MCID: GRN010
MIFTS: 47

Granular Cell Tumor

Categories: Rare diseases, Cancer diseases

Aliases & Classifications for Granular Cell Tumor

MalaCards integrated aliases for Granular Cell Tumor:

Name: Granular Cell Tumor 12 49 51 41 14 69
Granular Cell Carcinoma 12 14
Giant Granulocellular Abrikosov's Tumor 49
Malignant Variant of Abrikosov's Tumor 49
Abrikosoff's Granulous Cell Tumor 49
Granular Cell Adenocarcinoma 12
Neoplasm of Granular Cell 12
Carcinoma, Granular Cell 69
Granular Cell Tumor Nos 12
Abrikosoff's Tumor 49
Abrikosov’s Tumor 49
Abrikosov's Tumor 69

Classifications:



External Ids:

Disease Ontology 12 DOID:2411 DOID:4903
NCIt 46 C3474 C3681

Summaries for Granular Cell Tumor

NIH Rare Diseases : 49 Granular celltumors(GCTs) are soft tissue tumors that can occur anywhere in the body. They are thought to arise from the cells that surround and insulate the nerve cells in our body (Schwann cells). Most granular cell tumors are benign (non-cancerous), although some may be locally aggressive. Less than 2% are malignant (cancerous), but these are aggressive and often are associated with a poor prognosis. GCTs affect females more often than males and usually present as a solitary painless mass. A few individuals have multiple GCTs. In most cases, these tumors are located in the skin of the head and neck, especially inside the mouth (70%), the tongue being the most common site. They may also occur in the breast, heart, pituitary gland, stomach, esophagus, genitalia, and upper respiratory tract. GCTs may be  part of some genetic syndromes, such as LEOPARD syndrome and neurofibromatosis. Complete surgical removal of the tumor is usually curative, but in some cases the tumors may recur. Last updated: 10/25/2016

MalaCards based summary : Granular Cell Tumor, also known as granular cell carcinoma, is related to congenital epulis and epulis. An important gene associated with Granular Cell Tumor is ENO2 (Enolase 2), and among its related pathways/superpathways are Neuroscience and Cytoskeleton remodeling Neurofilaments. The drugs Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include breast, tongue and skin.

Disease Ontology : 12 A carcinoma that derives from epithelial cells, with polygonal accumulation of secondary lysosomes in the cytoplasm.

Wikipedia : 72 Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue... more...

Related Diseases for Granular Cell Tumor

Diseases in the Granular Cell Tumor family:

Congenital Granular Cell Tumor

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

(show top 50) (show all 264)
# Related Disease Score Top Affiliating Genes
1 congenital epulis 31.8 CD68 DES ENO2 UCHL1 VIM
2 epulis 30.6 DES ENO2 VIM
3 alveolar soft part sarcoma 30.0 DES ENO2 VIM
4 spindle cell carcinoma 29.8 DES ENO2 VIM
5 perineurioma 29.7 GFAP S100B VIM
6 cystic teratoma 29.6 ENO2 GFAP
7 odontogenic myxoma 29.5 S100B SERPINA3 VIM
8 leiomyosarcoma 29.3 DES ENO2 S100B VIM
9 gastrointestinal stromal tumor 28.9 DES ENO2 S100B UCHL1 VIM
10 congenital granular cell tumor 12.2
11 vulvar granular cell tumor 12.2
12 skin granular cell tumor 12.2
13 malignant gastric granular cell tumor 12.1
14 breast granular cell tumor 12.1
15 neurohypophysis granular cell tumor 12.0
16 mediastinal granular cell myoblastoma 11.5
17 cardiac granular cell neoplasm 11.5
18 myoblastoma 11.4
19 malignant granular cell esophageal tumor 11.4
20 malignant granular cell myoblastoma 11.3
21 malignant granular cell skin tumor 11.2
22 papillary tumor of the pineal region 10.5 ENO2 VIM
23 skin glomus tumor 10.5 DES VIM
24 infantile digital fibromatosis 10.5 DES VIM
25 lymphangiomatosis 10.5 DES VIM
26 angiolipoma 10.5 DES VIM
27 spindle cell lipoma 10.5 DES VIM
28 biphasic synovial sarcoma 10.4 DES VIM
29 benign metastasizing leiomyoma 10.4 DES VIM
30 malignant ectomesenchymoma 10.4 DES ENO2
31 ectomesenchymoma 10.4 DES ENO2
32 chondroid chordoma 10.4 ENO2 VIM
33 congenital fibrosarcoma 10.4 DES VIM
34 perivascular epithelioid cell tumor 10.4 DES VIM
35 adenofibroma 10.4 INHA VIM
36 reticulum cell sarcoma 10.4 DES VIM
37 microcystic adenoma 10.4 ENO2 INHA
38 hydromyelia 10.4 S100B VIM
39 mesenchymal chondrosarcoma 10.4 DES VIM
40 pseudosarcomatous fibromatosis 10.4 DES VIM
41 liver angiosarcoma 10.4 ENO2 VIM
42 primary hepatic neuroendocrine carcinoma 10.4 S100B VIM
43 fibrous meningioma 10.4 S100B VIM
44 infantile myofibromatosis 10.4 DES VIM
45 eccrine porocarcinoma 10.4 S100B VIM
46 smooth muscle tumor 10.4 DES VIM
47 sertoli cell tumor 10.4 INHA VIM
48 epithelioid malignant peripheral nerve sheath tumor 10.4 ENO2 S100B
49 middle cerebral artery infarction 10.4 ENO2 S100B
50 monophasic synovial sarcoma 10.4 S100B VIM

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



Diseases related to Granular Cell Tumor

Symptoms & Phenotypes for Granular Cell Tumor

Drugs & Therapeutics for Granular Cell Tumor

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

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
5
Doxil Approved June 1999 Phase 2, Phase 3 31703
6 Alkylating Agents Phase 2, Phase 3
7 Anti-Bacterial Agents Phase 2, Phase 3
8 Antibiotics, Antitubercular Phase 2, Phase 3
9 Isophosphamide mustard Phase 2, Phase 3 0
10 Topoisomerase Inhibitors Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride

Search NIH Clinical Center for Granular Cell Tumor

Cochrane evidence based reviews: granular cell tumor

Genetic Tests for Granular Cell Tumor

Anatomical Context for Granular Cell Tumor

MalaCards organs/tissues related to Granular Cell Tumor:

38
Breast, Tongue, Skin, Pituitary, Heart, Colon, Thyroid

Publications for Granular Cell Tumor

Articles related to Granular Cell Tumor:

(show top 50) (show all 679)
# Title Authors Year
1
Intramuscular granular cell tumor of the gluteal region. ( 29423168 )
2018
2
Granular cell tumor of the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration. ( 29380119 )
2018
3
Oral Granular Cell Tumor Mimicking a Giant Sialolith in a Child. ( 29336800 )
2018
4
Primary malignant perivascular epithelioid cell neoplasm (PEComa) of the bone mimicking granular cell tumor in core biopsy: A case report and literature review. ( 29435023 )
2018
5
Granular Cell Tumor of the Neurohypophysis With TFE-3 Expression: A Rare Case Report. ( 28612665 )
2017
6
A case of symptomatic granular cell tumor of the sellar region with large calcification. ( 28276095 )
2017
7
Multifocal Pulmonary Granular Cell Tumor Presenting with Postobstructive Pneumonia. ( 29163998 )
2017
8
Granular Cell Tumor of Cecum: A Common Tumor in a Rare Site with Diagnostic Challenge. ( 28975016 )
2017
9
Congenital Gingival Granular Cell Tumor: A Case Report. ( 28293667 )
2017
10
Extra-tongue oral granular cell tumor: Histological and immunohistochemical aspect. ( 27918739 )
2017
11
Congenital Non-Neural Granular Cell Tumor Mimicking Nevus Lipomatosus Superficialis. ( 29200768 )
2017
12
Large malignant granular cell tumor with suprascapular nerve and brachial plexus invasion: A case report and literature review. ( 29095317 )
2017
13
Sellar and Suprasellar Granular Cell Tumor of Neurohypophysis. ( 28303860 )
2017
14
Granular cell tumor in breast: a case report. ( 28442932 )
2017
15
Recurrent granular cell tumor: a case report and review of literature. ( 29057362 )
2017
16
Eosinophilic Esophagitis and Esophageal Granular Cell Tumor: An Unexpected Association. ( 28296675 )
2017
17
Sellar and suprasellar granular cell tumor of the neurohypophysis: A rare case report and review of the literature. ( 29271018 )
2017
18
Granular cell tumor of the urinary bladder. ( 28861316 )
2017
19
Pleural-based granular cell tumor. ( 28670071 )
2017
20
Extent of surgical resection and tumor size predicts prognosis in granular cell tumor of the sellar region. ( 28948361 )
2017
21
A Case of Granular Cell Tumor of the Clitoris in a Postmenopausal Woman. ( 28951863 )
2017
22
Granular cell tumor mimicking a squamous cell carcinoma of the tongue: a case report. ( 28057062 )
2017
23
Unexpected Presentation of Perianal Granular Cell Tumor in a Young Woman. ( 28284459 )
2017
24
Lung Metastases from Esophageal Granular Cell Tumor: An Undoubted Criterion for Malignancy. ( 28506712 )
2017
25
Noonan syndrome with multiple lentigines with PTPN11 (T468M) gene mutation accompanied with solitary granular cell tumor. ( 28681392 )
2017
26
Non-neural granular cell tumor of the uterine corpus mimicking uterine leiomyoma: A case report. ( 28781812 )
2017
27
Diagnosis and Treatment of Esophageal Granular Cell Tumor: A Case Report and Review of the Literature. ( 29359053 )
2017
28
Intraorbital Granular Cell Tumor Involving in Medial Rectus and Optic Nerve. ( 28639585 )
2017
29
Granular cell tumor of the esophagus. ( 28366624 )
2017
30
Granular cell tumor of the orbit: pathological features and treatment. ( 28417615 )
2017
31
Granular cell tumor at an unusual site masquerading as a neurofibroma. ( 28879925 )
2017
32
A Granular Cell Tumor of the Rectum: A Case Report and Review of the Literature. ( 29354608 )
2017
33
Esophageal Granular Cell Tumor: A Case and Review of the Literature. ( 29317947 )
2017
34
Coexistent Squamous Cell Carcinoma and Granular Cell Tumor of Head and Neck Region: Report of Two Very Rare Cases and Review of the Literature. ( 28783989 )
2017
35
A rare case of perianal granular cell tumor: case report and literature review. ( 28603602 )
2017
36
Fine-needle aspiration cytology of cutaneous granular cell tumor: Report of two cases with special emphasis on cytological differential diagnosis. ( 27011439 )
2016
37
Endoscopic resection of rectal granular-cell tumor using elastic band ligation. ( 26864296 )
2016
38
Granular cell tumor presenting in the scrotum of a pediatric patient: a case report and review of the literature. ( 27259474 )
2016
39
Abrikossoff's tumor mimicking endometrial tumor--literature review and case report of the first described granular cell tumor in scar tissue after caesarean section. ( 27172759 )
2016
40
Unusual Granular Cell Tumor of the Trachea Coexisting With Papillary Thyroid Carcinoma and Masquerading as Tracheal Invasion of Recurred Thyroid Carcinoma: A Case Report. ( 27149467 )
2016
41
Slowly Growing Nodule on the Trunk: Cutaneous Granular Cell Tumor. ( 27504442 )
2016
42
Esophageal Granular Cell Tumor: A Case Report and Review of Literature. ( 27752408 )
2016
43
Atypical granular cell tumor in the maxilla: The first report of primary intraosseous granular cell tumor. ( 27310657 )
2016
44
S-100 Negative Granular Cell Tumor (So-called Primitive Polypoid Non-neural Granular Cell Tumor) of the Oral Cavity. ( 27709427 )
2016
45
Granular Cell Tumor of the Bladder: A Rare Neoplasm Managed With Robotic Partial Cystectomy Using Near-infrared Filter Guidance. ( 27894977 )
2016
46
Granular cell tumor in a child: An uncommon cutaneous presentation. ( 27730035 )
2016
47
Granular cell tumor of cecum. ( 27324518 )
2016
48
Vulvar granular cell tumor. ( 27510686 )
2016
49
Benign Granular Cell Tumor of Breast. ( 27124422 )
2016
50
Esophageal Granular Cell Tumor: A Benign Tumor or an Insidious Cause for Concern? ( 27999519 )
2016

Variations for Granular Cell Tumor

Cosmic variations for Granular Cell Tumor:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM17483 PTCH1 skin,neck,carcinoma,NS c.1729-1G>T p.? 6
2 COSM17468 PTCH1 skin,neck,carcinoma,NS c.584G>T p.R195M 6
3 COSM29053 PTCH1 skin,neck,carcinoma,NS c.833G>A p.W278* 6
4 COSM29072 PTCH1 skin,neck,carcinoma,NS c.1067+1G>A p.? 6
5 COSM14452 PTCH1 skin,neck,carcinoma,NS c.2321G>A p.G774E 6
6 COSM516 KRAS skin,neck,carcinoma,NS c.34G>T p.G12C 6
7 COSM498 HRAS skin,neck,carcinoma,NS c.182A>T p.Q61L 6

Expression for Granular Cell Tumor

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

Pathways for Granular Cell Tumor

Pathways related to Granular Cell Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.79 ENO2 GFAP S100B UCHL1
2
Show member pathways
11.37 DES GFAP VIM
3
Show member pathways
10.91 DES VIM
4 10.7 GFAP S100B VIM

GO Terms for Granular Cell Tumor

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

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 9.33 DES GFAP VIM
2 photoreceptor inner segment GO:0001917 9.26 ENO2 INHA
3 myelin sheath GO:0043209 9.13 ENO2 GFAP UCHL1
4 neuronal cell body GO:0043025 8.92 ENO2 INHA S100B UCHL1

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

# Name GO ID Score Top Affiliating Genes
1 long-term synaptic potentiation GO:0060291 9.37 GFAP S100B
2 muscle filament sliding GO:0030049 9.32 DES VIM
3 astrocyte development GO:0014002 9.26 GFAP VIM
4 Bergmann glial cell differentiation GO:0060020 9.16 GFAP VIM
5 intermediate filament-based process GO:0045103 8.96 GFAP VIM
6 intermediate filament organization GO:0045109 8.8 DES GFAP VIM

Molecular functions related to Granular Cell Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural constituent of cytoskeleton GO:0005200 8.8 DES GFAP VIM

Sources for Granular Cell Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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