MCID: LTH001
MIFTS: 36

Lethal Midline Granuloma

Categories: Cancer diseases, Rare diseases, Respiratory diseases
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Aliases & Classifications for Lethal Midline Granuloma

MalaCards integrated aliases for Lethal Midline Granuloma:

Name: Lethal Midline Granuloma 11 14 16 75 31
Granuloma, Lethal Midline 43 71
Midfacial Necrotising Lesion 11
Malignant Granuloma of Face 11
Midline Lethal Granuloma 19

Classifications:



External Ids:

Disease Ontology 11 DOID:9072
ICD9CM 34 446.3
MeSH 43 D006103
NCIt 49 C8196
SNOMED-CT 68 58961005
ICD10 31 M31.2
UMLS 71 C0018197

Summaries for Lethal Midline Granuloma

MalaCards based summary: Lethal Midline Granuloma, also known as granuloma, lethal midline, is related to nasal type extranodal nk/t-cell lymphoma and fibrosarcoma of bone. An important gene associated with Lethal Midline Granuloma is SERPINA3 (Serpin Family A Member 3), and among its related pathways/superpathways are NF-kappaB Signaling and RhoGDI Pathway. The drugs Bevacizumab and Cetuximab have been mentioned in the context of this disorder. Affiliated tissues include endothelial, lung and breast, and related phenotypes are Decreased viability ratio and immune system

Wikipedia: 75 Lethal midline granuloma (LMG) is an historical term for a condition in which necrotic and highly... more...

Related Diseases for Lethal Midline Granuloma

Diseases related to Lethal Midline Granuloma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 135)
# Related Disease Score Top Affiliating Genes
1 nasal type extranodal nk/t-cell lymphoma 32.0 TNFRSF8 TIA1 NCAM1 GZMB CD2
2 fibrosarcoma of bone 30.8 TNFRSF8 SERPINA3
3 lymphomatoid granulomatosis 30.7 TIA1 GZMB
4 lymphoma, hodgkin, classic 30.3 TNFRSF8 TIA1 GZMB
5 lymphoma 30.1 TNFRSF8 TIA1 GZMB CD5
6 reticulosarcoma 30.0 TNFRSF8 TIA1 SERPINA3 GZMB
7 panniculitis 30.0 TIA1 NCAM1 GZMB
8 lymphoproliferative syndrome 29.9 TNFRSF8 TIA1 PRF1 CD5
9 lymphoproliferative syndrome, x-linked, 1 29.6 TNFRSF8 TIA1 PRF1 GZMB
10 paranasal sinus lymphoma 29.5 TNFRSF8 CD7 CD5
11 peripheral t-cell lymphoma 28.8 TNFRSF8 TIA1 NCAM1 GZMB CD7 CD5
12 mature t-cell and nk-cell lymphoma 28.6 TNFRSF8 TIA1 SPN NCAM1 GZMB CD7
13 celiac disease 1 28.1 TNFRSF8 TIA1 SERPINA3 PRF1 NCAM1 GZMB
14 lymphoma, non-hodgkin, familial 28.0 TNFRSF8 SPN SERPINA3 PRF1 NCAM1 GZMB
15 aggressive nk-cell leukemia 27.7 TNFRSF8 TIA1 PRF1 NCAM1 GZMB FCGR3B
16 polymorphic reticulosis 10.6
17 histiocytosis 10.4
18 histiocytic sarcoma 10.4
19 solitary mastocytoma of the skin 10.2 SERPINA3 CD2
20 fanconi anemia, complementation group e 10.2
21 orbit lymphoma 10.2 TIA1 NCAM1
22 solid adenocarcinoma with mucin production 10.2 NCAM1 CD2
23 indolent systemic mastocytosis 10.2 TNFRSF8 CD2
24 rete testis adenoma 10.2 TNFRSF8 NCAM1
25 mast-cell sarcoma 10.2 TNFRSF8 CD2
26 lichen sclerosus et atrophicus 10.2 TIA1 GZMB
27 rhinitis 10.2
28 chronic rhinitis 10.2
29 histiocytic and dendritic cell cancer 10.1 TNFRSF8 SERPINA3
30 hodgkin's lymphoma, mixed cellularity 10.1 TNFRSF8 SPN
31 giardiasis 10.1 TIA1 GZMB
32 aggressive systemic mastocytosis 10.1 TNFRSF8 CD2
33 systemic epstein-barr virus positive t-cell lymphoma of childhood 10.1 TNFRSF8 TIA1 CD2
34 mixed phenotype acute leukemia, t/myeloid 10.1 CD7 CD2
35 inflammatory bowel disease 16 10.1 CD7 CD2
36 critical covid-19 10.0 SERPINA3 FCGR3B
37 hypersensitivity vasculitis 10.0 SERPINA3 FCGR3B
38 intrahepatic bile duct adenoma 10.0 NCAM1 CD7
39 burkitt lymphoma 10.0
40 granulomatosis with polyangiitis 10.0
41 potocki-lupski syndrome 10.0
42 vasculitis, autoinflammation, immunodeficiency, and hematologic defects syndrome 10.0
43 necrotizing sialometaplasia 10.0
44 fungal infectious disease 10.0
45 thrombocytopenia 10.0
46 pulmonary fibrosis 10.0
47 syphilis 10.0
48 b-cell lymphoma 10.0
49 tertiary syphilis 10.0
50 vasculitis 10.0

Graphical network of the top 20 diseases related to Lethal Midline Granuloma:



Diseases related to Lethal Midline Granuloma

Symptoms & Phenotypes for Lethal Midline Granuloma

GenomeRNAi Phenotypes related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability ratio GR00368-A 9.1 CD5 FCGR3B PRF1 SPN TIA1 TNFRSF8

MGI Mouse Phenotypes related to Lethal Midline Granuloma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.61 CD5 CD7 FCGR3B GZMB PRF1 SERPINA3
2 hematopoietic system MP:0005397 9.28 CD5 CD7 FCGR3B GZMB PRF1 SERPINA3

Drugs & Therapeutics for Lethal Midline Granuloma

Drugs for Lethal Midline Granuloma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 35)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bevacizumab Approved, Investigational Phase 2 216974-75-3 135329020
2
Cetuximab Approved Phase 1, Phase 2 205923-56-4
3
Erlotinib Approved, Investigational Phase 1, Phase 2 183319-69-9, 183321-74-6 176870
4
Aspartic acid Approved, Nutraceutical Phase 1, Phase 2 56-84-8 5960
5
Arginine Approved, Investigational, Nutraceutical Phase 1, Phase 2 74-79-3 6322
6
Glycine Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 56-40-6 750
7 Immunoglobulins, Intravenous Phase 2
8 Immunoglobulins Phase 2
9 Angiogenesis Inhibitors Phase 2
10 Antibodies, Monoclonal Phase 2
11 Antibodies Phase 2
12 Antineoplastic Agents, Immunological Phase 2
13 Immunologic Factors Phase 2
14 Protein Kinase Inhibitors Phase 1, Phase 2
15 Radiopharmaceuticals Phase 1, Phase 2
16 Neurotransmitter Agents Phase 1, Phase 2
17
Arginyl-glycyl-aspartic acid Phase 1, Phase 2 3328704
18 Fluorodeoxyglucose F18 Phase 1, Phase 2
19 Fluorides Phase 1, Phase 2
20 N-Methylaspartate Phase 1, Phase 2
21
Everolimus Approved Phase 1 159351-69-6 70789204 6442177
22
Sirolimus Approved, Investigational Phase 1 53123-88-9 5284616 6436030
23
Lenograstim Approved, Investigational Phase 1 135968-09-1
24
Fluorouracil Approved Phase 1 51-21-8 3385
25
Hydroxyurea Approved Phase 1 127-07-1 3657
26
Irinotecan Approved, Investigational Phase 1 97682-44-5, 100286-90-6 60838
27
7-Hydroxystaurosporine Experimental, Investigational Phase 1 112953-11-4 4616292 72271
28 Cola Phase 1
29 Immunosuppressive Agents Phase 1
30 Antimetabolites Phase 1
31 Endothelial Growth Factors Phase 1
32 Mitogens Phase 1
33 Liver Extracts Phase 1
34 topoisomerase I inhibitors Phase 1
35
Gefitinib Approved, Investigational 184475-35-2 123631

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 A Randomized Phase II Trial of Bevacizumab to Control Brain Radiation Damage Completed NCT00492089 Phase 2 bevacizumab;placebo
2 A Phase I, and Biologic Correlative Study of Erlotinib, in Combination With Cetuximab and Bevacizumab in Patients With Metastatic Renal Cell Carcinoma Completed NCT00101348 Phase 1, Phase 2 erlotinib hydrochloride
3 Phase 1-2 18F-FPPRGD2 PET/CT or PET/MRI Imaging of αvβ3 Integrins Expression as a Biomarker of Angiogenesis Completed NCT01806675 Phase 1, Phase 2 18F-fludeoxyglucose (18F-FDG);18F-FPPRGD2
4 A Phase I Evaluation of Cetuximab and RAD001 in Patients With Solid Tumors Completed NCT01637194 Phase 1 everolimus
5 A Phase I Study of Surgery Plus Intraoperative Photodynamic Therapy (PDT) in Patients With Resectable, Primary or Recurrent Head and Neck Cancer Completed NCT00470496 Phase 1 HPPH;photodynamic therapy
6 A Phase I Study Of Bevacizumab (Recombinant Humanized Monoclonal Antibody To Vascular Endothelial Growth Factor) In Addition To Flourouracil And Hydroxyurea As Initial Chemotherapy With Concomitant Radiotherapy (B-FHX) For Poor Prognosis Head And Neck Cancer Completed NCT00023959 Phase 1 hydroxyurea;fluorouracil
7 Phase 1 Study of 17-dimethylaminoethylamino-17-demethoxygeldanamycin (17-DMAG, NSC #707545) in Patients With Solid Tumors. Completed NCT00089362 Phase 1 alvespimycin hydrochloride
8 A Phase I Clinical and Biological Evaluation of Combined EGFR Blockade With Erlotinib and Cetuximab in Patients With Advanced Cancer Completed NCT00397384 Phase 1 cetuximab;erlotinib hydrochloride
9 Phase I Study of OSI-774 (NSC 718781) for Solid Tumors in Patients With Hepatic or Renal Dysfunction Completed NCT00030498 Phase 1 erlotinib hydrochloride
10 A Phase I Study of UCN-01 in Combination With Irinotecan in Resistant Solid Tumor Malignancies (Part I) and in Triple Negative (ER-Negative, PgR-Negative, HER-2 Not-Amplified) Recurrent Breast Cancers (Part II) Completed NCT00031681 Phase 1 7-hydroxystaurosporine;irinotecan hydrochloride
11 Single Agent ZD-1839 (NSC-715055, IND-61187) in Patients With Advanced Head and Neck Carcinoma or Non-Small Cell Lung Cancer Aged 75 Years and Older (and in a Cohort of Patients 50 Years Old and Younger) Completed NCT00068497 gefitinib

Search NIH Clinical Center for Lethal Midline Granuloma

Cochrane evidence based reviews: granuloma, lethal midline

Genetic Tests for Lethal Midline Granuloma

Anatomical Context for Lethal Midline Granuloma

Organs/tissues related to Lethal Midline Granuloma:

MalaCards : Endothelial, Lung, Breast, Brain, Liver, Tonsil, Skin

Publications for Lethal Midline Granuloma

Articles related to Lethal Midline Granuloma:

(show top 50) (show all 203)
# Title Authors PMID Year
1
Nonhealing Ulcers with Rimming of the Adipose Tissues. 62
35950145 2022
2
Rhino-Orbital-Cerebral Mycosis and Extranodal Natural Killer or/and T-Cell Lymphoma, Nasal Type. 62
35783622 2022
3
Case Report: Rhizopus arrhizus Rhino-Orbital-Cerebral Mycosis and Lethal Midline Granuloma: Another Fungal Etiological Agent. 62
34150783 2021
4
Lethal Midline Granuloma: An Enigma in Diagnosis-A Rare Case Report. 62
31763310 2019
5
High grade cutaneous B-cell lymphoma presenting as lethal midline granuloma - case report of an unusual presentation. 62
30938452 2019
6
Extranodal NK/T-Cell Lymphoma, Nasal Type, Presenting as Refractory Pseudomonas aeruginosa Facial Cellulitis. 62
28748192 2017
7
Nasal NK/T-cell lymphoma: A tragic case. 62
27789156 2017
8
Nasal NK/T Cell Lymphoma Presenting with Perforation of Palate: A Case Report and Review of Literature. 62
28511419 2017
9
Lethal midline granuloma. 62
23440011 2013
10
Extranodal natural killer/T-cell lymphoma, nasal type: 'midline lethal granuloma.' A case report. 62
23327615 2013
11
Mucor irregularis infection and lethal midline granuloma: a case report and review of published literature. 62
22744721 2012
12
Epidemiology and pathogenesis of nasal NK/T-cell lymphoma: a mini-review. 62
21336457 2011
13
[Laryngotracheal NK/T lymphoma: clinical case]. 62
20307871 2011
14
Angiocentric nasal T-cell lymphoma in a patient withidiopathic CD4+ lymphocytopenia. 62
20404393 2009
15
Nasal NK/T cell lymphoma presenting as a lethal midline granuloma. 62
18388376 2008
16
Oral and maxillofacial pathology case of the month. Angiocentric T-Cell lymphoma (midline lethal granuloma). 62
17867546 2007
17
[Primary nasal natural killer/T-cell lymphomas: classification and clinicopathological features]. 62
17103008 2007
18
[Report on the 34th meeting of the German Clinical Immunology Workgroup, Frankfurt, 03.-04.11.2006]. 62
17160656 2007
19
Extranodal NK/T-cell lymphoma, nasal type. 62
17064952 2007
20
Lethal midline granuloma: a case of T-cell lymphoma. 62
18043881 2007
21
Prosthetic rehabilitation of a midfacial defect resulting from lethal midline granuloma--a clinical report. 62
17002746 2006
22
[Case series of localized nasal NK/T-cell lymphoma treated with preceding intensified local radiation therapy before systemic chemotherapy]. 62
16685174 2006
23
Lethal midline granuloma-nasal natural killer/T-cell lymphoma. 62
16601443 2006
24
Body cavity-based presentation of natural killer cell lymphoma. 62
15621817 2005
25
Clinicopathologic study of non-Hodgkin lymphoma in sinonasal and hard palate regions in 15 Japanese cases. 62
15735372 2005
26
[Lethal midline granuloma]. 62
15628680 2004
27
Midline lethal granuloma--a clinical enigma. 62
15164661 2003
28
Midline lethal granuloma complicating pregnancy: case report. 62
16167758 2003
29
Nasal and nasal-type natural killer (NK)/T-cell lymphoma: immunophenotype and Epstein-Barr virus (EBV) association. 62
14569739 2003
30
Centrofacial malignant T-cell lymphoma exhibiting recurrent fever and skin ulcer in a 3-year-old girl. 62
12487833 2002
31
Vision-threatening complications of nasal T/NK lymphoma. 62
12208253 2002
32
Lymphoid lesions of the head and neck: a model of lymphocyte homing and lymphomagenesis. 62
11904341 2002
33
Lethal midline granuloma in a human immunodeficiency virus-infected patient. 62
11545099 2001
34
[Angiocentric T/NK cell lymphoma: a special clinical-pathological entity of lethal midline granuloma. A case report]. 62
11488153 2001
35
Nasal natural killer cell lymphoma presenting as lethal midline granuloma. 62
11168665 2000
36
[Reactive hemaphagocytic syndrome in a patient with midline NK-cell lymphoma]. 62
11139660 2000
37
Specific c-kit mutations in sinonasal natural killer/T-cell lymphoma in China and Japan. 62
10811105 2000
38
Midline destructive lesions of the sinonasal tract: simplified terminology based on histopathologic criteria. 62
10696019 2000
39
Lethal midline granuloma: report of three cases. 62
10835805 2000
40
[Diagnostic difficulties in nasopharyngeal tumors]. 62
10822970 2000
41
Lethal midline granuloma starting as granuloma laryngis. 62
10778778 2000
42
[Nasal T-cell lymphoma of the lethal midline type. Case report and current aspects in the literature]. 62
10412659 1999
43
[Lethal midline granuloma: clinical management of three cases]. 62
10413926 1999
44
Lethal midline granuloma revisited: nasal T/Natural-killer cell lymphoma. 62
10561195 1999
45
Chronological changes in incidences of polymorphic reticulosis in Korea and Japan. 62
10202275 1999
46
Sinonasal malignant lymphomas: a distinct clinicopathological category. 62
10214792 1999
47
[Nasal T-cell lymphoma as a differential diagnosis of the midline granuloma syndrome]. 62
10226981 1999
48
[Intracranial and orbital extension of a nasal cavity adult T-cell leukemia: a case report]. 62
10024985 1999
49
Sinonasal lymphoma presenting as a lethal midline granuloma: case report. 62
9590351 1998
50
Treatment of localized non-Hodgkin's lymphomas of the head and neck: focusing on cases of non-lethal midline granuloma. 62
9727875 1998

Variations for Lethal Midline Granuloma

Expression for Lethal Midline Granuloma

Search GEO for disease gene expression data for Lethal Midline Granuloma.

Pathways for Lethal Midline Granuloma

Pathways related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.97 TNFRSF8 NCAM1 GZMB CD2
2
Show member pathways
11.96 PRF1 GZMB FCGR3B
3
Show member pathways
11.85 TIA1 PRF1 GZMB
4
Show member pathways
11.16 PRF1 GZMB
5 11.03 NCAM1 GZMB CD7
6 10.99 SPN NCAM1 CD5 CD2
7 10.95 PRF1 GZMB
8 10.69 PRF1 GZMB

GO Terms for Lethal Midline Granuloma

Cellular components related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 9.96 TNFRSF8 SPN PRF1 NCAM1 FCGR3B CD7
2 membrane GO:0016021 9.96 TNFRSF8 SPN PRF1 NCAM1 FCGR3B CD7
3 cytolytic granule GO:0044194 8.92 PRF1 GZMB

Biological processes related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytolysis GO:0019835 9.26 PRF1 GZMB
2 positive regulation of tumor necrosis factor production GO:0032760 9.1 TNFRSF8 SPN CD2

Sources for Lethal Midline Granuloma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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