MCID: FLL013
MIFTS: 44

Follicular Dendritic Cell Sarcoma

Categories: Blood diseases, Cancer diseases, Rare diseases

Aliases & Classifications for Follicular Dendritic Cell Sarcoma

MalaCards integrated aliases for Follicular Dendritic Cell Sarcoma:

Name: Follicular Dendritic Cell Sarcoma 12 12 58 15 17
Dendritic Cell Sarcoma, Not Otherwise Specified 12 70
Dendritic Cell Sarcoma, Follicular 44 70
Dendritic Cell Sarcoma 12 15
Dendritic Cell Sarcoma Not Otherwise Specified 58
Dendritic Cell Sarcoma, Interdigitating 70
Follicular Dendritic Cell Tumour 12

Characteristics:

Orphanet epidemiological data:

58
follicular dendritic cell sarcoma
Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:6262 DOID:7849
MeSH 44 D054740
NCIt 50 C27260 C9281
SNOMED-CT 67 128816008 397355008
ICD10 32 C96.4
MESH via Orphanet 45 D054740
ICD10 via Orphanet 33 C96.4
UMLS via Orphanet 71 C1260325 C1301364
UMLS 70 C1260325 C1260326 C1301364

Summaries for Follicular Dendritic Cell Sarcoma

Disease Ontology : 12 A dendritic cell sarcoma cancer that effects the follicular dendritic cells.

MalaCards based summary : Follicular Dendritic Cell Sarcoma, also known as dendritic cell sarcoma, not otherwise specified, is related to rare tumor and paraneoplastic pemphigus, and has symptoms including fatigue, fever and night sweats. An important gene associated with Follicular Dendritic Cell Sarcoma is CR1 (Complement C3b/C4b Receptor 1 (Knops Blood Group)), and among its related pathways/superpathways are Innate Immune System and Keratinization. The drugs Pembrolizumab and Antineoplastic Agents, Immunological have been mentioned in the context of this disorder. Affiliated tissues include tonsil, lymph node and spleen, and related phenotypes are hematopoietic system and immune system

Wikipedia : 73 Follicular dendritic cell sarcoma (FDCS) is an extremely rare neoplasm. While the existence of FDC... more...

Related Diseases for Follicular Dendritic Cell Sarcoma

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

(show top 50) (show all 256)
# Related Disease Score Top Affiliating Genes
1 rare tumor 31.6 KIT FCER2 CR2
2 paraneoplastic pemphigus 30.8 PPL EVPL DSP DSG3
3 pemphigus 30.8 PPL EVPL DSP DSG3
4 histiocytic and dendritic cell cancer 30.6 CR2 CR1 CLU CD68
5 dendritic cell tumor 30.6 FCER2 CR2 CR1 CD68
6 interdigitating dendritic cell sarcoma 30.6 CR2 CR1
7 liver inflammatory pseudotumor 30.5 CR2 CD34
8 reticulum cell sarcoma 30.4 FCER2 CR2 CR1
9 langerhans cell sarcoma 30.4 CR2 CR1 CD68
10 histiocytic sarcoma 30.3 CDKN2A CD68
11 small intestinal sarcoma 30.3 KIT CD68
12 follicular lymphoma 30.3 FCER2 CR2 CR1
13 meningioma, familial 30.3 DSP CDKN2A CD68 CD34
14 fibrous histiocytoma 30.2 KIT CD68 CD34
15 castleman disease 30.2 PPL FCER2 DSG3 CXCL13 CR2 CR1
16 retroperitoneal leiomyosarcoma 30.0 KIT CD34
17 bladder sarcoma 30.0 KIT CD68 CD34
18 mesenchymal cell neoplasm 30.0 KIT CD68 CD34
19 liposarcoma 30.0 CDKN2A CD68 CD34
20 angiosarcoma 30.0 KIT CD68 CD34
21 pemphigus vulgaris, familial 29.9 PPL EVPL DSP DSG3
22 dedifferentiated liposarcoma 29.9 KIT CDKN2A CD34
23 connective tissue benign neoplasm 29.8 KIT CD68 CD34
24 pancytopenia 29.7 KIT CD68 CD34
25 fibrosarcoma of bone 29.7 KIT CDKN2A CD68
26 thymoma 29.6 PPL KIT EVPL DSP CD274
27 b-cell lymphoma 29.5 FCER2 CXCL13 CR2 CDKN2A CD274
28 bullous pemphigoid 29.4 PPL FCER2 EVPL DSP DSG3
29 leukemia, chronic lymphocytic 29.4 FCER2 CR2 CR1 CD34 CD274
30 inflammatory myofibroblastic tumor 29.4 KIT FCER2 CR2 CR1 CD68 CD34
31 myelodysplastic syndrome 29.1 KIT CR1 CDKN2A CD68 CD34
32 malignant spindle cell melanoma 29.1 KIT CDKN2A CD68 CD34 CD274
33 malignant peripheral nerve sheath tumor 29.0 KIT CDKN2A CD34 CD274
34 sarcoma 11.3
35 spindle cell sarcoma 11.3
36 soft tissue sarcoma 10.6
37 myasthenia gravis 10.5
38 intracranial meningioma 10.5
39 secretory meningioma 10.5
40 lymphoplasmacyte-rich meningioma 10.5
41 thymoma, familial 10.4
42 parapharyngeal meningioma 10.4 CR2 CR1
43 posttransplant acute limbic encephalitis 10.4
44 anaplastic large cell lymphoma 10.3
45 angioimmunoblastic t-cell lymphoma 10.3
46 dysphagia 10.3
47 esophageal tuberculosis 10.3 CR2 CD68
48 histiocytosis 10.3
49 gallbladder lymphoma 10.2 FCER2 CR2
50 nodular tenosynovitis 10.2 CLU CD68

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



Diseases related to Follicular Dendritic Cell Sarcoma

Symptoms & Phenotypes for Follicular Dendritic Cell Sarcoma

UMLS symptoms related to Follicular Dendritic Cell Sarcoma:


fatigue; fever; night sweats

MGI Mouse Phenotypes related to Follicular Dendritic Cell Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.85 CD274 CD34 CD68 CDKN2A CR2 CXCL13
2 immune system MP:0005387 9.73 CD274 CD34 CD68 CDKN2A CLU CR2
3 integument MP:0010771 9.23 CD274 CD34 CDKN2A DSG3 DSP EVPL

Drugs & Therapeutics for Follicular Dendritic Cell Sarcoma

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Pembrolizumab Approved Phase 2 1374853-91-4
2 Antineoplastic Agents, Immunological Phase 2
3 Antibodies Phase 2
4 Immunoglobulins Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 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

Search NIH Clinical Center for Follicular Dendritic Cell Sarcoma

Cochrane evidence based reviews: dendritic cell sarcoma, follicular

Genetic Tests for Follicular Dendritic Cell Sarcoma

Anatomical Context for Follicular Dendritic Cell Sarcoma

MalaCards organs/tissues related to Follicular Dendritic Cell Sarcoma:

40
Tonsil, Lymph Node, Spleen, Thyroid, Breast, Small Intestine, Appendix

Publications for Follicular Dendritic Cell Sarcoma

Articles related to Follicular Dendritic Cell Sarcoma:

(show top 50) (show all 372)
# Title Authors PMID Year
1
Inflammatory pseudotumor-like follicular dendritic cell sarcoma mimicking a colonic polyp. 61
33674906 2021
2
Follicular dendritic cell sarcoma: Cytomorphologic features and diagnostic challenges. 61
33418601 2021
3
Clinicopathological characteristics of extranodal follicular dendritic cell sarcoma: A report of two cases. 61
33574921 2021
4
First case report of Epstein-Barr virus-positive inflammatory pseudotumour-like follicular dendritic cell sarcoma in Australia. 61
32786020 2021
5
Imaging features and radiologic-pathologic correlations of inflammatory pseudotumor-like follicular dendritic cell sarcoma. 61
33731032 2021
6
Tonsillar p16-Positive Follicular Dendritic Cell Sarcoma Mimicking HPV-Related Oropharyngeal Squamous Cell Carcinoma: A Case Report and Review of Reported Cases. 61
32189159 2021
7
18F-FDG PET/CT in Uterine Follicular Dendritic Cell Sarcoma. 61
33782302 2021
8
Follicular Dendritic Cell Sarcomas: CT and MRI Findings in 20 Patients. 61
33405946 2021
9
Fine needle aspiration of an intranodal follicular dendritic cell sarcoma: A case report with molecular analysis and review of the literature. 61
32816379 2021
10
A Case of Paraneoplastic Pemphigus with Follicular Dendritic Cell Sarcoma. 61
33621533 2021
11
A rare case of primary pulmonary inflammatory pseudotumor-like follicular dendritic cell sarcoma successfully treated by lobectomy. 61
33553370 2021
12
Metastatic follicular dendritic cell sarcoma treated with gemcitabine plus docetaxel with an outstanding survival: A case report and review of literature. 61
33489199 2021
13
Extranodal Follicular Dendritic Cell Sarcoma-A Review: "What the Mind Does Not Know the Eye Does Not See". 61
32991350 2021
14
Cytologic and histological features of rare nonepithelial and nonlymphoid tumors of the thyroid. 61
33493367 2021
15
Epstein-Barr virus-negative inflammatory pseudotumor-like variant of follicular dendritic cell sarcoma of the liver: A case report and literature review. 61
32540141 2021
16
Hematolymphoid Neoplasms Rarely Mimic Undifferentiated Pleomorphic Sarcoma of Soft Tissue. 61
32167380 2020
17
Follicular dendritic cell sarcoma: a novel orbital tumour. 61
33285088 2020
18
Intra-abdominal Follicular Dendritic Cell Sarcoma (FDCS): Series of 18 cases of a rare entity from Pakistan. 61
32905993 2020
19
Inflammatory pseudotumor-like follicular dendritic cell sarcoma of the liver. 61
33371694 2020
20
Multidisciplinary diagnosis and treatment of recurrent follicular dendritic cell sarcoma in abdomen: A case report. 61
33371089 2020
21
New Clinicopathologic Scenarios of EBV+ Inflammatory Follicular Dendritic Cell Sarcoma: Report of 9 Extrahepatosplenic Cases. 61
33264138 2020
22
Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma presenting as a solitary colonic mass: two rare cases and a literature review. 61
32506505 2020
23
Follicular dendritic cell sarcoma presenting as duodenal tumor. 61
32439273 2020
24
Multiple recurrent follicular dendritic cell sarcoma: A case report. 61
32765871 2020
25
Beware the inflammatory cell-rich colonic polyp: a rare case of EBV-positive inflammatory pseudotumour-like follicular dendritic cell sarcoma with increased IgG4-positive plasma cells. 61
32814623 2020
26
Dedifferentiated Liposarcoma With Meningothelial-Like Whorls: Five Additional Cases and Review of the Literature. 61
32419561 2020
27
Paraneoplastic Pemphigus Associated with Bilateral Corneal Perforations in Follicular Dendritic Cell Sarcoma. 61
33048604 2020
28
Disease Response to Pazopanib in Follicular Dendritic Cell Sarcoma. 61
33082759 2020
29
Descriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience. 61
32882761 2020
30
[Clinicopathological Features of Follicular Dendritic Cell Sarcoma]. 61
32895103 2020
31
Follicular dendritic cell sarcoma in the right chest wall: A case report. 61
32871936 2020
32
A case in which paraneoplastic pemphigus and bronchiolitis obliterans are the main manifestations of inflammatory pseudotumour-like follicular dendritic cell sarcoma. 61
32319082 2020
33
Follicular dendritic cell sarcoma of the uterine cervix: a case report. 61
32807169 2020
34
18F-FDG PET/CT in Follicular Dendritic Cell Sarcoma With Paraneoplastic Pemphigus as the First Manifestation. 61
32371617 2020
35
Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature. 61
32775392 2020
36
Follicular dendritic cell sarcoma presenting as a thyroid mass: an unusual case report and literature review. 61
32589853 2020
37
Granuloma With an Underlying Lymphoma: A Diagnostic Challenge and a Wider Histologic Spectrum Including Adult T-Cell Leukemia/Lymphoma. 61
30653030 2020
38
Follicular dendritic cell sarcoma and its response to immune checkpoint inhibitors nivolumab and ipilimumab. 61
32327462 2020
39
Retroperitoneal Follicular Dendritic Cell Sarcoma: A Case Report. 61
32280831 2020
40
Follicular dendritic cell sarcoma of the parotid gland: A case report. 61
31839406 2020
41
[Hepatic inflammatory pseudotumor-like follicular dendritic cell sarcoma]. 61
32164072 2020
42
Not always 'squame': The rare entity of follicular dendritic cell sarcoma of the tonsil presenting with cervical nodal metastases. 61
33391840 2020
43
[Special histopathological variants and potential diagnostic traps of classical follicular dendritic cell sarcoma]. 61
31914532 2020
44
Follicular Dendritic Cell Sarcoma With Co-Expression of CD4 and CD30 Mimics Anaplastic Large Cell Lymphoma. 61
32547956 2020
45
Primary Tonsillar Epithelioid Follicular Dendritic Cell Sarcoma: Report of a Rare Case Mimicking Undifferentiated Carcinoma and a Brief Review of the Literature. 61
30758753 2019
46
Follicular Dendritic Cell Sarcoma of Gastrointestinal Tract: an Uncommon Lesion, Commonly Missed. 61
30430359 2019
47
Inflammatory pseudotumor-like follicular dendritic cell sarcoma: A brief report of two cases. 61
31908727 2019
48
Uncommon retroperitoneal tumour: follicular dendritic cell sarcoma. 61
31690245 2019
49
Refractory mucositis associated with underlying follicular dendritic cell sarcoma of the thymus: Paraneoplastic pemphigus versus malignancy-exacerbated pemphigus vulgaris. 61
31687457 2019
50
Feasibility of laparoscopic isolated caudate lobe resection for rare hepatic mesenchymal neoplasms. 61
31667169 2019

Variations for Follicular Dendritic Cell Sarcoma

Expression for Follicular Dendritic Cell Sarcoma

Search GEO for disease gene expression data for Follicular Dendritic Cell Sarcoma.

Pathways for Follicular Dendritic Cell Sarcoma

Pathways related to Follicular Dendritic Cell Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.61 PPL KIT FCER2 DSP CR1 CLU
2
Show member pathways
11.91 PPL EVPL DSP DSG3
3
Show member pathways
11.81 CR2 CR1 CLU
4 11.74 CR2 CR1 CLU
5 11.56 KIT CD68 CD34
6
Show member pathways
11.47 PPL EVPL DSP
7 11.08 CXCL13 CR2 CR1 CD274
8 11.02 KIT FCER2 CR2 CR1 CD34

GO Terms for Follicular Dendritic Cell Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.18 PPL KIT FCER2 EVPL DSP DSG3
2 plasma membrane GO:0005886 10.07 PPL KIT FCER2 DSP DSG3 CR2
3 extracellular exosome GO:0070062 9.81 PPL FCER2 EVPL DSP DSG3 CR2
4 cell surface GO:0009986 9.65 KIT CR1 CLU CD34 CD274
5 external side of plasma membrane GO:0009897 9.62 KIT FCER2 CD34 CD274
6 cornified envelope GO:0001533 9.26 PPL EVPL DSP DSG3
7 desmosome GO:0030057 8.92 PPL EVPL DSP DSG3

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 cellular response to lipopolysaccharide GO:0071222 9.65 CXCL13 CD68 CD274
2 complement activation, classical pathway GO:0006958 9.63 CR2 CR1 CLU
3 regulation of complement activation GO:0030449 9.58 CR2 CR1 CLU
4 wound healing GO:0042060 9.54 PPL EVPL DSP
5 negative regulation of interleukin-2 production GO:0032703 9.49 CR1 CD34
6 keratinization GO:0031424 9.46 PPL EVPL DSP DSG3
7 complement activation, alternative pathway GO:0006957 9.43 CR2 CR1
8 complement receptor mediated signaling pathway GO:0002430 9.32 CR2 CR1
9 somatic stem cell division GO:0048103 9.26 KIT CDKN2A
10 cornification GO:0070268 9.26 PPL EVPL DSP DSG3
11 intermediate filament cytoskeleton organization GO:0045104 8.8 PPL EVPL DSP

Sources for Follicular Dendritic Cell Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....