MCID: MLG157
MIFTS: 38

Malignant Pheochromocytoma

Categories: Blood diseases, Cancer diseases, Endocrine diseases, Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Malignant Pheochromocytoma

MalaCards integrated aliases for Malignant Pheochromocytoma:

Name: Malignant Pheochromocytoma 12 15
Pheochromocytoma, Malignant 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0080347

Summaries for Malignant Pheochromocytoma

Disease Ontology : 12 An adrenal medulla cancer that arises within the adrenal medulla, releasing epinephrines and norepinephrines hormones.

MalaCards based summary : Malignant Pheochromocytoma, also known as pheochromocytoma, malignant, is related to extra-adrenal pheochromocytoma and pheochromocytoma-paraganglioma. An important gene associated with Malignant Pheochromocytoma is SDHB (Succinate Dehydrogenase Complex Iron Sulfur Subunit B), and among its related pathways/superpathways are Neuroscience and Validated targets of C-MYC transcriptional activation. The drugs Methylprednisolone and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include adrenal gland, bone and thyroid, and related phenotypes are growth/size/body region and mortality/aging

Related Diseases for Malignant Pheochromocytoma

Diseases in the Pheochromocytoma family:

Malignant Pheochromocytoma

Diseases related to Malignant Pheochromocytoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 353)
# Related Disease Score Top Affiliating Genes
1 extra-adrenal pheochromocytoma 32.8 SYP SDHB PNMT CHGA
2 pheochromocytoma-paraganglioma 31.1 SDHB PNMT
3 thyroid gland medullary carcinoma 30.7 SST CHGA CALCA
4 thyroid carcinoma, familial medullary 30.6 SST CHGA CALCA
5 ganglioneuroblastoma 30.6 TH SYP ENO2
6 multiple endocrine neoplasia, type iia 30.4 SYP SDHB CALCA
7 paragangliomas 1 30.3 SDHB CHGA
8 neurofibromatosis, type i 30.3 SST SDHB NCAM1
9 von hippel-lindau syndrome 30.3 TH SLC6A2 SDHB PNMT CHGA
10 gangliocytoma 30.1 TH SYP ENO2
11 adrenal cortical carcinoma 30.1 SYP MIR483 CHGA
12 thyroid carcinoma 29.7 LGALS3 CDH1 CALCA
13 adrenal cortical adenoma 29.6 SYP MIR483 ENO2 CHGA
14 paraganglioma 29.5 TH SYP SST SDHB PNMT NCAM1
15 adrenal carcinoma 29.2 SYP SST SDHB MIR483 ENO2 CHGA
16 ganglioneuroma 29.1 TH SYP SST PNMT NCAM1 ENO2
17 diarrhea 28.9 SST ERBB2 CDH1 CALCA
18 adenoma 28.8 SYP SST LGALS3 CHGA CDH1
19 neuroendocrine tumor 28.5 SYP SST SDHB NCAM1 ENO2 CHGA
20 meningioma, familial 28.2 TERT SYP SST ERBB2 ENO2 CDH1
21 pheochromocytoma 27.2 TH TERT SYP SST SLC6A2 SDHB
22 neuroblastoma 25.8 TH TERT SYP SST SLC6A2 SDHB
23 adrenal gland pheochromocytoma 11.8
24 paragangliomas 4 11.6
25 malignant pediatric adrenal gland pheochromocytoma 11.3
26 non-functioning pancreatic endocrine tumor 10.5 SST CHGA
27 primary hepatic neuroendocrine carcinoma 10.5 SYP CHGA
28 acinar cell cystadenocarcinoma 10.5 SYP CHGA
29 vulvar eccrine porocarcinoma 10.5 SYP CHGA
30 cervical large cell neuroendocrine carcinoma 10.5 SYP CHGA
31 mixed ductal-endocrine carcinoma 10.5 SYP CHGA
32 vaginal glandular tumor 10.5 ENO2 CHGA
33 rectum neuroendocrine neoplasm 10.5 SYP SST
34 vaginal tubulovillous adenoma 10.5 SYP CHGA
35 duodenal gastrinoma 10.5 SST CHGA
36 malignant melanocytic neoplasm of the peripheral nerve sheath 10.5 SYP ENO2
37 neonatal hypoxic and ischemic brain injury 10.5 TH ENO2
38 sinonasal undifferentiated carcinoma 10.5 SYP ENO2
39 cerebral neuroblastoma 10.5 SYP ENO2
40 glucagonoma 10.5 SST CHGA
41 gastric gastrinoma 10.5 ENO2 CHGA
42 papillary tumor of the pineal region 10.5 SYP ENO2
43 nodular ganglioneuroblastoma 10.5 ENO2 CHGA
44 pineal region teratoma 10.5 SYP ENO2
45 intracranial primitive neuroectodermal tumor 10.4 SYP ENO2
46 appendix adenocarcinoma 10.4 SYP CHGA
47 bile duct mucinous adenocarcinoma 10.4 SYP ENO2
48 ewing sarcoma of bone 10.4 ENO2 CHGA
49 extraosseous ewings sarcoma-primitive neuroepithelial tumor 10.4 SYP ENO2
50 nodular prostate 10.4 SYP ENO2

Graphical network of the top 20 diseases related to Malignant Pheochromocytoma:



Diseases related to Malignant Pheochromocytoma

Symptoms & Phenotypes for Malignant Pheochromocytoma

MGI Mouse Phenotypes related to Malignant Pheochromocytoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.1 CDH1 CDH2 CHGA ENO2 ERBB2 LGALS3
2 mortality/aging MP:0010768 10.07 CDH1 CDH2 CHGA ERBB2 LGALS3 MAX
3 nervous system MP:0003631 9.77 CDH2 CHGA ENO2 ERBB2 LGALS3 NCAM1
4 neoplasm MP:0002006 9.7 CDH1 ERBB2 LGALS3 NME1 SDHB TERT
5 normal MP:0002873 9.23 CDH1 ERBB2 PNMT SDHB SST SYP

Drugs & Therapeutics for Malignant Pheochromocytoma

Drugs for Malignant Pheochromocytoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
2
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
3
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
4
Carbidopa Approved Phase 2 28860-95-9 34359
5 Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
6
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
7
Iodine Approved, Investigational Phase 2 7553-56-2 807
8
Axitinib Approved, Investigational Phase 2 319460-85-0 6450551
9
Ipilimumab Approved Phase 2 477202-00-9
10
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
11
Epinephrine Approved, Vet_approved Phase 1, Phase 2 51-43-4 5816
12
nivolumab Approved Phase 1, Phase 2 946414-94-4
13
Racepinephrine Approved Phase 1, Phase 2 329-65-7 838
14
Parathyroid hormone Approved, Investigational Phase 2 9002-64-6
15
Tretinoin Approved, Investigational, Nutraceutical Phase 2 302-79-4 444795 5538
16
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
17
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
18 Methylprednisolone Acetate Phase 2
19 alpha-Methyltyrosine Phase 2
20 3-Iodobenzylguanidine Phase 2
21 cadexomer iodine Phase 2
22 Mitogens Phase 2
23 Endothelial Growth Factors Phase 2
24 Immunoglobulins Phase 2
25 Antibodies Phase 2
26 Antibodies, Monoclonal Phase 2
27 Antineoplastic Agents, Immunological Phase 2
28 Protein Kinase Inhibitors Phase 2
29 Angiogenesis Inhibitors Phase 2
30 Vaccines Phase 1, Phase 2
31 Epinephryl borate Phase 1, Phase 2
32 Radiopharmaceuticals Phase 2
33
Vinorelbine Approved, Investigational Phase 1 71486-22-1 60780 44424639
34
Clotrimazole Approved, Vet_approved Phase 1 23593-75-1 2812
35
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
36
Sirolimus Approved, Investigational Phase 1 53123-88-9 5284616 6436030 46835353
37
Everolimus Approved Phase 1 159351-69-6 70789204 6442177
38
Somatostatin Approved, Investigational Phase 1 38916-34-6, 51110-01-1 53481605
39
Octreotide Approved, Investigational Phase 1 83150-76-9 383414 6400441
40
Phenylalanine Approved, Investigational, Nutraceutical Phase 1 63-91-2 6140
41
Vatalanib Investigational Phase 1 212141-54-3 151194
42 Pancreatic Polypeptide Investigational Phase 1 59763-91-6
43 Anti-Infective Agents Phase 1
44 Immunologic Factors Phase 1
45 Immunosuppressive Agents Phase 1
46 Anti-Bacterial Agents Phase 1
47 Antifungal Agents Phase 1
48 Tubulin Modulators Phase 1
49 Antibiotics, Antitubercular Phase 1
50 Antimitotic Agents Phase 1

Interventional clinical trials:

(show all 19)
# Name Status NCT ID Phase Drugs
1 A Phase II Study of 131I-labeled Metaiodobenzylguanidine (MIBG) for Treatment of Patients With Metastatic or Unresectable Pheochromocytoma and Related Tumors Completed NCT01413503 Phase 2
2 A Phase 2 Study of Dovitinib in Adults With Advanced Malignant Pheochromocytoma or Paraganglioma Completed NCT01635907 Phase 2 Dovitinib
3 A Phase I Study Evaluating the Maximum Tolerated Dose, Dosimetry, Safety, and Efficacy of Ultratrace Iobenguane I 131 in Patients With Malignant Pheochromocytoma/Paraganglioma Completed NCT00458952 Phase 1, Phase 2 Ultratrace Iobenguane (MIBG) I 131
4 (131)I-Metaiodobenzylguanidine Treatment of Malignant Pheochromocytoma Completed NCT00028106 Phase 2 [131]I-MIBG;6-[18F]Fluorodopamine;[123]I-MIBG
5 First International Randomized Study in Malignant Progressive Pheochromocytoma and Paraganglioma (PPGL) Recruiting NCT01371201 Phase 2 Sunitinib;Placebo
6 Open Access Protocol of Targeted Radiotherapy With I-metaiodobenzylguanidine (I-MIBG) in Patients With Resistant Neuroblastoma or Malignant Chromaffin Cell Tumors Recruiting NCT00107289 Phase 2
7 Phase II Study of Axitinib (AG-013736) With Evaluation of the VEGF-pathway in Metastatic, Recurrent or Primary Unresectable Pheochromocytoma/Paraganglioma Recruiting NCT03839498 Phase 2 Axitinib
8 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2
9 A Investigator Initiated Phase II Study Of Sunitinib In Patients With Recurrent Paraganglioma/Pheochromocytoma Active, not recruiting NCT00843037 Phase 2 Sunitinib
10 A Phase 1/2 Trial of a Novel Therapeutic Vaccine (EO2401) in Combination With Immune Check Point Blockade, for Treatment of Patients With Locally Advanced or Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma Not yet recruiting NCT04187404 Phase 1, Phase 2
11 A Phase II Study to Evaluate the Effects of 177Lu-DOTATATE in Patients With Unresectable and Progressive Rare Metastatic Endocrine Carcinomas: Medullary Thyroid Cancer, Parathyroid Carcinoma, Pituitary Carcinoma, and Malignant Pheochromocytoma/Paraganglioma Not yet recruiting NCT04106843 Phase 2 Lutetium Lu 177 Dotatate
12 A Phase 2 Study of Pazopanib (GW786034) in Patients With Advanced and Progressive Malignant Pheochromocytoma or Paraganglioma Terminated NCT01340794 Phase 2 Pazopanib Hydrochloride
13 Phase 1 Study Evaluating the Safety, Distribution, Metabolism, and Radiation Dosimetry of ULTRATRACE Iobenguane I 131 in Patients With Malignant Pheochromocytoma/Paraganglioma or Metastatic Carcinoid Completed NCT00339131 Phase 1 Ultratrace iobenguane I 131
14 Phase I Clinical Trial of Temsirolimus and Vinorelbine in Advanced Solid Tumors. Completed NCT01155258 Phase 1 temsirolimus;vinorelbine ditartrate
15 A Phase I, Open Label, Maximum Tolerated Dose-Finding Study to Evaluate the Safety and Tolerability of 90Y-DOTA-tyr3-Octreotide Administered by Intravenous Infusion to Children With Refractory Somatostatin-Receptor Positive Tumors Completed NCT00049023 Phase 1
16 A Phase I Trial of the mTOR Inhibitor RAD001 in Combination With VEGF Receptor Tyrosine Kinase Inhibitor PTK787/ZK 222584 in Patients With Advanced Solid Tumors Completed NCT00655655 Phase 1 everolimus;vatalanib
17 A Phase I Study of [111In-DTPA-D-Phe]-Octreotide in Patients With Refractory Malignancies Expressing Somatostatin Receptors Terminated NCT00002947 Phase 1
18 Compassionate Use of 131I-MIBG for Patients With Malignant Pheochromocytoma Approved for marketing NCT01377532 131 I-Metaiodobenzylguanidine (131 I-MIBG)
19 The COMETE Network, Towards an Easy-to-use Adrenal Cancer/Tumor Identity Card Completed NCT02672020

Search NIH Clinical Center for Malignant Pheochromocytoma

Genetic Tests for Malignant Pheochromocytoma

Anatomical Context for Malignant Pheochromocytoma

MalaCards organs/tissues related to Malignant Pheochromocytoma:

40
Adrenal Gland, Bone, Thyroid, Pituitary, Liver, Testes, Endothelial

Publications for Malignant Pheochromocytoma

Articles related to Malignant Pheochromocytoma:

(show top 50) (show all 700)
# Title Authors PMID Year
1
History of the multiple endocrine neoplasia workshops and overview of MEN2019. 61
32508314 2020
2
Aggressive Paraganglioma of the Urinary Bladder with Local Recurrence and Pelvic Metastasis. 61
32548698 2020
3
MEN2-related pheochromocytoma: current state of knowledge, specific characteristics in MEN2B, and perspectives. 61
32388798 2020
4
Clinical Characteristics and Outcome of Patients With Pheochromocytoma: A Single Center Tertiary Care Experience. 61
32523845 2020
5
Simultaneous Occurrence of Germline Mutations of SDHB and TP53 in a Patient with Metastatic Pheochromocytoma. 61
31851316 2020
6
Large Malignant Pheochromocytoma Causing Cardiac Failure and Metastasis: A Case Report With Review of Literature. 61
32307136 2020
7
Metastatic pheochromocytoma to the pancreas diagnosed by endoscopic ultrasound-guided fine needle aspiration: A case report and review of literature. 61
31639290 2020
8
The Expression of Snail, Galectin-3, and IGF1R in the Differential Diagnosis of Benign and Malignant Pheochromocytoma and Paraganglioma. 61
32190664 2020
9
Malignant pheochromocytoma and paraganglioma: management options. 61
31599769 2020
10
Expression of PDK1 in malignant pheochromocytoma as a new promising potential therapeutic target. 61
30759304 2019
11
Catastrophic catecholamine-induced cardiomyopathy rescued by extracorporeal membrane oxygenation in recurrent malignant pheochromocytoma. 61
31620641 2019
12
Prognostic Factors of Malignant Pheochromocytoma and Paraganglioma: A Combined SEER and TCGA Databases Review. 61
30919391 2019
13
Correction: Prognostic Factors of Malignant Pheochromocytoma and Paraganglioma: A Combined SEER and TCGA Databases Review. 61
30986868 2019
14
A large pheochromocytoma requiring aortic and inferior vena caval reconstruction: A case report. 61
31335714 2019
15
Preclinical Evaluation of the Acute Radiotoxicity of the α-Emitting Molecular-Targeted Therapeutic Agent 211At-MABG for the Treatment of Malignant Pheochromocytoma in Normal Mice. 61
31078058 2019
16
Adrenalectomy for benign and malignant adrenal tumors. Experience from Misurata Cancer Center. 61
32011991 2019
17
Prognosis of Malignant Pheochromocytoma and Paraganglioma (MAPP-Prono Study): A European Network for the Study of Adrenal Tumors Retrospective Study. 61
30715419 2019
18
Malignant pheochromocytoma with negative biochemical markers: Is it time to reclassify pheochromocytomas? 61
31211069 2019
19
Transcriptome Analysis Reveals Significant Differences in Gene Expression of Malignant Pheochromocytoma or Paraganglioma. 61
31205467 2019
20
The Identification of Differentially Expressed Genes Showing Aberrant Methylation Patterns in Pheochromocytoma by Integrated Bioinformatics Analysis. 61
31803246 2019
21
Combinatorial Inhibition of mTORC2 and Hsp90 Leads to a Distinctly Effective Therapeutic Strategy in Malignant Pheochromocytoma. 61
30727894 2019
22
Anti-tumor effects and potential therapeutic response biomarkers in α-emitting meta-211At-astato-benzylguanidine therapy for malignant pheochromocytoma explored by RNA-sequencing. 61
31037122 2019
23
Single-Agent Thalidomide for Treatment of Malignant Paraganglioma of the Organ of Zuckerkandl. 61
31582983 2019
24
Apogossypolone acts as a metastasis inhibitor via up-regulation of E-cadherin dependent on the GSK-3/AKT complex. 61
30787981 2019
25
A clinical prediction model to estimate the metastatic potential of pheochromocytoma/paraganglioma: ASES score. 61
29929757 2018
26
Malignant Pheochromocytoma with Widespread Bony and Pulmonary Metastases. 61
30483454 2018
27
[Germline gene testing of the RET, VHL, SDHD and SDHB genes in patients with pheochromocytoma/paraganglioma]. 61
30122763 2018
28
Successful treatment of malignant pheochromocytoma with sacrum metastases: A case report. 61
30170467 2018
29
Antitumor effects of radionuclide treatment using α-emitting meta-211At-astato-benzylguanidine in a PC12 pheochromocytoma model. 61
29350258 2018
30
Giant malignant pheochromocytoma in an elderly patient: A case report. 61
29742697 2018
31
Propofol Prevents the Progression of Malignant Pheochromocytoma In Vitro and In Vivo. 61
29565198 2018
32
What determines mortality in malignant pheochromocytoma? - Report of a case with eighteen-year survival and review of the literature. 61
29768630 2018
33
Prevention of serious skeletal-related events by interventional radiology techniques in patients with malignant paraganglioma and pheochromocytoma. 61
29305799 2018
34
Paraganglioma as a risk factor for bone metastasis. 61
29187703 2018
35
Treatment for Patients With Malignant Pheochromocytomas and Paragangliomas: A Perspective From the Hallmarks of Cancer. 61
29892268 2018
36
Recent advances in the management of malignant pheochromocytoma and paraganglioma: focus on tyrosine kinase and hypoxia-inducible factor inhibitors. 61
30109021 2018
37
131-I MIBG therapy of malignant pheochromocytoma and paraganglioma tumours - a single-centre study. 61
29645065 2018
38
Unusual presentation of pheochromocytoma: thirteen years of anxiety requiring psychiatric treatment. 61
29644077 2018
39
Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma. 61
29099418 2018
40
Takotsubo syndrome induced by malignant pheochromocytoma in a patient with type 2 papillary renal cell carcinoma - a case report. 61
29785166 2018
41
Interferon-alpha Treatment for Disease Control in Metastatic Pheochromocytoma/Paraganglioma Patients. 61
28748315 2017
42
Complete remission of metastatic pheochromocytoma in 123I-metaiodobenzylguanidine scintigraphy after a single session of 131I-metaiodobenzylguanidine therapy: a case report. 61
29258609 2017
43
Malignant pheochromocytoma with multiple vertebral metastases causing acute incomplete paralysis during pregnancy: Literature review with one case report. 61
29095319 2017
44
Enhanced wild-type p53 expression by small activating RNA dsP53-285 induces cell cycle arrest and apoptosis in pheochromocytoma cell line PC12. 61
29048679 2017
45
Recurrent Malignant Pheochromocytoma with Lymph Nodal Metastasis in a Child: A Rare Case. 61
28974878 2017
46
Surgery for pheochromocytoma: A 20-year experience of a single institution. 61
29518759 2017
47
Ultrastructure of pheochromocytoma: undescribed morphologic features. 61
28429074 2017
48
Proteasome Inhibitors: A Potential Medical Therapy for Malignant Pheochromocytoma. 61
28977615 2017
49
Malignant Pheochromocytoma and Paraganglioma: 272 Patients Over 55 Years. 61
28605453 2017
50
Aggressive Imaging Features in a Malignant Pheochromocytoma With a Novel Mutation of the SDHB Gene. 61
28650885 2017

Variations for Malignant Pheochromocytoma

Expression for Malignant Pheochromocytoma

Search GEO for disease gene expression data for Malignant Pheochromocytoma.

Pathways for Malignant Pheochromocytoma

Pathways related to Malignant Pheochromocytoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.56 TH SYP NCAM1 ENO2 CALCA
2 11.34 TERT NME1 MAX
3 11.23 TH SYP NCAM1
4 10.8 NCAM1 CDH2 CDH1

GO Terms for Malignant Pheochromocytoma

Cellular components related to Malignant Pheochromocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 perinuclear region of cytoplasm GO:0048471 9.43 TH SYP NME1 ERBB2 CHGA CDH1
2 terminal bouton GO:0043195 9.33 TH SYP CALCA
3 neuron projection GO:0043005 9.1 TH SYP SLC6A2 NCAM1 CDH2 CALCA

Biological processes related to Malignant Pheochromocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.35 TH SST SLC6A2 NME1 CDH1
2 catecholamine biosynthetic process GO:0042423 9.16 TH PNMT
3 epinephrine biosynthetic process GO:0042418 8.62 TH PNMT

Molecular functions related to Malignant Pheochromocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.23 TERT SYP NME1 MAX ERBB2 CDH2
2 gamma-catenin binding GO:0045295 9.16 CDH2 CDH1
3 protein phosphatase binding GO:0019903 9.13 LGALS3 ERBB2 CDH2

Sources for Malignant Pheochromocytoma

3 CDC
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10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
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32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
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44 MESH via Orphanet
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56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
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70 Tocris
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72 UMLS via Orphanet
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