Langerhans cell sarcoma. Medical search. Frequent questions

Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis. (wikipedia.org)
It should not be confused with Langerhans cell histiocytosis, which is cytologically benign. (wikipedia.org)
Dendritic cell neoplasms are classified by the World Health Organization as follows: Langerhans cell histiocytosis Langerhans cell sarcoma Interdigitating dendritic cell sarcoma/tumor Follicular dendritic cell sarcoma/tumor Dendritic cell sarcoma The exact incidence of LCS is unknown due to the rarity of the condition. (wikipedia.org)
The related condition, Langerhans Cell Histiocytosis (LCH) is estimated to have an incidence of around 5 cases per 1 million people per year. (wikipedia.org)
Langerhans cell sarcoma can occur de novo, or can occur as a malignant transformation of Langerhans cell histiocytosis. (wikipedia.org)
Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. (scirp.org)
We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution. (scirp.org)
Final diagnosis was therefore Langerhans cell histiocytosis of the dorsal spine complicated by spinal cord compression. (scirp.org)
A chest CT scan revealed a tumoral process measuring 25 mm at the lower lobe of the left lung evoking a Langerhans cell histiocytosis ( Figure 3 ). (scirp.org)
The Memorial Sloan Kettering Make-an-IMPACT Initiative is a rare-cancer global outreach initiative that provides free genomic profiling for patients with Langerhans Cell Histiocytosis (LCH), Erdheim-Chester Disease (ECD), Rosai-Dorfman Disease (RDD), Juvenile Xanthogranuloma (JXG), and Histiocytic Sarcoma (HS). (histio.org)
Sakata N, Toguchi N, Kimura M, Nakayama M, Kawa K, Takemura T. Development of Langerhans cell histiocytosis associated with chronic active Epstein-Barr virus infection. (mayabouchenaki.com)
Murakami I, Gogusev J, Fournet JC, Glorion C, Jaubert F. Detection of molecular cytogenetic aberrations in langerhans cell histiocytosis of bone. (mayabouchenaki.com)
Erdem AP, Kasimoglu Y, Sepet E, Gencay K, Sahin S, Dervisoglu S. Oral manifestations may be the first sign of Langerhans cell histiocytosis. (mayabouchenaki.com)
Langerhans cell histiocytosis mimicking molluscum contagiosum. (harvard.edu)
Currently, the preferred term is Langerhans cell histiocytosis. (thedoctorsdoctor.com)
Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. (thedoctorsdoctor.com)
OBJECTIVES: To describe the morphologic characteristics of skin lesions, extent of extracutaneous disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH), and to examine clinical predictors of disease prognosis. (thedoctorsdoctor.com)
Epidemiologic study of Langerhans cell histiocytosis in children. (thedoctorsdoctor.com)
OBJECTIVE: The etiology and pathogenesis of Langerhans cell histiocytosis (LCH) remain poorly understood. (thedoctorsdoctor.com)
Pathology of the tissue biopsy demonstrated proliferation of macrophages or dendritic cells, and positive staining with CD1a confirmed a diagnosis of Langerhans' cell histiocytosis (See Figure 2) . (reviewofophthalmology.com)
Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, describes a rare spectrum of conditions with abnormal proliferation of histiocytes, occuring in various locations throughout the body. (reviewofophthalmology.com)
National death data collected by the US Vital Statistics Reporting System and aggregated using wonder.cdc.gov were analyzed for underlying cause of death due to malignant histiocytosis (MH), Langerhans cell histiocytosis (LCH) and Letterer-Siwe disease (LS, a form of LCH) for 3 periods: 1979-1988, 1989-1998, and 1999-2006. (johnshopkins.edu)
Symptoms of Langerhans' Cell Histiocytosis vary depending on the part of the body affected and the severity of the disease. (homeremedylifestyle.com)
Langerhans' cell histiocytosis can cause symptoms in the skin, bones, lungs, and other parts of the body. (homeremedylifestyle.com)
The symptoms of Langerhans' cell histiocytosis include pain, swelling, and brown or red skin sores. (homeremedylifestyle.com)
Langerhans' cell histiocytosis is a rare disease . (homeremedylifestyle.com)
Children with Langerhans' cell histiocytosis have a high risk of developing cancer. (homeremedylifestyle.com)
Single-system Langerhans' cell histiocytosis responds well to chemotherapy. (homeremedylifestyle.com)
Multisystem Langerhans' cell histiocytosis, however, is fatal in severe cases. (homeremedylifestyle.com)
The treatment for Langerhans' cell histiocytosis will depend on the organs affected. (homeremedylifestyle.com)
Having a diagnosis of Langerhans' Cell Histiocytosis can be a scary time for a family. (homeremedylifestyle.com)
A diagnosis of Langerhans' Cell Histiocytosis is dependent on a number of factors. (homeremedylifestyle.com)
A diagnosis of Langerhans' cell histiocytosis should be made only after the patient has undergone a biopsy . (homeremedylifestyle.com)
Another way to diagnose Langerhans cell histiocytosis is by examining the lymph nodes. (homeremedylifestyle.com)
The best way to treat Langerhans' cell histiocytosis is to have the disease treated by experts who understand the disease. (homeremedylifestyle.com)
Approximately one out of every 200,000 children develops Langerhans cell histiocytosis every year. (homeremedylifestyle.com)
We pursued target-capture next generation sequencing (NGS) on tissue specimen of patients with various histiocytic neoplasms- Erdheim-Chester disease (ECD), Langerhans cell histiocytosis (LCH), Rosai-Dorfman disease (RDD), histiocytic sarcoma (HS), and Langerhans cell sarcoma (LCS). (tempus.com)
An idiopathic variation of Langerhans cell histiocytosis , commonly seen as a non-neoplastic osteolytic lesion of bone. (logicalimages.com)
cell histiocytosis and giant cell tumor. (neobiotechnologies.com)
Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. (msdmanuals.com)
Pulmonary Langerhans Cell Histiocytosis Pulmonary Langerhans cell histiocytosis (PLCH) is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. (msdmanuals.com)
Langerhans cell histiocytosis (LCH) is a dendritic cell (antigen-presenting cell) disorder. (msdmanuals.com)
Symptoms and signs of Langerhans cell histiocytosis vary considerably depending on which organs are infiltrated. (msdmanuals.com)

SummaryDiffuse large B‑cell lymphoma (DLBCL) comprises 30-40% of non-Hodgkin's lymphoma. (deepdyve.com)
In lymphomas I have conducted studies in rare aggressive primary cutaneous tumors such as gamma/delta T cell lymphoma (expanding the description of the epidermotropic variant) as well as more indolent entities such as lymphomatoid papulosis (type D) and CD4-positive small to medium size T cell lymphoproliferative disorders. (mdanderson.org)
Keyword: Non-Hodgkin's Lymphoma, high-grade B-cell lymphoma, plexopathy. (bvsalud.org)
INTRODUCTION: Integration of molecular characterization of lymphomas in clinical diagnostics may improve subclassification and risk-stratification, and we implemented a next generation sequencing (NGS) analysis as part of routine diagnostic work-up of all mature B-cell non-Hodgkin's lymphoma (B-NHL). (bvsalud.org)
Richter's Transformation of chronic lymphocytic leukemia (CLL) to diffuse large B-cell lymphoma or Hodgkin lymphoma (both are B-cell origin) are well described in literature. (najms.com)
Michelis FV, Kourti G, Skertsou M. Richter transformation of chronic lymphocytic leukemia into composite diffuse large B-cell and Hodgkin lymphoma. (najms.com)
Feldman AL, Arber DA, Pittaluga S, Martinez A, Burke JS, Raffeld M. Clonally related follicular lymphomas and histiocytic/dendritic cell sarcomas: Evidence for transdifferentiation of the follicular lymphoma clone. (najms.com)
Transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma to interdigitating dendritic cell sarcoma: Evidence for transdifferentiation of the lymphoma clone. (najms.com)
Shao H, Xi L, Raffeld M, Feldman AL, Ketterling RP, Knudson R. Clonally related histiocytic/dendritic cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma: A study of seven cases. (najms.com)
Wang E, Hutchinson CB, Huang Q, Sebastian S, Rehder C, Kanaly A. Histiocytic sarcoma arising in indolent small b-cell lymphoma: Report of two cases with molecular/genetic evidence suggestive of a 'transdifferentiation' during the clonal evolution. (najms.com)
Histiocytic/dendritic cell sarcoma arising from follicular lymphoma involving the bone: A case report and review of literature. (najms.com)
IgVH mutational status and clonality analysis of Richter's transformation: diffuse large B-cell lymphoma and Hodgkin lymphoma in association with B-cell chronic lymphocytic leukemia (B-CLL) represent 2 different pathways of disease evolution. (najms.com)
In lymphoma, the cells in the lymphatic system grow abnormally, dividing too rapidly and growing without any order or control (Longe 2005). (newworldencyclopedia.org)
DNA-microarray analysis of Burkitt's lymphoma and diffuse large B-cell lymphoma (DLBCL) showing differences in gene expression patterns. (newworldencyclopedia.org)
green indicates genes that are overexpressed in normal cells compared to lymphoma cells and red indicates genes that are overexpressed in lymphoma cells compared to normal cells. (newworldencyclopedia.org)
The diagnosis of Hairy Cell Leukemia and HCL-like diosrders, including HCL variant (vHCL) and Splenic Diffuse Red Pulp Lymphoma (SDRPL), is based on the examination of the peripheral blood and bone marrow smears allowing the identification of hairy cells and the flow cytometric analysis. (fortuneonline.org)
Splenic marginal zone lymphoma with circulating villous lymphoid cells (SMZL) is a distinct and very different pathological entity since the WHO 2008 classification: it is characterized by an expansion of the splenic white pulp with the infiltration of the red pulp. (fortuneonline.org)
38= Cutaneous T-cell lymphoma, NOS (C44. (cancercentrum.se)
Chemotherapy regimens that have documented responses include lymphoma protocols with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) and rituximab with CHOP (R-CHOP) as well as sarcoma protocols of doxorubicin and ifosfamide or gemcitabine and a taxane [ 6 ]. (biomedcentral.com)

Histiocytic neoplasms are rare disorders of the monocyte-macrophage-dendritic cell lineage that pose significant management challenges to the clinicians. (tempus.com)
1 ] and was since classified in the 2008 World Health Organization classification of haematolymphoid neoplasms with tumours of histiocytes and dendritic cells, alongside other dendritic cell tumours including Langerhans cell tumours, interdigitating dendritic cell sarcoma and other rare tumours. (biomedcentral.com)

Cells from these tumor samples will display features characteristic of Langerhans cells, but with additional signs of malignancy, such as increased mitoses, cellular atypia, and pleomorphic nuclei. (wikipedia.org)
As such, CNS tumor classification heavily relies on accurate grading , which reflects the degree of abnormal behavior displayed by the tumor cells themselves. (medscape.com)
Genotyping of 69 histiocytic lesions revealed that 23/48 Langerhans cell lesions were BRAF -V600E-mutant whereas all non-Langerhans cell lesions (including dendritic cell sarcoma, juvenile xanthogranuloma, Rosai-Dorfman disease, and granular cell tumor) were wild-type. (oncotarget.com)
Background/aim: Insulin-like growth factor-1 receptor (IGF-1R) is a pivotal receptor tyrosine kinase involved in the cell cycle and malignant tumor transformation. (tubitak.gov.tr)
Anti-tumor activity of the HSP90 inhibitor SNX-2112 in pediatric cancer cell lines. (ucdavis.edu)
Biopsy of the lesion is necessary to confirm the diagnosis as other orbital lesions in children, such as neuroblastoma, Ewing sarcoma and Wilms tumor, and other bony tumors may also present similarly on clinical exam and radiographic imaging. (reviewofophthalmology.com)
This classification attempts to classify lymphomas by cell type (i.e. the normal cell type that most closely resembles the tumor). (newworldencyclopedia.org)

B-cell heavy chain rearrangement study on blood sample (pure CLL) and liver metastatic NEC (pure NEC, no contamination from CLL) revealed that these two tumors shared similar monoclonal peaks. (najms.com)
Dendritic cell tumors associated with low-grade b-cell malignancies. (najms.com)
They play a major role in defending the host from both tumors and virally infected cells. (newworldencyclopedia.org)
These tumors demonstrate papillary structures with a delicate fibrovascular core lined by columnar or cuboidal epithelial cells with vesicular basal nuclei. (radiopaedia.org)

Interdigitating dendritic cell sarcoma subtotally replacing lymph node shows that the neoplasm tends to spare lymphoid follicles. (basicmedicalkey.com)
Primitive Spindle Cell Neoplasm of Ileum with Extensive Heterotopic Cartilage, Presenting as Acute Abdomen in a 6-Day-Old Neonate. (ucdavis.edu)
Follicular dendritic cell sarcoma (FDCS) is an uncommon neoplasm of mesenchymal stem cell origin whose clinical course displays much variability. (biomedcentral.com)

Antigen-presenting cells are also termed Dendritic cells, of which Langerhans cells are a subtype. (wikipedia.org)

Interdigitating dendritic cell sarcoma involving lymph node shows that the neoplastic cells are spindled and epithelioid with abundant eosinophilic cytoplasm. (basicmedicalkey.com)

Bone marrow aspirations in Ewing sarcomas: Are they still necessary? (nih.gov)

Homocysteine is associated with severity of microvasculopathy in sickle cell disease patients. (ucdavis.edu)

It has previously been mistaken with metastatic melanoma and metastatic large-cell carcinoma. (wikipedia.org)
This condition is characterized by a large, noncancerous patch of abnormally dark skin that is present from birth and an increased risk of a type of skin cell cancer called melanoma (described below). (medlineplus.gov)
Additional gene mutations in cells within the nevus after birth can lead to melanoma in people with giant congenital melanocytic nevus. (medlineplus.gov)

LCH is characterized by the proliferation of CD1a-positive activated Langerhans (not atypical Langerhans cell, morphologically)-like cells (LCH cells) generating inflammatory granuloma. (mayabouchenaki.com)
Microscopic review of the tissue specimen revealed proliferation of histiocytes (left) with positive CD1a staining, a marker for Langerhans' cells (right). (reviewofophthalmology.com)
Specifically, the RAS/MAPK pathway regulates the growth and division (proliferation) of cells, the process by which cells mature to carry out specific functions (differentiation), cell movement (migration), and the self-destruction of cells (apoptosis). (medlineplus.gov)
This mutation leads to production of a BRAF protein that is abnormally active, which disrupts regulation of cell proliferation and may allow histiocytes to grow and divide uncontrollably, leading to the abnormal accumulation of histiocytes that occurs in Erdheim-Chester disease. (medlineplus.gov)
This mutation leads to production of a BRAF protein that is abnormally active, which disrupts regulation of cell proliferation. (medlineplus.gov)
The unregulated cell proliferation of early melanocytes leads to a large patch of darkly pigmented skin characteristic of giant congenital melanocytic nevus. (medlineplus.gov)

This rare condition is characterized by the abnormal production and accumulation of immune system cells called histiocytes in many of the body's tissues. (medlineplus.gov)

In immunohistochemical study, these eosinophilic cells expressed CD1a and S100 protein. (scirp.org)
Positive immunohistological staining with neuronal markers S100 and CD1a are specific for Langerhans' cells and help to confirm the diagnosis. (reviewofophthalmology.com)

He is the In Charge of the Paediatric Stem Cell Transplantation program which has to its credit transplanting the smallest baby in the world (transplanted for Neuroblastoma at the age of 4 months) and the youngest thalassemia transplant in India at 9 months. (kokilabenhospital.com)

Sagransky MJ, Deng AC, Magro CM. Primary cutaneous langerhans cell sarcoma: a report of four cases and review of the literature. (umassmed.edu)

Kaposi sarcoma in association with molluscum contagiosum: an uncommon diagnosis in a single biopsy and potential diagnostic pitfall. (harvard.edu)
In cultures, a subpopulation of Kaposi sarcoma cells express CD14. (neobiotechnologies.com)

In conclusion, BRAF mutations in histiocytic proliferations are restricted to lesions of the Langerhans-cell type. (oncotarget.com)

Typically, neoplastic cells lack CD1a, desmin and CD45 staining. (biomedcentral.com)

Because Langerhans cells are most commonly found in the mucosa and the skin, LCS is thought to usually begin here with further spread to other areas of the body via the lymphatic system. (wikipedia.org)
Generalized reticular cell sarcoma of lymph nodes associated with lymphatic leukemia. (najms.com)
Ironically, the lymphatic system is fundamentally important for combating cancer cells-as well as foreign bodies, such as viruses and bacteria , and combating heart disease and arthritis as well. (newworldencyclopedia.org)
check the tag ADOLESCENCE HN - 2008 BX - Nutrition in Adolescence FX - Adolescent Nutrition Physiology MH - Peritoneal Stomata UI - D054048 MN - A01.047.025.600.700 MN - A10.810 MS - Natural openings in the subdiaphragmatic lymphatic plexus in the PERITONEUM, delimited by adjacent mesothelial cells. (bvsalud.org)

We treat all cancers occurring in children and young people (including brain tumours, solid tumours and leukemias's and offer chemotherapy, radiotherapy, stem-cell transplant and surgical treatments as required. (leedsth.nhs.uk)
Some high-grade monomorphic tumours, (such as Ewing's sarcoma), and some other specific tumour types cannot be graded accurately in this way and the tumour grade is defined by the specific histological type or subtype. (biomedcentral.com)
80 000 cancer-related deaths per year This was a cross-sectional study of chil- sarcoma), germ cell tumours (GCTs), worldwide. (who.int)

Throughout the body in locations such as mucous membranes, skin, lymph nodes, thymus, and spleen are cells known as antigen-presenting cells. (wikipedia.org)

The biopsy specimen from the right maxillary sinus showed soft tissue infiltration with myeloid leukemic cells, consistent with a chloroma. (contemporarypediatrics.com)

Broadly speaking, they can be divided into Langerhans and non-Langerhans cell histiocytoses. (thedoctorsdoctor.com)
Langerhans cell histiocytoses are all defined by the presence of a unique ultrastructural organelle, the Birbeck granule. (thedoctorsdoctor.com)
Baer MR, Krantz SB, Cousar JB, Glick AD, Collins RD. Malignant histiocytoses developing in patients with b-cell lymphomas. (najms.com)

Our data from micro-dissected LC in both dermatopathic lymphadenopathy [12] and LCS [13] suggest that monocytes, precursor Langerhans cells, or Langerhans cells are one of the reservoir cells for MCPyV. (mayabouchenaki.com)

Giving monoclonal antibody therapy with chemotherapy may kill more cancer cells. (policylab.us)

Monoclonal antibodies, such as blinatumomab, may induce changes in the body's immune system and may interfere with the ability of cancer cells to grow and spread. (policylab.us)
The antibody is a new monoclonal antibody against a C-type lectin found on the surface of Langerhans cells. (homeremedylifestyle.com)

They all share histologically a significant infiltration of affected tissues by langerhans cells. (scirp.org)

The BRAF gene provides instructions for making a protein that helps transmit chemical signals from outside the cell to the cell's nucleus. (medlineplus.gov)
The BRAF gene mutation that causes this condition is somatic, meaning that it occurs during a person's lifetime and is present only in certain cells. (medlineplus.gov)
13 ] have reported v-Raf murine sarcoma viral oncogene homolog B (BRAF) V600E mutations in 5 from 27 (18.5 %) cases tested. (biomedcentral.com)

This summary addresses squamous cell cancer of the vulva and vulvar intraepithelial neoplasias (VIN), some of which are thought to be precursors to invasive squamous cell cancers. (cancer.gov)
The term "oncotarget" encompasses all molecules, pathways, cellular functions, cell types, and even tissues that can be viewed as targets relevant to cancer as well as other diseases. (oncotarget.com)
High-density array analysis of DNA methylation in Tamoxifen-resistant breast cancer cell lines. (umassmed.edu)
La présente étude transversale a été menée auprès de 138 enfants atteints de cancer et traités dans l'Unité d'oncologie pédiatrique du Centre d'oncologie de l'Université de Mansoura, en Égypte. (who.int)
En revanche, les patients de moins de cinq ans et ceux avec un diagnostic de cancer provisoire posé initialement bénéficiaient du délai total médian le plus court. (who.int)
Nous suggérons de mettre en place des programmes de formation médicale continue, d'améliorer l'accès aux services de diagnostic, et de faciliter l'orientation-recours de façon à donner la priorité aux patients suspects de cancer et ainsi raccourcir le délai de diagnostic. (who.int)
At the age of 10, Deryn Blackwell was diagnosed with leukaemia and two years later developed a rare form of cancer called Langerhans cell sarcoma. (xpeerion.com)
In the most severe form of the disease, the cancer cells can spread to other parts of the body. (homeremedylifestyle.com)

Histologically, Langerhans cells characteristically display Birbeck granules and nuclei with a longitudinal groove. (wikipedia.org)

All primary malignant bone tumour cases should be discussed at a properly constituted sarcoma multidisciplinary team (MDT) meeting. (biomedcentral.com)
Follicular dendritic cells (FDC) form a tight meshwork in primary and secondary lymphoid follicles and interact with T and B lymphocytes through antigen presentation and generation and regulation of the germinal centre reaction [ 2 , 3 ]. (biomedcentral.com)

Diagnosis of Langerhans Cell Sarcoma is predominantly a pathologic diagnosis. (wikipedia.org)
Thus, a diagnosis is made by skin biopsy showing a large number of yeasts of uniform size and thick cell walls that form chains linked by thin bridges that are shown by staining with methenamine silver ( 2 , 4 , 9 , 18 ). (cdc.gov)
An accurate diagnosis is necessary given that different clinical management is required: the first step in a definitive diagnosis is based on the examination of the peripheral blood and bone marrow smears allowing the identification of hairy cells and a specific Flow Cytometric Analysis (FCA). (fortuneonline.org)

Exchange transfusion therapy and its effects on real-time microcirculation in pediatric sickle cell anemia patients: an intravital microscopy study. (ucdavis.edu)
Comparison of real-time microvascular abnormalities in pediatric and adult sickle cell anemia patients. (ucdavis.edu)

Clinical factors such as a high International Prognostic Index (IPI) or molecular factors as cell of origin (COO) have an influence on the clinical outcome after conventional immunochemotherapy. (deepdyve.com)
The HCL-Japanese variant form (jpHCL) is rare and less well defined: there are common points with vHCL but several aspects are different in terms of morphology of hairy cells, degree of leukocytosis or clinical course. (fortuneonline.org)
Follicular dendritic cell sarcoma is a rare tumour with clinical behaviour covering a spectrum from indolent to aggressive disease. (biomedcentral.com)

In giant congenital melanocytic nevus, a somatic V600E mutation occurs during embryonic development in cells that will develop into pigment-producing skin cells (melanocytes). (medlineplus.gov)

Anatomy1

Diseases6

Histiocytosis43

Lymphoma19

Neoplasms2

Tumor7

Tumors4

Neoplasm3

Termed Dendritic cells1

Interdigitating1

Ewing1

Sickle cell di1

Melanoma3

Proliferation6

Histiocytes1

S1002

Neuroblastoma1

Cutaneous1

Kaposi2

Lesions1

CD1a1

Lymphatic4

Tumours3

Lymph1

Soft tissue1

Histiocytoses3

Monocytes1

Chemotherapy1

Monoclonal2

Infiltration1

BRAF3

Cancer8

Characteristically1

Primary2

Diagnosis3

Pediatric2

Clinical3

V600E1