MCID: LNG004
MIFTS: 40

Langerhans Cell Sarcoma

Categories: Blood diseases, Cancer diseases, Rare diseases
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Aliases & Classifications for Langerhans Cell Sarcoma

MalaCards integrated aliases for Langerhans Cell Sarcoma:

Name: Langerhans Cell Sarcoma 11 19 58 43 14 16 71 75 33
Malignant Langerhans Cell Sarcoma 19

Characteristics:


Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 11 DOID:7146
MeSH 43 D054752
NCIt 49 C6921
SNOMED-CT 68 724649000
ICD10 31 C96.4
MESH via Orphanet 44 D054752
ICD10 via Orphanet 32 C96.4
UMLS via Orphanet 72 C1260327
Orphanet 58 ORPHA86897
ICD11 33 588958190
UMLS 71 C1260327

Summaries for Langerhans Cell Sarcoma

GARD: 19 A rare dendritic cell tumor characterized by an aggressive, high-grade neoplasm derived from Langerhans cells, most commonly extranodal and multifocal, involving the skin and underlying soft tissue, as well as lung, liver, spleen, and bone. Primary nodal involvement is seen in a minority of patients. Immune-phenotyping and the presence of Birbeck granules on ultrastructural examination reveal the Langerhans cell derivation of the neoplastic cells. Prognosis is generally poor.

MalaCards based summary: Langerhans Cell Sarcoma, also known as malignant langerhans cell sarcoma, is related to histiocytosis and langerhans cell histiocytosis. An important gene associated with Langerhans Cell Sarcoma is CD207 (CD207 Molecule), and among its related pathways/superpathways are Innate Immune System and Class I MHC mediated antigen processing and presentation. Affiliated tissues include spleen, bone marrow and lung.

Orphanet: 58 A rare dendritic cell tumor characterized by an aggressive, high-grade neoplasm derived from Langerhans cells, most commonly extranodal and multifocal, involving the skin and underlying soft tissue, as well as lung, liver, spleen, and bone. Primary nodal involvement is seen in a minority of patients. Immune-phenotyping and the presence of Birbeck granules on ultrastructural examination reveal the Langerhans cell derivation of the neoplastic cells. Prognosis is generally poor.

Disease Ontology: 11 A histiocytic and dendritic cell cancer that derives from the lymph nodes, derives from the skin, derives from the liver, derives from the spleen and derives from bones.

Wikipedia: 75 Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis. It should not be confused with... more...

Related Diseases for Langerhans Cell Sarcoma

Diseases related to Langerhans Cell Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 120)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 31.0 CD207 CD1C CD1A
2 langerhans cell histiocytosis 29.5 CD68 CD207 CD1E CD1D CD1C CD1B
3 histiocytic and dendritic cell cancer 27.8 CR2 CR1 CD68 CD207 CD1E CD1D
4 sarcoma 10.8
5 leukemia, chronic lymphocytic 10.2
6 follicular lymphoma 10.2
7 burkitt lymphoma 10.2
8 leukemia, acute myeloid 10.2
9 lymphoma 10.2
10 leukemia 10.2
11 hematologic cancer 10.2
12 chronic lymphocytic leukemia/small lymphocytic lymphoma 10.2
13 b-cell lymphoma 10.2
14 acute myeloid leukemia with recurrent genetic anomaly 10.2
15 dendritic cell tumor 10.2
16 rare tumor 10.2
17 inflamed seborrheic keratosis 10.1 CD1E CD1A
18 lobomycosis 10.0 CR2 CD1E
19 mixed type thymoma 10.0 CD1E CD1B CD1A
20 igg4-related disease 10.0 CD1E CD1A
21 lichen nitidus 10.0 CD1E CD1B CD1A
22 epithelial malignant thymoma 10.0 CD1E CD1B CD1A
23 cortical thymoma 10.0 CD1E CD1B CD1A
24 mixed phenotype acute leukemia, t/myeloid 10.0 CD1E CD1B CD1A
25 kimura disease 10.0 CD1E CD1B CD1A
26 geographic tongue 10.0 CD1E CD1B CD1A
27 interstitial pneumonitis, desquamative, familial 10.0 CD1E CD1B CD1A
28 mite infestation 10.0 CD1E CD1B CD1A
29 folliculitis 10.0 CD1E CD1B CD1A
30 oral hairy leukoplakia 10.0 CR2 CD1A
31 verruciform xanthoma of skin 10.0 CD68 CD1E
32 lentigines 9.9
33 wilms tumor 1 9.9
34 sjogren syndrome 9.9
35 wilms tumor 5 9.9
36 leukemia, chronic myeloid 9.9
37 aplastic anemia 9.9
38 leukemia, acute lymphoblastic 9.9
39 aspergillosis 9.9
40 myelodysplastic syndrome 9.9
41 bacterial sepsis 9.9
42 invasive aspergillosis 9.9
43 marginal zone b-cell lymphoma 9.9
44 nodal marginal zone lymphoma 9.9
45 nodular malignant melanoma 9.9
46 sialadenitis 9.9
47 pancytopenia 9.9
48 acute leukemia 9.9
49 brain cancer 9.9
50 melanoma 9.9

Graphical network of the top 20 diseases related to Langerhans Cell Sarcoma:



Diseases related to Langerhans Cell Sarcoma

Symptoms & Phenotypes for Langerhans Cell Sarcoma

Drugs & Therapeutics for Langerhans Cell Sarcoma

Search Clinical Trials, NIH Clinical Center for Langerhans Cell Sarcoma

Cochrane evidence based reviews: langerhans cell sarcoma

Genetic Tests for Langerhans Cell Sarcoma

Anatomical Context for Langerhans Cell Sarcoma

Organs/tissues related to Langerhans Cell Sarcoma:

MalaCards : Spleen, Bone Marrow, Lung, Liver, Bone, Skin, Lymph Node

Publications for Langerhans Cell Sarcoma

Articles related to Langerhans Cell Sarcoma:

(show top 50) (show all 123)
# Title Authors PMID Year
1
Left Forearm Langerhans Cell Sarcoma Demonstrated by 18 F-FDG PET/CT. 62
35835140 2022
2
Prolonged remission with pembrolizumab and radiation therapy in a patient with multisystem Langerhans cell sarcoma. 62
35615932 2022
3
Langerhans cell sarcoma arising after antecedent Langerhans cell histiocytosis in lymphoepithelial sialadenitis of Sjögren's syndrome. 62
35583172 2022
4
Differential Diagnosis of Rare Diseases Involving Bilateral Lower Extremities with Similar 99mTc-MDP Bone Scan Patterns: Analysis of a Case Series. 62
35453958 2022
5
Unusual presentation of Langerhans cell sarcoma as bilateral submandibular gland swelling - A rare case report. 62
35645054 2022
6
Case Report: Langerhans Cell Sarcoma With Intracranial and Extracranial Communication on the Left Frontal Bone. 62
35747436 2022
7
Langerhans cell sarcoma with BRAF-V600E mutation and hemophagocytosis: An autopsy report. 62
35645053 2022
8
Chronic lymphocytic leukemia/small lymphocytic lymphoma complicated with skin Langerhans cell sarcoma: A case report. 62
35005006 2021
9
A Pedigree Analysis and Clonal Correlations of the Coexistence of B-Cell Lymphoma and Histiocytic/Dendritic Cell Tumor. 62
33939500 2021
10
Concurrent follicular lymphoma and Langerhans cell sarcoma. 62
35845209 2021
11
Histiocytic disorders. 62
34620874 2021
12
The Expression of Insulin-Like Growth Factor 2 Messenger RNA-Binding Protein 3 in Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma. 62
34497165 2021
13
Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature. 62
34289900 2021
14
From the archives of MD Anderson Cancer Center: A case of concurrent follicular lymphoma and Langerhans cell sarcoma with a review of the literature. 62
33676171 2021
15
Langerhans cell sarcoma involving skin and showing epidermotropism: A comprehensive review. 62
32644218 2021
16
Histiocytosis and Neoplasms of Macrophage-Dendritic Cell Lineages: Multimodality Imaging with Emphasis on PET/CT. 62
33606566 2021
17
[Primary cutaneous Langerhans cell sarcoma: report of a case]. 62
33677899 2021
18
Epstein-Barr Virus-Positive Langerhans Cell Sarcoma: Is There a Link? A Case Report. 62
35117987 2021
19
Nodal Langerhans cell neoplasm: detailing the diagnostic quandaries. 62
35004363 2021
20
Histiocytic and dendritic cell neoplasms: Reappraisal of a Japanese series based on t(14;18) and neoplastic PD-L1 expression. 62
33238073 2021
21
Bone marrow coexistence of chronic lymphocytic leukemia and Langerhans cell sarcoma. 62
32621179 2020
22
A case of Langerhans cell sarcoma on the scalp: Whole-exome sequencing reveals a role of ultraviolet in the pathogenesis. 62
33410565 2020
23
Adult Langerhans cell histiocytosis presenting with multisystem involvement and sarcomatoid features: a case report. 62
32979930 2020
24
Langerhans cell sarcoma of the vulva: Case report and review of the literature. 62
32346590 2020
25
Concurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma in a patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma. 62
31441945 2020
26
TIM-3 expression and its association with overall survival in primary osteosarcoma. 62
31612039 2019
27
Unusual Presentation of a Rare Tumor: Histiocytic Sarcoma Presenting as a Finger Growth. 62
31890359 2019
28
Efficacy of a Modified Scoring System to Facilitate Surgical Decision-making for Diaphyseal Malignancies: When is Devitalized Tumor-bearing Autograft of Value? 62
31402605 2019
29
RASA1 loss in a BRAF-mutated Langerhans cell sarcoma: a mechanism of resistance to BRAF inhibitor. 62
30977771 2019
30
Incidence, Clinical Features, and Outcomes of Langerhans Cell Sarcoma in the United States. 62
31005630 2019
31
Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report. 62
30855438 2019
32
Combined Tumors in Hematolymphoid Neoplasms: Case Series of Histiocytic and Langerhans Cell Sarcomas Arising From Low-Grade B-Cell Lymphoma. 62
31633108 2019
33
[Durable remission of Langerhans cell sarcoma attained by autologous hematopoietic stem cell transplantation following surgical resection]. 62
31068562 2019
34
Pegylated interferon-α2b for local control of Langerhans cell sarcoma. 62
30355544 2018
35
Successful treatment of an elderly Langerhans cell sarcoma patient by EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) chemotherapy. 62
30305476 2018
36
Synchronous case of follicular lymphoma and Langerhans cell sarcoma in the same lymph node. 62
30255584 2018
37
KRAS mutation in secondary malignant histiocytosis arising from low grade follicular lymphoma. 62
30322385 2018
38
Liquid-based cytology in the diagnosis of Langerhans cell sarcoma: A case report. 62
29673124 2018
39
Merkel cell polyomavirus and Langerhans cell neoplasm. 62
30134914 2018
40
[Langerhans cell sarcoma: a clinicopathologic analysis of four cases]. 62
29534353 2018
41
CDKN2A/B Deletion and Double-hit Mutations of the MAPK Pathway Underlie the Aggressive Behavior of Langerhans Cell Tumors. 62
29194093 2018
42
Histiocytic lesions of the orbit: A study of 9 cases. 62
29755270 2018
43
Prognostic factors for histiocytic and dendritic cell neoplasms. 62
29228722 2017
44
Langerhans cell sarcoma of the skin in association with superficial atypical Langerhans cell proliferation. 62
29218135 2017
45
[Langerhans cell sarcoma: two cases report and literature rewiew]. 62
28926905 2017
46
Cytological findings of langerhans cell sarcoma in a case of quintuple cancer. 62
28233936 2017
47
Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion. 62
28626593 2017
48
A Case of Pulmonary Langerhans Cell Sarcoma Simultaneously Diagnosed with Cutaneous Langerhans Cell Histiocytosis Studied by Whole-Exome Sequencing. 62
28614815 2017
49
Sustained response to salvage therapy for dabrafenib-resistant metastatic Langerhans cell sarcoma. 62
27502715 2016
50
High frequency of clonal IG and T-cell receptor gene rearrangements in histiocytic and dendritic cell neoplasms. 62
27823979 2016

Variations for Langerhans Cell Sarcoma

Cosmic variations for Langerhans Cell Sarcoma:

8 (show top 50) (show all 109)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM87903333 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
2 COSM112319175 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
3 COSM145022367 TP53 soft tissue,liver,sarcoma,NS c.529G>A p.V177M 17:7674885-7674885 9
4 COSM93250304 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
5 COSM111826264 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
6 COSM112257487 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
7 COSM142840682 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
8 COSM144718997 TP53 soft tissue,liver,sarcoma,NS c.242A>T p.K81M 17:7676010-7676010 9
9 COSM144313969 TP53 soft tissue,liver,sarcoma,NS c.529G>A p.V177M 17:7674885-7674885 9
10 COSM111763428 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
11 COSM142892050 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
12 COSM105695818 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
13 COSM143947706 TP53 soft tissue,liver,sarcoma,NS c.169G>A p.V57M 17:7674885-7674885 9
14 COSM87975568 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
15 COSM144090111 TP53 soft tissue,liver,sarcoma,NS c.169G>A p.V57M 17:7674885-7674885 9
16 COSM93188160 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
17 COSM105625297 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
18 COSM122738565 TP53 soft tissue,liver,sarcoma,NS c.250G>A p.V84M 17:7674885-7674885 9
19 COSM144656180 TP53 soft tissue,liver,sarcoma,NS c.529G>A p.V177M 17:7674885-7674885 9
20 COSM121881499 TP53 soft tissue,liver,sarcoma,NS c.250G>A p.V84M 17:7674885-7674885 9
21 COSM145076613 TP53 soft tissue,liver,sarcoma,NS c.242A>T p.K81M 17:7676010-7676010 9
22 COSM122275696 TP53 soft tissue,liver,sarcoma,NS c.250G>A p.V84M 17:7674885-7674885 9
23 COSM144364047 TP53 soft tissue,liver,sarcoma,NS c.242A>T p.K81M 17:7676010-7676010 9
24 COSM143160205 TP53 soft tissue,liver,sarcoma,NS c.169G>A p.V57M 17:7674885-7674885 9
25 COSM143433111 TP53 soft tissue,liver,sarcoma,NS c.242A>T p.K81M 17:7676010-7676010 9
26 COSM142564545 TP53 soft tissue,liver,sarcoma,NS c.529G>A p.V177M 17:7674885-7674885 9
27 COSM143375373 TP53 soft tissue,liver,sarcoma,NS c.529G>A p.V177M 17:7674885-7674885 9
28 COSM106116663 TP53 soft tissue,liver,sarcoma,NS c.359A>T p.K120M 17:7676010-7676010 9
29 COSM144017945 TP53 soft tissue,liver,sarcoma,NS c.613G>A p.V205M 17:7674885-7674885 9
30 COSM142627250 TP53 soft tissue,liver,sarcoma,NS c.242A>T p.K81M 17:7676010-7676010 9
31 COSM144072930 TP53 soft tissue,liver,sarcoma,NS c.340+15A>T p.? 17:7676010-7676010 9
32 COSM106057273 TP53 soft tissue,liver,sarcoma,NS c.646G>A p.V216M 17:7674885-7674885 9
33 COSM97107943 NRAS soft tissue,skin,sarcoma,NS c.38G>C p.G13A 1:114716123-114716123 9
34 COSM93992510 SRC soft tissue,lung,sarcoma,NS c.205T>G p.S69A 20:37384358-37384358 8
35 COSM98338978 SRC soft tissue,lung,sarcoma,NS c.205T>G p.S69A 20:37384358-37384358 8
36 COSM98373331 SRC soft tissue,lung,sarcoma,NS c.205T>G p.S69A 20:37384358-37384358 8
37 COSM93554172 SRC soft tissue,lung,sarcoma,NS c.205T>G p.S69A 20:37384358-37384358 8
38 COSM89629521 SF3B1 soft tissue,liver,other,neoplasm c.2014G>T p.A672S 2:197402619-197402619 8
39 COSM93451505 RAC1 soft tissue,lung,sarcoma,NS c.533C>T p.A178V 7:6402343-6402343 8
40 COSM92729038 RAC1 soft tissue,lung,sarcoma,NS c.476C>T p.A159V 7:6402343-6402343 8
41 COSM117948203 PTPRD soft tissue,lung,sarcoma,NS c.2939C>T p.A980V 9:8404587-8404587 8
42 COSM132326062 PTPRD soft tissue,lung,sarcoma,NS c.4160C>T p.A1387V 9:8404587-8404587 8
43 COSM91028178 PTPRD soft tissue,lung,sarcoma,NS c.2942C>T p.A981V 9:8404587-8404587 8
44 COSM101931022 PTPRD soft tissue,lung,sarcoma,NS c.4160C>T p.A1387V 9:8404587-8404587 8
45 COSM132607215 PTPRD soft tissue,lung,sarcoma,NS c.2912C>T p.A971V 9:8404587-8404587 8
46 COSM102156539 PTPRD soft tissue,lung,sarcoma,NS c.2930C>T p.A977V 9:8404587-8404587 8
47 COSM102254463 PTPRD soft tissue,lung,sarcoma,NS c.2939C>T p.A980V 9:8404587-8404587 8
48 COSM92957889 PTPRD soft tissue,lung,sarcoma,NS c.4160C>T p.A1387V 9:8404587-8404587 8
49 COSM102059370 PTPRD soft tissue,lung,sarcoma,NS c.2939C>T p.A980V 9:8404587-8404587 8
50 COSM106841030 PTCH1 soft tissue,lung,sarcoma,NS c.2468T>G p.F823C 9:95458260-95458260 8

Expression for Langerhans Cell Sarcoma

Search GEO for disease gene expression data for Langerhans Cell Sarcoma.

Pathways for Langerhans Cell Sarcoma

GO Terms for Langerhans Cell Sarcoma

Cellular components related to Langerhans Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 10.68 TIMD4 CR2 CR1 CD68 CD207 CD1E
2 plasma membrane GO:0005887 10.68 CR1 CD1E CD1D CD1C CD1A
3 extracellular space GO:0005615 10.21 CR2 CR1 CD1E CD1D CD1C CD1B
4 membrane GO:0016021 10.02 CD1A CD1B CD1C CD1D CD1E CD207
5 membrane GO:0016020 10.02 CD1A CD1B CD1C CD1D CD1E CD207
6 lysosome GO:0005764 9.92 CD1B CD1C CD1D CD1E CD68
7 endosome membrane GO:0010008 9.91 CD68 CD1D CD1C CD1B CD1A
8 endosome GO:0005768 9.63 CD68 CD1E CD1D CD1C CD1B CD1A
9 external side of plasma membrane GO:0009897 9.4 CD207 CD1E CD1D CD1C CD1B CD1A

Biological processes related to Langerhans Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 10.06 CD1E CD1C CD1B CD1A
2 immune response GO:0006955 9.97 CR2 CD1E CD1D CD1C CD1B CD1A
3 positive regulation of T cell mediated cytotoxicity GO:0001916 9.85 CD1E CD1D CD1C CD1B CD1A
4 immune system process GO:0002376 9.8 CD1A CD1B CD1C CD1D CD1E CR1
5 complement activation, alternative pathway GO:0006957 9.73 CR2 CR1
6 complement receptor mediated signaling pathway GO:0002430 9.71 CR2 CR1
7 negative regulation of complement activation, classical pathway GO:0045959 9.67 CR2 CR1
8 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.65 CD1E CD1D CD1C CD1B CD1A
9 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 9.32 CD1E CD1D CD1C CD1B CD1A

Molecular functions related to Langerhans Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lipopeptide binding GO:0071723 9.85 CD1E CD1D CD1C CD1B CD1A
2 exogenous lipid antigen binding GO:0030884 9.65 CD1E CD1D CD1C CD1B CD1A
3 endogenous lipid antigen binding GO:0030883 9.32 CD1E CD1D CD1C CD1B CD1A

Sources for Langerhans Cell Sarcoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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