MCID: HST016
MIFTS: 38

Histiocytic Sarcoma

Categories: Blood diseases, Cancer diseases, Rare diseases

Aliases & Classifications for Histiocytic Sarcoma

MalaCards integrated aliases for Histiocytic Sarcoma:

Name: Histiocytic Sarcoma 58 70 32

Characteristics:

Orphanet epidemiological data:

58
histiocytic sarcoma
Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 32 C96.8
MESH via Orphanet 45 D054747
ICD10 via Orphanet 33 C96.8
UMLS via Orphanet 71 C0334663
Orphanet 58 ORPHA86896
UMLS 70 C0334663

Summaries for Histiocytic Sarcoma

MalaCards based summary : Histiocytic Sarcoma is related to fibrosarcoma of bone and histiocytosis. An important gene associated with Histiocytic Sarcoma is CD163 (CD163 Molecule), and among its related pathways/superpathways are Pathways in cancer and Endometrial cancer. The drugs Pembrolizumab and Vemurafenib have been mentioned in the context of this disorder. Affiliated tissues include myeloid, spleen and bone marrow, and related phenotypes are hematopoietic system and immune system

Wikipedia : 73 Histiocytic sarcoma is a tumor derived from histiocytes. The tumor is often positive for CD163 and can... more...

Related Diseases for Histiocytic Sarcoma

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

(show top 50) (show all 287)
# Related Disease Score Top Affiliating Genes
1 fibrosarcoma of bone 31.5 CDKN2A CD68
2 histiocytosis 31.0 CD163 BRAF
3 rosai-dorfman disease 30.4 CD163 BRAF
4 langerhans cell histiocytosis 30.1 CD68 CD163 BRAF
5 histiocytic and dendritic cell cancer 30.1 CD68 CD163
6 langerhans cell sarcoma 29.9 CD68 CD163
7 synovitis 29.8 CD68 CD163
8 thyroid carcinoma 29.8 PTEN BRAF
9 fibrous histiocytoma 29.8 CD68 CD163
10 meningioma, familial 29.7 PTEN CDKN2A CD68
11 teratoma 29.6 PTEN CDKN2A
12 precursor t-cell acute lymphoblastic leukemia 29.5 PTEN CDKN2A
13 central nervous system lymphoma 29.5 CDKN2A CD68
14 small cell cancer of the lung 29.5 PTEN CDKN2A
15 glioma 29.4 PTEN CDKN2A BRAF
16 adenocarcinoma 29.2 PTEN CDKN2A BRAF
17 colorectal adenocarcinoma 29.2 PTEN CDKN2A BRAF
18 renal cell carcinoma, papillary, 1 29.2 PTEN CDKN2A BRAF
19 gastric adenocarcinoma 29.2 PTEN CDKN2A BRAF
20 malignant astrocytoma 29.2 PTEN CDKN2A BRAF
21 myelodysplastic syndrome 29.2 PTEN CDKN2A CD68
22 high grade glioma 29.2 PTEN CDKN2A BRAF
23 gastrointestinal stromal tumor 29.2 PTEN CDKN2A BRAF
24 liposarcoma 29.2 PTEN CDKN2A CD68
25 sarcoma 11.2
26 spindle cell sarcoma 11.2
27 reticulosarcoma 11.1
28 rare tumor 10.5
29 splenomegaly 10.5
30 b-cell lymphoma 10.4
31 follicular lymphoma 10.4
32 leukemia, acute lymphoblastic 10.4
33 lymphoma 10.3
34 thrombocytopenia 10.3
35 ataxia and polyneuropathy, adult-onset 10.2
36 diffuse large b-cell lymphoma 10.2
37 intracranial meningioma 10.2
38 pancytopenia 10.2
39 secretory meningioma 10.2
40 lymphoplasmacyte-rich meningioma 10.2
41 germ cells tumors 10.2
42 leukemia 10.2
43 brain sarcoma 10.2
44 hemolytic anemia 10.2
45 lymphoma, hodgkin, classic 10.2
46 anaplastic large cell lymphoma 10.2
47 melanoma 10.2
48 interdigitating dendritic cell sarcoma 10.2
49 renal cell carcinoma, nonpapillary 10.1
50 leukemia, acute monocytic 10.1

Graphical network of the top 20 diseases related to Histiocytic Sarcoma:



Diseases related to Histiocytic Sarcoma

Symptoms & Phenotypes for Histiocytic Sarcoma

MGI Mouse Phenotypes related to Histiocytic Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.72 BRAF CD163 CD68 CDKN2A PTEN
2 immune system MP:0005387 9.65 BRAF CD163 CD68 CDKN2A PTEN
3 muscle MP:0005369 9.46 BRAF CD163 CDKN2A PTEN
4 pigmentation MP:0001186 9.13 BRAF CDKN2A PTEN
5 respiratory system MP:0005388 8.92 BRAF CD163 CDKN2A PTEN

Drugs & Therapeutics for Histiocytic Sarcoma

Drugs for Histiocytic Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 25)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Pembrolizumab Approved Phase 2 1374853-91-4
2
Vemurafenib Approved Phase 2 918504-65-1 23252090 42611257
3
Olaparib Approved Phase 2 763113-22-0 23725625
4
Mebendazole Approved, Vet_approved Phase 2 31431-39-7 4030
5
Palbociclib Approved, Investigational Phase 2 571190-30-2 11431660 5005498 5330286
6
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
7
Piperazine Approved, Vet_approved Phase 2 110-85-0 4837
8
Lenalidomide Approved Phase 2 191732-72-6 216326
9
Mesna Approved, Investigational Phase 2 3375-50-6 598
10
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
11
Ifosfamide Approved Phase 2 3778-73-2 3690
12
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
13
Ensartinib Investigational Phase 2 1370651-20-9
14
tipifarnib Investigational Phase 2 192185-72-1 159324
15 Antineoplastic Agents, Immunological Phase 2
16 Immunoglobulins Phase 2
17 Antibodies Phase 2
18 Poly(ADP-ribose) Polymerase Inhibitors Phase 2
19 DMP 777 Phase 2 157341-41-8
20 Protein Kinase Inhibitors Phase 2
21 Piperazine citrate Phase 2
22 Angiogenesis Inhibitors Phase 2
23 Immunologic Factors Phase 2
24
Liposomal doxorubicin Phase 2 31703
25
Isophosphamide mustard Phase 2 100427

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
2 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib;Olaparib;Palbociclib;Samotolisib;Selpercatinib;Selumetinib Sulfate;Tazemetostat;Tipifarnib;Ulixertinib;Vemurafenib
3 A Phase II Study of Lenalidomide for Adult Histiocyte Disorders Active, not recruiting NCT02523040 Phase 2 Lenalidomide
4 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Active, not recruiting NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
5 A Phase 2 Study to Assess the Safety and Efficacy of Cobimetinib in Refractory Langerhans Cell Histiocytosis, LCH-Associated Neurodegenerative Disease, and Other Histiocytic Disorders. Not yet recruiting NCT04079179 Phase 2 Cobimetinib

Search NIH Clinical Center for Histiocytic Sarcoma

Genetic Tests for Histiocytic Sarcoma

Anatomical Context for Histiocytic Sarcoma

MalaCards organs/tissues related to Histiocytic Sarcoma:

40
Myeloid, Spleen, Bone Marrow, Lymph Node, Brain, Heart, Kidney

Publications for Histiocytic Sarcoma

Articles related to Histiocytic Sarcoma:

(show top 50) (show all 648)
# Title Authors PMID Year
1
Histiocytic sarcoma following combination chemotherapy for primary mediastinal germ cell tumor: a diagnostic dilemma. 61
33786289 2021
2
Identification of common predisposing loci to hematopoietic cancers in four dog breeds. 61
33793571 2021
3
Correlation between Tumour Associated Macrophage (TAM) Infiltration and Mitotic Activity in Canine Soft Tissue Sarcomas. 61
33806407 2021
4
Clinical, histopathological, and immunohistochemical studies of histiocytic sarcoma in four-toed hedgehogs (Atelerix albiventris): A retrospective study. 61
33390364 2021
5
Intratumoral Canine Distemper Virus Infection Inhibits Tumor Growth by Modulation of the Tumor Microenvironment in a Murine Xenograft Model of Canine Histiocytic Sarcoma. 61
33808256 2021
6
Case report: Haemophagocytic histiocytic sarcoma in an english setter. 61
33750030 2021
7
Histiocytic Sarcoma Arising From a Long Bone: Report of Two Cases. 61
33750231 2021
8
Successful Treatment of Incidental Histiocytic Sarcoma Concomitant with Laryngeal Carcinoma. 61
33678749 2021
9
Overview of Gastrointestinal Lymphoproliferative disorders✰. 61
33714627 2021
10
Histiocytosis and Neoplasms of Macrophage-Dendritic Cell Lineages: Multimodality Imaging with Emphasis on PET/CT. 61
33606566 2021
11
Histiocytic sarcoma arising in a child affected by Burkitt lymphoma, with t(8;14)(q24;q32) positivity in both tumors. 61
33621154 2021
12
The mutational landscape of histiocytic sarcoma associated with lymphoid malignancy. 61
32929178 2021
13
Histiocytic Sarcoma: Challenging Course, Dismal Outcome. 61
33671860 2021
14
Short-term response to alemtuzumab in CD52-positive secondary histiocytic sarcoma in a child: Is it time to consider new targets? 61
32897114 2021
15
Histiocytic sarcoma with brainstem and spinal canal involvement detected by [18F]-FDG PET/CT. 61
33550372 2021
16
Nodal histiocytic sarcoma with prominent eosinophilic infiltration: expression of eotaxin-2 on tumor cells. 61
33436014 2021
17
Leukemic Phase of Histiocytic Sarcoma of the Digestive System: A Rare Manifestation of a Rare Disease. 61
33017829 2021
18
Disseminated Haemophagocytic Histiocytic Sarcoma in an African Pygmy Hedgehog (Atelerix albiventris). 61
33494908 2021
19
Disseminated histiocytic sarcoma in Asian palm civet (Paradoxurus hermaphroditus). 61
33229793 2021
20
Circulating tumor DNA is detectable in canine histiocytic sarcoma, oral malignant melanoma, and multicentric lymphoma. 61
33441840 2021
21
Original Observation of Primary Bladder Histiocytic Sarcoma: First Case Report. 61
33614360 2021
22
A Rare Case of Histiocytic Sarcoma Secondary to Gastrointestinal Stromal Tumor in the Stomach: Transdifferentiation or Synchronicity? 61
33747577 2021
23
Spectrum of histiocytic neoplasms associated with diverse haematological malignancies bearing the same oncogenic mutation. 61
32852896 2021
24
Cytopathologic findings in histiocytic sarcoma of thyroid mimicking as anaplastic carcinoma: A report of a rare case with review of literature. 61
33277975 2020
25
A case of spindle cell dominant histiocytic sarcoma showing a complete remission after first-line chemotherapy with doxorubicin and ifosfamide. 61
32431236 2020
26
Outcome comparison between radiation therapy and surgery as primary treatment for dogs with periarticular histiocytic sarcoma: An Italian Society of Veterinary Oncology study. 61
32396662 2020
27
Hematolymphoid Neoplasms Rarely Mimic Undifferentiated Pleomorphic Sarcoma of Soft Tissue. 61
32167380 2020
28
Leukemic histiocytic sarcoma in a captive common squirrel monkey (Saimiri sciureus) with Saimiriine Gammaherpesvirus 2 (Rhadinovirus), Saimiri sciureus lymphocryptovirus 2 (Lymphocryptovirus) and Squirrel monkey retrovirus (β-Retrovirus) coinfection. 61
32412106 2020
29
Wobbly hedgehog syndrome with disseminated histiocytic sarcoma and lateral ventricular meningioma in an African pygmy hedgehog. 61
33140710 2020
30
Periarticular histiocytic sarcoma in a domestic rabbit. 61
33210288 2020
31
Clinical outcomes in dogs with localized splenic histiocytic sarcoma treated with splenectomy with or without adjuvant chemotherapy. 61
32986268 2020
32
Immunophenotyping of Nonneoplastic and Neoplastic Histiocytes in Cats and Characterization of a Novel Cell Line Derived From Feline Progressive Histiocytosis. 61
32885737 2020
33
Primary localized histiocytic sarcoma of the lumbar vertebrae in a four-toed hedgehog (Atelerix albiventris). 61
32848108 2020
34
Histiocytic Sarcoma 61
33070490 2020
35
Molecular diagnosis using RNAscope in-situ hybridization in canine malignancies. 61
33012982 2020
36
Histiocytic sarcoma progressing from follicular lymphoma and mimicking acquired hemophagocytic lymphohistiocytosis. 61
32808104 2020
37
Ophthalmopathologic characterization of multicentric or metastatic neoplasms with an extraocular origin in dogs and cats. 61
32687655 2020
38
PTPN11 mutations in canine and human disseminated histiocytic sarcoma. 61
32212266 2020
39
Descriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience. 61
32882761 2020
40
Mesenchymal to epithelial transition driven by canine distemper virus infection of canine histiocytic sarcoma cells contributes to a reduced cell motility in vitro. 61
32627957 2020
41
Histiocytic sarcoma arising from thyroid gland with BRAF V600E mutation. 61
32600657 2020
42
Ultrasonographic honeycomb pattern of the spleen in cats: correlation with pathological diagnosis in 33 cases. 61
31537164 2020
43
Histological and Immunohistochemical Features of Normal Histiocytes and Langerhans Cells, and Histiocytic Sarcomas in Four-Toed Hedgehogs (Atelerix albiventris). 61
32800106 2020
44
Oral histiocytic sarcoma in a cat. 61
33282334 2020
45
18F-FDG PET-CT in a multifocal peritoneal histiocytic sarcoma. A very rare case with complete response to second-line chemotherapy. 61
32624440 2020
46
Diagnosis and treatment of hemophagocytic histiocytic sarcoma in a cat. 61
33194216 2020
47
CLIPPERS, a syndrome of lymphohistiocytic disorders. 61
32234602 2020
48
Primary Histiocytic Sarcoma in Adult Polycystic Kidney Disease: Case Report and Review of Literature. 61
32677548 2020
49
Anaplastic Sarcoma and Sertoli Cell Tumor in a Central Bearded Dragon (Pogona vitticeps). 61
33633500 2020
50
Canine histiocytic sarcoma cell lines with SHP2 p.Glu76Gln or p.Glu76Ala mutations are sensitive to allosteric SHP2 inhibitor SHP099. 61
31339650 2020

Variations for Histiocytic Sarcoma

Expression for Histiocytic Sarcoma

Search GEO for disease gene expression data for Histiocytic Sarcoma.

Pathways for Histiocytic Sarcoma

Pathways related to Histiocytic Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 12.31 PTEN CDKN2A BRAF
2
Show member pathways
12.19 PTEN CDKN2A BRAF
3
Show member pathways
12.1 PTEN CDKN2A BRAF
4
Show member pathways
11.93 PTEN CDKN2A BRAF
5 11.23 CDKN2A BRAF
6 11.18 PTEN CDKN2A BRAF
7 10.82 PTEN CDKN2A BRAF

GO Terms for Histiocytic Sarcoma

Biological processes related to Histiocytic Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.54 PTEN CDKN2A BRAF
2 regulation of protein stability GO:0031647 9.4 PTEN CDKN2A
3 negative regulation of G1/S transition of mitotic cell cycle GO:2000134 9.32 PTEN CDKN2A
4 long-term synaptic potentiation GO:0060291 9.26 PTEN BRAF
5 cellular response to nerve growth factor stimulus GO:1990090 9.16 PTEN BRAF
6 negative regulation of cyclin-dependent protein serine/threonine kinase activity GO:0045736 8.96 PTEN CDKN2A
7 regulation of axon regeneration GO:0048679 8.62 PTEN BRAF

Sources for Histiocytic 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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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