MCID: FLL013
MIFTS: 46

Follicular Dendritic Cell Sarcoma

Categories: Blood diseases, Cancer diseases, Immune 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 59 15
Dendritic Cell Sarcoma, Not Otherwise Specified 12 73
Dendritic Cell Sarcoma, Follicular 44 73
Dendritic Cell Sarcoma 12 15
Dendritic Cell Sarcoma Not Otherwise Specified 59
Dendritic Cell Sarcoma, Interdigitating 73
Follicular Dendritic Cell Tumour 12

Characteristics:

Orphanet epidemiological data:

59
follicular dendritic cell sarcoma
Age of onset: All ages;

Classifications:



External Ids:

Disease Ontology 12 DOID:6262 DOID:7849
ICD10 33 C96.4
MeSH 44 D054740
NCIt 50 C9281 C27260
UMLS via Orphanet 74 C1301364 C1260325
ICD10 via Orphanet 34 C96.4
MESH via Orphanet 45 D054740

Summaries for Follicular Dendritic Cell Sarcoma

Disease Ontology : 12 A histiocytic and dendritic cell cancer that is mainly located in lymph nodes.

MalaCards based summary : Follicular Dendritic Cell Sarcoma, also known as dendritic cell sarcoma, not otherwise specified, is related to dendritic cell tumor and pemphigus, and has symptoms including fever, fatigue and night sweats. An important gene associated with Follicular Dendritic Cell Sarcoma is CR2 (Complement C3d Receptor 2), and among its related pathways/superpathways are Innate Immune System and Keratinization. The drugs Pembrolizumab and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include lymph node, tonsil and liver.

Wikipedia : 76 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 77)
# Related Disease Score Top Affiliating Genes
1 dendritic cell tumor 32.8 CR1 CR2 FCER2
2 pemphigus 30.6 DSP EVPL PPL
3 paraneoplastic pemphigus 30.6 DSP EVPL PPL
4 castleman disease 30.5 CR1 CR2 PPL
5 interdigitating dendritic cell sarcoma 30.2 CR1 CR2
6 reticulum cell sarcoma 30.0 CR1 CR2 FCER2
7 histiocytic sarcoma 30.0 CR1 CR2
8 cellulitis 29.9 DSP PPL
9 thymoma 29.7 EVPL KIT PPL
10 sarcoma 11.1
11 myasthenia gravis 10.3
12 myasthenia gravis congenital 10.3
13 spindle cell sarcoma 10.3
14 parapharyngeal meningioma 10.2 CR1 CR2
15 langerhans cell sarcoma 10.2 CR1 CR2
16 t-cell/histiocyte rich large b cell lymphoma 10.2 CR1 CR2
17 lateral medullary syndrome 10.1 CR1 CR2
18 lymphoma 10.1
19 melanoma 10.1
20 spindle cell liposarcoma 10.1 CR2 KIT
21 smooth muscle tumor 10.1 CR2 KIT
22 pemphigus foliaceus 10.0 EVPL PPL
23 subcorneal pustular dermatosis 10.0 EVPL PPL
24 plexiform schwannoma 10.0 DSP KIT
25 inflammatory myofibroblastic tumor 10.0 CR1 CR2 KIT
26 bronchiolitis obliterans 10.0 EVPL PPL
27 perineurioma 10.0 DSP KIT
28 intussusception 10.0
29 kaposi sarcoma 10.0
30 cryptorchidism, unilateral or bilateral 10.0
31 nasopharyngeal carcinoma 10.0
32 autoimmune lymphoproliferative syndrome, type v 10.0
33 arthritis 10.0
34 leukemia 10.0
35 lymphoproliferative syndrome 10.0
36 angioimmunoblastic t-cell lymphoma 10.0
37 squamous cell carcinoma 10.0
38 mediastinum sarcoma 10.0
39 carcinosarcoma 10.0
40 small intestinal sarcoma 10.0
41 thyroiditis 10.0
42 mediastinitis 10.0
43 appendicitis 10.0
44 myeloid leukemia 10.0
45 amyloidosis 10.0
46 liver inflammatory pseudotumor 10.0
47 unicentric castleman disease 10.0
48 leukemia, chronic lymphocytic 2 9.9
49 leukemia, chronic lymphocytic 9.9
50 lymphocytic leukemia 9.9

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:


fever, fatigue, night sweats

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 Antibodies Phase 2
3 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:

41
Lymph Node, Tonsil, Liver, T Cells, Spleen, Myeloid, B Cells

The Foundational Model of Anatomy Ontology organs/tissues related to Follicular Dendritic Cell Sarcoma:

19
Lymph Nodes

Publications for Follicular Dendritic Cell Sarcoma

Articles related to Follicular Dendritic Cell Sarcoma:

(show top 50) (show all 228)
# Title Authors Year
1
A Rare Case of Retroperitoneal Follicular Dendritic Cell Sarcoma Identified by 99mTc-HYNIC-TOC SPECT/CT. ( 29863574 )
2018
2
Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature. ( 29928216 )
2018
3
Image Gallery: Paraneoplastic pemphigus and follicular dendritic cell sarcoma. ( 29441554 )
2018
4
Retroperitoneal follicular dendritic cell sarcoma in a young woman: Diagnosis and treatment challenges. ( 29395417 )
2018
5
Follicular dendritic cell sarcoma of the duodenum: an extremely rare entity. ( 29440239 )
2018
6
Follicular dendritic cell sarcoma presenting as a painless lump in the parotid. ( 29848530 )
2018
7
Indolent T-lymphoblastic proliferation associated with low grade follicular dendritic cell sarcoma and Castleman disease. ( 29486963 )
2018
8
Follicular dendritic cell sarcoma of the porta hepatis. ( 29437813 )
2018
9
Cytological diagnosis of follicular dendritic cell sarcoma: A case report and review of literature. ( 29683532 )
2018
10
Follicular Dendritic Cell Sarcoma With Indolent T-Lymphoblastic Proliferation Is Associated With Paraneoplastic Autoimmune Multiorgan Syndrome. ( 30222603 )
2018
11
Follicular Dendritic Cell Sarcoma of Gastrointestinal Tract: an Uncommon Lesion, Commonly Missed. ( 30430359 )
2018
12
Unusual Presentation of Follicular Dendritic Cell Sarcoma as a Cystic Neck Swelling. ( 30473898 )
2018
13
Profiles of genomic alterations in primary esophageal follicular dendritic cell sarcoma: A case report. ( 30508944 )
2018
14
Hemorrhagic intracranial follicular dendritic cell sarcoma: A case report. ( 29119046 )
2017
15
Comprehensive Genomic Profiling of a Rare Thyroid Follicular Dendritic Cell Sarcoma. ( 28975018 )
2017
16
Cytomorphological Findings of Follicular Dendritic Cell Sarcoma on Fine-Needle Aspiration Cytology. ( 29275417 )
2017
17
Follicular dendritic cell sarcoma presenting as colonic intussusception. ( 29256544 )
2017
18
Follicular dendritic cell sarcoma of the liver with metachronous small bowel and splenic metastases: a case report and literature review. ( 28653001 )
2017
19
Identification of novel follicular dendritic cell sarcoma markers, FDCSP and SRGN, by whole transcriptome sequencing. ( 28145886 )
2017
20
p16 expression in follicular dendritic cell sarcoma: a potential mimicker of human papillomavirus-related oropharyngeal squamous cell carcinoma. ( 28554574 )
2017
21
Characteristics, management, and outcomes of patients with follicular dendritic cell sarcoma. ( 28382648 )
2017
22
Angioimmunoblastic T-Cell Lymphoma: A Questionable Association with Follicular Dendritic Cell Sarcoma. ( 28197348 )
2017
23
Primary follicular dendritic cell sarcoma of the urinary bladder: the first case report and potential diagnostic pitfalls. ( 28438212 )
2017
24
Mediastinitis After Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration of a Follicular Dendritic Cell Sarcoma. ( 28843365 )
2017
25
Recurrent Follicular Dendritic Cell Sarcoma of the Parotid Gland Imaged with 18F-FDG PET/CT. ( 29242731 )
2017
26
Follicular dendritic cell sarcoma of the small intestine detected by double-balloon enteroscopy. ( 28543909 )
2017
27
Follicular Dendritic Cell Sarcoma. ( 28353378 )
2017
28
Small intestine follicular dendritic cell sarcoma with liver metastasis: A case report. ( 28767565 )
2017
29
"Medusa-Head" cells, "Starfish" cells, and interconnecting long cytoplasmic processes as diagnostic cytologic clues for follicular dendritic cell sarcoma in fine needle aspiration samples. ( 28160447 )
2017
30
Follicular dendritic cell sarcoma of the head and neck. Literature review and report of the tonsil occurrence in a Ugandan patient. ( 28748971 )
2017
31
Follicular Dendritic Cell Sarcoma of the Larynx: Apropos a Rare Case with Review of the Literature. ( 28984342 )
2017
32
Genomic analysis of follicular dendritic cell sarcoma by molecular inversion probe array reveals tumor suppressor-driven biology. ( 28621320 )
2017
33
Inflammatory Pseudotumor-Like Follicular Dendritic Cell Sarcoma of the Spleen: Computed Tomography Imaging Characteristics in 5 Patients. ( 29287022 )
2017
34
Response to rituximab in an anti-muscle-specific receptor tyrosine kinase- and anti-acetylcholine recepto-double-positive myasthenia gravis patient concomitant with follicular dendritic cell sarcoma. ( 26818174 )
2016
35
Follicular dendritic cell tumour/sarcoma: a commonly misdiagnosed tumour in the thorax Intrathoracic follicular dendritic cell sarcoma. ( 27206572 )
2016
36
Paraneoplastic arthritis as first symptom of a liver inflammatory pseudotumor-like follicular dendritic cell sarcoma. ( 27125361 )
2016
37
Clinicopathological characteristics of mediastinal follicular dendritic cell sarcoma: report of three cases. ( 27068522 )
2016
38
Follicular dendritic cell sarcoma: clinicopathologic study of 15 cases with emphasis on novel expression of MDM2, somatostatin receptor 2A, and PD-L1. ( 27402219 )
2016
39
Paraneoplastic pemphigus and myasthenia gravis, associated with inflammatory pseudotumor-like follicular dendritic cell sarcoma: response to rituximab. ( 27525088 )
2016
40
High efficacy of tumor-targeting Salmonella typhimurium A1-R on a doxorubicin- and dactolisib-resistant follicular dendritic-cell sarcoma in a patient-derived orthotopic xenograft PDOX nude mouse model. ( 27105519 )
2016
41
Follicular dendritic cell sarcoma treated with a variety of chemotherapy. ( 27734516 )
2016
42
(18)F-FDG PET/CT follow-up of follicular dendritic cell sarcoma. ( 27793634 )
2016
43
Follicular dendritic cell sarcoma of the spleen: A case report and review of the literature. ( 27602139 )
2016
44
A Rare Case of Epstein-Barr Virus Negative Inflammatory Pseudotumor-like Follicular Dendritic Cell Sarcoma Presenting as a Solitary Colonic Mass in a 53-Year-Old Woman; Case Report and Review of Literature. ( 27299190 )
2016
45
Cytological features of inflammatory pseudotumor-like follicular dendritic cell sarcoma of spleen: A case report. ( 27775241 )
2016
46
Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case. ( 27001632 )
2016
47
Poster 10 Successful Rehabilitation Outcome from Rare Brain Tumor (Intracranial Follicular Dendritic Cell Sarcoma): A Case Report. ( 27672780 )
2016
48
Clinicopathological Profile and Outcomes of Follicular Dendritic Cell Sarcoma of the Head and Neck Region - A Study of 10 Cases with Literature Review. ( 27656545 )
2016
49
Follicular Dendritic Cell Sarcoma of Appendix Presenting as Acute Appendicitis: An Exceptionally Rare Case Report. ( 27225654 )
2016
50
Treatment of breast cancer 2 (BRCA2)-mutant follicular dendritic cell sarcoma with a poly ADP-ribose polymerase (PARP) inhibitor: a case report. ( 27488020 )
2016

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

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 9.91 CD274 CLU CR1 CR2 DSP EVPL
2 cell surface GO:0009986 9.67 CD274 CLU CR1 KIT
3 external side of plasma membrane GO:0009897 9.61 CD274 FCER2 KIT
4 intermediate filament GO:0005882 9.5 DSP EVPL PPL
5 ficolin-1-rich granule membrane GO:0101003 9.4 CR1 DSP
6 cornified envelope GO:0001533 9.33 DSP EVPL PPL
7 extracellular exosome GO:0070062 9.23 CD274 CLU CR1 CR2 DSP EVPL
8 desmosome GO:0030057 9.13 DSP EVPL PPL
9 plasma membrane GO:0005886 10 CD274 CR1 CR2 DSP FCER2 KIT

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

# Name GO ID Score Top Affiliating Genes
1 keratinization GO:0031424 9.58 DSP EVPL PPL
2 complement activation, classical pathway GO:0006958 9.54 CLU CR1 CR2
3 keratinocyte differentiation GO:0030216 9.46 DSP EVPL
4 peptide cross-linking GO:0018149 9.43 DSP EVPL
5 cornification GO:0070268 9.43 DSP EVPL PPL
6 regulation of complement activation GO:0030449 9.33 CLU CR1 CR2
7 complement receptor mediated signaling pathway GO:0002430 9.32 CR1 CR2
8 wound healing GO:0042060 9.13 DSP EVPL PPL
9 intermediate filament cytoskeleton organization GO:0045104 8.8 DSP EVPL PPL

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

# Name GO ID Score Top Affiliating Genes
1 virus receptor activity GO:0001618 8.96 CR1 CR2
2 structural molecule activity GO:0005198 8.8 DSP EVPL PPL

Sources for Follicular Dendritic Cell Sarcoma

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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