MCID: DND018
MIFTS: 42

Dendritic Cell Tumor

Categories: Rare diseases, Cancer diseases

Aliases & Classifications for Dendritic Cell Tumor

MalaCards integrated aliases for Dendritic Cell Tumor:

Name: Dendritic Cell Tumor 53
Dendritic Cell Neoplasm 53 73
Dendritic Cell Sarcoma, Not Otherwise Specified 73

Classifications:



External Ids:

UMLS 73 C1531553

Summaries for Dendritic Cell Tumor

NIH Rare Diseases : 53 A dendritic celltumor develops from the cells of the immune system.  This condition typically begins in the lymph system and may spread to nearby organs or distant parts of the body (metastasize).  There are five subtypes of dendritic cell tumors: follicular dendritic cell tumor, interdigitating dendritic cell tumor, Langerhans' cell histiocytosis, Langerhans' cell sarcoma, and dendritic cell sarcoma not specified otherwise.  The symptoms and severity of the condition depend on the subtype and location of the tumor.  Treatment may include surgery, radiation therapy, and/or chemotherapy.

MalaCards based summary : Dendritic Cell Tumor, also known as dendritic cell neoplasm, is related to castleman disease and spleen cancer. An important gene associated with Dendritic Cell Tumor is VIM (Vimentin), and among its related pathways/superpathways are Innate Immune System and Cytoskeletal Signaling. The drugs Central Nervous System Stimulants and Trastuzumab have been mentioned in the context of this disorder. Affiliated tissues include myeloid, t cells and skin, and related phenotypes are hematopoietic system and immune system

Related Diseases for Dendritic Cell Tumor

Diseases related to Dendritic Cell Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 165)
# Related Disease Score Top Affiliating Genes
1 castleman disease 30.5 CR1 CR2
2 spleen cancer 30.0 CD68 CR2
3 langerhans cell histiocytosis 29.2 CD1A CD207 IFNG S100B
4 inflammatory myofibroblastic tumor 29.1 ACTC1 CR1 CR2 S100B VIM
5 histiocytosis 29.0 CD1A CD207 FSCN1 PTPRC S100B
6 lymphoblastic lymphoma 29.0 DNTT MPO PTPRC
7 indeterminate cell histiocytosis 11.5
8 parapharyngeal meningioma 10.9 CR1 CR2
9 primitive neuroectodermal tumor of the cervix uteri 10.9 PTPRC VIM
10 multicentric reticulohistiocytosis 10.9 PTPRC VIM
11 retroperitoneal fibrosis 10.9 PTPRC VIM
12 liver inflammatory pseudotumor 10.9 CR2 PTPRC
13 malignant peritoneal mesothelioma 10.9 MUC1 VIM
14 hydromyelia 10.9 S100B VIM
15 papillary tumor of the pineal region 10.9 MUC1 VIM
16 bednar tumor 10.8 S100B VIM
17 localized hypertrophic neuropathy 10.8 MUC1 S100B
18 sclerosing perineurioma 10.8 MUC1 S100B
19 primary hepatic neuroendocrine carcinoma 10.8 S100B VIM
20 malignant mixed mullerian tumor 10.8 MUC1 VIM
21 adenoid squamous cell carcinoma 10.8 KRT20 VIM
22 intracranial cysts 10.8 MUC1 S100B
23 reticular perineurioma 10.8 MUC1 S100B
24 nodular hidradenoma 10.8 MUC1 VIM
25 primary cutaneous anaplastic large cell lymphoma 10.8 MUC1 PTPRC
26 endometrial mucinous adenocarcinoma 10.8 MUC1 VIM
27 non-functioning pituitary adenoma 10.8 MUC1 S100B
28 biphasic synovial sarcoma 10.7 MUC1 VIM
29 interdigitating dendritic cell sarcoma 10.7 CR1 CR2 PTPRC
30 epithelioid malignant peripheral nerve sheath tumor 10.7 MUC1 S100B
31 extraskeletal ewing sarcoma 10.7 MUC1 PTPRC VIM
32 mucinous bronchioloalveolar adenocarcinoma 10.7 KRT20 MUC1
33 ovarian lymphoma 10.7 CR2 PTPRC
34 mast-cell sarcoma 10.7 MPO VIM
35 juvenile xanthogranuloma 10.7 FSCN1 S100B VIM
36 lymphangiectasis 10.7 ACTC1 VIM
37 giant cell tumor 10.7 MUC1 PTPRC VIM
38 bile duct cystadenocarcinoma 10.6 KRT20 MUC1
39 rosai-dorfman disease 10.6 CR2 FSCN1 S100B
40 bladder lymphoma 10.6 CR2 KRT20
41 spindle cell rhabdomyosarcoma 10.6 MUC1 S100B
42 follicular dendritic cell sarcoma 10.6 CR1 CR2 FCER2
43 glomangiomyoma 10.6 ACTC1 VIM
44 dermoid cyst 10.6 KRT20 MUC1 VIM
45 fibrous meningioma 10.6 MUC1 S100B VIM
46 chordoid meningioma 10.6 MUC1 S100B VIM
47 eccrine porocarcinoma 10.6 MUC1 S100B VIM
48 myxopapillary ependymoma 10.6 MUC1 S100B VIM
49 monophasic synovial sarcoma 10.6 MUC1 S100B VIM
50 spiradenoma 10.6 MUC1 S100B VIM

Graphical network of the top 20 diseases related to Dendritic Cell Tumor:



Diseases related to Dendritic Cell Tumor

Symptoms & Phenotypes for Dendritic Cell Tumor

MGI Mouse Phenotypes related to Dendritic Cell Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.65 FCER2 IFNG MPO PTPRC VIM CD207
2 immune system MP:0005387 9.36 FSCN1 IFNG MPO PTPRC VIM CD207

Drugs & Therapeutics for Dendritic Cell Tumor

Drugs for Dendritic Cell Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 46)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Central Nervous System Stimulants Phase 3
2
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
3
Adenosine Approved, Investigational Phase 2 58-61-7 60961
4
Vidarabine Approved, Investigational Phase 2,Phase 1,Not Applicable 24356-66-9 32326 21704
5
Fludarabine Approved Phase 2,Phase 1,Not Applicable 21679-14-1, 75607-67-9 30751
6
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
7
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
8
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 50-18-0, 6055-19-2 2907
9
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
10
Mycophenolic acid Approved Phase 2,Not Applicable 24280-93-1 446541
11
Mycophenolate mofetil Approved, Investigational Phase 2,Not Applicable 128794-94-5 5281078
12
Pembrolizumab Approved Phase 2 1374853-91-4
13
nivolumab Approved Phase 2 946414-94-4
14
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
15
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
16 Vaccines Phase 2,Phase 1,Not Applicable
17 Ado-trastuzumab emtansine Phase 2
18 Maytansine Phase 2
19 Antimitotic Agents Phase 2
20 Antineoplastic Agents, Phytogenic Phase 2
21 Alkylating Agents Phase 2,Phase 1
22 Folic Acid Antagonists Phase 2
23 Dermatologic Agents Phase 2,Not Applicable
24 Nucleic Acid Synthesis Inhibitors Phase 2
25 Vidarabine Phosphate Phase 2,Phase 1,Not Applicable
26 Anti-Bacterial Agents Phase 2,Not Applicable
27 Vitamin B Complex Phase 2
28 Antibiotics, Antitubercular Phase 2,Not Applicable
29 Antibodies Phase 2,Phase 1
30 Immunoglobulins Phase 2,Phase 1
31 Antibodies, Monoclonal Phase 2
32 Immunosuppressive Agents Phase 2,Phase 1,Not Applicable
33 Anti-Infective Agents Phase 2,Phase 1,Not Applicable
34 Antitubercular Agents Phase 2,Not Applicable
35 Antirheumatic Agents Phase 2,Phase 1,Not Applicable
36 Antimetabolites Phase 2,Phase 1,Not Applicable
37 Antiviral Agents Phase 2,Phase 1,Not Applicable
38 Antimetabolites, Antineoplastic Phase 2,Phase 1,Not Applicable
39 Calcineurin Inhibitors Phase 2,Not Applicable
40 Antineoplastic Agents, Alkylating Phase 2,Phase 1
41 Folate Nutraceutical Phase 2
42 Vitamin B9 Nutraceutical Phase 2
43
Miconazole Approved, Investigational, Vet_approved Not Applicable 22916-47-8 4189
44 Cyclosporins Not Applicable
45 Antifungal Agents Not Applicable
46 Heptavalent Pneumococcal Conjugate Vaccine Not Applicable

Interventional clinical trials:

(show all 22)
# Name Status NCT ID Phase Drugs
1 Autologous Dendritic Cell-Tumor Cell Immunotherapy for Metastatic Melanoma Terminated NCT01875653 Phase 3
2 Vaccine Therapy in Treating Patients With Stage III or Stage IV Melanoma Unknown status NCT00085397 Phase 2
3 An Open-label Phase II Study of Lorvotuzumab Mertansine Completed NCT02420873 Phase 2 Lorvotuzumab Mertansine (IMGN901)
4 DT388IL3 Fusion Protein in Treating Patients With Acute Myeloid Leukemia or Myelodysplastic Syndromes Completed NCT00397579 Phase 1, Phase 2 DT388IL3
5 SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm or Acute Myeloid Leukemia Recruiting NCT02113982 Phase 1, Phase 2 SL-401
6 A Phase 2 Study of Pembrolizumab in Patients With Histiocyte/Dendritic Cell Neoplasms and Biologically Selected Subtypes of Relapsed/Refractory Aggressive Lymphomas Recruiting NCT03316573 Phase 2 Pembrolizumab
7 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
8 Selective Depletion of CD45RA+ T Cells From Allogeneic Peripheral Blood Stem Cell Grafts From HLA-Matched Related and Unrelated Donors in Preventing GVHD Recruiting NCT02220985 Phase 2 Cyclophosphamide;Fludarabine Phosphate;Methotrexate;Mycophenolate Mofetil;Tacrolimus;Thiotepa
9 Vaccination of Patients With Renal Cell Cancer With Dendritic Cell Tumor Fusions and GM-CSF Active, not recruiting NCT00458536 Phase 1, Phase 2 Granulocyte Macrophage Colony Stimulating Factor (GM-CSF)
10 The Use of Dendritic Cell/Tumor Fusions as a Novel Tumor Vaccine in Patients With Multiple Myeloma Completed NCT00459069 Phase 1
11 Immune Responses To Antigen-Bearing Dendritic Cells in Patients With Malignancy Completed NCT00700167 Phase 1
12 MK2206 in Treating Younger Patients With Recurrent or Refractory Solid Tumors or Leukemia Completed NCT01231919 Phase 1 Akt inhibitor MK2206
13 Study to Evaluate the Safety and Clinical Activity of UCART123 in Patients With BPDCN Recruiting NCT03203369 Phase 1
14 Genetically Modified T-cell Immunotherapy in Treating Patients With Relapsed/Refractory Acute Myeloid Leukemia and Persistent/Recurrent Blastic Plasmacytoid Dendritic Cell Neoplasm Recruiting NCT02159495 Phase 1 cyclophosphamide;Fludarabine Phosphate
15 Study of IMGN632 in Patients With Relapse/Refractory AML, BPDCN, ALL, Other CD123+ Hem Malignancies Recruiting NCT03386513 Phase 1 IMGN632
16 PH 1 Study to Evaluate Safety and Tolerability of XmAb14045 in Patients With CD123-expressing Hematologic Malignancies Recruiting NCT02730312 Phase 1
17 Vaccination With Dendritic Cell/Tumor Fusions With Autologous Stem Cell Transplants in Patients With Multiple Myeloma Active, not recruiting NCT00458653 Phase 1
18 Study of Venetoclax, a BCL2 Antagonist, for Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Not yet recruiting NCT03485547 Phase 1 Venetoclax
19 Descriptive Study of the Efficacy of Treatments for Blastic Dendritic Cell Neoplasm (BPDCN) Completed NCT02859623
20 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715 Not Applicable
21 Fludarabine Phosphate and Total-Body Radiation Followed by Donor Peripheral Blood Stem Cell Transplant and Immunosuppression in Treating Patients With Hematologic Malignancies Active, not recruiting NCT00014235 Not Applicable fludarabine phosphate;cyclosporine;mycophenolate mofetil
22 Vaccine Therapy With or Without Cryosurgery in Treating Patients With Residual, Relapsed, or Refractory B-Cell Non-Hodgkin Lymphoma Terminated NCT01239875 Not Applicable

Search NIH Clinical Center for Dendritic Cell Tumor

Genetic Tests for Dendritic Cell Tumor

Anatomical Context for Dendritic Cell Tumor

MalaCards organs/tissues related to Dendritic Cell Tumor:

41
Myeloid, T Cells, Skin, Spleen, B Cells, Bone, Bone Marrow

Publications for Dendritic Cell Tumor

Articles related to Dendritic Cell Tumor:

(show top 50) (show all 309)
# Title Authors Year
1
First pediatric experience of SL-401, a CD123-targeted therapy, in patients with blastic plasmacytoid dendritic cell neoplasm: report of three cases. ( 29720227 )
2018
2
Benefit of electron beam therapy in combination with medium-dose systemic corticosteroid as a palliative treatment for elderly patients with blastic plasmacytoid dendritic cell neoplasm: case report and review of the literature. ( 29876950 )
2018
3
Blastic plasmacytoid dendritic cell neoplasm arising from clonal hematopoiesis. ( 29705980 )
2018
4
Interdigitating dendritic cell tumor: A rare case report with review of literature. ( 29893342 )
2018
5
Clinicopathologic retrospective analysis of blastic plasmacytoid dendritic cell neoplasms. ( 29760611 )
2018
6
Blastic plasmacytoid dendritic cell neoplasm: update on therapy especially novel agents. ( 29455234 )
2018
7
Immunophenotypic CD56 Variation Within a Single Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 29490374 )
2018
8
Recurrent Indeterminate Dendritic Cell Tumor of the Skin. ( 29621879 )
2018
9
Blastic plasmacytoid dendritic cell neoplasm: a rare case of gingival lesion with leukaemic presentation. ( 29735506 )
2018
10
Clonal evolution in the transition from cutaneous disease to acute leukaemia suggested by liquid biopsy in blastic plasmacytoid dendritic cell neoplasm. ( 29419438 )
2018
11
Inflammatory pseudotumor-like follicular dendritic cell tumor of the spleen. ( 29391320 )
2018
12
Recurrent 8q24 rearrangement in blastic plasmacytoid dendritic cell neoplasm: association with immunoblastoid cytomorphology, MYC expression, and drug response. ( 29795241 )
2018
13
Blastic plasmacytoid dendritic cell neoplasm with centrocyte-like morphology clinically simulating a melanocytic nevus. ( 29293270 )
2018
14
Blastic plasmacytoid dendritic cell neoplasm. ( 29636962 )
2018
15
Report On Three Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 29666036 )
2018
16
Atypical presentation of blastic plasmacytoid dendritic cell neoplasm: A potential diagnostic pitfall in nasal cavity. ( 29884520 )
2018
17
8q24/MYC rearrangement is a recurrent cytogenetic abnormality in blastic plasmacytoid dendritic cell neoplasms. ( 29407586 )
2018
18
Exploring the effect of chidamide on blastic plasmacytoid dendritic cell neoplasm: a case report and literature review. ( 29379294 )
2018
19
Indeterminate Dendritic Cell Tumor: A Report of 2 New Cases Lacking the ETV3-NCOA2 Translocation and a Literature Review. ( 29877893 )
2018
20
A blastic plasmacytoid dendritic cell neoplasm-like phenotype identifies a subgroup of npm1-mutated acute myeloid leukemia patients with worse prognosis. ( 29080220 )
2018
21
Blastic plasmacytoid dendritic cell neoplasm with unusual morphology, MYC rearrangement and TET2 and DNMT3A mutations. ( 29411884 )
2018
22
An Analysis of Blastic Plasmacytoid Dendritic Cell Neoplasm with Translocations Involving the MYC Locus Identifies t(6;8)(p21;q24) as a Recurrent Cytogenetic Abnormality. ( 29884995 )
2018
23
Skin lesions serve as clues to relapse of pediatric blastic plasmacytoid dendritic cell neoplasm. ( 29436012 )
2018
24
Indeterminate Dendritic Cell Tumor in Thoracic Spine: A Case Report and Review of Literature. ( 28755918 )
2017
25
CD4-Negative Variant of Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm With a Novel PBRM1 Mutation in an 11-Year-Old Girl. ( 28419186 )
2017
26
A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry. ( 28409040 )
2017
27
BET Inhibitors Target Blastic Plasmacytoid Dendritic Cell Neoplasms. ( 27913389 )
2017
28
Blastic plasmacytoid dendritic cell neoplasm in children: A review of two cases. ( 28856005 )
2017
29
Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm: A Systematic Literature Review. ( 28906324 )
2017
30
Successful treatment with biweekly CHOP for bone marrow relapse of blastic plasmacytoid dendritic cell neoplasm. ( 28321093 )
2017
31
Novel Pathways and Potential Therapeutic Strategies for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): CD123 and Beyond. ( 29064022 )
2017
32
Analysis of First-Year Twitter Metrics of a Rare Disease Community for Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) on Social Media: #BPDCN. ( 29064025 )
2017
33
Blastic plasmacytoid dendritic cell neoplasm: a pathological illustration of two clinical cases. ( 28762083 )
2017
34
Recurrent MYB rearrangement in blastic plasmacytoid dendritic cell neoplasm. ( 28344318 )
2017
35
Blastic Plasmacytoid Dendritic Cell Neoplasm Presenting as Erythematous Nodules with Gallbladder Involvement. ( 28761306 )
2017
36
Concomitant Occurrence of Blastic Plasmacytoid Dendritic Cell Neoplasm and Acute Myeloid Leukaemia after Lenalidomide Treatment for. ( 28879716 )
2017
37
Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor: Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia. ( 28885734 )
2017
38
Blastic plasmacytoid dendritic cell neoplasm presenting as leukemia without cutaneous lesion. ( 29237977 )
2017
39
Haematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a North American multicentre collaborative study. ( 28980314 )
2017
40
Blastic plasmacytoid dendritic cell neoplasm with t(11;19)(q23;p13.3);KMT2A(MLL) rearranged: a diagnostic challenge. ( 27774582 )
2017
41
Complete monocular vision loss due to optic nerve involvement of blastic plasmacytoid dendritic cell neoplasm. ( 29276838 )
2017
42
Clinical features and treatment outcomes of blastic plasmacytoid dendritic cell neoplasm: a single-center experience in Korea. ( 28870016 )
2017
43
Splenic Rupture Secondary to Blastic Plasmacytoid Dendritic Cell Neoplasm. ( 28963621 )
2017
44
Hepatic inflammatory pseudotumour-like follicular dendritic cell tumor: A case report. ( 28413665 )
2017
45
Aggressive Indeterminate Dendritic Cell Tumor Mimicking Scalp Angiosarcoma. ( 28966519 )
2017
46
Blastic plasmacytoid dendritic cell neoplasm in the CSF. ( 29043229 )
2017
47
Blastic Plasmacytoid Dendritic Cell Neoplasm Evaluated by FDG PET/CT. ( 28394841 )
2017
48
Childhood Blastic Plasmacytoid Dendritic Cell Neoplasm Mimicking Acute Rheumatic Fever: Report of an Unusual Clinical Presentation and Review of Literature. ( 28859047 )
2017
49
Localized skin-limited blastic plasmacytoid dendritic cell neoplasm: A subset with possible durable remission without transplantation. ( 28752118 )
2017
50
Blastic plasmacytoid dendritic cell neoplasm following acquired erythropoietic protoporphyria. ( 28966462 )
2017

Variations for Dendritic Cell Tumor

Expression for Dendritic Cell Tumor

Search GEO for disease gene expression data for Dendritic Cell Tumor.

Pathways for Dendritic Cell Tumor

GO Terms for Dendritic Cell Tumor

Cellular components related to Dendritic Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.28 ACTC1 CR1 CR2 FCER2 FSCN1 MPO

Biological processes related to Dendritic Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.56 FCER2 FSCN1 MUC1 VIM
2 intermediate filament organization GO:0045109 9.32 KRT20 VIM
3 regulation of regulatory T cell differentiation GO:0045589 9.16 CR1 IFNG
4 complement receptor mediated signaling pathway GO:0002430 8.96 CR1 CR2
5 positive regulation of killing of cells of other organism GO:0051712 8.62 FCER2 IFNG

Sources for Dendritic Cell Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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