In langerhans cell histiocytosis formerly known as histiocytosis x, langerhans cells multiply abnormally. Plch may be a reactive process with a subset showing clonality, but extrapulmonary forms of langerhans cell histiocytosis are thought to be neoplastic. Radiation oncologyhistiocytosis wikibooks, open books for. We are intechopen, the worlds leading publisher of open access books. It is a proliferative disorder of monoclonal origin, but not necessarily neoplastic. Diagnosis of langerhans cell histiocytosis on fine needle. Langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Histiocyte tumors are among the more uncommon neoplasms affecting lymphoid tissues, probably representing less than 1% of all malignancies involving lymph nodes or soft tissues.
During childhood, langerhans cell histiocytosis peaks between ages 1 and 3. Langerhans cell histiocytosis lch of the female genital tract is rare. Dendritic cells are a form of histiocyte, or white blood cell. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. 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. Langerhans cell histiocytosis lch is a rare cancer that begins in lch cells. The langerhans cell displays immunoreactivity cytoplasmic and nuclear for s100 protein and the monoclonal antibody cd1a, hladr ia and peanut agglutinin. Langerhans cell histiocytosis histiocytosis x guide.
Langerhans cell histiocytosis lch formerly histiocytosis x is characterized by intense and abnormal proliferation of bone marrowderived histiocytes langerhans cells, together with a variable number of leucocytes, eosinophils, neutrophils, lymphocytes, plasma cells and giant multinucleated cells. Langerhans cell histiocytosis lch is the most common histiocytic disorder, characterized by the pathologic clonal proliferation and accumulation of immature langerhans cells within organs. People with lch produce too many langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. Of the reported cases, most have been treated successfully with surgery, radiotherapy, andor chemotherapy. Langerhans cell sarcoma arising from langerhans cell. In medicine, histiocytosis is an excessive number of histiocytes tissue macrophages, and the term is also often used to refer to a group of rare diseases which share this sign as a characteristic. Langerhans cell histiocytosis lch is a group of rare disorders histologically characterized by the proliferation of langerhans cells. Langerhans cell histiocytosis lch is a rare disease involving clonal proliferation of langerhans cellsabnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes.
Citescore measures average citations received per document published. Lch cells are a type of dendritic cell which fights infection. Targeting brafv600e in clonal lcs and their precursors is a potential treatment option for. Rollins1 1department of medical oncology, danafarber cancer institute, and department of medicine, brigham and womens hospital, harvard medical school, boston, massachusetts. They also occur in the papillary dermis, particularly around blood vessels. Selected aspects of the histopathology of langerhans cell histiocytosis representing diagnostic difficulty andor controversy are presented with emphasis on the composition of pathological lesions. Bohn, julie teruyafeldstein and sergio sanchezsosa. Langerhans cell sarcoma lcs typically presents as cytologic atypia and clinical aggressiveness and may involve multiple organs during the progression of the disease. Csf1r is required for differentiation and migration of. The nonlch consist of a long list of diverse disorders which have been dif. Langerhans cell histiocytosis is a clonal proliferation of cells that morphologically and immunophenotypically resemble langerhans cells more common in childhood 1 3 years old and involves nodal and extranodal sites most common site is bone. Langerhans cell histiocytosis lch is a rare disorder with heterogeneous clinical manifestations arico and egeler, 1998.
The genetics of langerhans cell histiocytosis italian. A case of langerhans cell histiocytosis with multifocal. These include mutations of the braf, map2k1, ras and araf genes. A case of multifocal langerhans cell histiocytosis in a twoyearold child is presented where fine needle aspiration was helpful in achieving a rapid and accurate diagnosis in an appropriate clinical and radiological setting. Despite this unified nosology, important questions about lch remain unanswered. It is characterized by the accumulation of pathologic langerhans cells, commonly in the bones, skin, liver, spleen, lungs, bone marrow, and brain. Langerhans cell histiocytosis lch describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Summary langerhans cell histiocytosis lch is a clonal disease characterized by the proliferation and accumulation of langerhans cells in a. Osteolytic lesions can lead to pathological fractures. Langerhans cell histiocytosis lch is a disease with a variable clinical manifestation, being localised ss or disseminated ms.
The langerhansnonlangerhans dichotomy noted in prior classification of. Plch is characterized by bronchiolocentric nodules andor cysts in an upper and. Gastrointestinal gi tract involvement by langerhans cell histiocytosis lch is a rare condition. Research that demonstrated langerhans cell involvement as well as histiocytes led to a proposal that the term langerhans cell histiocytosis lch be used in place of histiocytosis x. Nosology and pathology of langerhans cell histiocytosis.
Langerhans cell histiocytosis lch is an inflammatory neoplasm of. Ultrastructurally, the birbeck or x granule is straight or curved pentalaminary rod of variable length, often vesicular at one end frequently resembling a racquet. Langerhans cell sarcoma arising from langerhans cell histiocytosis. Langerhans cell histiocytosis genetics home reference nih. To date, no largescale studies have been done on how often lch occurs in. The histopathology of langerhans cell histiocytosis. It broadly fits into the category of histiocytoses. According to the histiocytosis association of america, 1 in 200,000 children in the united. The diagnosis of lch is confirmed by immunohistochemistry ihc by demonstrating the presence of dendritic cell markers such as s100 protein, in addition to cd1a and langerin. The highly characteristic common and rare cytological. Contrary to previous beliefs, recent literature reveals that the pathogenesis of lch might involve a clonal process implicating braf c. Primary skin lcs without any extracutaneous site association is extremely rare and only a few such cases have been described in the literature. In bone this may cause pain and adjacent softtissue swelling, but.
Langerhans cell histiocytosis and langerhans cell sarcoma. Occasionally and confusingly, the term histiocytosis is sometimes used to refer to individual diseases. However, a study from sweden shows a higher annual incidence of 8. Sometimes there are mutations changes in lch cells as they form. Langerhans cell histiocytosis lch is a heterogeneous disease, involving the accumulation of langerhans cells in various organs. Langerhans cell histiocytosis harpers textbook of pediatric. Variable clinical picture with single or multiple lesions or disseminated disease. The physicians perception of the disease varies considerably depending on their experience, the presentation of the disease or the shortterm treatment outcome. Publications biology and treatment of the histiocytosis. They can be found in the skin, lungs, stomach, bone, eyes and intestines. It is typically noted in male patients with systemic disease and is associated with both poor prognosis and high morbidity. Radiation oncologyhistiocytosis wikibooks, open books. A flow cytometric study of langerhans cell histiocytosis.
Gastrointestinal tract langerhans cell histiocytosis. Pubmed abstract allen ce, li l, peters tl, et al cell specific gene expression in langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal langerhans cells. Langerhans cell histiocytosis of the skin sciencedirect. Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of. The cytology of langerhans cell histiocytosis histiocytosis. The complexity of the lesions was due largely to the absence of data regarding clonality and genetic. Pathologic histiocytes in lch are mononucleated cells with coffee bean or. You may not embed one of our images on your web page without a link back to our site. Pathology multiple centrilobular, discrete nodular infiltrates peribronchiolar with stellate extension into surrounding alveolar walls. Isolated langerhans cell histiocytosis of the stomach. Composed of langerhans cells uniform, bland nuclei pale chromatin, thin nuclear membrane, inconspicuous nucleoli. These cells play a role in the bodys immune system.
The disease is characterised by formation of eosinophilic. Pathology of langerhans cell histiocytosis histiocytosis x. Langerhans cells are white blood cells in the immune system that normally play an important role in protecting the body against viruses, bacteria and other invaders. Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Langerhans cell histiocytosis of the female genital tract. This can avoid unnecessary biopsy and guide the management especially where access to histopathology is limited.
Langerhans cell histiocytosis is due to uncontrolled monoclonal proliferation of langerhans cells distinctive cells of monocytemacrophage lineage and should be considered a malignancy although its biological behaviour is very variable 1,3. Langerhans cell histiocytosis information from the histiocytosis association. Langerhans cell histiocytosis lch combines in one nosological category a group of diseases that have widely disparate clinical manifestations but are all characterized by accumulation of proliferating cells with surface markers and ultrastructural features similar to cutaneous langerhans cells lcs. Pulmonary langerhans cell histiocytosis harpreet s suri1, eunhee s yi2, gregorz s nowakowski3 and robert vassallo1,4 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. Langerhans cell histiocytosis lch is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions or skin rashes demonstrating infiltration with histiocytes with beanshaped nuclei on biopsy with or without histiocytic infiltration of extraskeletal tissues most notably the skin, lungs, lymph nodes, liver, spleen, bone marrow, brain.
This disease may involve the vulva, vagina, cervix, endometrium, and ovary. We present a case of unusual primary lcs in skin occurring in a middleaged male. Lch can occur at any age but is more frequent in the pediatric population. Langerhans cells are dendritic cells antigenpresenting immune cells of the skin, and contain organelles called birbeck granules. Sep 11, 2017 langerhans cell histiocytosis lch is a disorder that primarily affects children, but is also found in adults of all ages. Langerhans cell histiocytosis in children a disease with many. If you would like a large, unwatermarked image for your web page or blog, please purchase the appropriate license. Fascin, a 55kd actinbundling protein, represents a highly selective marker for dendritic cells of lymphoid tissues and peripheral blood and is involved in the formation of dendritic processes in maturing epidermal langerhans cells. This uptodate and comprehensive dvdrom disc provides a superb collection of authoritative documents from the nations cancer experts on childhood langerhans cell histiocytosis lch histiocytosis x, eosinophilic granuloma, abtletterersiwe, handschullerchristian, diffuse reticuloendotheliosis. Multiple organ systems can be affected, resulting in a spectrum of clinical manifestations. Langerhans cell histiocytosis genetic and rare diseases. Revised classification of histiocytoses and neoplasms of the.
It occurs predominantly in young smokers, without gender predominance. The clinical presentation of lch is highly variable, ranging from a singlesystem limited disease, to severe, multiorgan diseases with high mortality. Pulmonary langerhans cell histiocytosis surgical pathology. Langerhans cell histiocytosis, tongue langerhans cell histiocytosis, tongue. Uncontrolled clonal proliferatoin of langerhans cells. Involvement manifestation possible other condition skin vesicles and bullae most common in early infancy erythema toxicum herpes simplex chickenpox infection dermatitis most frequently scalp, diaper or axilla, may occur up to late infancy seborrheic dermatitis. A nonlangerhans cell histiocytosis, benign cephalic histiocytosis is characterized by scattered yellowbrown papules and macules concentrated on face, scalp, neck, and upper trunk that appear in the first 2 years of life, though typically after birth. Pulmonary langerhans cell histiocytosis an update from the pathologists perspective anja c. Lch usually affects children but very rarely involves adults.
Langerhans cell histiocytosis is occasionally misspelled as langerhan or langerhans cell histiocytosis, even in authoritative textbooks. Langerhans cell histiocytosis lch is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. Recent advances in the understanding of langerhans cell histiocytosis. Jan 22, 2020 langerhans cell histiocytosis is rare, affecting one in 250,000 children and one in 1 million adults in the united states. Langerhans cell histiocytosis lch is a rare disorder characterized by an abnormal proliferation of pathologic langerhans cells. The world health organization, according to the type of histiocyte involved, classifies this pathology into dendritic cell disorders, macrophagerelated disorders, and malignant histiocytic disorders. They are present in all layers of the epidermis and are most prominent in the stratum spinosum. In people with lch, these cells multiply excessively and build up in certain areas of the body. Isolated gastrointestinal involvement in lch is rare and usually presents in childhood as a multisystem disease. Lesional cell phenotypes and the factors influencing variations are noted. Either term refers to three separate illnesses listed in order of increasing severity. Later on, these cells were identified in all stratified squamous epithelium of mammals. Langerhans cell histiocytosis lch, or histiocytosis x, is now generally considered to be a nonmalignant condition.
Skin biopsy diagnosis of langerhans cell neoplasms intechopen. Langerhans cell histiocytosis lch and lymphangioleiomyomatosis lam. Symptoms range from isolated bone lesions to multisystem disease. Request pdf nosology and pathology of langerhans cell histiocytosis the classification of the histiocytoses has evolved based on new understanding of the cell of origin as a bone marrow. Report from the international registry of the histiocyte society. Tenyear experience at dallas childrens medical center. Dendritic cells dcs play an important role in local defense mechanisms in the epithelium. Langerhans cell histiocytosis lch is a rare histiocytic disorder of unknown etiopathogenesis.
Its clinical presentation is variable and ranges from isolated skin or bone disease to a lifethreatening multisystem condition. Aug 20, 2010 the median amount of langerhans cell histiocytosis versus rosaidorfman disease involvement was 7. Langerhans cell histiocytosis lch is a rare disorder in which the body makes too many dendritic cells. Clinical manifestations, pathologic features, and diagnosis of. Pulmonary langerhans cell histiocytosis plch is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. Differential diagnosis for manifestations of langerhans cell histiocytosis.
Although it can strike at any age, it is most common in children and young adults, with about 70% of cases occurring before age 17. A flow cytometric fcm study of a single case has, however, been published which claimed to provide evidence to contradict this. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver. Handschullerchristians disease, which involves multiple organ systems and, most typically, the skull base. Nodal involvement may be sole manifestation of disease or associated with systemic disease. The lesions of langerhans cell histiocytosis have a wide spectrum of histologic changes, but a constant feature is a sinusoidal proliferation of langerhans cells in the appropriate cellular background fig. Langerhans cell histiocytosis lch is a clonal disorder believed to be derived from cells of the dendritic system. Langerhans cells are a subset of antigenpresenting dendritic cells belonging to the histiocytic system and are classified into langerhans cell histiocytosis and langerhans cell sarcoma. Oral surg oral med oral pathol oral radiol endod 2005. Langerhans cell lc histiocytosis lch and lc sarcoma lcs constitute proliferative disorders of lcs. Langerhans cell histiocytosis shares some clinical and immunohistochemical features such as s100 expression with sinus histiocytosis. Medical management of langerhans cell histiocytosis from.
They are found in the skin, lymph nodes, spleen, bone marrow and lungs. The pathologic hallmark of plch is the accumulation of langerhans and. Multiple organs and systems may be involved by the disease. Langerhans cell histiocytosis epidemiology bmj best practice. The nature of lch and lcs has been actively discussed as exemplified by the title of an article, the langerhans cell histiocytosis x files revealed1 table 1. The langerhans cell is a dendritic antigenpresenting cell of bone marrow origin. Langerhans cell histiocytosis is occasionally misspelled as langerhan or langerhans cell histiocytosis, even in authoritative. The epidemiology of langerhans cell histiocytosis in children in denmark, 197589. Percutaneous techniques for the diagnosis and treatment of localized langerhans cell histiocytosis eosinophilic granuloma of bone. A case report langerhans cell sarcoma lcs is a neoplastic proliferation of langerhans cells that have overtly malignant cytologic features. It has been referred to by several eponyms handschullerchristian disease, abtletterersiwe disease or letterersiwe disease, and histiocytosis x.
The cytomorphology of cases of langerhans cell histiocytosis is described. Histiocytic disorders are a group of diseases derived from macrophages and dendritic cells resulting in a wide range of clinical conditions. The hallmark of lch is the proliferation and accumulation of a specific histiocyte. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. Skin biopsy diagnosis of langerhans cell neoplasms. Bone marrow assessment in langerhans cell histiocytosis. Also called eosinophilic granuloma, histiocytosis x, langerhans cell granulomatosis. The histiocytic disorders are generally defined by their constitutive cell type, on the basis of widely recognized pathologic and histological criteria.
Pulmonary langerhans cell histiocytosis orphanet journal of rare. A continuous effort by the clinical investigators, especially in the pediatric field, has led to improved, standardized definitions of the clinical and pathologic diagnostic criteria chu et al. Langerhans cell histiocytosis lch is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions. Langerhans cell histiocytosis an overview sciencedirect. Langerhans cell histiocytosis is a rare disease within the adult population, with very few cases reported as solitary hypophyseal lesions in adults. Radiotherapy has been thought to be curative at the relatively low dose of 20gy. Langerhans cell histiocytosis is a clonal hematopoietic disorder characterized by proliferation of langerhans cells in one or more organs. Here we report a case of recurrent hypophyseal langerhans. Langerhans cells lcs were first described by paul langerhans, in 1868, as dendritically shaped cells, which were located in the squamous epithelia of epidermis. 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. Langerhans cell histiocytosis lch is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrowderived histiocytes langerhans cells. Jul 28, 2004 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. Pulmonary langerhans cell histiocytosis plch is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived langerhans cells, which carry mutations of braf gene andor nras, kras and map2k1 genes.
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