MCID: GRN010
MIFTS: 40

Granular Cell Tumor

Categories: Cancer diseases, Rare diseases, Skin diseases

Aliases & Classifications for Granular Cell Tumor

MalaCards integrated aliases for Granular Cell Tumor:

Name: Granular Cell Tumor 12 20 54 44 15 17 70
Abrikosov's Tumor 20 70
Giant Granulocellular Abrikosov's Tumor 20
Malignant Variant of Abrikosov's Tumor 20
Abrikosoff's Granulous Cell Tumor 20
Neoplasm of Granular Cell 12
Carcinoma, Granular Cell 70
Abrikosoff's Tumor 20

Classifications:



External Ids:

Disease Ontology 12 DOID:2411
MeSH 44 D016586
NCIt 50 C3474
SNOMED-CT 67 12169001
UMLS 70 C0085167 C0205644 C2930931

Summaries for Granular Cell Tumor

GARD : 20 Granular cell tumors (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 a ffect 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.

MalaCards based summary : Granular Cell Tumor, also known as abrikosov's tumor, is related to skin granular cell tumor and congenital granular cell tumor. 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 Mechlorethamine and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include tongue, pituitary and breast, and related phenotypes are behavior/neurological and normal

Wikipedia : 73 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 620)
# Related Disease Score Top Affiliating Genes
1 skin granular cell tumor 32.6 ENO2 CD68
2 congenital granular cell tumor 32.6 ENO2 CD68
3 malignant granular cell myoblastoma 32.3 SERPINA3 MLANA ENO2 CD68
4 congenital epulis 32.1 VIM SERPINA3 ENO2 DES CD68
5 myoblastoma 31.9 SYP ENO2 CD68 CALB2
6 pleomorphic lipoma 30.8 SERPINA3 CD68
7 epulis 30.8 VIM SERPINA3 ENO2 DES CD68
8 lipomatosis, multiple 30.6 SERPINA3 MLANA DES
9 leiomyoma 30.5 VIM S100A1 DES
10 connective tissue benign neoplasm 30.4 SERPINA3 MLANA ENO2 CD68 CALB2
11 carcinoid syndrome 30.3 SYP ENO2
12 skin benign neoplasm 30.2 SERPINA3 MLANA ENO2 CD68
13 mesenchymal cell neoplasm 30.2 SERPINA3 MLANA CD68
14 alveolar soft part sarcoma 30.2 VIM SYP ENO2 DES
15 epidermoid cysts 30.2 S100B S100A1
16 secretory meningioma 30.1 VIM SERPINA3
17 small cell carcinoma 30.0 SYP NKX2-1 ENO2
18 leydig cell tumor 30.0 SYP INHA CALB2
19 neuroendocrine tumor 29.9 SYP NKX2-1 ENO2
20 odontogenic myxoma 29.9 VIM SERPINA3 S100B
21 fibroma 29.9 VIM SERPINA3 S100A1 DES CD68 CALB2
22 oncocytoma 29.9 VIM SYP S100A1 NKX2-1
23 hemangioma 29.9 VIM SYP NKX2-1 ENO2 CD68
24 neurofibroma 29.8 VIM SYP S100B S100A1 MLANA
25 perineurioma 29.8 VIM S100B S100A1 GFAP
26 congenital fibrosarcoma 29.8 VIM SERPINA3 S100A1 DES
27 epithelioid sarcoma 29.7 VIM SYP CD68 CALB2
28 spindle cell sarcoma 29.7 VIM SERPINA3 S100B DES
29 lung benign neoplasm 29.7 SYP NKX2-1 ENO2
30 malignant astrocytoma 29.6 VIM SERPINA3 S100B GFAP
31 mucoepidermoid carcinoma 29.6 SERPINA3 S100A1 NKX2-1
32 malignant fibrous histiocytoma 29.5 VIM SERPINA3 S100B DES CD68
33 benign teratoma 29.5 SYP NKX2-1 ENO2 CALB2
34 ewing sarcoma 29.4 VIM SYP S100B ENO2 DES
35 paraganglioma 29.3 SYP S100B GFAP ENO2
36 papillary carcinoma 29.3 SYP S100A1 NKX2-1 CALB2
37 gastrointestinal stromal tumor 29.3 VIM UCHL1 SYP S100B S100A1 ENO2
38 fibrous histiocytoma 29.3 VIM SERPINA3 S100B ENO2 DES CD68
39 teratoma 29.2 SYP NKX2-1 GFAP ENO2 CALB2
40 leiomyosarcoma 29.1 VIM SERPINA3 S100B S100A1 MLANA ENO2
41 neurilemmoma 29.0 VIM SERPINA3 S100B S100A1 GFAP ENO2
42 cutaneous fibrous histiocytoma 29.0 VIM SERPINA3 S100B S100A1 MLANA DES
43 cystic teratoma 29.0 SYP NKX2-1 GFAP ENO2 CALB2
44 hydrocephalus 28.8 SYP SERPINA3 S100B GFAP ENO2
45 meningioma, familial 28.0 VIM SYP SERPINA3 S100B S100A1 GFAP
46 neurohypophysis granular cell tumor 11.5
47 breast granular cell tumor 11.4
48 malignant gastric granular cell tumor 11.4
49 mediastinal granular cell myoblastoma 11.4
50 vulvar granular cell tumor 11.4

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



Diseases related to Granular Cell Tumor

Symptoms & Phenotypes for Granular Cell Tumor

MGI Mouse Phenotypes related to Granular Cell Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.7 CALB2 DES ENO2 GFAP NKX2-1 S100A1
2 normal MP:0002873 9.23 CALB2 GFAP INHA NKX2-1 S100A1 S100B

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
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
4
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
5
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
6
Isophosphamide mustard Phase 2, Phase 3 100427
7
Liposomal doxorubicin Phase 2, Phase 3 31703
8 Antibiotics, Antitubercular Phase 2, Phase 3
9 Alkylating Agents Phase 2, Phase 3
10 Anti-Bacterial Agents Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pazopanib Neoadjuvant Trial in Non-Rhabdomyosarcoma Soft Tissue Sarcomas (PAZNTIS): A Phase II/III Randomized Trial of Preoperative Chemoradiation or Preoperative Radiation Plus or Minus Pazopanib (NSC# 737754) Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;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:

40
Tongue, Pituitary, Breast, Skin, Lung, Colon, Thyroid

Publications for Granular Cell Tumor

Articles related to Granular Cell Tumor:

(show top 50) (show all 1587)
# Title Authors PMID Year
1
Immunohistochemical expression of Galectin-3 and HBME-1 in granular cell tumors: a new finding. 61 54
18581283 2008
2
Granular cell tumor of the urethra. 54 61
17511737 2007
3
HMB-45 and Melan-A are useful in the differential diagnosis between granular cell tumor and malignant melanoma. 54 61
17284958 2007
4
A rare case of multiple congenital epulis. 61 54
15590438 2004
5
Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. 61 54
15214825 2004
6
Recurrence and rapid metastasis formation of a granular cell tumor of the vulva. 54 61
12551796 2003
7
Expression of inhibin-alpha by granular cell tumors of the gallbladder and extrahepatic bile ducts. 61 54
11688581 2001
8
Granular cell tumor of the urinary bladder. 54 61
11168695 2001
9
Malignant granular cell tumor metastatic to the orbit. 61 54
10711894 2000
10
Granular cell tumor of the rectum: a case report and review of the literature. 61 54
10955603 2000
11
Histiocytic reaction in pelvic peritoneum associated with oxidized regenerated cellulose. 61 54
7817938 1995
12
[Granular cell tumor of the gingiva in the newborn. Report of a case in Martinique]. 54 61
7857416 1994
13
Coincident occurrence of granular cell tumor of the stomach with an early gastric carcinoma. 61 54
1547986 1992
14
Congenital granular cell tumor with systemic involvement. Immunohistochemical and ultrastructural study. 54 61
1929790 1991
15
Rectal Granular Cell Carcinoma Requiring Abdominoperineal Resection. 61
33745283 2021
16
Primary Sarcomas of the Larynx: A Clinicopathologic Study of 27 Cases. 61
33686585 2021
17
Benign and Malignant Granular Cell Tumor of the Hypopharynx: Two Faces of a Rare Entity. 61
32240515 2021
18
Squash smear cytology of pituitary granular cell tumor: A case report and review of literature with special emphasis on cytological differential diagnosis in pituitary region. 61
32926559 2021
19
18F-FDG PET/CT Assessment of Malignant Granular Cell Tumor of the Soft Tissue. 61
33323734 2021
20
Peroral endoscopic tumor resection of a granular cell tumor arising from the 4th layer: Case report with video. 61
33713483 2021
21
Immunohistochemical ALK Expression in Granular Cell Atypical Fibroxanthoma: A Diagnostic Pitfall for ALK-Rearranged Non-neural Granular Cell Tumor. 61
33767072 2021
22
Congenital cutaneous non-neural granular cell tumor mimicking herpetic infection: Case report and review of the literature. 61
33738854 2021
23
Colonic granular cell tumor identified in an adenomatous polyp. 61
33545239 2021
24
Mediastinal granular cell tumor diagnosed with endoscopic ultrasound-guided fine needle aspiration via a modified technique based on wet suction: A case report and literature review. 61
33527755 2021
25
Cervical esophaguectomy plus tracheal resection and free jeyuno graft as treatment of esophageal granular cell tumor. 61
33268150 2021
26
Abrikossoff Tumor (Granular Cell Tumor) Presenting in the Esophagus. 61
33604226 2021
27
Granular Cell Tumor of the Breast Coexisting with Ductal Carcinoma in situ: Case Report. 61
33776721 2021
28
Cytologic and histological features of rare nonepithelial and nonlymphoid tumors of the thyroid. 61
33493367 2021
29
Neuropathology of Pituitary Adenomas and Sellar Lesions. 61
33476394 2021
30
Rare case of granular cell tumor of perianal region: a case report and literature review. 61
33459105 2021
31
Clinical-Pathological, Immunohistochemical, and Genetic Characterization of a Series of Posterior Pituitary Tumors. 61
33212494 2021
32
Granular cell tumor of the breast: Tip and tricks on conventional and magnetic resonance imaging. 61
33289271 2020
33
Colonic Granular Cell Tumor With Ileocecal Valve Involvement. 61
33279775 2020
34
Malignant Granular Cell Tumor of the Esophagus: A Case Report. 61
33012751 2020
35
Peripheral Nerve Sheath Tumors of Head and Neck: Imaging-Based Review of World Health Organization Classification. 61
33196600 2020
36
Imaging features of granular cell tumor in the breast: Case report. 61
33217851 2020
37
Primitive Polypoid Granular Cell Tumor. 61
33253654 2020
38
Granular Cell Tumor: A Clinical Study of 81 Patients. 61
33253647 2020
39
Concurrent corticotroph pituitary tumor and granular cell tumor: A very uncommon association. 61
33239251 2020
40
Three additional cases of non-neural granular cell tumor with novel immunohistochemical findings. 61
32643817 2020
41
Laryngeal granular cell tumors in children: A literature review. 61
32705988 2020
42
Rare Pediatric Vaginal Granular Cell Tumor: A Case Report. 61
32603872 2020
43
A Rare Perineurioma/Granular Cell Tumor Hybrid Peripheral Nerve Sheath Tumor. 61
31977319 2020
44
"The unusual suspects"-Mammographic, sonographic, and histopathologic appearance of atypical breast masses. 61
32470708 2020
45
A Case of Synchronous Malignant and Benign Granular Cell Tumor of the Vulva. 61
32947331 2020
46
Lower Leg Reconstruction With Free Tissue Transfer Using Reverse Flow Recipient Vein: A Case Report. 61
32975137 2020
47
Granular Cell Tumor of Female Urethra - the Second Such Reported Case. 61
33312260 2020
48
A Rare Intramuscular Tumor in Gracilis Muscle: Granular Cell Tumor. 61
33364702 2020
49
Granular Cell Tumor of Vulva: Mesquerading Reactive Histiocytic Proliferation. 61
33364724 2020
50
Underwater endoscopic mucosal resection for esophageal granular cell tumor sized more than 1 cm. 61
32512623 2020

Variations for Granular Cell Tumor

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 11.63 UCHL1 SYP S100B NKX2-1 GFAP ENO2
2
Show member pathways
11.37 VIM GFAP DES
3 11.15 VIM SYP S100B GFAP

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 neuron projection terminus GO:0044306 8.96 UCHL1 SYP
2 intermediate filament cytoskeleton GO:0045111 8.8 VIM GFAP DES

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

# Name GO ID Score Top Affiliating Genes
1 astrocyte development GO:0014002 9.26 VIM GFAP
2 Bergmann glial cell differentiation GO:0060020 9.16 VIM GFAP
3 intermediate filament-based process GO:0045103 8.96 VIM GFAP
4 intermediate filament organization GO:0045109 8.8 VIM GFAP DES

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

# Name GO ID Score Top Affiliating Genes
1 S100 protein binding GO:0044548 8.96 S100B S100A1
2 structural constituent of cytoskeleton GO:0005200 8.8 VIM GFAP DES

Sources for Granular Cell Tumor

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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