LCH patients, with a lower threshold of sequencing sensitivity of 1%-2% relative mutation abundance. A novel germ line (T599AB-RAF mutant allele was detected in one patient, at a relative mutation abundance close to 50% in the LCH granuloma, blood monocytes and lymphocytes. However, (T599AB-RAF did not destabilize the inactive conformation of the B-RAF kinase, and did not induce increased ERK phosphorylation or C-RAF transactivation. CONCLUSIONS: Our data confirmed presence of the (V600EB-RAF mutation in LCH granuloma of some patients, and identify two novel B-RAF mutations. They indicate that (V600EB-RAF and (600DLATB-RAF mutations are somatic mutants enriched in LCH CD1a(+ cells and absent from the patient blood. Further studies are needed to assess the functional consequences of the germ-line (T599AB-RAF allele.

  6. Langerhans cell histiocytosis with multifocal sites. A case report; Histiocitose de celulas de Langerhans com acometimento multifocal. Relato de um caso

    Energy Technology Data Exchange (ETDEWEB)

    Albuquerque, Silvio Cavalcanti; Silveira, Andre; Ribeiro Neto, Joao Vicente [Instituto Materno Infantil de Pernambuco (IMIP), Recife (Brazil)

    1998-05-01

    The authors report a case of Langerhans cell histiocytosis in which the patient, at the time of the diagnosis, had lesions at the central nervous system, mediastinum and long bones, all detectable in simple roentgenogram. The recent reclassification of this disease is discussed together with it`s radiological findings. (author) 4 refs., 6 figs.

  7. Isolated Langerhans Histiocytosis in Thyroid: Thyroidectomy or Chemotherapy?

    OpenAIRE

    Attakkil, Anoop; Thorawade, Vandana; Jagade, Mohan; Kar, Rajesh; Parelkar, Kartik

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder of mononuclear phagocytic system whose clinical presentation varies from the localised involvement of a single bone to a widely disseminated disease. Langerhans cell histiocytosis rarely involves the thyroid gland and isolated involvement of thyroid is even rarer. We report a case of an eight-year-old male child diagnosed with Langerhans cell histiocytosis limited to thyroid gland with review of literature. Should thyroidectomy be done or...

  8. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female.

    Science.gov (United States)

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  9. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report

    Science.gov (United States)

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign. PMID:27065773

  10. A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome

    Science.gov (United States)

    Granata, Francesca; Morabito, Rosa; Grasso, Giovanni; Alafaci, Elisabetta; Salpietro, Francesco M.; Alafaci, Concetta

    2016-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented. Case Description: The patient, with an insidious history of headache and a growing soft mass in the left frontal region, presented with a sudden generalized tonic-clonic epileptic seizure. Neuroradiological investigations showed an osteolytic lesion of the left frontal bone and an underlying brain lesion associated with recent signs of bleeding. The patient was operated on with a complete removal of the lesion. The postoperative course was uneventful. Conclusions: The clinical, neuroradiological, and intraoperative findings are presented, along with a review of the literature. Although rare, LCH should be considered in the differential diagnosis when a scalp lesion occurs with a progressive growing. PMID:27127696

  11. Langerhans cell histiocytosis: retrospective evaluation of 123 patients at a single institution.

    Science.gov (United States)

    Braier, J; Chantada, G; Rosso, D; Bernaldez, P; Amaral, D; Latella, A; Balancini, B; Masautis, A; Goldberg, J

    1999-01-01

    The aim of this study was to retrospectively evaluate clinical characteristics at diagnosis and outcome of patients with Langerhans cell histiocytosis (LCH). From October 1987 to March 1996, 133 patients with confirmed LCH were admitted to Hospital JP Garrahan in Buenos Aires (123 evaluable). Median age was 5 years (range 15 days to 18 years). Initial organ involvement included bone 114 patients, ear 34, skin 30, liver 18, lung 14, lymph nodes 14, spleen 12, diabetes insipidus 9, and bone marrow 2. Nineteen patients had organ dysfunction, pulmonary 14, hematological 14, and hepatic 12. Two groups were defined: Group A included patients with single system disease (uni- or multifocal) and group B multisystem (with or without organ dysfunction). In group A (n = 82), 24 patients were treated with chemotherapy (prednisone and vinblastine), 21 with surgery, 15 received radiotherapy, and 22 were only observed. Patients of group B (n = 41) were treated with chemotherapy consisting of prednisone and vinblastine, DALHX 83, or LCH1-based chemotherapy. At a median follow-up of 3 years (range 1 month-8 5/12 years) 93% of patients of group A and 39% of group B survive free of reactivation. In group B, 22% had a reactivation and 39% died of progressive disease. Sequelae were detected in 35 patients (28%), which included diabetes insipidus in 17, hearing loss in 13, bony sequelae in 11, sclerosing cholangitis in 6, and lung fibrosis with bullae in 6. Two patients had a subsequent malignant disease. A total of 17 (14%) patients died and 16 of them belonged to the group B: 13 died of progressive disease, 2 due to sclerosing cholangitis (with sepsis in one case and encephalitis in the other one), 1 with progressive disease and associated myelofibrosis, and 1 patient of group A with active disease and brain stem tumor. Patients who had organ dysfunction had a reactivation free survival of 32%. All these patients survived with sequelae. Logistic regression analysis showed that organ

  12. Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

    Directory of Open Access Journals (Sweden)

    Devendra K Tyagi

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  13. Central diabetes insipidus as a very late relapse limited to the pituitary stalk in Langerhans cell histiocytosis.

    Science.gov (United States)

    Nakagawa, Shunsuke; Shinkoda, Yuichi; Hazeki, Daisuke; Imamura, Mari; Okamoto, Yasuhiro; Kawakami, Kiyoshi; Kawano, Yoshifumi

    2016-07-01

    Central diabetes insipidus (CDI) and relapse are frequently seen in multifocal Langerhans cell histiocytosis (LCH). We present two females with multifocal LCH who developed CDI 9 and 5 years after the initial diagnosis, respectively, as a relapse limited to the pituitary stalk. Combination chemotherapy with cytarabine reduced the mass in the pituitary stalk. Although CDI did not improve, there has been no anterior pituitary hormone deficiency (APHD), neurodegenerative disease in the central nervous system (ND-CNS) or additional relapse for 2 years after therapy. It was difficult to predict the development of CDI in these cases. CDI might develop very late in patients with multifocal LCH, and therefore strict follow-up is necessary, especially with regard to symptoms of CDI such as polydipsia and polyuria. For new-onset CDI with LCH, chemotherapy with cytarabine might be useful for preventing APHD and ND-CNS. PMID:27089406

  14. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation

    Science.gov (United States)

    Bakhaidar, Mohamad G.; Alghamdi, Fahad A.; Baeesa, Saleh S.

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  15. Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child

    International Nuclear Information System (INIS)

    We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion. (orig.)

  16. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation.

    Science.gov (United States)

    Bakhaidar, Mohamad G; Alghamdi, Fahad A; Baeesa, Saleh S

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  17. Whole-body MRI in children with Langerhans cell histiocytosis for the evaluation of the skeletal system

    International Nuclear Information System (INIS)

    Purpose: the usefulness of whole-body MRI (WB-MRI) for the detection of skeletal lesions in patients with Langerhans cell histiocytosis should be documented on the basis of case presentations. Materials and methods: in six patients with histologically proven Langerhans cell histiocytosis, 14 WB-MRI examinations were performed to evaluate the skeletal system within disease staging (6 primary, 8 follow-up examinations). The examinations were performed on a 1.5 Tesla, 32-channel whole-body scanner. The examination protocol consisted of T1-weighted and STIR sequences in coronal and sagittal orientation. For comparison, radiographs of the initial skeletal lesions and those that were additionally detected on WB-MRI were available. Results: in 4 patients no additional skeletal lesions were found on WB-MRI besides the initial lesion leading to the diagnosis of unifocal single system disease. In 2 patients WB-MRI was able to identify additional skeletal lesions. In a 5 1/2 year-old boy with the primary lesion located in the cervical spine, a second lesion was detected in the lumbar spine on the initial scan and in the skull and proximal femur during follow-up examination. In a 12 year-old girl with a primary lesion of the thoracic spine, WB-MRI diagnosed additional lesions in the pelvic bone and the tibia. In both patients the diagnosis of multifocal skeletal involvement led to chemotherapy. During follow-up examination, the healing response under therapy could be demonstrated. Comparison with conventional imaging showed that especially lesions located in the spine or the pelvis were not detectable on radiographs even when knowing the MR results. (orig.)

  18. Activated Conventional T-Cells Are Present in Langerhans Cell Histiocytosis Lesions Despite the Presence of Immune Suppressive Cytokines.

    Science.gov (United States)

    Quispel, Willemijn T; Stegehuis-Kamp, Janine A; Santos, Susy J; Egeler, R Maarten; van Halteren, Astrid G S

    2015-10-01

    Langerhans cell histiocytosis (LCH) lesions are characterized by neoplastic CD1a(+)/Langerin(+) histiocytes (LCH-cells) and display many features of chronic inflammation. Cancer cells can escape immune-surveillance through intra-tumoral secretion of immune-suppressive cytokines. We therefore studied by immunohistochemistry the local cytokine milieu and phenotypic characteristics of T-cells and LCH-cells present in LCH lesions collected from 25 therapy naïve patients. LCH biopsies predominantly expressed interleukin-10 (IL-10) (10/25), transforming growth factor-beta (TGF-β) (9/25), or both cytokines (6/25). The absolute number of CD3(+)T-cells and the CD3(+)FOXP3(-) conventional cell (T-CONV) versus the CD3(+)FOXP3(+) regulatory T-cell (T-REG) was comparable for each suppressive cytokine profile (5:1). IL-10-expressing lesions contained, however, a higher proportion of T-CONV expressing the activation markers CD25 98% (38%-100%) and inducible costimulatory molecule (ICOS) 86% (47%-100%) than lesions wherein solely TGF-β was detected (CD25(+) 20% (6%-54%); ICOS(+) 29% (7%-51%)). Virtually all T-REG expressed CD25 and ICOS in IL-10 lesions, whereas TGF-β(+) lesions contained a lower proportion of ICOS(+) T-REG (P=0.05). IL-10(+) lesions contained more LCH-cells expressing high intensity of ICOS ligand (ICOSL) compared with TGF-β(+) lesions (P=0.03). ICOS expression by lesion-infiltrating T-CONV and T-REG positively correlated to the extent of ICOSL expression by LCH-cells (P=0.004). Our study points out that the combined detection of interlesional IL-10 and ICOSL expression by LCH-cells is associated with the highest prevalence of activated T-CONV. Immune profiling of LCH-affected tissues obtained at the time of diagnosis may set the stage for the development of new types of therapies, which aim at local boosting of immune cells that recognize and eliminate neoplastic LCH-cells. PMID:26381039

  19. Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature.

    Science.gov (United States)

    Gao, Limin; Li, Huifang; Li, Gandi; Liu, Weiping; Li, Jinnan; Zhang, Wenyan

    2015-01-01

    We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck lymph node were characterized by large convoluted nuclei with cerebriform indentations of the nuclear envelope and longitudinal grooves. The nuclei contained small eosinophilic nucleoli and moderate amount cytoplasm. Immunohistochemically, the histiocytoid cells were positive for Langerin, CD1a and S-100. Acid-fast bacilli were found in sputum and lung biopsy tissue. To the best of our knowledge, this is the first case of PLCH with cervical lymph node involvement, and coexisted with pulmonary tuberculosis, right pneumothorax. A contribution of this case and review three of the five cases of PLCH with extrapulmonary involvement to lymph nodes resolved spontaneously after smoking cessation constitute a novel addition that it is inappropriate to regard pulmonary/nodal LCH as multi-organ or disseminated disease, and the treatment methods are the same whether the PLCH patient with lymph node involvement or not. PMID:25973117

  20. Langerhans cell histiocytosis of the mandible in bone scintigraphy and CT

    International Nuclear Information System (INIS)

    The authors present a case of a 4-year old boy with a quickly growing tumor of the jaw. The CT examination revealed a destructive tumor in the body of the mandible involving soft tissues. A diagnosis of eosinophilic granuloma of the mandible was confirmed by a biopsy of the tumor. Skeletal scintigraphy showed areas of increased and decreased radiotracer uptake. The fusion of CT and scintigraphy images showed that the cold focus corresponds with the osteolytic area and the hot focuses are larger than the areas of osseous reconstruction shown in CT. Conclusion: In cases of histiocytosis skeletal scintigraphy and CT are complementary methods that enable one to make an assessment of the extent of the disease. (author)

  1. Radiotherapy for management of Langerhans' cell histiocytosis. Two case reports and a literature review; Strahlentherapie bei der Langerhanszell-Histiozytose. Zwei Einzelfallberichte - Literaturuebersicht

    Energy Technology Data Exchange (ETDEWEB)

    Heyd, R.; Strassmann, G.; Martin, T.; Zamboglou, N. [Staedtische Kliniken Offenbach, Offenbach am Main (Germany). Strahlenklinik; Donnerstag, F. [Staedtische Kliniken Offenbach, Offenbach am Main (Germany). Inst. fuer Roentgendiagnostik

    2000-07-01

    The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7x1.8 Gy and 7x2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required. (orig.) [German] Der Einsatz der Strahlentherapie zur Behandlung von Langerhanszell-Histiozytosen ist seit 1930 in der Literatur beschrieben und ihre Wertigkeit ist durch eine Reihe von Untersuchungen belegt. Zwei weibliche, erwachsene Patientinnen aus unserem Krankengut wurden aufgrund eines eosinophilen Granulom des Stuetzapparates in insgesamt vier Lokalisationen mit Gesamtdosen von 7x1,8 Gy und 7x2,0 Gy einer postoperativen und primaeren Radiotherapie unterzogen. Bei Nachbeobachtungszeitraeumen von 3 Monaten bis 6 Jahren wurde in allen Lokalisationen jeweils eine komplette Remission der Erkrankung erzielt. Die Analyse von 18 vorangegangenen Studien, in denen 216 Patienten an insgesamt 310 Knochenherden behandelt wurden, ergab, dass in

  2. Macrophages in Langerhans cell histiocytosis are differentiated toward M2 phenotype: their possible involvement in pathological processes.

    Science.gov (United States)

    Ohnishi, Koji; Komohara, Yoshihiro; Sakashita, Naomi; Iyama, Ken-Ichi; Murayama, Toshihiko; Takeya, Motohiro

    2010-01-01

    Although numerous macrophages are found in the lesions of Langerhans cell histiocytosis (LCH), their activation phenotypes and their roles in the disease process have not been clarified. Paraffin-embedded LCH samples were examined on immunohistochemistry and it was found that CD163 can be used to distinguish infiltrated macrophages from neoplastic Langerhans cells (LC). The number of CD163-positve macrophages was positively correlated with the number of multinucleated giant cells (MGC), indicating that most MGC are derived from infiltrated macrophages. A significant number of CD163-positive macrophages were positive for interleukin (IL)-10 and phospho-signal transducer and activator of transcription-3 (pSTAT3), an IL-10-induced signal transduction molecule. This indicates that these macrophages are polarized to anti-inflammatory macrophages of M2 phenotype. Tumor-derived macrophage-colony-stimulating factor (M-CSF) was considered to responsible for inducing M2 differentiation of infiltrated macrophages. The number of CD163-positive macrophages in different cases of LCH varied, and interestingly the density of CD163-positive macrophages was inversely correlated with the Ki-67-positivity of LC. Although the underlying mechanism is not fully elucidated, macrophage-derived IL-10 was considered to be involved in the suppression of tumor cell proliferation via activation of STAT3. PMID:20055949

  3. Pulmonary langerhans cell histiocytosis in adults: high-resolution CT - pathology comparisons and evolutional changes at CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyo Jin; Lee, Ho Yun; Kim, Tae Sung [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Lee, Kyung Soo [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Samsung Medical Center, Department of Radiology, Seoul (Korea, Republic of); Johkoh, Takeshi [Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Department of Radiology, Hyoko (Japan); Tomiyama, Noriyuki [Osaka University Graduate School of Medicine, Department of Radiology, Osaka (Japan); Han, Joungho [Sungkyunkwan University School of Medicine, Department of Pathology, Samsung Medical Center, Seoul (Korea, Republic of)

    2011-07-15

    To compare high-resolution (HR) CT and histopathological findings and to evaluate serial CT findings in pulmonary Langerhans cell histiocytosis (PLCH). We reviewed CT of lung lesions in 27 adults (M:F = 20:7, mean age, 41 {+-} 12.3 years) with PLCH. After evaluating lung abnormalities including nodules, micronodules, thick-walled, thin-walled, and bizarre-shaped cysts and reticulation, observers compared CT findings obtained at lung biopsy sites with histopathological findings. The final CT was compared with the initial CT to determine disease extent changes. The most frequently observed patterns of lung abnormalities were micronodules (n = 24, 89%), thick-walled (n = 22, 82%), and thin-walled (n = 22, 82%) cysts. Even thin-walled and bizarre cysts harboured active inflammatory Langerhans cell sheets and eosinophils in their walls. In thin-walled cysts, we noted pericystic inflammatory cell infiltrations along the alveolar walls, as well as pericystic emphysema. Thin-walled or bizarre cysts demonstrated a tendency to coalesce with surrounding cysts via their cystic wall destruction. Fourteen (52%) patients showed improvement and nine (33%) showed progressing disease. More than half of patients with pulmonary PLCH show improvement at follow-up CT. Even thin-walled cysts harbour active inflammatory cells on histopathology and exhibit improvement at follow-up CT. (orig.)

  4. Pulmonary langerhans cell histiocytosis in adults: high-resolution CT - pathology comparisons and evolutional changes at CT

    International Nuclear Information System (INIS)

    To compare high-resolution (HR) CT and histopathological findings and to evaluate serial CT findings in pulmonary Langerhans cell histiocytosis (PLCH). We reviewed CT of lung lesions in 27 adults (M:F = 20:7, mean age, 41 ± 12.3 years) with PLCH. After evaluating lung abnormalities including nodules, micronodules, thick-walled, thin-walled, and bizarre-shaped cysts and reticulation, observers compared CT findings obtained at lung biopsy sites with histopathological findings. The final CT was compared with the initial CT to determine disease extent changes. The most frequently observed patterns of lung abnormalities were micronodules (n = 24, 89%), thick-walled (n = 22, 82%), and thin-walled (n = 22, 82%) cysts. Even thin-walled and bizarre cysts harboured active inflammatory Langerhans cell sheets and eosinophils in their walls. In thin-walled cysts, we noted pericystic inflammatory cell infiltrations along the alveolar walls, as well as pericystic emphysema. Thin-walled or bizarre cysts demonstrated a tendency to coalesce with surrounding cysts via their cystic wall destruction. Fourteen (52%) patients showed improvement and nine (33%) showed progressing disease. More than half of patients with pulmonary PLCH show improvement at follow-up CT. Even thin-walled cysts harbour active inflammatory cells on histopathology and exhibit improvement at follow-up CT. (orig.)

  5. A case of Langerhans' cell histiocytosis associated with Hodgkin's lymphoma: Fine-needle aspiration cytologic and histopathological features.

    Science.gov (United States)

    Das, Dilip K; Sheikh, Zafar A; Alansary, Taiba A; Amir, Thasneem; Al-Rabiy, Fatma N; Junaid, Thamradeen A

    2016-02-01

    Langerhans cell histiocytosis (LCH) can be associated with a variety of malignant neoplasms, the most common being malignant lymphoma, especially Hodgkin's lymphoma (HL). In this report, we describe the fine needle aspiration (FNA) cytologic features of a case with concurrent LCH and HL in a lymph node. A 20-year-old man presented with an enlarged left upper cervical lymph node. FNA smears from the swelling revealed numerous CD1a+ and S-100+ Langerhans-type cells (LCs) along with many eosinophils, neutrophils, and lymphocytes; there were also large atypical cells with enlarged nuclei having prominent nucleoli. The cytodiagnosis was LCH and the possibility of association with or trans-differentiation into a lymphoma was suggested. The histopathological diagnosis of the excised left cervical lymph node was classical HL-nodular sclerosis type (CHL-NS) with LCH. The lacunar type Reed-Sternberg (RS) cells were positive for CD30 and CD15, and the LCs were positive for CD1a and S-100 protein. PET/CT imaging demonstrated hypermetabolic lymph nodes in neck, abdomen, thorax and pelvis as well as pulmonary nodules and a splenic mass. The patient received 13 courses of chemotherapy and two years later, the enhanced CT revealed regressive course of the disease. PMID:26608102

  6. [Central diabetes insipidus in adult patients--the first sign of Langerhans cell histiocytosis and Erdheim-Chester disease. Three case studies and literature review].

    Science.gov (United States)

    Adam, Z; Balsíková, K; Krejcí, M; Pour, L; Stĕpánková, S; Svacina, P; Hermanová, M; Vanícek, J; Krupa, P; Stanícek, J; Koukalová, R; Neubauer, J; Krivanová, A; Mayer, J; Hájek, R

    2010-02-01

    Central diabetes insipidus with an onset in adulthood is very rare. Unlike in children, central diabetes insipidus in adults is more frequently caused by inflammatory processes and neoplastic infiltrations that do not originate from the neuronal tissue than primary neuronal tissue tumours. Rare histiocytic neoplasias (Langerhans cell histiocytosis, xanthogranulomatosis and Erdheim-Chester disease) have a specific affinity to hypothalamus and the pituitary stalk not only in paediatric patients but also when occurring in adults. We describe 3 cases of central diabetes insipidus with an onset in adulthood. Diabetes insipidus was the first sign of Langerhans cell histiocytosis in 2 patients, and it was the first sign of Erdheim-Chester disease in one patient. MR imaging showed pathological infiltration and dilated pituitary stalks in all 3 patients. PET-CT proved useful in differential diagnosis, showing further extracranial pathological changes either on the basis of significant glucose accumulation or on the basis of CT imaging. The Langerhans cell histiocytosis in the first patient has also manifested itself as an infiltration of the perianal area with intensive accumulation of fluorodeoxyglucose (FDG) - SUV 8.6 and gingival inflammation indistinguishable from parodontosis. Histology of the perianal infiltrate confirmed Langerhans cell histiocytosis. Infiltration of the pituitary stalk disappeared from the MR image after 4 cycles of 2-chlordeoxyadenosin (5 mg/m2 5 consecutive days). The PET-CT of the 2nd patient showed only borderline accumulation of FDG in the ENT area, while simultaneously performed CT imaging showed cystic restructuring of the pulmonary parenchyma and nodulations consistent with pulmonary Langerhans cell histiocytosis. Bronchoalveolar lavage identified higher number of CD1 and S100 positive elements, consistent, once again, with pulmonary LCH also affecting pituitary stalk and ear canal. The PET-CT of the third patient showed increased activity

  7. Histiocitose das células de Langerhans na região anogenital Langerhans cell histiocytosis of the anogenital region

    Directory of Open Access Journals (Sweden)

    M. S. Neto

    1998-12-01

    Full Text Available A Histiocitose das células de Langerhans (HCL acometendo o trato genital tem uma incidência rara com apenas 48 casos relatados na literatura1-5 . Considerando somente as lesões da região anogenital, esse número cai para dois casos descritos1,2. Reportamos o 3º caso de HCL anogenital em uma paciente de 31 anos, branca, cuja comprovação diagnóstica foi feita através de microscopia eletrônica. O tratamento realizado foi quimioterapia sistêmica e excisão cirúrgica local.The Langerhans cell histiocytosis (LCH of the genital tract is rare, with only 48 cases related in the literature1-5. There were reported only 2 cases in the anogenital region1,2. We reported the third case of LCH in the anogenital region; patient was female, 31 years-old, caucasian and the diagnosis was confirmed by electron microscopic magnification. The treatment was local surgical excision and systemic chemotherapy.

  8. 111In-Octreotide and 99mTc-MDP scintigraphy in the detection of bone lesions in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Aim: Langerhans cell histiocytosis (LCH) is a granulomatous disease which can involve multiples sites of the body. Diagnostic imaging is of utmost importance in the management of these patients. Since now radiographic skeletal survey and bone scintigraphy (BS) have been used to assess bone involvement (both with low specificity). Magnetic resonance and CT have been used to assess visceral involvement but with the limitation that can not give information about the functional status. Recently two groups of investigators (Lastoria et al. and Calming et al.) have proposed somatostatin receptor scintigraphy (SSRS) to detect active lesions and for monitoring the response to treatment, due to the somatostatin analogue octreotide binds to the cell membrane of activated lymphocytes expressing somatostatine receptors. The aim of this study is to assess bone and somatostatine receptor scintigraphy in the detection of bone involvement in LCH in children. Visceral involvement has not been assessed due to none of the patients presented it at the moment of the SRS. Methods: 17 scintigraphies (11 SSRS and 6 BS) were performed in seven patients (3 girls and 4 boys) aged at diagnosis: 18 month- 12 years (mean age 6,2 years). The findings obtained in the scintigraphies were compared with clinical evolution and other imaging techniques. Results: All the BS detected the diagnosed lesions, that decreased the uptake after the treatment. In three cases BS detected one unknown bone lesion. Two SSRS could not detect a lesion on right rib in two patients, both at the moment of diagnosis. SSRS was true negative in one patient (clinical and other imaging remission) and true positive in the other four cases. SSRS detected three new unknown bone lesions. SRSS showed decreased uptake after treatment and increased uptake in the relapses. Conclusions: Somatostatin receptor and Bone scintigraphy can be used to detect active LCH bone lesions in children and can help in monitoring the response to

  9. Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem Langerhans cell histiocytosis: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study.

    Science.gov (United States)

    Morimoto, Akira; Shioda, Yoko; Imamura, Toshihiko; Kudo, Kazuko; Kawaguchi, Hiroshi; Sakashita, Kazuo; Yasui, Masahiro; Koga, Yuhki; Kobayashi, Ryoji; Ishii, Eiichi; Fujimoto, Junichiro; Horibe, Keizo; Bessho, Fumio; Tsunematsu, Yukiko; Imashuku, Shinsaku

    2016-07-01

    The JLSG-96 study reported very low mortality rates for children newly diagnosed with multifocal Langerhans cell histiocytosis (LCH). The JLSG-02 study was performed to further improve the prognosis from 2002 to 2009. The present study compared the therapeutic results of these two studies in terms of multisystem disease. All patients were treated with 6 weeks of the Induction A regimen, comprising cytarabine, vincristine and prednisolone, followed by maintenance therapy. Poor responders to Induction A were switched to Induction B. JLSG-02 has been revised from JLSG-96 in the following respects: prednisolone dosage during Induction A increased; duration of maintenance therapy extended from 24 to 48 weeks; cyclosporine introduced to Induction B for progressive disease. One hundred forty-seven children with multisystem LCH were evaluated. Of these, 84 were positive for risk of organ involvement (RO) and 63 were RO-negative. At the 6-week point, 76.2 % of RO+ and 93.7 % of RO- patients responded to Induction A. Five-year event-free survival (EFS) was 46.2 % [95 % confidence (CI), 35.5-56.9] for RO+ and 69.7 % (58.4-81.1) for RO-, which was significantly superior to that in JLSG-96 [26.8 % (13.3-40.4) and 38.9 % (16.4-61.4), respectively]. The intensified induction and prolonged maintenance regimens in JLSG-02 improved EFS in patients with multisystem LCH. PMID:27040279

  10. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Abbritti, M. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Mazzei, M.A. [Dept. Human Pathology and Oncology, Section of Radiological Sciences, Siena University, Siena (Italy); Bargagli, E., E-mail: bargagli2@gmail.com [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Refini, R.M.; Penza, F.; Perari, M.G. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Volterrani, L. [Dept. Human Pathology and Oncology, Section of Radiological Sciences, Siena University, Siena (Italy); Rottoli, P. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy)

    2012-08-15

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical-radiological and immunological data from 12 Caucasian patients (6 M, 7 smokers and 5 ex-smokers, mean age 36 {+-} 8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p < 0.05) and at the end of follow-up (time 1, p < 0.05) and with FEV1 values at time 0 (p < 0.05) and time 1 (p < 0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value.

  11. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical–radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical–radiological and immunological data from 12 Caucasian patients (6 M, 7 smokers and 5 ex-smokers, mean age 36 ± 8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p < 0.05) and at the end of follow-up (time 1, p < 0.05) and with FEV1 values at time 0 (p < 0.05) and time 1 (p < 0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value.

  12. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Abbritti, M; Mazzei, M A; Bargagli, E; Refini, R M; Penza, F; Perari, M G; Volterrani, L; Rottoli, P

    2012-08-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical-radiological and immunological data from 12 Caucasian patients (6M, 7 smokers and 5 ex-smokers, mean age 36±8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p<0.05) and at the end of follow-up (time 1, p<0.05) and with FEV1 values at time 0 (p<0.05) and time 1 (p<0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value. PMID:21549535

  13. [Pneumothorax secondary to pulmonary histiocytosis X].

    Science.gov (United States)

    Bianchi, M; Cataldi, M

    1999-01-01

    The authors report a case of pneumothorax in a young man suffering from pulmonary histiocytosis. They take this observation as a starting point for discussing about the so-called "histiocytosis X". The term "histiocytosis" is a term for a variety of proliferative disorders of histiocytes or macrophages. There is a small cluster of conditions characterized by proliferation of a special type of histiocyte called the "Langerhans' cell". The proliferation of Langerhans' cells results from disturbances in immunoregulation. Particularly, there are three clinicopathologic entities: *Letterer-Siwe disease (acute disseminated Langerhans' cells histiocytosis), *multifocal eosinophilic granuloma or Hand-Schüller-Christian disease (multifocal Langerhans' cells histiocytosis), *unifocal eosinophilic granuloma (unifocal Langerhans' cells histiocytosis). In the past, these were collected in the not specific term of histiocytosis X. Most patients with Langerhans' cell granulomatosis have diseases limited to the lung, only a small proportion have extrapulmonary involvement or a systemic disorder. Typically, there are expanding, erosive accumulations of Langerhans' cells within the medullary cavities of bones. In this reported case, a patient, two years before, was affected by osteolytic bone lesion in the left femur. Pulmonary histiocytosis is often seen in smokers in the third and fourth decade of life. The 20% of patients are asymptomatic and found to have an abnormal chest radiograph; a lesser proportion presents with pneumothorax. Some patients develop progressive fibrotic lung disease and respiratory failure. Chest radiographs may show bilateral reticular or reticulonodular infiltrates, sometimes with cystic changes, often with sparing of the costophrenic angles, but high resolution computerized tomography scans may be virtually diagnostic based on the predilection of the cysts and nodules for the upper lung zones. Sometimes the typical Langerhans' cells may be recovered in

  14. Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature

    OpenAIRE

    Gao, Limin; Li, Huifang; LI, GANDI; Liu, Weiping; Li, Jinnan; Zhang, Wenyan

    2015-01-01

    We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck...

  15. Histiocytosis: a review focusing on neuroimaging findings

    Directory of Open Access Journals (Sweden)

    Larissa Barcessat Gabbay

    2014-07-01

    Full Text Available Objective: Histiocytosis is a systemic disease that usually affects the central nervous system. The aim of this study is to discuss the neuroimaging characteristics of Langerhans cell histiocytosis (LCH, the most common of these diseases; and the non-Langerhans cells histiocytosis (NLCH, which includes entities such as hemophagocytic syndrome, Erdheim-Chester and Rosai-Dorfman diseases. Method: Literature review and illustrative cases with pathologic confirmation. Results: In LCH, the most common findings are 1 osseous lesions in the craniofacial bones and/or skull base; 2 intracranial, extra-axial changes; 3 intra-axial parenchymal changes (white and gray matter; 4 atrophy. Among the NLCH, diagnosis usually requires correlation with clinical and laboratory criteria. The spectrum of presentation includes intraparenchymal involvement, meningeal lesions, orbits and paranasal sinus involvement. Conclusion: It is important the recognition of the most common imaging patterns, in order to include LCH and NLCH in the differential diagnosis, whenever pertinent.

  16. Role of PET/CT in congenital histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Udayasankar, Unni K. [Emory University School of Medicine, Department of Radiology, Atlanta, GA (United States); Alazraki, Adina L.; Simoneaux, Stephen F. [Emory University School of Medicine, Department of Radiology, Atlanta, GA (United States); Children' s Healthcare of Atlanta at Egleston, Department of Radiology, Atlanta, GA (United States)

    2010-12-15

    Congenital Langerhans cell histiocytosis (LCH) is a rare, but often severe, form of LCH. Although a more benign single-system congenital LCH has been described, most cases present as multisystem disease with poorer prognosis and are often treated with systemic chemotherapy. Imaging plays a central role in diagnosis, initial staging and assessment of treatment response. PET/CT is increasingly utilized for pediatric LCH. We report a unique case with PET/CT used as an imaging tool in staging and evaluating treatment response in congenital multisystem LCH. (orig.)

  17. Role of PET/CT in congenital histiocytosis

    International Nuclear Information System (INIS)

    Congenital Langerhans cell histiocytosis (LCH) is a rare, but often severe, form of LCH. Although a more benign single-system congenital LCH has been described, most cases present as multisystem disease with poorer prognosis and are often treated with systemic chemotherapy. Imaging plays a central role in diagnosis, initial staging and assessment of treatment response. PET/CT is increasingly utilized for pediatric LCH. We report a unique case with PET/CT used as an imaging tool in staging and evaluating treatment response in congenital multisystem LCH. (orig.)

  18. Two rare cases of laryngeal intralymphatic histiocytosis

    DEFF Research Database (Denmark)

    Reznitsky, Martin; Daugaard, Søren; Charabi, Birgitte Wittenborg

    2016-01-01

    We report two rare cases of intralymphatic histiocytosis causing, respectively, recurrent and persistent episodes of upper airway swelling and breathing difficulties. Case 1 was a 39-year-old man who was referred with recurrent upper airway swelling causing difficulty in breathing. A direct....... Extensive investigations were performed but discovered no abnormal findings. He received CO2 laser treatment twice and the swelling decreased. Intralymphatic histiocytosis is extremely rare in upper airway pathology. It is an important differential diagnosis in patients with recurrent and chronic laryngeal...

  19. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    International Nuclear Information System (INIS)

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  20. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    Energy Technology Data Exchange (ETDEWEB)

    Samet, Jonathan [Northwestern University Feinberg School of Medicine, Department of Medical Imaging, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Weinstein, Joanna [Northwestern University Feinberg School of Medicine, Departments of Pediatrics, Division of Hematology/Oncology/Stem Cell Transplant, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Fayad, Laura M. [The Johns Hopkins University School of Medicine, The Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States)

    2016-05-15

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  1. Histiocitosis de células de Langerhans localizada en hueso malar: Presentación de un caso Localized langerhans cell histiocytosis of the zygomatic bone: A case report

    Directory of Open Access Journals (Sweden)

    María Loreto Castellón Zirpel

    2011-09-01

    Full Text Available La histiocitosis de células de Langerhans localizada (HCLL, conocida como granuloma eosinófilo, representa entre el 50 y el 60% de todos los casos de histiocitosis de células de Langerhans. El tratamiento clásico para la HCLL ha sido el curetaje o la resección de las lesiones óseas. Hay publicaciones de casos tratados con inyección intralesional de corticosteroides, combinado con curetaje. Se presenta un caso clínico de un paciente de tres años de edad con diagnóstico de HCLL que compromete en su extensión el hueso malar, tratado con infiltraciones de corticosteroides y posterior curetaje de la lesión. A un año de realizado el tratamiento, el paciente se encuentra asintomático y con una regeneración ósea del hueso malar, evidenciable en la tomografía axial computarizada.Localized Langerhans cell histiocytosis (LLCH, also known as eosinophilic granuloma, represents 50 to 60% of all cases of Langerhans cell histiocytosis. The standard treatment for LLCH has been lesion curettage or resection. Cases treated with intralesional corticosteroid injections combined with curettage have been described. We report the case of a three-year-old patient diagnosed of LLCH with extensive zygomatic bone involvement, who was treated with corticosteroid infiltrations and subsequent curettage of the lesion. One year after treatment, the patient is asymptomatic with zygomatic reossification evidenced on computed tomography.

  2. Histiocitose das células de Langerhans: experiência de 16 anos Langerhans cell histiocytosis: a 16-year experience

    Directory of Open Access Journals (Sweden)

    Márcia Kanadani Campos

    2007-02-01

    Full Text Available OBJETIVOS: Descrever a apresentação clínica da histiocitose das células de Langerhans e comparar sua evolução de acordo com a idade, estadiamento e resposta ao tratamento. MÉTODOS: Análise retrospectiva dos dados referentes a 33 crianças com histiocitose das células de Langerhans acompanhadas no Hospital das Clínicas da Universidade Federal de Minas Gerais no período de 1988 a 2004. RESULTADOS: A idade ao diagnóstico variou de 2 meses a 16 anos (mediana: 2,5 anos. Dezessete crianças eram do sexo masculino. O tempo de seguimento variou de 21 dias a 16,2 anos (mediana: 3,4 anos. As manifestações clínicas mais comuns ao diagnóstico foram lesões osteolíticas, linfadenomegalia e lesões cutâneas. A sobrevida global para todo o grupo foi de 86,1% aos 16 anos (IC95% 66,6-94,6. Os óbitos ocorreram em pacientes com doença multissistêmica e disfunção orgânica ao diagnóstico. Os pacientes que apresentaram resposta "melhor" à sexta semana de tratamento apresentaram uma probabilidade estimada de sobrevida global significativamente maior em relação aos que apresentaram progressão da doença. A sobrevida global foi significativamente maior para os pacientes com doença em um único sistema. A probabilidade de sobrevida livre de eventos para todo o grupo foi de 30,9% aos 16 anos (IC95% 15,6-47,5, sendo significativamente maior para os portadores de doença em um único sistema. A idade não se associou com a sobrevida livre de eventos. A seqüela mais comum foi o diabetes insipidus. Não foram observados casos de neoplasias secundárias. CONCLUSÃO: A histiocitose das células de Langerhans apresenta uma grande variedade de manifestações clínicas, com alta taxa de recidivas e baixa taxa de mortalidade.OBJECTIVES: To describe the clinical course of Langerhans cell histiocytosis and to compare its outcome according to age, staging of the disease and treatment response. METHODS: Retrospective analysis of data on 33 children with

  3. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease) and diffuse large B-cell lymphoma: a case report

    OpenAIRE

    Moore James C; Zhao Xiaohui; Nelson Edward L

    2008-01-01

    Abstract Introduction Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease, is a rare and benign source of lymphadenopathy first described in 1969, which mimics neoplastic processes. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy or extranodal lesions consisting of tissue infiltrates composed of a polyclonal population of histiocytes. Since its description greater than 400 cases have been described, sometim...

  4. Progressive nodular histiocytosis associated with eale′s disease

    Directory of Open Access Journals (Sweden)

    Abhilasha Williams

    2015-01-01

    Full Text Available Progressive nodular histiocytosis (PNH is a rare normolipemic macrophage disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (LCHs which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement. We report a 30-year-old gentleman who presented with skin lesions all over the body associated with gradual bilateral painless loss of vision. On examination, approximately 30 to 40, skin-colored, firm, non-tender papules and nodules were noted over the body especially on the face and trunk. A skin biopsy revealed a cellular tumor in the dermis composed of oval to spindle-shaped cells, positive for CD68 but negative for S-100, CD34, CD21, CD35 and HMB45, supporting a diagnosis of spindle cell histiocytic tumor. Ophthalmic examination revealed a generalized arteriolar attenuation in both eyes. He received Tab Imatinib 400 mg OD for 5 months followed by Tab Pazopanib 800 mg OD for 4 months and both the drugs were stopped due to lack of any response in the skin lesions. We report this case due to its rarity, characteristic clinical presentation, and its association with Eale′s disease. Primary treatment remains surgical excision of bothersome lesions and optimal systemic treatment is still unknown.

  5. Blood Stem Cell Transplant in Treating Patients With Hematologic Cancer

    Science.gov (United States)

    2014-06-05

    Adult Langerhans Cell Histiocytosis; Childhood Langerhans Cell Histiocytosis; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms

  6. A Rare Case of Langerhans Cell Histiocytosis of the Skull in an Adult: a Systematic Review

    OpenAIRE

    Corinna Chiong; Shruti Jayachandra; Eslick, Guy D.; Darweesh Al-Khawaja; Vidyasagar Casikar

    2013-01-01

    We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1 mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell ...

  7. Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child

    Energy Technology Data Exchange (ETDEWEB)

    Trusen, Andreas; Beissert, Matthias; Darge, Kassa [Department of Paediatric Radiology, Institute of Radiodiagnostics, University of Wuerzburg, Josef-Schneider-Strasse 2, 97080 Wuerzburg (Germany); Hebestreit, Helge [University Children' s Hospital, University of Wuerzburg, Wuerzburg (Germany); Marx, Alexander [Institute of Pathology, University of Wuerzburg, Wuerzburg (Germany)

    2003-07-01

    We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion. (orig.)

  8. p53 expression in biopsies from children with Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Bank, Micha I; Lundegaard, Pia Rengtved; Carstensen, Henrik;

    2002-01-01

    single-system lesions only without relapse, whereas others develop fatal multiorgan disease. The expression of p53 tumor suppressor gene product detected immunohistochemically can be used as a guideline to alterations in DNA repair control and apoptosis. The authors have chosen to analyze p53 expression...... diagnosis of LCH based on CD1a positivity. The slides were stained with p53 antibody and semiquantitatively evaluated using a grading system from 1 to 5 as an estimate for 0% to 20%, 20% to 40%, 40% to 60%, 60% to 80%, and 80% to 100% p53-positive for pathologic Langerhans cells (pLC), respectively. RESULTS......: The p53 protein was expressed in various degrees in pLC in all lesions. The degree of p53 expression could not be correlated to either clinical manifestation or outcome. CONCLUSIONS: An increased expression of p53 in pLC indicates an altered DNA repair control with or without abnormal control of...

  9. BRAF V600E mutation correlates with suppressive tumor immune microenvironment and reduced disease-free survival in Langerhans cell histiocytosis.

    Science.gov (United States)

    Zeng, Kaixuan; Wang, Zhe; Ohshima, Koichi; Liu, Yixiong; Zhang, Weichen; Wang, Lu; Fan, Linni; Li, Mingyang; Li, Xia; Wang, Yingmei; Yu, Zhou; Yan, Qingguo; Guo, Shuangping; Wei, Jie; Guo, Ying

    2016-07-01

    Langerhans cell histiocytosis (LCH) is a neoplasm of myeloid origin characterized by a clonal proliferation of CD1a(+)/CD207(+) dendritic cells. Recurrent BRAF V600E mutation has been reported in LCH. In the present report, we confirm the feasibility of the high-specificity monoclonal antibody VE1 for detecting BRAF V600E mutation in 36/97 (37.1%) retrospectively enrolled patients with LCH; concordant immunohistochemistry and Sanger sequencing results were seen in 94.8% of cases. We then assessed the tumor immune microenvironment status in LCH, and found that the GATA binding protein 3 (GATA3)(+)/T-bet(+) ratio could distinguish between clinical multi-system/single-system (SS) multifocal and SS unifocal LCH. Notably, we found that BRAF V600E mutation is significantly correlated with increased programmed cell death 1 ligand 1 (PDL1) expression and forkhead box protein 3 (FOXP3)(+) regulatory T cells (p hazard ratio [HR] = 2.38, 95% confidence interval [CI] 1.02-5.56, p = 0.044; HR = 3.06, 95%CI 1.14-7.14, p = 0.025, respectively), and the superiority of PDL1 in sensitivity and specificity as biomarker for DFS in LCH was demonstrated by receiver operator characteristic (ROC) curves when compared with BRAF V600E and risk category. Collectively, this study identifies for the first time relationship between BRAF V600E mutation and a suppressive tumor immune microenvironment in LCH, resulting in disruption of host-tumor immune surveillance, which is DFS. Our findings may provide a rationale for combining immunotherapy and BRAF-targeted therapy for treating patients with BRAF V600E mutant LCH. PMID:27622040

  10. Histiocytic sarcoma that mimics benign histiocytosis.

    Science.gov (United States)

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge. PMID:8793665

  11. Diagnósticos diferenciales de la histiocitosis a células de Langerhans The differential diagnostics of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    C. N. Chirino

    2007-06-01

    Full Text Available La histiocitosis a células de Langerhans (HCL debe diferenciarse de las siguientes entidades: eritema tóxico neonatorum (ETN, dermatitis seborreica (DS, foliculitis pustulosa eosinofílica (FPE, incontinencia pigmenti (IP, mastocitosis/urticaria pigmentosa (M/UP, acrodermatitis enteropática (ADE, síndrome de Wiskott-Aldrich (WAS, acropustulosis infantil (API. Además se deben considerar la enfermedad de Rosai- Dorfman (ERD, xantomas diseminados, melanosis pustulosa neonatal (MPN, candidiasis congénita, listeriosis neonatal, herpes simple perinatal y la varicela neonatal. Debido a que los métodos auxiliares de laboratorio no siempre están disponibles o los resultados laboratoriales algunas veces son extemporáneos, y puesto que el médico práctico a menudo necesita tomar decisiones precozmente, es que la epidemiología resulta útil, pues brinda el marco adecuado para ordenar y jerarquizar las sospechas diagnósticas frente a un caso concreto, con un paciente determinado, en un momento específico.The differential diagnostics of Langerhans cell histiocytosis should include the following disorders: erythema toxicum neonatorum, seborrheic dermatitis, eosinophilic pustular folliculitis, incontinentia pigmenti, mastocytosis / urticaria pigmentosa, acrodermatitis enteropathica, Wiskott-Aldrich syndrome, infantile acropustulosis, Rosai- Dorfman disease, xanthoma disseminatum, neonatal pustular melanosis, congenital candidiasis, perinatal listeriosis, perinatal herpes simplex, neonatal varicella. Since the auxiliary methods of lab are not always available, or lab results are sometimes extemporaneous, the physicians often needs to make quick decisions. The epidemiology is useful because it offers the appropriate mark to prioritize the diagnostic in specific cases

  12. Whole-body MRI in children with Langerhans cell histiocytosis for the evaluation of the skeletal system; Ganzkoerper-MRT bei Langerhans-Zell-Histiozytose im Kindes- und Jugendalter zur Beurteilung des Skelettsystems

    Energy Technology Data Exchange (ETDEWEB)

    Steinborn, M. [Inst. fuer Diagnostische und Interventionelle Radiologie, Abt. Kinderradiologie, Staedtisches Klinikum Muenchen GmbH, Klinikum Schwabing (Germany); Woertler, K.; Rummeny, E.J. [Inst. fuer Roentgendiagnostik der Technischen Univ. Muenchen, Klinikum Rechts der Isar (Germany); Nathrath, M. [Abt. fuer Paediatrische Onkologie und Haematologie, Kinderklinik und Poliklinik des Klinikums Rechts der Isar der Technischen Univ. Muenchen, Kinderklinik Schwabing (Germany); KKG Osteosarkom, Helmholtz Zentrum, Muenchen (Germany); Schoeniger, M. [Abt. fuer Paediatrische Onkologie und Haematologie, Kinderklinik und Poliklinik des Klinikums Rechts der Isar der Technischen Univ. Muenchen, Kinderklinik Schwabing (Germany); Hahn, H. [KKG Osteosarkom, Helmholtz Zentrum, Muenchen (Germany)

    2008-07-15

    Purpose: the usefulness of whole-body MRI (WB-MRI) for the detection of skeletal lesions in patients with Langerhans cell histiocytosis should be documented on the basis of case presentations. Materials and methods: in six patients with histologically proven Langerhans cell histiocytosis, 14 WB-MRI examinations were performed to evaluate the skeletal system within disease staging (6 primary, 8 follow-up examinations). The examinations were performed on a 1.5 Tesla, 32-channel whole-body scanner. The examination protocol consisted of T1-weighted and STIR sequences in coronal and sagittal orientation. For comparison, radiographs of the initial skeletal lesions and those that were additionally detected on WB-MRI were available. Results: in 4 patients no additional skeletal lesions were found on WB-MRI besides the initial lesion leading to the diagnosis of unifocal single system disease. In 2 patients WB-MRI was able to identify additional skeletal lesions. In a 5 1/2 year-old boy with the primary lesion located in the cervical spine, a second lesion was detected in the lumbar spine on the initial scan and in the skull and proximal femur during follow-up examination. In a 12 year-old girl with a primary lesion of the thoracic spine, WB-MRI diagnosed additional lesions in the pelvic bone and the tibia. In both patients the diagnosis of multifocal skeletal involvement led to chemotherapy. During follow-up examination, the healing response under therapy could be demonstrated. Comparison with conventional imaging showed that especially lesions located in the spine or the pelvis were not detectable on radiographs even when knowing the MR results. (orig.)

  13. Complete disappearance of lung abnormalities on high-resolution computed tomography: A case of histiocytosis X

    OpenAIRE

    2007-01-01

    A case of pulmonary Langerhans cell histiocytosis, proved by both lung high-resolution computed tomography and lung biopsy, is described. Following smoking cessation, lung nodules and cysts gradually disappeared on serial computed tomography scans, with complete clearance of the lesions after 12 months. The role of tobacco smoking is discussed, in detail, against the background of the literature.

  14. The diagnostic value of {sup 18}F-FDG PET and MRI in paediatric histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, Wolfgang Peter; Melzer, Henriette Ingrid; Bartenstein, Peter; Pfluger, Thomas [Ludwig-Maximilians-University of Munich, Department of Nuclear Medicine, Munich (Germany); Schmid, Irene [Ludwig-Maximilians-University of Munich, Department of Paediatric Oncology, Munich (Germany); Coppenrath, Eva [Ludwig-Maximilians-University of Munich, Department of Radiology, Munich (Germany)

    2013-03-15

    To analyse the diagnostic value of {sup 18}F-FDG PET and MRI for the evaluation of active lesions in paediatric Langerhans cell histiocytosis. We compared 21 {sup 18}F-FDG PET scans with 21 MRI scans (mean time interval 17 days) in 15 patients (11 male, 4 female, age range 4 months to 19 years) with biopsy-proven histiocytosis. Primary criteria for the lesion-based analysis were signs of vital histiocyte infiltrates (bone marrow oedema and contrast enhancement for MRI; SUV greater than the mean SUV of the right liver lobe for PET). PET and MR images were analysed separately and side-by-side. The results were validated by biopsy or follow-up scans after more than 6 months. Of 53 lesions evaluated, 13 were confirmed by histology and 40 on follow-up investigations. The sensitivity and specificity of PET were 67 % and 76 % and of MRI were 81 % and 47 %, respectively. MRI showed seven false-positive bone lesions after successful chemotherapy. PET showed five false-negative small bone lesions, one false-negative lesion of the skull and three false-negative findings for intracerebral involvement. PET showed one false-positive lesion in the lymphoid tissue of the head and neck region and two false-positive bone lesions after treatment. Combined PET/MR analysis decreased the number of false-negative findings on primary staging, whereas no advantage over PET alone was seen in terms of false-positive or false-negative results on follow-up. Our retrospective analysis suggests a pivotal role of {sup 18}F-FDG PET in lesion follow-up due to a lower number of false-positive findings after chemotherapy. MRI showed a higher sensitivity and is indispensable for primary staging, evaluation of brain involvement and biopsy planning. Combined MRI/PET analysis improved sensitivity by decreasing the false-negative rate during primary staging indicating a future role of simultaneous whole-body PET/MRI for primary investigation of paediatric histiocytosis. (orig.)

  15. The diagnostic value of 18F-FDG PET and MRI in paediatric histiocytosis

    International Nuclear Information System (INIS)

    To analyse the diagnostic value of 18F-FDG PET and MRI for the evaluation of active lesions in paediatric Langerhans cell histiocytosis. We compared 21 18F-FDG PET scans with 21 MRI scans (mean time interval 17 days) in 15 patients (11 male, 4 female, age range 4 months to 19 years) with biopsy-proven histiocytosis. Primary criteria for the lesion-based analysis were signs of vital histiocyte infiltrates (bone marrow oedema and contrast enhancement for MRI; SUV greater than the mean SUV of the right liver lobe for PET). PET and MR images were analysed separately and side-by-side. The results were validated by biopsy or follow-up scans after more than 6 months. Of 53 lesions evaluated, 13 were confirmed by histology and 40 on follow-up investigations. The sensitivity and specificity of PET were 67 % and 76 % and of MRI were 81 % and 47 %, respectively. MRI showed seven false-positive bone lesions after successful chemotherapy. PET showed five false-negative small bone lesions, one false-negative lesion of the skull and three false-negative findings for intracerebral involvement. PET showed one false-positive lesion in the lymphoid tissue of the head and neck region and two false-positive bone lesions after treatment. Combined PET/MR analysis decreased the number of false-negative findings on primary staging, whereas no advantage over PET alone was seen in terms of false-positive or false-negative results on follow-up. Our retrospective analysis suggests a pivotal role of 18F-FDG PET in lesion follow-up due to a lower number of false-positive findings after chemotherapy. MRI showed a higher sensitivity and is indispensable for primary staging, evaluation of brain involvement and biopsy planning. Combined MRI/PET analysis improved sensitivity by decreasing the false-negative rate during primary staging indicating a future role of simultaneous whole-body PET/MRI for primary investigation of paediatric histiocytosis. (orig.)

  16. Smoking and interstitial lung disease. The effect of cigarette smoking on the incidence of pulmonary histiocytosis X and sarcoidosis.

    Science.gov (United States)

    Hance, A J; Basset, F; Saumon, G; Danel, C; Valeyre, D; Battesti, J P; Chrétien, J; Georges, R

    1986-01-01

    Cigarette smoking produces marked alterations in the lung parenchyma and in the population of immune and inflammatory cells present in the lower respiratory tract. These cigarette-induced changes appear to influence the incidence of two different interstitial lung diseases, histiocytosis X and sarcoidosis. Smoking is a strong risk factor for the development of pulmonary histiocytosis X, since the incidence of smoking is very high among patients with histiocytosis X: 90% of the patients with histiocytosis X were smokers; 46% of the controls were smokers (p less than .001). In contrast, smoking appears to reduce the incidence of sarcoidosis: 31% of the patients with sarcoidosis were smokers (p less than .05 compared to controls). In an effort to understand how cigarette smoking influences the incidence of these two disorders, we compared the numbers and types of immune and inflammatory cells recovered by bronchoalveolar lavage from nonsmoking and smoking controls and patients with histiocytosis X and sarcoidosis. Although nonsmoking patients with histiocytosis X did not have a significant increase in the number of alveolar macrophages recovered by lavage (p greater than .2 compared to normals), smoking patients had an increase in the number of alveolar macrophages similar to that observed in the control population. In contrast, the number of macrophages recovered from patients with sarcoidosis who smoked was considerably less than that observed in normal smokers (p less than .05 comparing patients with sarcoidosis and controls who smoked 1-20 cigarettes/day). This difference in the intensity of the cigarette-induced macrophage alveolitis observed in the two patient groups may be important in explaining the opposite effects of cigarette smoking on the incidence of histiocytosis X and sarcoidosis. PMID:3488004

  17. Histiocytosis-X in Saudi children.

    OpenAIRE

    al-Mulhim I; Sabbah R; al-Akkad S

    1991-01-01

    A retrospective analysis of 21 Saudi children with Histiocytosis-X were reviewed. 13 were males, 8 were females, with a ratio of 1.62:1. Five had unifocal disease while 16 were with multifocal disease. Bone involvement was seen in 19 patients (90.4%), and three patients had organ dysfunction. One patient had family history of Histiocytosis-X. Follow-up was from 1-10 years (Median 3 years). Three patients died, three had recurrences and eight patients had various disabilities (38%...

  18. Evolución clínica y radiológica de una mujer con histiocitosis pulmonar de células de Langerhans durante 18 años Clinical and radiological evolution of a woman with pulmonary Langerhans cell histiocytosis during 18 years

    Directory of Open Access Journals (Sweden)

    XIMENA CEA B

    2008-01-01

    Full Text Available La histiocitosis pulmonar de células de Langerhans (HPCL es una enfermedad pulmonar difusa quística poco frecuente. En esta revisión, presentamos un caso de HPCL diagnosticada en una adolescente, no fumadora, que consultó por disnea rápidamente progresiva asociada a dolor pleurítico izquierdo, debido a un neumotorax bilateral. El diagnóstico se confirmó por biopsia quirúrgica. Se indicó tratamiento con corticoides (prednisona durante el primer año. Tras 18 años de seguimiento destaca una importante mejoría clínica, con una evolución radiológica y espirométrica favorable, pero no completa. Los casos que se presentan con neumotorax espontáneo recurrente son raros. La evolución natural de esta enfermedad es variable y su tratamiento aún es controversial, siendo la terapia con inmunosupresores, tal como corticoesteroides y agentes citotóxicos de valor limitado, ya que son escasos los estudios que avalan su eficaciaThe Pulmonary Langerhans cell histiocytosis (PLCH is an uncommon pulmonary diffuse cystic disease. This review presents one case of PLCH diagnosed in a non-smoker female adolescent, who consulted for rapidly progressive dyspnea associated to left pleuritic pain that corresponded to a bilateral pneumothorax. The diagnosis was confirmed by surgical biopsy of the lung. Treatment with corticoids (prednisone was indicated during the first year. After 18 years from diagnosis she presented an important clinical improvement, with a favourable but not complete radiological and spirometric improvement. The cases that make their debut with recurrent spontaneous pneumothorax are infrequent. The natural evolution of this disease is variable and the treatment is still controversial, been the immunosuppressive therapy, as corticoesteroids and cytotoxic agents of limited value, since are few studies that confirm their effectiveness

  19. Histiocitose de células de Langerhans com acometimento vulvar e com resposta terapêutica à talidomida: relato de caso Langerhans cells histiocytosis with vulvar involvement and responding to thalidomide therapy: case report

    Directory of Open Access Journals (Sweden)

    Lana Bezerra Fernandes

    2011-08-01

    Full Text Available A histiocitose de células de Langerhans é representante de um raro grupo de síndromes histiocitárias, sendo caracterizada pela proliferação das células de Langerhans. Suas manifestações variam de lesão solitária a envolvimento multissistêmico, sendo o acometimento vulvar incomum. Segue-se o relato de caso refratário da doença limitada à pele, em mulher de 57 anos. A paciente apresentava história de pápulas eritematosas ulceradas em couro cabeludo, face, vulva, tronco e axila há seis anos. O diagnóstico da doença é difícil, sendo confirmado neste caso através de estudo imuno-histoquímico e se obteve resposta terapêutica e eficaz, com a administração de talidomidaLangerhans cell histiocytosis is a member of a group of rare histiocytic syndromes and is characterized for the proliferation of histiocytes called Langerhans'cells. Its manifestations vary from a solitary injury to systemic involvement, and vulvar lesions are uncommon. We describe a refractory case of cutaneous limited disease in a 57-year-old woman. She presented with a 6-year history of an erythematous papular eruption of the scalp, face, vulva, trunk and axillae. The diagnosis is difficult and in this case it was confirmed through immunohistochemical study and clinical improvement was achieved with thalidomide

  20. Histiocitose de células de Langerhans: rápida resolução após cessação do tabagismo Langerhans-cell histiocytosis: rapid resolution after smoking cessation

    Directory of Open Access Journals (Sweden)

    José Miguel Chatkin

    2005-04-01

    Full Text Available Descreve-se um caso de histiocitose pulmonar de células de Langerhans com relação estreita entre cessação do tabagismo e melhora radiológica. A tomografia computadorizada de alta resolução revelava múltiplos nódulos pequenos localizados em lobos superiores e campos médios de ambos os pulmões. O exame microscópico dessas lesões mostrava infiltração histiocitária, que se corava fortemente pela proteína S100. Apesar de a remissão espontânea poder ser uma possível explicação para essa evolução, este relato de caso mostra um rápido desaparecimento das lesões após o abandono do tabagismo.We describe a case of pulmonary Langerhans cell histiocytosis with a close temporal relationship between smoking cessation and radiological improvement. High-resolution computed tomography revealed multiple small nodules located in the upper and middle lobes of both lungs. Microscopy of these lesions showed histiocytic infiltration that reacted strongly to staining for S100 protein. The histiocytes resembling Langerhans cells showed strong reactivity for S100 protein. Smoking cessation was recommended and the patient complied. Chest X-ray and computed tomography performed 6 and 24 months later revealed almost complete resolution of the radiographic abnormalities. Despite the possibility that this evolution was attributable to spontaneous remission, in this case, the lesions did, in fact, disappear rapidly after smoking cessation.

  1. Pulmonary histiocytosis X - imaging aspects of pulmonary involvement

    International Nuclear Information System (INIS)

    Pulmonary histiocytosis X is an idiopathic disease which is and uncommon but important cause of pulmonary fibrosis in young adults. Chest radiographs and high resolution computed tomographic (HRCT) scans of the lungs of 7 patients diagnosed as pulmonary histiocytosis X were examined retrospectively. The authors reviewed the pathologic, clinical and radiographic features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. Pulmonary histiocytosis X can be suspected on the basis of chest radiographic findings; predominantly upper lobe nodules and cysts present an increased sensitivity and are virtually pathognomonic of this disorder. Chest HRCT allows good assessment of the evolution of pulmonary histiocytosis X and is also valuable in distinguishing histiocytosis from other disorders that produces nodules or cysts. (author)

  2. Combined basal cell carcinoma and Langerhans cell histiocytosis of the scrotum in a patient with occupational exposure to coal tar and dust

    Energy Technology Data Exchange (ETDEWEB)

    Izikson, L.; Vanderpool, J.; Brodsky, G.; Mihm, M.C.; Zembowicz, A. [Harvard University, Boston, MA (US). Massachusetts General Hospital

    2004-09-01

    The patient was a 77-year-old male former smoker, with history of several basal cell carcinomas (BCCs) in sun-protected areas around the waistline, who presented with another small ulceration on the anterior right upper scrotum near the base of the penis. BCC was suspected clinically and the lesion was treated with cryosurgery. The tumor recurred, became raised, and began to bleed. An excisional biopsy was performed. It showed nodular BCC surrounded by a cellular proliferation of round histiocytic cells with convoluted, lobulated and reniform nuclei and abundant cytoplasm . The patient had no history of exposure to ionizing radiation, chemotherapy, immunosuppressive medications, prior lymphoma or other malignancy. However, he spent 4 years on a ship loading coal into the furnace of a steam engine, during which he slept in adjacent quarters that were covered with coal dust. Additionally, he had a several-year history of occupational skin exposure to machine oil, oil refinery waste, sulfur waste, hydraulic fluid, and asbestos. He also reported a history of nude sunbathing. The scrotal lesion was re-excised and the patient remains disease-free more than 1 year after the diagnosis.

  3. 160例成人朗格汉斯细胞组织细胞增生症患者临床特征分析%A clinicopathological analysis of 160 cases of adult Langerhans cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    许霞; 聂秀; 熊文; 潘华雄; 王威亚; 杨群培; 廖殿英; 刘卫平

    2015-01-01

    目的 观察成人朗格汉斯细胞组织细胞增生症(LCH)患者的临床特征,以提高其早期诊断与治疗水平.方法 对1992年1月至2013年12月间年龄≥18岁的160例LCH患者资料进行回顾性分析.结果 160例患者中男110例,女50例(男、女比例为2.2∶1),中位年龄33(18~73)岁.160例患者共有222个病变部位,包括骨骼172处(77.5%),淋巴结13处(5.8%),口腔8处(3.6%),皮肤及肝脏各5处(2.2%),眼眶及肺各4处(1.8%),胸锁关节3处(1.4%),消化道、耳及甲状腺各2处(0.9%),肾上腺及舌下腺各1处(0.5%).1个脏器受累150例(93.8%),2个以上脏器受累10例(6.2%).外院临床误诊77例(48.1%),其中骨肿瘤(包括骨巨细胞瘤、纤维结构不良、软骨母细胞瘤、骨母细胞瘤及骨肉瘤)28例,骨结核13例,脑膜瘤9例,骨囊肿、慢性骨髓炎及尿崩症各5例,皮肤病及恶性淋巴瘤各4例,皮肤慢性溃疡、慢性中耳炎、肺癌及口腔癌各1例.结论 成人LCH患者以男性居多,以单系统病灶占绝对优势,多发生于骨骼系统.成人LCH临床表现多样化,易于延误诊断.%Objectives To observe the clinical features of Langerhans cell histiocytosis (LCH),and to improve its early diagnosis and treatment.Methods Retrospective analysis of 160 cases of adult LCH from pathology department,West China Hospital of Sichuan University and Union Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 1992 to December 2013 were performed,and their clinical features were analyzed.Results Of 160 cases,there were 110 male and 50 female,the male to female ratio was 2.2∶1.The mean age was 35 (18-73) years.There were total 222 lesion sites,including 172 (77.5%) osteal lesions,followed by 13 (5.8%) lymph nodes and 8 (3.6%) oral cavity lesions.The other involved organs were skin(5,2.2%),liver(5,2.2%),fossa orbitalis (4,1.8%),lungs (4,1.8%),sternoclavicular joint (3,1.4

  4. Solitary pulmonary MALT lymphoma presenting crystal-storing histiocytosis.

    Science.gov (United States)

    Nagaharu, Keiki; Kageyama, Yuki; Watanabe, Takuya; Yamaguchi, Takanori; Ito, Ryugo; Baba, Youichirou; Masuya, Masahiro; Ohashi, Riuko; Kawakami, Keiki

    2016-08-01

    Crystal-storing histiocytosis (CSH) is characterized by the accumulation of large histiocytes with intracytoplasmic crystallized immunoglobulin and is typically associated with hematological malignancies. A 69-year-old man, who had a history of left nephrectomy and chemotherapy for renal pelvic cancer six years earlier, had received a CT scan every year thereafter and a small nodule was found in the left lower lobe of his lungs two years prior to the current presentation. Because of progression of this pulmonary nodule, he underwent pulmonary lobectomy on suspicion of lung cancer. He was ultimately diagnosed as having CSH accompanied by mucosa-associated lymphoid tissue lymphoma stage IAE. In the absence of further treatment, he has been well with no recurrence of the disease for 10 months postoperatively. Because CSH could reportedly be an initial presentation of hematological malignancies, careful observation and evaluation for the presence of these blood disorders is essential. PMID:27599420

  5. Hallazgos en el macizo facial y la base del cráneo de pacientes pediátricos con histiocitosis de células de Langerhans Craniofacial and skull base findings in Langerhans cell histiocytosis in pediatric patients

    Directory of Open Access Journals (Sweden)

    Daniel Forlino

    2013-03-01

    Full Text Available La histiocitosis de células de Langerhans (HCL es una entidad poco frecuente, con una incidencia anual de 2,6 a 5,4 por millón de niños en la población general. Tiene manifestaciones óseas (lesiones osteolíticas solitarias o múltiples en huesos planos, largos e irregulares o multisistémicas. Se describen los hallazgos imagenológicos de una serie retrospectiva de 17 pacientes pediátricos, de 1 a 12 años de edad, con histiocitosis de células de Langerhans en el macizo facial y la base del cráneo. Las manifestaciones incluyeron lesiones osteolíticas y masa de partes blandas, que ocupaban las cavidades adyacentes, como la órbita, los senos paranasales, la caja timpánica y la mastoides. En el maxilar inferior puede provocar reabsorción del reborde alveolar con apariencia de dientes flotantes. La complejidad anatómica del área de estudio requiere su valoración mediante tomografía computada (TC y resonancia magnética (RM con contraste. La histiocitosis de células de Langerhans debe considerarse dentro de los diagnósticos diferenciales de las lesiones del macizo facial y la base del cráneo, especialmente en pacientes pediátricos.Langerhans cell histiocytosis (CLH is an uncommon entity, of unknown etiology, with an incidence of 2.6 at 5.4 per 1,000,000 children/year in the general population. It may have bone manifestations (solitary or multiple osteolytic lesions in flat, long and irregular bones or multiorgan manifestations. We report the radiological findings in retrospective series of 17 pediatric patients aged 1 to 12 years old, with CLH in craniofacial and skull base. Radiological findings included osteolytic and soft tissue lesions occupying the orbit, sinuses, tympanic cavity and mastoid. In the mandible, reabsorption of the alveolar ridge with the appearance of floating teeth was observed. The anatomical complexity of the area studied required evaluation by CT and MRI with contrast. LCH disease should be considered in

  6. MRI features of Langerhans cell histiocytosis in central nervous system in children%儿童中枢神经系统郎格汉斯细胞组织细胞增生症的MRI表现

    Institute of Scientific and Technical Information of China (English)

    曾洪武; 干芸根; 黄文献; 何玲; 刘鸿圣

    2016-01-01

    Objective To explore the MRI features of Langerhans cell histiocytosis (LCH) in central nervous system (CNS) in children. Methods Clinical and MRI data of 25 cases with LCH in CNS from three children's hospitals between January 2009 and December 2014 were retrospectively studied. All cases were confirmed by surgery or biopsy. All cases underwent non⁃contrast and contrast pituitary and/or cranial MRI examinations. The location, morphology, MRI signal and enhanced patterns of LCH lesions were observed and analyzed. Result A total of 17 cases had hypothalamic-pituitary LCH, with 2 of them complicated with pineal cyst, 2 complicated with brain parenchymal lesions, and one complicated with both pineal cyst and brain parenchymal lesions. MR images showed that neurohypophysis lost its original hyper⁃intensity on T1WI, and nodular or homogeneous thickening was seen in the pituitary stalks. Dura matter was involved in 3 cases, 2 of them had single lesion, and the other one got multiple lesions. Neoplasm in choroid plexus was seen in trigone of left lateral ventricles in one case. Three cases with pineal gland involved demonstrated cystic change. Four cases had gray matter involved, with cerebellar dentate nuclei involvement in 2 cases, and both thalamus and basal ganglia involvement in the other two cases. Three cases showed white matter involvement without obvious Virchow-Robin space enlargement and brain atrophy. Conclusions MR imaging of LCH in CNS shows certain specific characteristics. The diagnosis can be made comprehensively based on both clinic features and other imaging findings.%目的:探讨儿童中枢神经系统郎格汉斯细胞组织细胞增生症(LCH)的MRI表现。方法回顾性分析2009年1月至2014年12月3家儿童医院经手术或穿刺活检证实的中枢神经系统LCH病例25例,所有病例均行垂体和(或)头颅MR平扫及增强扫描。重点观察神经系统受累病灶部位、形态、MRI信号变化及强化情况

  7. Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis and familial Rosai-Dorfman disease.

    Directory of Open Access Journals (Sweden)

    Neil V Morgan

    2010-02-01

    Full Text Available The histiocytoses are a heterogeneous group of disorders characterised by an excessive number of histiocytes. In most cases the pathophysiology is unclear and treatment is nonspecific. Faisalabad histiocytosis (FHC (MIM 602782 has been classed as an autosomal recessively inherited form of histiocytosis with similarities to Rosai-Dorfman disease (RDD (also known as sinus histiocytosis with massive lymphadenopathy (SHML. To elucidate the molecular basis of FHC, we performed autozygosity mapping studies in a large consanguineous family and identified a novel locus at chromosome 10q22.1. Mutation analysis of candidate genes within the target interval identified biallelic germline mutations in SLC29A3 in the FHC kindred and in two families reported to have familial RDD. Analysis of SLC29A3 expression during mouse embryogenesis revealed widespread expression by e14.5 with prominent expression in the central nervous system, eye, inner ear, and epithelial tissues including the gastrointestinal tract. SLC29A3 encodes an intracellular equilibrative nucleoside transporter (hENT3 with affinity for adenosine. Recently germline mutations in SLC29A3 were also described in two rare autosomal recessive disorders with overlapping phenotypes: (a H syndrome (MIM 612391 that is characterised by cutaneous hyperpigmentation and hypertrichosis, hepatomegaly, heart anomalies, hearing loss, and hypogonadism; and (b PHID (pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome. Our findings suggest that a variety of clinical diagnoses (H and PHID syndromes, FHC, and familial RDD can be included in a new diagnostic category of SLC29A3 spectrum disorder.

  8. Bilateral pulmonary nodules after the successful treatment of a mediastinal seminoma

    OpenAIRE

    Encarnacion Santos Reche; Gema Bruixola; Carmen Salvador; Nuria Mancheño; Oscar Niño; Corina Escoin; Jorge Aparicio

    2015-01-01

    Germ cell tumors are unusual, but they represent the most common neoplasm in young men. Since the introduction of cisplatin-based chemotherapy, most cases are expected to be cured today. Intensive monitoring of these patients during follow-up is required to rule out relapses or late complications of therapy. We present the case of a 21-year-old male who developed extensive lung dissemination by Langerhans cell histiocytosis eight months after the successful treatment of a bulky mediastinal se...

  9. MRI features of neurodegenerative Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    CNS complications of LCH include ''space occupying'' lesions corresponding to histiocytic granulomas and ''neurodegenerative'' presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH. (orig.)

  10. MRI features of neurodegenerative Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Martin-Duverneuil, N.; Guillevin, R.; Chiras, J. [GH Pitie-Salpetriere, 47 Bd de l' Hopital, Department of Neuroradiology, Paris (France); Idbaih, A.; Hoang-Xuan, K. [GH Pitie-Salpetriere, 47 Bd de l' Hopital, Department of Neurology, Paris (France); Donadieu, J. [GH Trousseau, Department of Hematology/Oncology Pediatrics, Paris (France); Genereau, T. [Groupe d' etude des Histiocytoses langerhansiennes, Nantes (France)

    2006-09-15

    CNS complications of LCH include ''space occupying'' lesions corresponding to histiocytic granulomas and ''neurodegenerative'' presentation (ND-LCH) characterized by a progressive cerebellar ataxia. Studies analyzing specifically the MRI presentation of ND-LCH are scarce. We present here the MRIs of 13 patients registered as isolated ND-LCH. Posterior fossa was involved in 12 patients (92%), showing a symmetrical T2 hyperintensity of the cerebellar white matter areas in seven cases with a circumscribed T1 hyperintensity of the dentate nuclei in five cases, definite hyperintense T2 areas in the adjacent pontine tegmentum white matter in nine cases associated with a hyperintensity of the pontine pyramidal tracts in four cases. A cerebellar atrophy was noted in eight cases. The supratentorial region was involved in 11 patients, showing T2 hyperintense lesions in the cerebral white matter in eight cases and a discrete symmetrical T1 hyperintense signal in the globus pallidus in eight patients. A diffuse cortical atrophy was present in three cases and a marked focal atrophy of the corpus callosum in three cases. This series allows us to establish a not previously reported evocative semeiologic MR presentation to precisely orientate to the diagnosis of the pure neurodegenerative form of LCH. (orig.)

  11. Histiocytosis X and Bronchopulmonary Adenocarcinoma: A Rare Coexistence

    Directory of Open Access Journals (Sweden)

    Akýn Kaya

    2002-01-01

    Full Text Available There exists a rarely observed association between pulmonary histiocytosis X and bronchopulmonary cancer. However, the frequency of bronchopulmonary cancer in these patients is higher than in the general population. A 28-year-old patient who currently smokes ten packs of cigarettes a year came to our department of pneumology with complains of cough and hemoptysis. An x-ray of the thorax revealed bilateral cysts and a shadow in the upper part of the right pulmonary field. In addition, a chest tomography showed multiple cysts dispersed throughout the two pulmonary fields and an irregular mass with a diameter of four centimetres in the upper right lobe. Bronchopulmonary adenocarcinoma was diagnosed during a cytologic exam of the bronchial washing. We decided to perform a thoracotomy on the patient, since there was no far metastasis. An upper lobectomy and wedge resection of the upper segment of the lower right lobe, which had been invaded by the tumour, were performed. Histology confirmed the diagnosis of adenocarcinoma. A pulmonary biopsy was carried out on the tumour-free site and showed the presence of histiocytosis X. There is a hypothesis that a neoplasm developed on the pulmonary fibrosis could be an epiphenomenon of bronchopulmonary cancer in patients who smoke and have pulmonary histiocytosis X. It is interesting to note that histiocytosis X and bronchopulmonary cancer were diagnosed at the same time, since the bronchopulmonary cancer may have occurred within a few years following the diagnosis of histiocytosis X, even if she was a smoker. Hemoptysis, which is found in 5% of patients with histiocytosis X, may suggest cancer. This young patient, a smoker, who complained of hemoptysis, is a particularly rare case of the association between pulmonary histiocytosis X and bronchopulmonary cancer whose pathogenesis is not clear cut. It is thus important to note that smoking can have major consequences, even in young people.

  12. Treatment Of Sickle Cell Disease

    KAUST Repository

    Essack, Magbubah

    2014-12-04

    The present invention includes embodiments for treatment and/or prevention of sickle cell disease that employ Hydroxyfasudil or Isocoronarin D alone or either in conjunction with each other or an inducer of HbF production. The compounds may act synergistically, and the compounds employed circumvent the side effects seen with Hydroxyurea.

  13. Hairy Cell Leukemia Treatment Option Overview

    Science.gov (United States)

    ... Childhood ALL Treatment Childhood AML Treatment Research Hairy Cell Leukemia Treatment (PDQ®)–Patient Version General Information About Hairy Cell Leukemia Go to Health Professional Version Key Points ...

  14. Treatment Option Overview (Renal Cell Cancer)

    Science.gov (United States)

    ... Genetics of Kidney Cancer Research Renal Cell Cancer Treatment (PDQ®)–Patient Version General Information About Renal Cell ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  15. Treatment Options for Renal Cell Cancer

    Science.gov (United States)

    ... Genetics of Kidney Cancer Research Renal Cell Cancer Treatment (PDQ®)–Patient Version General Information About Renal Cell ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  16. Stem Cell Treatments: What to Ask

    Science.gov (United States)

    ... Search Toggle Nav Stem Cell Treatments: What to Ask You have the right to a full explanation ... Cells and Medicine > Stem Cell Treatments: What to Ask There is certain information you should look into ...

  17. Metastatic renal cell cancer treatments.

    Science.gov (United States)

    Sternberg, C N

    2003-01-01

    Metastatic renal cell carcinoma has been considered to be resistant to chemotherapy, with responses observed in only limited numbers of patients. For this reason, therapeutic options have ranged from no treatment, to immunotherapy with cytokines such as IL-2 and interferon-alpha, chemotherapy alone or in combination with cytokines, and to a variety of new investigational approaches. Interferon and interleukin-2 (IL-2) have led to long-term survival in selected patients. Immunotherapy with cytokines, monoclonal antibodies, new agents, dendritic cell therapy, and allotransplantation offer promise. Novel therapeutic strategies include combining cytokines, and antiangiogenic approaches such as thalidomide and antivascular endothelial growth factor therapy. Pathologic, cytogenic and molecular studies have proven that renal cell carcinoma is not a single tumor entity. Efforts to improve results also include the identification of prognostic factors, which allow treatment to be better directed towards those patients most likely to benefit. Increasing understanding of cancer biology is beginning to allow for a more targeted approach to the therapy of metastatic renal cell carcinoma. Adequate positioning of known treatments is essential and many trials of new targeted therapies are underway. PMID:14988745

  18. Pulmonary histiocytosis X - imaging aspects of pulmonary involvement; Histiocitose X - aspectos radiologicos do acometimento pulmonar

    Energy Technology Data Exchange (ETDEWEB)

    Sabedotti, Ismail Fernando; Maeda, Lucimara; Ferreira, Daniel Miranda; Montandon, Cristiano; Marins, Jose Luiz C. [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia

    1999-06-01

    Pulmonary histiocytosis X is an idiopathic disease which is and uncommon but important cause of pulmonary fibrosis in young adults. Chest radiographs and high resolution computed tomographic (HRCT) scans of the lungs of 7 patients diagnosed as pulmonary histiocytosis X were examined retrospectively. The authors reviewed the pathologic, clinical and radiographic features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. Pulmonary histiocytosis X can be suspected on the basis of chest radiographic findings; predominantly upper lobe nodules and cysts present an increased sensitivity and are virtually pathognomonic of this disorder. Chest HRCT allows good assessment of the evolution of pulmonary histiocytosis X and is also valuable in distinguishing histiocytosis from other disorders that produces nodules or cysts. (author)

  19. Treatment Option Overview (Extragonadal Germ Cell Tumors)

    Science.gov (United States)

    ... following PDQ summaries: Ovarian Germ Cell Tumors Treatment Testicular Cancer Treatment Age and gender can affect the risk ... summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and ...

  20. Cutaneous reactive histiocytosis in dogs: a retrospective evaluation of 32 cases.

    Science.gov (United States)

    Palmeiro, Brian S; Morris, Daniel O; Goldschmidt, Michael H; Mauldin, Elizabeth A

    2007-10-01

    Thirty-two cases of canine cutaneous histiocytosis were retrospectively evaluated. Median age at onset was 4 years. Lesions included nodules and plaques affecting the head/face, trunk and limbs, and erythema, swelling and depigmentation of the nasal planum/nares. Systemic involvement was not ruled out in all cases. All dogs had complete resolution of dermatological lesions after initial treatment (median 45 days). Initial treatment included prednisone +/- antibiotics (12 of 32 dogs), prednisone and tetracycline/niacinamide (four of 32), prednisone and azathioprine (three of 32), tetracycline/niacinamide +/- vitamin E/essential fatty acids (six of 32), antibiotics +/- antihistamines (three of 32), cyclosporine and ketoconazole (one of 32), topical therapy (two of 32), and no treatment (one of 32). Seventeen dogs received maintenance therapy which consisted of tetracycline/niacinamide +/- vitamin E/essential fatty acids (12 of 17), cyclosporine/ketoconazole (two to three times a week) (two of 17), azathioprine daily (one of 17), prednisone/azathioprine (two times a week) (one of 17), and prednisone daily (one of 17). Median follow up was 25 months. Nine dogs had a recurrence of cutaneous histiocytosis (median days to recurrence 130 days), with seven of nine having more than one recurrence. At study completion, six dogs were deceased (no lesions at the time of death) and 26 of 32 were alive with no lesions. Ten of 26 dogs were on maintenance treatment (eight tetracycline/niacinamide, one azathioprine, one vitamin E). Previous dermatological disease and season had no detectable influence on recurrence. Recurrence was significantly more likely in dogs with nasal planum/nares lesions than dogs without these lesions. Tetracycline/niacinamide was an effective treatment option for dogs in this study population. PMID:17845621

  1. Treatment Option Overview (Small Cell Lung Cancer)

    Science.gov (United States)

    ... Cancer Prevention Lung Cancer Screening Research Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Small Cell Lung Cancer Go to Health Professional Version Key Points Small ...

  2. Retinal stem cells and potential cell transplantation treatments.

    Science.gov (United States)

    Lin, Tai-Chi; Hsu, Chih-Chien; Chien, Ke-Hung; Hung, Kuo-Hsuan; Peng, Chi-Hsien; Chen, Shih-Jen

    2014-11-01

    The retina, histologically composed of ten delicate layers, is responsible for light perception and relaying electrochemical signals to the secondary neurons and visual cortex. Retinal disease is one of the leading clinical causes of severe vision loss, including age-related macular degeneration, Stargardt's disease, and retinitis pigmentosa. As a result of the discovery of various somatic stem cells, advances in exploring the identities of embryonic stem cells, and the development of induced pluripotent stem cells, cell transplantation treatment for retinal diseases is currently attracting much attention. The sources of stem cells for retinal regeneration include endogenous retinal stem cells (e.g., neuronal stem cells, Müller cells, and retinal stem cells from the ciliary marginal zone) and exogenous stem cells (e.g., bone mesenchymal stem cells, adipose-derived stem cells, embryonic stem cells, and induced pluripotent stem cells). The success of cell transplantation treatment depends mainly on the cell source, the timing of cell harvesting, the protocol of cell induction/transplantation, and the microenvironment of the recipient's retina. This review summarizes the different sources of stem cells for regeneration treatment in retinal diseases and surveys the more recent achievements in animal studies and clinical trials. Future directions and challenges in stem cell transplantation are also discussed. PMID:25238708

  3. Current treatments for renal cell carcinoma

    OpenAIRE

    Noble, Helen; Walsh, Ian

    2015-01-01

    Renal cell carcinoma (RCC), also known as kidney cancer, renal adenocarcinoma or hypernephroma, and metastatic renal cell carcinoma is a global burden. This article aims to provide a brief overview of RCC. It outlines epidemiology and presentation; invesitgation and staging; treatments and prognosis. The article also includes a focus on currently available drug treatments, and serves as an introduction to the topic.

  4. Pulmonary Langerhans Histiocytosis: an uncommon cause of interstitial pneumonia in a patient with Sjögren syndrome.

    Science.gov (United States)

    González García, Andrés; Callejas Rubio, José Luis; Ríos Fernández, Raquel; Ortego Centeno, Norberto

    2016-03-01

    Sjögren syndrome is a chronic, systemic, and autoimmune disorder that targets exocrine glands by remarkable B cell hyperactivity. Eventually, it is associated with extra-glandular clinical manifestations that affect essentially any organ system, including pulmonary involvement. Interstitial lung disease is one of the most serious pulmonary complications, and the early diagnosis is essential to initiate a prompt therapy. On the other hand, Sjögren syndrome could present concomitantly with several rheumatologic diseases such as systemic lupus erythematosus or rheumatoid arthritis. Pulmonary Langerhans Histiocytosis is a rare clonal proliferative disease characterized by pulmonary involvement by cells phenotypically similar to Langerhans cells. We describe the case of a nonsmoker 62-year-old woman with Sjögren syndrome who presented concomitantly a Pulmonary Langerhans Histiocytosis mimicking a pulmonary complication of its Sjögren. Fortunately, she had a well response to corticosteroids and azathioprine regimen. The aim of the paper is to emphasize the importance of the good differential diagnosis related to the pulmonary involvement. To the best of our knowledge, this is the first description of these two entities in the literature. PMID:25894436

  5. Treatment of Aggressive NK-Cell Leukemia

    DEFF Research Database (Denmark)

    Boysen, Anders Kindberg; Jensen, Paw; Johansen, Preben;

    2011-01-01

    Aggressive NK-cell leukemia is a rare malignancy with neoplastic proliferation of natural killer cells. It often presents with constitutional symptoms, a rapid declining clinical course, and a poor prognosis with a median survival of a few months. The disease is usually resistant to cytotoxic...... literature concerning treatment of aggressive NK-cell leukemia....

  6. Basal cell carcinoma-treatment with cryosurgery

    Directory of Open Access Journals (Sweden)

    Kaur S

    2003-03-01

    Full Text Available Basal cell carcinoma is a common cutaneous malignancy, frequently occurring over the face in elderly individuals. Various therapeutic modalities are available to treat these tumors. We describe three patients with basal cell carcinoma successfully treated with cryosurgery and discuss the indications and the use of this treatment modality for basal cell carcinomas.

  7. Basal cell carcinoma-treatment with cryosurgery

    OpenAIRE

    Kaur S; Thami G; Kanwar A

    2003-01-01

    Basal cell carcinoma is a common cutaneous malignancy, frequently occurring over the face in elderly individuals. Various therapeutic modalities are available to treat these tumors. We describe three patients with basal cell carcinoma successfully treated with cryosurgery and discuss the indications and the use of this treatment modality for basal cell carcinomas.

  8. Giant Cell Tumor: Role of Conservative Treatment

    Institute of Scientific and Technical Information of China (English)

    Anatolii Diedkov[1; Pavlo Kovalchuk[1; Marija Kukushkina[2; Sergey Bojchuk[1; Viktor Kostyuk[1

    2014-01-01

    Giant cell tumor is aggressive bone tumor. Surgical treatment is considered to be the only effective method of treatment ofthese tumors. The problem of inoperable patients with giant cell tumors is a challenge. A total of 8 patients had giant cell bone tumorsof pelvis and sacrum. 3 patients were treated by bisphosphonates, radiation therapy and embolization of tumor-nutrient arteries. 5patients received denosumab. The efficiency was assessed according to clinical data and CT scan control. Median follow up is 28months. All 8 patients had reduction of pain intensity. Treatment with denosumab demonstrated more than 30% tumor regression. Allof the patients are in remission.

  9. Pathological Consequence of Misguided Dendritic Cell Differentiation in Histiocytic Diseases

    OpenAIRE

    Berres, Marie-Luise; Allen, Carl E.; Merad, Miriam

    2013-01-01

    Histiocytic disorders represent a group of complex pathologies characterized by the accumulation of histiocytes, an old term for tissue-resident macrophages and dendritic cells. Langerhans cell histiocytosis is the most frequent of histiocytosis in humans and has been thought to arise from the abnormal accumulation of epidermal dendritic cells called Langerhans cells. In this chapter, we discuss the origin and differentiation of Langerhans cells and dendritic cells and present accumulated evi...

  10. Stem cell treatment of degenerative eye disease

    Directory of Open Access Journals (Sweden)

    Ben Mead

    2015-05-01

    Full Text Available Stem cell therapies are being explored extensively as treatments for degenerative eye disease, either for replacing lost neurons, restoring neural circuits or, based on more recent evidence, as paracrine-mediated therapies in which stem cell-derived trophic factors protect compromised endogenous retinal neurons from death and induce the growth of new connections. Retinal progenitor phenotypes induced from embryonic stem cells/induced pluripotent stem cells (ESCs/iPSCs and endogenous retinal stem cells may replace lost photoreceptors and retinal pigment epithelial (RPE cells and restore vision in the diseased eye, whereas treatment of injured retinal ganglion cells (RGCs has so far been reliant on mesenchymal stem cells (MSC. Here, we review the properties of non-retinal-derived adult stem cells, in particular neural stem cells (NSCs, MSC derived from bone marrow (BMSC, adipose tissues (ADSC and dental pulp (DPSC, together with ESC/iPSC and discuss and compare their potential advantages as therapies designed to provide trophic support, repair and replacement of retinal neurons, RPE and glia in degenerative retinal diseases. We conclude that ESCs/iPSCs have the potential to replace lost retinal cells, whereas MSC may be a useful source of paracrine factors that protect RGC and stimulate regeneration of their axons in the optic nerve in degenerate eye disease. NSC may have potential as both a source of replacement cells and also as mediators of paracrine treatment.

  11. Ovarian Germ Cell Tumors Treatment

    Science.gov (United States)

    ... c) cancer cells are found in the pelvic peritoneum. In stage I , cancer is found in one ... in the abdomen ) or in washings of the peritoneum ( tissue lining the peritoneal cavity). Stage II Enlarge ...

  12. Malignant histiocytosis X. Report of a rapidly fatal case in an elderly man.

    Science.gov (United States)

    Wood, C; Wood, G S; Deneau, D G; Oseroff, A; Beckstead, J H; Malin, J

    1984-07-15

    A 71-year-old white man developed an increasing number of 1-to-10 mm, erythematous nodules, many with central ulceration, most prominent on the head and trunk. Biopsy of a nodule showed infiltration of the dermis and epidermis by large cells with multilobulated nuclei and numerous mitoses. Electron microscopy showed that most tumor cells contained Langerhans' cell granules. Immunohistochemical studies demonstrated a pattern of antigen expression similar to that of Langerhans' cells including Ia and Leu-6 (T6) antigens. Chest x-ray showed diffuse pulmonary infiltration and similar tumor cells were present in the sputum and urine. He developed increasing dyspnea and jaundice despite chemotherapy, and died 6 months after the onset of the disease. Autopsy showed massive tumor infiltration of the lungs, liver, spleen, and lymph nodes, and focal involvement of the myocardium, skin and bladder. Clinical and cytologic features indicated this case to be a rare example of highly malignant histiocytosis X in an elderly man. PMID:6202393

  13. Bilateral pulmonary nodules after the successful treatment of a mediastinal seminoma

    Directory of Open Access Journals (Sweden)

    Encarnacion Santos Reche

    2015-03-01

    Full Text Available Germ cell tumors are unusual, but they represent the most common neoplasm in young men. Since the introduction of cisplatin-based chemotherapy, most cases are expected to be cured today. Intensive monitoring of these patients during follow-up is required to rule out relapses or late complications of therapy. We present the case of a 21-year-old male who developed extensive lung dissemination by Langerhans cell histiocytosis eight months after the successful treatment of a bulky mediastinal seminoma.  

  14. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Priya; Babyn, Paul [University of Toronto, Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)

    2008-07-15

    Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date. (orig.)

  15. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease

    International Nuclear Information System (INIS)

    Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date. (orig.)

  16. Treatment Options for Plasma Cell Neoplasms (Including Multiple Myeloma)

    Science.gov (United States)

    ... Treatment Health Professional Plasma Cell Neoplasms Treatment Research Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Patient Version General Information About Plasma Cell Neoplasms Go to Health Professional Version Key ...

  17. Application of whole-body bone scintigraphy in diagnosis and staging of Langerhans cell histiocytosis in infants%全身骨显像在婴幼儿朗格汉斯组织细胞增多症诊断和分期中的应用

    Institute of Scientific and Technical Information of China (English)

    丁献敏; 刘保平; 韩星敏; 程兵; 阮翘

    2011-01-01

    目的 探讨全身骨显像在婴幼儿朗格汉斯组织细胞增多症(LCH)诊断和分期中的应用价值.方法 对20例经手术病理或骨髓穿刺、皮疹压片细胞学确诊为LCH的婴幼儿行全身骨显像检查,对骨显像阳性者加行局部X线或CT检查.利用ROI技术计算全身骨显像阳性病灶的F/N比值并求出位于相同部位病灶的F/N比值均值.结果 20例患儿全身骨显像10例阳性,4例为多发病灶,6例为单发病灶.单纯颅骨放射分布异常3例,颅骨合并其他部位骨3例,肩胛骨2例,肋骨1例,下肢骨1例;单发颅骨病灶的F/N比值均值为0.36,长骨病灶的F/N比值均值为3.52.结论 全身骨显像有助于婴幼儿LCH的诊断及分期.%Objective To assess the application value of whole-body bone scintigraphy in diagnosis and staging of Langerhans cell histiocytosis (LCH) in infants. Methods Whole-body bone scintigraphy was performed in 20 infants of LCH confirmed with surgical pathology or bone marrow aspiration and laboratory data. The infants with positive bone scintigraphy underwent X-ray or CT, and the F/N value of positive lesions and lesions located in the same area were calculated by ROI technology. Results Ten of 20 patients showed positive in bone scans, including 4 with multi-focal lesions and 6 with single lesion. Among 10 patients with positive scintigrams, there were 3 patients with abnormal bone uptakes limited to cranial, 3 with cranial and other parts of abnormal bone uptakes, 2 with scapula abnormal uptakes, as well as rib and lower limb bone involvement (each n= 1). The average ratios of F/N on cranial alone was 0. 36, while focused on long bones was 3. 52. Conclusion Whole-body bone scintigraphy is useful in the diagnosis and staging of LCH in infants.

  18. CZTSSe thin film solar cells: Surface treatments

    Science.gov (United States)

    Joglekar, Chinmay Sunil

    Chalcopyrite semiconducting materials, specifically CZTS, are a promising alternative to traditional silicon solar cell technology. Because of the high absorption coefficient; films of the order of 1 micrometer thickness are sufficient for the fabrication of solar cells. Liquid based synthesis methods are advantageous because they are easily scalable using the roll to roll manufacturing techniques. Various treatments are explored in this study to enhance the performance of the selenized CZTS film based solar cells. Thiourea can be used as a sulfur source and can be used to tune band gap of CZTSSe. Bromine etching can be used to manipulate the thickness of sintered CZTSSe film. The etching treatment creates recombination centers which lead to poor device performance. Various after treatments were used to improve the performance of the devices. It was observed that the performance of the solar cell devices could not be improved by any of the after treatment steps. Other surface treatment processes are explored including KCN etching and gaseous H2S treatments. Hybrid solar cells which included use of CIGS nanoparticles at the interface between CZTSSe and CdS are also explored.

  19. Radiofrequency treatment alters cancer cell phenotype

    Science.gov (United States)

    Ware, Matthew J.; Tinger, Sophia; Colbert, Kevin L.; Corr, Stuart J.; Rees, Paul; Koshkina, Nadezhda; Curley, Steven; Summers, H. D.; Godin, Biana

    2015-07-01

    The importance of evaluating physical cues in cancer research is gradually being realized. Assessment of cancer cell physical appearance, or phenotype, may provide information on changes in cellular behavior, including migratory or communicative changes. These characteristics are intrinsically different between malignant and non-malignant cells and change in response to therapy or in the progression of the disease. Here, we report that pancreatic cancer cell phenotype was altered in response to a physical method for cancer therapy, a non-invasive radiofrequency (RF) treatment, which is currently being developed for human trials. We provide a battery of tests to explore these phenotype characteristics. Our data show that cell topography, morphology, motility, adhesion and division change as a result of the treatment. These may have consequences for tissue architecture, for diffusion of anti-cancer therapeutics and cancer cell susceptibility within the tumor. Clear phenotypical differences were observed between cancerous and normal cells in both their untreated states and in their response to RF therapy. We also report, for the first time, a transfer of microsized particles through tunneling nanotubes, which were produced by cancer cells in response to RF therapy. Additionally, we provide evidence that various sub-populations of cancer cells heterogeneously respond to RF treatment.

  20. Prognostic factors for healing of bone lesions in histiocytosis X

    International Nuclear Information System (INIS)

    Healing characteristics and rates of resolution were retrospectively reviewed in 38 bone lesions in 18 patients with histiocytosis X. Signs which tended to indicate future healing were a change from a nontrabecular pattern to a trabecular pattern by 10 weeks, a nonsclerotic lesion to one with sclerosis by 13 weeks, and loss of distinct margins by 24 weeks. Complete healing then occurred within 36 to 40 weeks after development of these favorable signs. The earliest occurrence of a favorable sign was 6 weeks. A trabecular pattern was found in 90% of the lesions, outside the skull, which eventually healed. Fifty-eight percent (22 of 38) of the lesions completely healed in an average of 13.6 months. Five months was the most rapid occurrence of healing in any of our cases. (orig.)

  1. Glioma treatment strategies using mesenchymal stem cells

    International Nuclear Information System (INIS)

    Because of the growth characteristics of malignant gliomas that are highly invasive and deeply infiltrate the surrounding brain area; the surgical resection of these gliomas with preservation of neural functions is almost always noncurative. The residual tumor cells are usually resistant to standard adjuvant radio-chemotherapy, and therefore, the tumors inevitably recur after a certain period and finally cause the death of the patients. Neural and mesenchymal stem cells have been extensively studied for the development of new strategies for treating malignant gliomas because of these cells possess the intrinsic property of homing toward tumor cells. By using neural and mesenchymal stem cells as vehicles for drug carriers, it is possible to deliver anticancer drugs to the tumor cells that infiltrate functioning normal brain tissue and are difficult to remove. Several cytokines and suicide genes have been tested, and promising results have been reported in animal brain tumor models. However, further studies involving safety issues such as secondary cancer formation are required before human trials of stem cell therapies. In the present paper, the author has reviewed the recent concepts involved in the treatment of malignant gliomas with stem cells, especially mesenchymal stem cells that are much easier to obtain from the patients themselves. (author)

  2. The mantle cells lymphoma: a proposed treatment

    International Nuclear Information System (INIS)

    A literature review was performed on mantle cells lymphoma in the therapeutic schemes. The literature that has been used is published in journals of medicine specializing in hematology, oncology, radiation therapy, molecular biology and internal medicine. The literature review was performed to propose a scheme of treatment according to Costa Rica. Epigenetic alterations have been revealed in patients with mantle lymphoma on current researches. The mantle lymphoma pathology has been described in various forms of clinical and histological presentation, stressing the importance of detailing the different methods and diagnostic reports. Working groups have proposed and developed various chemotherapy regimens and concluded that CHOP alone is without effect in mantle cell lymphoma unlike R-hyper-CVAD, CHOP / DHAP, high-dose Ara-C. Researchers have tried to develop new treatments based vaccines, use of modified viruses, specific monoclonal antibodies. The classic treatment has been triple intrathecal therapy. The central nervous system has been one of the most momentous sites of mantle cell lymphoma infiltration because poorer patient prognosis

  3. Pulmonary histiocytosis-X: high-resolution CT findings correlated with pulmonary function test

    International Nuclear Information System (INIS)

    The purposes of this study are to describe the high-resolution computed tomographic (HRCT) findings of pulmonary histiocytosis X and to evaluate the relationship between the extent of the lesion on HRCT and pulmonary function test (PFT). We retrospectively analyzed the HRCT findings of 8 patients with pathologically proved pulmonary histiocytosis X. The morphological pattern and incidence of the lesions were evaluated. The extent of the lesions were correlated with the results of PFT by calculating Spearman correlation. The most common HRCT findings were thick-walled cysts (n = 8) followed by nodules (n = 7). Other associated findings were reticulation, ground-glass opacification, and conglomerate nodules. The extent of the disease on HRCT was correlated inversely with percent predicted value of functional vital capacity. Common HRCT findings of pulmonary histiocytosis X were cysts with irregular wall thickening and nodules. The extent of the disease on HRCT correlated with the severity of restrictive pattern of PFT

  4. High expression of markers of apoptosis in Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Petersen, Bodil Laub; Lundegaard, Pia Rengtved; Bank, M I;

    2003-01-01

    develop organ dysfunction. In these cases the prognosis is poor. Our objective has been to study the immunohistochemical expression of Fas and Fas-ligand (Fas-L) in order to determine whether the level of expression of these proteins could predict the outcome of the disease. We also wanted to determine...

  5. Radionuclide bone scintigraphy in patients with histiocytosis X

    International Nuclear Information System (INIS)

    The term histiocytosis X (HX) refers to a spectrum of disorders varying from unifocal eosinophilic granuloma (UEG), multifocal eosinophilic granuloma (MEG), to the Abt-Letterer-Siwe syndrome. In a series of 16 patients with different types of HX and skeletal lesions, whole body bone scintigraphy was performed at the time of diagnosis and during follow up. Results were compared with radiographic findings. In patients with MEG with or without extra-skeletal dissemination bone scintigraphy revealed cold spots or hot spots, but half of the lesions were not visualised scintigraphically, resulting in false negative scans. In UEG the lesions were visualised as areas of increased uptake or as a cold spot with increased uptake at its borders. No false negative scans were encountered. The reliability of skeletal scintigraphy in patients with HX seems to depend on the type of the disorder: in UEG bone scintigraphy is a safe procedure. In MEG false negative bone scans have to be expected, and radiography is superior. (orig.)

  6. Nonsurgical Treatment Options for Basal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Mary H. Lien

    2011-01-01

    Full Text Available Basal cell carcinoma (BCC remains the most common form of nonmelanoma skin cancer (NMSC in Caucasians, with perhaps as many as 2 million new cases expected to occur in the United States in 2010. Many treatment options, including surgical interventions and nonsurgical alternatives, have been utilized to treat BCC. In this paper, two non-surgical options, imiquimod therapy and photodynamic therapy (PDT, will be discussed. Both modalities have demonstrated acceptable disease control rates, cosmetically superior outcomes, and short-term cost-effectiveness. Further studies evaluating long-term cure rates and long-term cost effectiveness of imiquimod therapy and PDT are needed.

  7. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) presenting with skeletal lesions

    International Nuclear Information System (INIS)

    A 2-year-old child presenting with bone pain and bone lesions was found to have sinus histiocytosis with massive lymphadenopathy (SHML). SHML presenting with skeletal symptoms is unusual. Management has been conservative and the child has been symptom free for 30 months, although the bone lesions have not completely regressed. (orig.)

  8. Emerging surgical treatments for renal cell carcinoma.

    Science.gov (United States)

    Husain, Fatima Z; Badani, Ketan K; Sfakianos, John P; Mehrazin, Reza

    2016-04-01

    Treatment of renal cell carcinoma has evolved considerably over the last few years. While total nephrectomy is necessary at times, nephron-sparing surgery, with a goal of renal function preservation, should always be considered. Although open partial nephrectomy is considered the gold standard approach for nephron-sparing surgery, laparoscopic- or robotic-assisted techniques allow urologists to perform renal surgery less invasively, with excellent long-term oncological outcomes. Cryotherapy and radiofrequency ablation are less invasive management approaches for carefully selected patients with small renal masses. Active surveillance should be considered in elderly or patients who are unfit for surgery. Ultimately, the method chosen for management of a renal mass is an informed decision made by the physician and patient. PMID:26892144

  9. Treatment Resistance Mechanisms of Malignant Glioma Tumor Stem Cells

    Directory of Open Access Journals (Sweden)

    Philip G.R. Schmalz

    2011-02-01

    Full Text Available Malignant gliomas are highly lethal because of their resistance to conventional treatments. Recent evidence suggests that a minor subpopulation of cells with stem cell properties reside within these tumors. These tumor stem cells are more resistant to radiation and chemotherapies than their counterpart differentiated tumor cells and may underlie the persistence and recurrence of tumors following treatment. The various mechanisms by which tumor stem cells avoid or repair the damaging effects of cancer therapies are discussed.

  10. Treatment Resistance Mechanisms of Malignant Glioma Tumor Stem Cells

    OpenAIRE

    Schmalz, Philip G.R.; Park, John K.; Shen, Michael J.

    2011-01-01

    Malignant gliomas are highly lethal because of their resistance to conventional treatments. Recent evidence suggests that a minor subpopulation of cells with stem cell properties reside within these tumors. These tumor stem cells are more resistant to radiation and chemotherapies than their counterpart differentiated tumor cells and may underlie the persistence and recurrence of tumors following treatment. The various mechanisms by which tumor stem cells avoid or repair the damaging effects o...

  11. Stem cell technology and engineering for cancer treatment

    OpenAIRE

    Sinh Truong Nguyen; Phuc Van Pham

    2015-01-01

    Stem cells are not only widely used for regenerative medicine, but are also considered as a useful tool for cancer treatment. For a long time, stem cells have been utilized to renew the immune system for radiation or chemotherapy treated patients. Recently, stem cells are being engineered to carry therapeutic reagents to target tumor sites. Cancer vaccines based on the knowledge of cancer stem cells have been studied and applied for cancer treatment. Induced pluripotent stem cells have been u...

  12. Treatment Resistance Mechanisms of Malignant Glioma Tumor Stem Cells

    International Nuclear Information System (INIS)

    Malignant gliomas are highly lethal because of their resistance to conventional treatments. Recent evidence suggests that a minor subpopulation of cells with stem cell properties reside within these tumors. These tumor stem cells are more resistant to radiation and chemotherapies than their counterpart differentiated tumor cells and may underlie the persistence and recurrence of tumors following treatment. The various mechanisms by which tumor stem cells avoid or repair the damaging effects of cancer therapies are discussed

  13. Treatment Option Overview (Non-Small Cell Lung Cancer)

    Science.gov (United States)

    ... Prevention Lung Cancer Screening Research Non-Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Non-Small Cell Lung Cancer Go to Health Professional Version Key Points Non- ...

  14. Treatment Options by Stage (Small Cell Lung Cancer)

    Science.gov (United States)

    ... Cancer Prevention Lung Cancer Screening Research Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Small Cell Lung Cancer Go to Health Professional Version Key Points Small ...

  15. Treatment Options by Stage (Non-Small Cell Lung Cancer)

    Science.gov (United States)

    ... Prevention Lung Cancer Screening Research Non-Small Cell Lung Cancer Treatment (PDQ®)–Patient Version General Information About Non-Small Cell Lung Cancer Go to Health Professional Version Key Points Non- ...

  16. Genetically Modified T Cells for the Treatment of Malignant Disease

    OpenAIRE

    Wieczorek, Agnieszka; Uharek, Lutz

    2013-01-01

    The broaden application of adoptive T-cell transfer has been constrained by the technical abilities to isolate and expand antigen-specific T cells potent to selectively kill tumor cells. With the recent progress in the design and manufacturing of cellular products, T cells used in the treatment of malignant diseases may be regarded as anticancer biopharmaceuticals. Genetical manipulation of T cells has given T cells desired specificity but also enable to tailor their activation and proliferat...

  17. Clinical characteristics and survival of children with Langerhans cell hystiocytosis

    Directory of Open Access Journals (Sweden)

    Krstovski Nada

    2008-01-01

    Full Text Available INTRODUCTION Langerhans cell histiocytosis is a rare disease in children, initial presentation is variable, clinical course, prognosis and survival are mostly unpredictable. OBJECTIVE To summarise clinical characteristics and treatment results in children with Langerhans cell histiocytosis. METHOD Retrospectively there were analyzed patients with LCH diagnosed and treated at Hematology Department of University Children's Hospital in Belgrade from 1990 to 2006. Clinical presentation, therapy and survival according to Kaplan-Meier's statistical test was analysed. RESULTS 30 patients were treated, aged from 4 months to 14 years, mean 3.9 years, median 2.3 years, 18 (60% males, 12 (40% females. A single system disease was diagnosed in 16 (53% patients, of whom 6 patients with multifocal bone disease. All patients were in complete remission averagely following162 and 82 months respectively. Multisystem disease was found in 14 (47% patients. The lymph nodes and skin were more frequently involved organs than the central nervous system (diabetes insipidus, lung, liver and spleen. The number of involved organs ranged from 2 to 8, mean 4.2. Four patients died due to disease progression 3, 16, 36 and 66 months after diagnosis. Nine patents with multisystem disease were in remission with 117 months of follow-up. One patient was lost on follow-up. CONCLUSION The clinical course of patients with a single system disease is usually benign while a multisystem disease has to be aggressively treated with precise initial evaluation and staging before therapy.

  18. Stem Cell Therapy in Treatment of Different Diseases

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Sahraian

    2012-02-01

    Full Text Available Stem cells are undifferentiated cells with the ability of proliferation, regeneration, conversion to differentiated cells and producing various tissues. Stem cells are divided into two categories of embryonic and adult. In another categorization stem cells are divided to Totipotent, Multipotent and Unipotent cells.So far usage of stem cells in treatment of various blood diseases has been studied (such as lymphoblastic leukemia, myeloid leukemia, thalassemia, multiple myeloma and cycle cell anemia. In this paper the goal is evaluation of cell therapy in treatment of Parkinsons disease, Amyotrophic lateral sclerosis, Alzheimer, Stroke, Spinal Cord Injury, Multiple Sclerosis, Radiation Induced Intestinal Injury, Inflammatory Bowel Disease, Liver Disease, Duchenne Muscular Dystrophy, Diabetes, Heart Disease, Bone Disease, Renal Disease, Chronic Wounds, Graft-Versus-Host Disease, Sepsis and Respiratory diseases. It should be mentioned that some disease that are the target of cell therapy are discussed in this article.

  19. Ionomycin Treatment Renders NK Cells Hyporesponsive

    OpenAIRE

    Gema Romera-Cárdenas; L Michael Thomas; Sheila Lopez-Cobo; Eva M García-Cuesta; Eric O Long; Reyburn, Hugh T.

    2016-01-01

    Natural killer cells are cytotoxic lymphocytes important in immune responses to cancer and multiple pathogens. However, chronic activation of NK cells can induce a hyporesponsive state. The molecular basis of the mechanisms underlying the generation and maintenance of this hyporesponsive condition are unknown, thus an easy and reproducible mechanism able to induce hyporesponsiveness on human NK cells would be very useful to gain understanding of this process. Human NK cells treated with ionom...

  20. 78 FR 44575 - Sickle Cell Disease Treatment Demonstration Program

    Science.gov (United States)

    2013-07-24

    ... HUMAN SERVICES Health Resources and Services Administration Sickle Cell Disease Treatment Demonstration... Services (HHS). ACTION: Request for Class Deviation for Non-Competitive Extension: Sickle Cell Disease... nine programs that are funded through competitive grant awards under the Sickle Cell Disease...

  1. LITERATURE REVIEW ON STEM CELL TREATMENT & ORAL SUBMUCOUS FIBROSIS (OSMF

    Directory of Open Access Journals (Sweden)

    Prathipaty James

    2015-08-01

    Full Text Available Stem cell therapy is a part of regenerative medicine that involves the use of undifferentiated cells in order to cure the disease. Stem cell - based therapies are being investigated for the treatment of many conditions, including neurodegenerative conditions such as Parkinson's disease, cardiovascular disease, liver disease, diabetes, autoimmune diseases and for nerve regeneration. (1 In orofacial region these therapies are being used for tooth and periodontal regeneration, temporomandibular joint reconstruction, alve olar bone regeneration. Craniofacial stem cells including dental pulp derived stem cells have the potential to cure a number of diseases. Present day treatment modalities for oral mucosal lesions like ulcerative lesions, premalignancies and malignancies mainly consist of steroids and antioxidants (which provide only a short term and symptomatic relief and surgery with or without chemo/radiotherapy (which leave the patient with certain amount of morbidity. Advances in stem cell technology have opened new vistas for treatment of these lesions. Various studies have shown the successful role of stem cell therapies in the treatment of precancerous conditions, oral ulcers, wounds and mucositis. (2 The recent concept of cancer stem cells (CSCs has directed sci entific communities toward a new area of research and possible potential treatment modalities for oral cancer. (3 The present article will discuss the role of stem cell applications in oral mucosal lesions. KEYWORDS: R eview - stem cells - properties - types - appl ications - role in osmf - results

  2. Stem Cell Treatment for Type 1 Diabetes

    Directory of Open Access Journals (Sweden)

    MingLi

    2014-03-01

    Full Text Available Type 1 diabetes mellitus (T1DM is a common chronic disease in children, characterized by a loss of  cells, which results in defects in insulin secretion and hyperglycemia. Chronic hyperglycemia causes diabetic complications, including diabetic nephropathy, neuropathy and retinopathy. Curative therapies mainly include diet and insulin administration. Although hyperglycemia can be improved by insulin administration, exogenous insulin injection cannot successfully mimic the insulin secretion from normal  cells, which keeps blood glucose levels within the normal range all the time. Islet and pancreas transplantation achieves better glucose control, but there is a lack of organ donors. Cell based therapies have also been attempted to treat T1DM. Stem cells such as embryonic stem cells, induced pluripotent stem cells and tissue stem cells (TSCs such as bone marrow-, adipose tissue- and cord blood-derived stem cells, have been shown to generate insulin-producing cells. In this review, we summarize the most-recently available information about T1DM and the use of TSCs to treat T1DM.

  3. Ionomycin Treatment Renders NK Cells Hyporesponsive.

    Directory of Open Access Journals (Sweden)

    Gema Romera-Cárdenas

    Full Text Available Natural killer cells are cytotoxic lymphocytes important in immune responses to cancer and multiple pathogens. However, chronic activation of NK cells can induce a hyporesponsive state. The molecular basis of the mechanisms underlying the generation and maintenance of this hyporesponsive condition are unknown, thus an easy and reproducible mechanism able to induce hyporesponsiveness on human NK cells would be very useful to gain understanding of this process. Human NK cells treated with ionomycin lose their ability to degranulate and secrete IFN-γ in response to a variety of stimuli, but IL-2 stimulation can compensate these defects. Apart from reductions in the expression of CD11a/CD18, no great changes were observed in the activating and inhibitory receptors expressed by these NK cells, however their transcriptional signature is different to that described for other hyporesponsive lymphocytes.

  4. Ionomycin Treatment Renders NK Cells Hyporesponsive.

    Science.gov (United States)

    Romera-Cárdenas, Gema; Thomas, L Michael; Lopez-Cobo, Sheila; García-Cuesta, Eva M; Long, Eric O; Reyburn, Hugh T

    2016-01-01

    Natural killer cells are cytotoxic lymphocytes important in immune responses to cancer and multiple pathogens. However, chronic activation of NK cells can induce a hyporesponsive state. The molecular basis of the mechanisms underlying the generation and maintenance of this hyporesponsive condition are unknown, thus an easy and reproducible mechanism able to induce hyporesponsiveness on human NK cells would be very useful to gain understanding of this process. Human NK cells treated with ionomycin lose their ability to degranulate and secrete IFN-γ in response to a variety of stimuli, but IL-2 stimulation can compensate these defects. Apart from reductions in the expression of CD11a/CD18, no great changes were observed in the activating and inhibitory receptors expressed by these NK cells, however their transcriptional signature is different to that described for other hyporesponsive lymphocytes. PMID:27007115

  5. Stem Cell Therapy for Treatment of Ocular Disorders

    Directory of Open Access Journals (Sweden)

    Padma Priya Sivan

    2016-01-01

    Full Text Available Sustenance of visual function is the ultimate focus of ophthalmologists. Failure of complete recovery of visual function and complications that follow conventional treatments have shifted search to a new form of therapy using stem cells. Stem cell progenitors play a major role in replenishing degenerated cells despite being present in low quantity and quiescence in our body. Unlike other tissues and cells, regeneration of new optic cells responsible for visual function is rarely observed. Understanding the transcription factors and genes responsible for optic cells development will assist scientists in formulating a strategy to activate and direct stem cells renewal and differentiation. We review the processes of human eye development and address the strategies that have been exploited in an effort to regain visual function in the preclinical and clinical state. The update of clinical findings of patients receiving stem cell treatment is also presented.

  6. Stem Cell Therapy for Treatment of Ocular Disorders

    Science.gov (United States)

    Sivan, Padma Priya; Syed, Sakinah; Mok, Pooi-Ling; Higuchi, Akon; Murugan, Kadarkarai; Alarfaj, Abdullah A.; Munusamy, Murugan A.; Awang Hamat, Rukman; Umezawa, Akihiro; Kumar, Suresh

    2016-01-01

    Sustenance of visual function is the ultimate focus of ophthalmologists. Failure of complete recovery of visual function and complications that follow conventional treatments have shifted search to a new form of therapy using stem cells. Stem cell progenitors play a major role in replenishing degenerated cells despite being present in low quantity and quiescence in our body. Unlike other tissues and cells, regeneration of new optic cells responsible for visual function is rarely observed. Understanding the transcription factors and genes responsible for optic cells development will assist scientists in formulating a strategy to activate and direct stem cells renewal and differentiation. We review the processes of human eye development and address the strategies that have been exploited in an effort to regain visual function in the preclinical and clinical state. The update of clinical findings of patients receiving stem cell treatment is also presented. PMID:27293447

  7. Current treatment approach to non-clear cell renal carcinoma

    OpenAIRE

    I. V. Tsimafeyeu

    2015-01-01

    Non-clear cell renal cell carcinoma has various histologic subtypes. Tumor biology plays significant role in the disease development. However, despite the one surgical approach both to clear cell and non-clear cell renal carcinoma, patients’ outcomes within one stage of the disease may vary. Furthermore, tumor sensitivity and its response to therapy are highly dependent on the same histologic subtype.The article gives detailed data on the current treatment of papillary, chromophobe and other ...

  8. Multifunctional Nucleic Acids for Tumor Cell Treatment

    DEFF Research Database (Denmark)

    Pofahl, Monika; Wengel, Jesper; Mayer, Günter

    2014-01-01

    We report on a multifunctional nucleic acid, termed AptamiR, composed of an aptamer domain and an antimiR domain. This composition mediates cell specific delivery of antimiR molecules for silencing of endogenous micro RNA. The introduced multifunctional molecule preserves cell targeting, anti......-proliferative and antimiR function in one 37-nucleotide nucleic acid molecule. It inhibits cancer cell growth and induces gene expression that is pathologically damped by an oncomir. These findings will have a strong impact on future developments regarding aptamer- and antimiR-related applications for tumor...

  9. Treatment Option Overview (Ovarian Germ Cell Tumors)

    Science.gov (United States)

    ... c) cancer cells are found in the pelvic peritoneum. In stage I , cancer is found in one ... in the abdomen ) or in washings of the peritoneum ( tissue lining the peritoneal cavity). Stage II Enlarge ...

  10. Nine Things to Know About Stem Cell Treatments

    Science.gov (United States)

    ... with their treatments. 7 Patient testimonials and other marketing provided by clinics may be misleading It can ... Resources About the ISSCR Get Involved Media © 2015 International Society for Stem Cell Research Terms of Use ...

  11. Training stem cells for treatment of malignant brain tumors

    Institute of Scientific and Technical Information of China (English)

    Shengwen; Calvin; Li; Mustafa; H; Kabeer; Long; T; Vu; Vic; Keschrumrus; Hong; Zhen; Yin; Brent; A; Dethlefs; Jiang; F; Zhong; John; H; Weiss; William; G; Loudon

    2014-01-01

    The treatment of malignant brain tumors remains a challenge. Stem cell technology has been applied in the treatment of brain tumors largely because of the ability of some stem cells to infiltrate into regions within the brain where tumor cells migrate as shown in preclinical studies. However, not all of these efforts can translate in the effective treatment that improves the quality of life for pa-tients. Here, we perform a literature review to identify the problems in the field. Given the lack of efficacy of most stem cell-based agents used in the treatment of malignant brain tumors, we found that stem cell distribution(i.e., only a fraction of stem cells applied capable of targeting tumors) are among the limiting factors. We provide guidelines for potential improvements in stem cell distribution. Specifically, we use an engineered tissue graft platform that replicates the in vivo microenvironment, and provide our data to validate that this culture platform is viable for producing stem cells that have better stem cell distribution than with the Petri dish culture system.

  12. Stem cells show promising results for lymphoedema treatment

    DEFF Research Database (Denmark)

    Toyserkani, Navid Mohamadpour; Quaade, Marlene Louise; Sheikh, Søren Paludan;

    2015-01-01

    Abstract Lymphoedema is a debilitating condition, manifesting in excess lymphatic fluid and swelling of subcutaneous tissues. Lymphoedema is as of yet still an incurable condition and current treatment modalities are not satisfactory. The capacity of mesenchymal stem cells to promote angiogenesis......, secrete growth factors, regulate the inflammatory process, and differentiate into multiple cell types make them a potential ideal therapy for lymphoedema. Adipose tissue is the richest and most accessible source of mesenchymal stem cells and they can be harvested, isolated, and used for therapy in a...... single stage procedure as an autologous treatment. The aim of this paper was to review all studies using mesenchymal stem cells for lymphoedema treatment with a special focus on the potential use of adipose-derived stem cells. A systematic search was performed and five preclinical and two clinical...

  13. Bortezomib for the treatment of mantle cell lymphoma: an update.

    Science.gov (United States)

    Hambley, Bryan; Caimi, Paolo F; William, Basem M

    2016-08-01

    Bortezomib is a first in class proteasome inhibitor, initially approved by the US Food and Drug Administration for the treatment of plasma cell myeloma. Bortezomib has been approved for the treatment of relapsed and refractory mantle cell lymphoma (MCL) and, more recently, in the upfront setting as well. Treatment algorithms for MCL have rapidly evolved over the past two decades, and the optimal regimen remains to be defined. The choice of treatment regimen is based on disease risk stratification models, the expected toxicity of antineoplastic agents, the perceived patient ability to tolerate the planned treatments and the availability of novel agents. As new drugs with novel mechanisms of action and variable toxicity profiles come into use, treatment decisions for a given patient have become increasingly complex. This article provides an overview of the evolving use of bortezomib in the rapidly changing management landscape of MCL. PMID:27493710

  14. Volume changes of human endothelial cells induced by photodynamic treatment

    Science.gov (United States)

    Leunig, Andreas; Staub, Frank; Plesnila, Nick; Peters, Jurgen; Feyh, Jens; Gutmann, Ralph; Goetz, Alwin E.

    1996-01-01

    Photodynamic therapy (PDT) has shown promising results in treatment of malignant tumors. However, the mechanisms leading to tumor destruction during PDT are still not completely understood. In addition to effects on the microcirculation, damage to cellular structures has been observed following exposure of cells to PDT. A phenomenon preceding these events might possibly be cell swelling. We therefore studied the influence of treatment with Photofrin (PF) and laser light on volume changes and cell viability of endothelial cells. Endothelial cells were obtained from human umbilical cord veins (HUVEC) by an adaption of the method of Maruyama (1963). After subcultivation the cells were harvested and transferred as a cell suspension into a specially designed incubation chamber. Cells received either PF in concentrations of 1.5 or 3.0 (mu) g/ml and laser illumination (630 nm; 40 mW/cm2, 4 Joule), PF alone, or laser treatment only. Following start of PF incubation and after phototreatment cell samples were taken for volume measurements using flow cytometry and for studies of cellular morphology using scanning electron microscopy. Simultaneously, cell viability was monitored by the trypan blue exclusion test and colorimetric MTT assay. (abstract truncated)

  15. Dasatinib and Doxorubicin Treatment of Sarcoma Initiating Cells: A Possible New Treatment Strategy

    DEFF Research Database (Denmark)

    Aggerholm-Pedersen, Ninna; Demouth, Christina; Safwat, Akmal;

    2016-01-01

    . However, inhibition with the TKI dasatinib in combination with doxorubicin decreased cell viability of the hMSC-TERT20-CE8 cell line. Conclusion. Our results demonstrate that dasatinib, but not EGFR-directed treatment, can decrease cell viability of stromal cancer stem cells less sensitive to doxorubicin.......Background. One of the major challenges affecting sarcoma treatment outcome, particularly that of metastatic disease, is resistance to chemotherapy. Cancer-initiating cells are considered a major contributor to this resistance. Methods. An immortalised nontransformed human stromal (mesenchymal......) stem cell line hMSC-TERT4 and a transformed cell line hMSC-TERT20-CE8, known to form sarcoma-like tumours when implanted in immune-deficient mice, were used as models. Receptor tyrosine kinase (RTK) activation was analysed by RTK arrays and cellular viability after tyrosine kinases inhibitor (TKI...

  16. Stem cells: novel players in the treatment of erectile dysfunction

    Institute of Scientific and Technical Information of China (English)

    Haiyang Zhang; Maarten Albersen; XunboJin; Guiting Lin

    2012-01-01

    Stem cells are defined by their capacity for both self-renewal and directed differentiation; thus,they represent great promise for regenerative medicine.Historically,stem cells have been categorized as either embryonic stem cells (ESCs) or adult stem cells (ASCs).It was previously believed that only ESCs hold the ability to differentiate into any cell type,whereas ASCs have the capacity to give rise only to cells of a given germ layer.More recently,however,numerous studies demonstrated the ability of ASCs to differentiate into cell types beyond their tissue origin.The aim of this review was to summarize contemporary evidence regarding stem cell availability,differentiation,and more specifically,the potential of these cells in the diagnosis and treatment of erectile dysfunction (ED) in both animal models and human research.We performed a search on PubMed for articles related to definition,Iocalisation and circulation of stem cells as well as the application of stem cells in both diagnosis and treatment of ED.Strong evidence supports the concept that stem cell therapy is potentially the next therapeutic approach for ED.To date,a large spectrum of stem cells,including bone marrow mesenchymal stem cells,adipose tissue-derived stem cells and muscle-derived stem cells,have been investigated for neural,vascular,endothelial or smooth muscle regeneration in animal models for ED.In addition,several subtypes of ASCs are localized in the penis,and circulating endogenous stem cells can be employed to predict the outcome of ED and ED-related cardiovascular diseases.

  17. TRAIL treatment provokes mutations in surviving cells

    OpenAIRE

    Lovric, M M; Hawkins, C J

    2010-01-01

    Chemotherapy and radiotherapy commonly damage DNA and trigger p53-dependent apoptosis through intrinsic apoptotic pathways. Two unfortunate consequences of this mechanism are resistance due to blockade of p53 or intrinsic apoptosis pathways, and mutagenesis of non-malignant surviving cells which can impair cellular function or provoke second malignancies. Death ligand-based drugs, such as tumor necrosis factor-related apoptosis inducing ligand (TRAIL), stimulate extrinsic apoptotic signaling,...

  18. LITERATURE REVIEW ON STEM CELL TREATMENT & ORAL SUBMUCOUS FIBROSIS (OSMF)

    OpenAIRE

    Prathipaty James; Kameswararao

    2015-01-01

    Stem cell therapy is a part of regenerative medicine that involves the use of undifferentiated cells in order to cure the disease. Stem cell - based therapies are being investigated for the treatment of many conditions, including neurodegenerative conditions such as Parkinson's disease, cardiovascular disease, liver disease, diabetes, autoimmune diseases and for nerve regeneration. (1) In orofacial region these therapies are being used for tooth and periodonta...

  19. Superficial treatment of mammalian cells using plasma needle

    Energy Technology Data Exchange (ETDEWEB)

    Stoffels, E; Kieft, I E; Sladek, R E J [Department of Biomedical Engineering, Eindhoven University of Technology, PO Box 513, 5600 MB Eindhoven (Netherlands)

    2003-12-07

    Interactions of a small-size, non-thermal plasma (plasma needle) with living cells in culture are studied. We have demonstrated the non-destructive character of the plasma needle: under moderate conditions (low-power and low concentration of molecular species) the plasma needle does not heat biological samples and does not induce cell death. Treatment of living cells is restricted to the cell exterior (membrane). As a result of the interactions of plasma radicals with cell adhesion molecules, cell attachment is temporarily interrupted; the loose cells can be removed, reattached or transferred. This effect may prove very useful in fine surgery, where a part of the tissue must be removed with high-precision, without damage to the adjacent cells and without inflammatory reaction.

  20. Aerosol-Based Cell Therapy for Treatment of Lung Diseases.

    Science.gov (United States)

    Kardia, Egi; Halim, Nur Shuhaidatul Sarmiza Abdul; Yahaya, Badrul Hisham

    2016-01-01

    Aerosol-based cell delivery technique via intratracheal is an effective route for delivering transplant cells directly into the lungs. An aerosol device known as the MicroSprayer(®) Aerosolizer is invented to transform liquid into an aerosol form, which then can be applied via intratracheal administration for drug delivery. The device produces a uniform and concentrated distribution of aerosolized liquid. Using the capability of MicroSprayer(®) Aerosolizer to transform liquid into aerosol form, our group has designed a novel method of cell delivery using an aerosol-based technique. We have successfully delivered skin-derived fibroblast cells and airway epithelial cells into the airway of a rabbit with minimum risk of cell loss and have uniformly distributed the cells into the airway. This chapter illustrates the application of aerosol device to deliver any type of cells for future treatment of lung diseases. PMID:27062596

  1. Detection and Treatment of Mycoplasma Contamination in Cultured Cells

    Directory of Open Access Journals (Sweden)

    Hsuan Jung

    2003-04-01

    Full Text Available Background: Mycoplasmas, the smallest and simplest prokaryotes that reside in endosomesof mammalian cells, are widespread contaminants found in cell cultures.About 30% of all cell cultures, varying from 15 to 80%, are reportedlycontaminated with mycoplasmas. Here, we present our experience in successfullydetecting and treating mycoplasmal infection in various cell lines.Methods: The nested polymerase chain reaction (PCR detection and microscopicexamination, including phase-contrast, fluorescent, as well as differentialinterference contrast, were used for detecting potential mycoplasma contaminationof cell lines used in our laboratory. As soon as mycoplasma was identified,antibiotic treatment was initiated.Results: Mycoplasmal contamination was detected in six of 15 cell lines using thenested PCR amplification of mycoplasma DNA, which was further demonstratedusing 4, 6-Diamidino-2-phenylindole (DAPI staining and fluorescentmicroscopy. Alternate treatment with two antibiotics, macrolide (tiamulinand tetracycline (minocycline, effectively eliminated mycoplasma, whichwas validated by both PCR and microscopic studies.Conclusions: The nested PCR using genomic DNA extracted from cultured cells as templatesis a rapid and sensitive method for detecting mycoplasma contamination.Treatment with combined antibiotics can completely eradicatemycoplasmal infection from cultured cells. For the ease of use, PCR and/orDAPI staining appear suitable for detecting potential mycoplasmal contaminationin laboratories that rely heavily on the cell culture system.

  2. Transplantation of retinal pigment epithelial cells - a possible future treatment for age-related macular degeneration

    DEFF Research Database (Denmark)

    Wiencke, Anne Katrine

    2001-01-01

    ophthalmology, age-related macular degeneration, retinal pigment epithelial cells, transplantation, treatment......ophthalmology, age-related macular degeneration, retinal pigment epithelial cells, transplantation, treatment...

  3. Transplantation of retinal pigment epithelial cells - a possible future treatment for age-related macular degeneration

    DEFF Research Database (Denmark)

    Wiencke, Anne Katrine

    2001-01-01

    ophthalmology, age-related macular degeneration, transplantation, retinal pigment epithelial cells, treatment......ophthalmology, age-related macular degeneration, transplantation, retinal pigment epithelial cells, treatment...

  4. Asthma in Sickle Cell Disease: Implications for Treatment

    Directory of Open Access Journals (Sweden)

    Kathryn Blake

    2011-01-01

    Full Text Available Objective. To review issues related to asthma in sickle cell disease and management strategies. Data Source. A systematic review of pertinent original research publications, reviews, and editorials was undertaken using MEDLlNE, the Cochrane Library databases, and CINAHL from 1947 to November 2010. Search terms were [asthma] and [sickle cell disease]. Additional publications considered relevant to the sickle cell disease population of patients were identified; search terms included [sickle cell disease] combined with [acetaminophen], [pain medications], [vitamin D], [beta agonists], [exhaled nitric oxide], and [corticosteroids]. Results. The reported prevalence of asthma in children with sickle cell disease varies from 2% to approximately 50%. Having asthma increases the risk for developing acute chest syndrome , death, or painful episodes compared to having sickle cell disease without asthma. Asthma and sickle cell may be linked by impaired nitric oxide regulation, excessive production of leukotrienes, insufficient levels of Vitamin D, and exposure to acetaminophen in early life. Treatment of sickle cell patients includes using commonly prescribed asthma medications; specific considerations are suggested to ensure safety in the sickle cell population. Conclusion. Prospective controlled trials of drug treatment for asthma in patients who have both sickle cell disease and asthma are urgently needed.

  5. Stem Cell Therapies for Treatment of Liver Disease

    Directory of Open Access Journals (Sweden)

    Clara Nicolas

    2016-01-01

    Full Text Available Cell therapy is an emerging form of treatment for several liver diseases, but is limited by the availability of donor livers. Stem cells hold promise as an alternative to the use of primary hepatocytes. We performed an exhaustive review of the literature, with a focus on the latest studies involving the use of stem cells for the treatment of liver disease. Stem cells can be harvested from a number of sources, or can be generated from somatic cells to create induced pluripotent stem cells (iPSCs. Different cell lines have been used experimentally to support liver function and treat inherited metabolic disorders, acute liver failure, cirrhosis, liver cancer, and small-for-size liver transplantations. Cell-based therapeutics may involve gene therapy, cell transplantation, bioartificial liver devices, or bioengineered organs. Research in this field is still very active. Stem cell therapy may, in the future, be used as a bridge to either liver transplantation or endogenous liver regeneration, but efficient differentiation and production protocols must be developed and safety must be demonstrated before it can be applied to clinical practice.

  6. Bone Marrow Stem Cell as a Potential Treatment for Diabetes

    Directory of Open Access Journals (Sweden)

    Ming Li

    2013-01-01

    Full Text Available Diabetes mellitus (DM is a group of metabolic diseases in which a person has high blood glucose levels resulting from defects in insulin secretion and insulin action. The chronic hyperglycemia damages the eyes, kidneys, nerves, heart, and blood vessels. Curative therapies mainly include diet, insulin, and oral hypoglycemic agents. However, these therapies fail to maintain blood glucose levels in the normal range all the time. Although pancreas or islet-cell transplantation achieves better glucose control, a major obstacle is the shortage of donor organs. Recently, research has focused on stem cells which can be classified into embryonic stem cells (ESCs and tissue stem cells (TSCs to generate functional β cells. TSCs include the bone-marrow-, liver-, and pancreas-derived stem cells. In this review, we focus on treatment using bone marrow stem cells for type 1 and 2 DM.

  7. Cell Adhesion on Polycaprolactone Modified by Plasma Treatment

    Directory of Open Access Journals (Sweden)

    Nina Recek

    2016-01-01

    Full Text Available We have investigated the influence of various plasma treatments of electrospun polycaprolactone (PCL scaffolds on the adhesion and proliferation of human umbilical endothelial cells (HUVEC. The PCL scaffolds were treated in plasmas created in O2, NH3 or SO2 gas at identical conditions. Surface functionalization of plasma-treated samples was determined using X-ray photoelectron spectroscopy. Cell adhesion and morphology were investigated by scanning electron microscopy and the influence of plasma treatment on cell adhesion and viability was evaluated with cell viability assay (MTT assay. The results showed the highest metabolic activity of HUVEC on PCL samples treated with O2 and NH3 plasma. Accordingly, the cells reflected the best adhesion and morphology on O2 and NH3 plasma-treated PCL samples already at 3 h. Moreover, treatment with O2 and NH3 plasma even stimulated endothelial cell proliferation on PCL surfaces by 60% as measured at 24 h, showing significant improvement in endothelialization of this material. Contrarily, SO2 plasma appeared to be less promising in comparison with O2 and NH3 plasma; however, it was still better than without any plasma treatment. Thus, our results importantly contribute to the biocompatibility improvement of the PCL polymer, commonly used for scaffolds in tissue engineering.

  8. Cell size dynamics and viability of cells exposed to hypotonic treatment and electroporation for electrofusion optimization:

    OpenAIRE

    Hudej, Rosana; Kandušer, Maša; Miklavčič, Damijan; Trontelj, Katja; Ušaj, Marko

    2009-01-01

    Background. Various electrofusion parameters have to be adjusted to obtain theoptimal electrofusion efficiency. Based on published data, good electrofusion conditions can be achieved with the hypotonic treatment. However, the duration of the hypotonic treatment before electroporation and buffer hypoosmolarity have to be adjusted in order to cause cell swelling, to avoid regulatory volume decrease and to preserve cell viability. The aims of our study were to determine cell size dynamics and vi...

  9. Cell size dynamics and viability of cells exposed to hypotonic treatment and electroporation for electrofusion optimization

    OpenAIRE

    Trontelj, Katja; Kandušer, Maša; Miklavčič, Damijan; Hudej, Rosana; Ušaj, Marko

    2015-01-01

    Background. Various electrofusion parameters have to be adjusted to obtain theoptimal electrofusion efficiency. Based on published data, good electrofusion conditions can be achieved with the hypotonic treatment. However, the duration of the hypotonic treatment before electroporation and buffer hypoosmolarity have to be adjusted in order to cause cell swelling, to avoid regulatory volume decrease and to preserve cell viability. The aims of our study were to determine cell size dynamics and vi...

  10. CD34+ Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation for Non-Malignant Disease

    Science.gov (United States)

    2016-03-10

    Bone Marrow Failure Syndrome; Severe Aplastic Anemia; Severe Congenital Neutropenia; Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Schwachman Diamond Syndrome; Primary Immunodeficiency Syndromes; Acquired Immunodeficiency Syndromes; Histiocytic Syndrome; Familial Hemophagocytic Lymphocytosis; Lymphohistiocytosis; Macrophage Activation Syndrome; Langerhans Cell Histiocytosis (LCH); Hemoglobinopathies; Sickle Cell Disease; Sickle Cell-beta-thalassemia

  11. The excision width in surgical treatment of basal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Mališ M.

    2006-01-01

    Full Text Available Basal cell carcinoma originates from pluripotent cells of basal layer of epiderm, external covering of hair follicles, sebaceous glands or other skin adnexa. It is characterized by local infiltrating and sometimes destructive growth. There are several types of basal cell carcinomas that may be manifested in over 12 clinical forms. Surgical treatment depends to a large extent on the histological type, localization and its clinical manifestation. The analysis included 250 patients of both gender and different age, operated for basal cell carcinoma. Clinical characteristics of basal cell carcinoma and the width of the excision were described. It was concluded that the width of the excision of basal cell cancer was in relation to histological type. .

  12. Economics of Treatments for Non-Small Cell Lung Cancer

    OpenAIRE

    Christos Chouaid; Kukovi Atsou; Gilles Hejblum; Alain Vergnenegre

    2009-01-01

    The purpose of this article is to review the economics of treatments for non-small cell lung cancer (NSCLC). We systematically analysed the cost effectiveness of treatments for the different stages of NSCLC, with particular emphasis on more recently approved agents. Numerous economic analyses in NSCLC have been conducted, with a variety of methods and in a number of countries. In patients with localized disease, adjuvant chemotherapy appears to have greater cost effectiveness than observation...

  13. Sediment microbial fuel cells for wastewater treatment: challenges and opportunities

    OpenAIRE

    Xu, Bojun; Ge, Zheng; He, Zhen

    2015-01-01

    Sediment microbial fuel cells (SMFCs) have been intensively investigated for the harvest of energy from natural sediment, but studies of their application for wastewater treatment mainly occurred in the past 2-3 years. SMFCs with simple structures can generate electrical energy while decontaminating wastewater. Most SMFCs used for wastewater treatment contain plants to mimic constructed wetlands. Both synthetic and real wastewaters have been used as substrates in SMFCs that achieved satisfact...

  14. Carboplatin treatment of antiestrogen-resistant breast cancer cells

    DEFF Research Database (Denmark)

    Larsen, Mathilde S; Yde, Christina Westmose; Christensen, Ib J;

    2012-01-01

    sensitivity to carboplatin, as it was previously shown with cisplatin, and whether low Bcl-2 expression levels have a potential value as marker for increased carboplatin sensitivity. Breast cancer cells resistant to the pure antiestrogen fulvestrant, and two out of four cell lines resistant to the...... antiestrogen tamoxifen, were more sensitive to carboplatin treatment compared to the parental MCF-7 cell line. This indicates that carboplatin may be an advantageous treatment in antiestrogen‑resistant breast cancer; however, a marker for increased sensitivity would be needed. Low Bcl-2 expression was...... combination with Bcl-xL and Bax, could explain the observed responses to carboplatin in all tamoxifen‑resistant cell lines, indicating that more markers are needed to predict the response to carboplatin in tamoxifen‑resistant breast cancer....

  15. Stem cells for the treatment of musculoskeletal pain

    Institute of Scientific and Technical Information of China (English)

    Luminita Labusca; Florin Zugun-Eloae; Kaveh Mashayekhi

    2015-01-01

    Musculoskeletal-related pain is one of the most disablinghealth conditions affecting more than one third of theadult population worldwide. Pain from various mechanismsand origins is currently underdiagnosed and undertreated.The complexity of molecular mechanisms correlating painand the progression of musculoskeletal diseases is notyet fully understood. Molecular biomarkers for objectiveevaluation and treatment follow-up are needed as a steptowards targeted treatment of pain as a symptom or as adisease. Stem cell therapy is already under investigationfor the treatment of different types of musculoskeletalrelatedpain. Mesenchymal stem cell-based therapiesare already being tested in various clinical trials that usemusculoskeletal system-related pain as the primary orsecondary endpoint. Genetically engineered stem cells,as well as induced pluripotent stem cells, offer promisingnovel perspectives for pain treatment. It is possible that amore focused approach and reassessment of therapeuticgoals will contribute to the overall efficacy, as well asto the clinical acceptance of regenerative medicinetherapies. This article briefly describes the principal typesof musculoskeletal-related pain and reviews the stemcell-based therapies that have been specifically designedfor its treatment.

  16. Cell Adhesion on Polycaprolactone Modified by Plasma Treatment

    OpenAIRE

    Nina Recek; Matic Resnik; Helena Motaln; Tamara Lah-Turnšek; Robin Augustine; Nandakumar Kalarikkal; Sabu Thomas; Miran Mozetič

    2016-01-01

    We have investigated the influence of various plasma treatments of electrospun polycaprolactone (PCL) scaffolds on the adhesion and proliferation of human umbilical endothelial cells (HUVEC). The PCL scaffolds were treated in plasmas created in O2, NH3 or SO2 gas at identical conditions. Surface functionalization of plasma-treated samples was determined using X-ray photoelectron spectroscopy. Cell adhesion and morphology were investigated by scanning electron microscopy and the influence of p...

  17. Oral squamous cell carcinoma following treatment of acute lymphoblastic leukaemia

    International Nuclear Information System (INIS)

    With substantially increased survival after most paediatric cancers over the past decades have come the late sequelae of treatment. Of all late complications of treatment, second malignancies are generally considered to be the most serious. We report on a 20-year-old man with an oral squamous cell carcinoma 17 years after initial chemotherapy and irradiation for acute lymphoblastic leukaemia. Although occurrence of the oral malignancy in this patient could have been treatment-related, one should keep in mind that the occurrence of second tumours may also be based on a shared genetic aetiology. (au) 9 refs

  18. Microbial fuel cells as pollutant treatment units: Research updates.

    Science.gov (United States)

    Zhang, Quanguo; Hu, Jianjun; Lee, Duu-Jong

    2016-10-01

    Microbial fuel cells (MFC) are a device that can convert chemical energy in influent substances to electricity via biological pathways. Based on the consent that MFC technology should be applied as a waste/wastewater treatment unit rather than a renewable energy source, this mini-review discussed recent R&D efforts on MFC technologies for pollutant treatments and highlighted the challenges and research and development needs. Owing to the low power density levels achievable by larger-scale MFC, the MFC should be used as a device other than energy source such as being a pollutant treatment unit. PMID:26906446

  19. Oral squamous cell carcinoma following treatment of acute lymphoblastic leukaemia

    Energy Technology Data Exchange (ETDEWEB)

    Waal, R.I.F. van der; Waal, I. van der [Univ. Hospital Vrije Univ., Dept. of Oral and Maxillofacial Surgery/Oral Pathology, Amsterdam (Netherlands); Veerman, A.J.P. [Univ. Hospital Vrije Univ., Dept. of Paediatric Oncology, Amsterdam (Netherlands); Snow, G.B. [Univ. Hospital Vrije Univ., Dept. of Otorhinolaryngology, Amsterdam (Netherlands)

    1997-02-01

    With substantially increased survival after most paediatric cancers over the past decades have come the late sequelae of treatment. Of all late complications of treatment, second malignancies are generally considered to be the most serious. We report on a 20-year-old man with an oral squamous cell carcinoma 17 years after initial chemotherapy and irradiation for acute lymphoblastic leukaemia. Although occurrence of the oral malignancy in this patient could have been treatment-related, one should keep in mind that the occurrence of second tumours may also be based on a shared genetic aetiology. (au) 9 refs.

  20. Mesenchymal stem cells in diabetes treatment: progress and perspectives

    Directory of Open Access Journals (Sweden)

    Yu CHENG

    2016-08-01

    Full Text Available Diabetes is a chronic metabolic disorder caused by relative or absolute insulin deficient or reduced sensitivity of target cells to insulin. Mesenchymal stem cells (MSCs are adult stem cells with multiple differentiation potential, self-renewable and immunoregulatory properties. Accumulating evidences from clinic or animal experiments recent years showed that MSCs infusion could ameliorate hyperglycemia in diabetes. The research progress of MSCs in diabetes treatment is summarized and a corresponding perspective is herewith proposed in present paper. DOI: 10.11855/j.issn.0577-7402.2016.07.16