MCID: MLG157
MIFTS: 37

Malignant Pheochromocytoma

Categories: Cancer diseases, Endocrine diseases, Genetic 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 HIF-1 signaling pathway. The drugs Iodine and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include adrenal gland, pituitary and thyroid, and related phenotypes are nervous system and neoplasm

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 333)
# Related Disease Score Top Affiliating Genes
1 extra-adrenal pheochromocytoma 31.9 SYP SDHB PNMT CHGA
2 pheochromocytoma-paraganglioma 30.7 VHL SDHB PNMT
3 ganglioneuroblastoma 30.5 TH SYP ENO2
4 thyroid gland medullary carcinoma 30.4 SYP SST CHGA CALCA
5 nonsyndromic paraganglioma 30.3 SDHB CHGA
6 hereditary paraganglioma-pheochromocytoma syndromes 30.3 VHL SDHB MAX
7 thyroid carcinoma, familial medullary 30.2 VHL SST CHGA CALCA
8 gangliocytoma 30.1 TH SYP ENO2
9 von hippel-lindau syndrome 29.9 VHL TH SLC6A2 SDHB PNMT CHGA
10 adrenal cortical carcinoma 29.8 SYP MIR483 CHGA
11 oncocytoma 29.8 VHL SYP SDHB
12 multiple endocrine neoplasia 29.7 VHL TH SST SDHB PNMT CHGA
13 adrenal cortical adenoma 29.7 SYP MIR483 ENO2 CHGA
14 neuroendocrine tumor 29.6 SYP SST SDHB NCAM1 ENO2 CHGA
15 meningioma, familial 29.5 TERT SYP SST NCAM1
16 adenoma 29.5 VHL SYP SST LGALS3 CHGA
17 paraganglioma 29.4 VHL TH SYP SST SDHB PNMT
18 neurofibromatosis, type i 29.4 VHL SST SDHB NCAM1 ENO2
19 multiple endocrine neoplasia, type iia 29.3 VHL SYP SDHB PNMT MAX CHGA
20 ganglioneuroma 29.2 TH SYP SST PNMT NCAM1 ENO2
21 pheochromocytoma 28.9 VHL TH TERT SYP SST SLC6A2
22 neuroblastoma 28.0 TH TERT SYP SST SLC6A2 SDHB
23 adrenal gland pheochromocytoma 11.4
24 primary hepatic neuroendocrine carcinoma 10.4 SYP CHGA
25 acinar cell cystadenocarcinoma 10.4 SYP CHGA
26 vulvar eccrine porocarcinoma 10.4 SYP CHGA
27 vaginal tubulovillous adenoma 10.4 SYP CHGA
28 mixed ductal-endocrine carcinoma 10.4 SYP CHGA
29 vaginal glandular tumor 10.4 ENO2 CHGA
30 non-functioning pancreatic endocrine tumor 10.4 SST CHGA
31 neonatal hypoxic and ischemic brain injury 10.4 TH ENO2
32 gastric gastrinoma 10.4 ENO2 CHGA
33 appendix adenocarcinoma 10.4 SYP CHGA
34 malignant melanocytic neoplasm of the peripheral nerve sheath 10.4 SYP ENO2
35 duodenal gastrinoma 10.3 SST CHGA
36 nodular ganglioneuroblastoma 10.3 ENO2 CHGA
37 optic nerve astrocytoma 10.3 SYP ENO2
38 cerebral neuroblastoma 10.3 SYP ENO2
39 neuronal intestinal dysplasia, type b 10.3 SYP ENO2
40 goblet cell carcinoid 10.3 ENO2 CHGA
41 ewing sarcoma of bone 10.3 ENO2 CHGA
42 lung meningioma 10.3 SYP ENO2
43 sinonasal undifferentiated carcinoma 10.3 SYP ENO2
44 rectum neuroendocrine neoplasm 10.3 SYP SST
45 glucagonoma 10.3 SST CHGA
46 papillary tumor of the pineal region 10.3 SYP ENO2
47 cloacogenic carcinoma 10.3 SYP ENO2
48 pineal region teratoma 10.3 SYP ENO2
49 extraosseous ewings sarcoma-primitive neuroepithelial tumor 10.3 SYP ENO2
50 intracranial primitive neuroectodermal tumor 10.3 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 9.73 CHGA ENO2 ERBB2 LGALS3 NCAM1 NME1
2 neoplasm MP:0002006 9.7 ERBB2 LGALS3 NME1 SDHB TERT TH
3 normal MP:0002873 9.23 ERBB2 PNMT SDHB SST SYP TERT

Drugs & Therapeutics for Malignant Pheochromocytoma

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

(show all 19)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Iodine Approved, Investigational Phase 1, Phase 2 7553-56-2 807
2
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
3
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
5
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
6
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
7
Carbidopa Approved Phase 2 28860-95-9 34359
8
Racepinephrine Approved Phase 1, Phase 2 329-65-7 838
9
Epinephrine Approved, Vet_approved Phase 1, Phase 2 51-43-4 5816
10
nivolumab Approved Phase 1, Phase 2 946414-94-4
11
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
12
Tretinoin Approved, Investigational, Nutraceutical Phase 2 302-79-4 444795 5538
13
Cadexomer iodine Experimental Phase 1, Phase 2 94820-09-4
14
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
15 3-Iodobenzylguanidine Phase 1, Phase 2
16 Methylprednisolone Acetate Phase 2
17 alpha-Methyltyrosine Phase 2
18 Epinephryl borate Phase 1, Phase 2
19 Vaccines Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 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
2 (131)I-Metaiodobenzylguanidine Treatment of Malignant Pheochromocytoma Completed NCT00028106 Phase 2 [131]I-MIBG;6-[18F]Fluorodopamine;[123]I-MIBG
3 A Phase 2 Study of Dovitinib in Adults With Advanced Malignant Pheochromocytoma or Paraganglioma Completed NCT01635907 Phase 2 Dovitinib
4 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 Recruiting NCT04187404 Phase 1, Phase 2
5 A Phase 2 Study of Pazopanib (GW786034) in Patients With Advanced and Progressive Malignant Pheochromocytoma or Paraganglioma Terminated NCT01340794 Phase 2 Pazopanib Hydrochloride
6 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 Withdrawn NCT04106843 Phase 2 Lutetium Lu 177 Dotatate
7 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
8 Compassionate Use of 131I-MIBG for Patients With Malignant Pheochromocytoma Approved for marketing NCT01377532 131 I-Metaiodobenzylguanidine (131 I-MIBG)
9 An Observational Study of Patients With Recurrent Progressive or Refractory Neuroblastoma or Malignant Pheochromocytoma or Paraganglioma Who Receive 131 I- MIBG Not yet recruiting NCT04770831

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, Pituitary, Thyroid, Bone, Kidney, Pancreas, Breast

Publications for Malignant Pheochromocytoma

Articles related to Malignant Pheochromocytoma:

(show top 50) (show all 711)
# Title Authors PMID Year
1
Malignant pheochromocytoma with liver invasion treated successfully by combined retroperitoneal laparoscopic control of arterial in-flow followed by open hepatectomy: A case report. 61
33743242 2021
2
The use of immunotherapy treatment in malignant pheochromocytomas/paragangliomas: a case report. 61
33775249 2021
3
Sonoelastography for pelvic metastatic malignant pheochromocytoma: A case report. 61
33480348 2021
4
Recurrent Malignant Pheochromocytoma With Unusual Peritoneal Carcinomatosis Detected on 131I-MIBG SPECT/CT. 61
33086275 2021
5
Persistent hypoglycemia due to an IGF-II-secreting malignant pheochromocytoma: a case report and literature review. 61
33363755 2020
6
Collet-Sicard Syndrome as the Presentation of Malignant Pheochromocytoma. 61
32983356 2020
7
Resection of Pulmonary Metastases 12 Years after Initial Surgery for a Benign Pheochromocytoma. 61
33071244 2020
8
Aggressive Paraganglioma of the Urinary Bladder with Local Recurrence and Pelvic Metastasis. 61
32548698 2020
9
Ocular muscle metastasis as the initial presentation of a malignant pheochromocytoma: A unique case. 61
32983478 2020
10
Obstructive shock caused by right atrial thrombosis secondary to malignant pheochromocytoma in a dog. 61
32794177 2020
11
MEN2-related pheochromocytoma: current state of knowledge, specific characteristics in MEN2B, and perspectives. 61
32388798 2020
12
History of the multiple endocrine neoplasia workshops and overview of MEN2019. 61
32508314 2020
13
Malignant Pheochromocytoma With Metastasis to the Thoracic Cage. 61
32683908 2020
14
Clinical Characteristics and Outcome of Patients With Pheochromocytoma: A Single Center Tertiary Care Experience. 61
32523845 2020
15
Large Malignant Pheochromocytoma Causing Cardiac Failure and Metastasis: A Case Report With Review of Literature. 61
32307136 2020
16
Simultaneous Occurrence of Germline Mutations of SDHB and TP53 in a Patient with Metastatic Pheochromocytoma. 61
31851316 2020
17
Metastatic pheochromocytoma to the pancreas diagnosed by endoscopic ultrasound-guided fine needle aspiration: A case report and review of literature. 61
31639290 2020
18
The Expression of Snail, Galectin-3, and IGF1R in the Differential Diagnosis of Benign and Malignant Pheochromocytoma and Paraganglioma. 61
32190664 2020
19
Malignant pheochromocytoma and paraganglioma: management options. 61
31599769 2020
20
Primitive Neuroectodermal Tumour Invading the Inferior Vena Cava. 61
32789120 2020
21
Expression of PDK1 in malignant pheochromocytoma as a new promising potential therapeutic target. 61
30759304 2019
22
Catastrophic catecholamine-induced cardiomyopathy rescued by extracorporeal membrane oxygenation in recurrent malignant pheochromocytoma. 61
31620641 2019
23
A large pheochromocytoma requiring aortic and inferior vena caval reconstruction: A case report. 61
31335714 2019
24
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
25
Prognostic Factors of Malignant Pheochromocytoma and Paraganglioma: A Combined SEER and TCGA Databases Review. 61
30919391 2019
26
Correction: Prognostic Factors of Malignant Pheochromocytoma and Paraganglioma: A Combined SEER and TCGA Databases Review. 61
30986868 2019
27
Adrenalectomy for benign and malignant adrenal tumors. Experience from Misurata Cancer Center. 61
32011991 2019
28
Prognosis of Malignant Pheochromocytoma and Paraganglioma (MAPP-Prono Study): A European Network for the Study of Adrenal Tumors Retrospective Study. 61
30715419 2019
29
Malignant pheochromocytoma with negative biochemical markers: Is it time to reclassify pheochromocytomas? 61
31211069 2019
30
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
31
Combinatorial Inhibition of mTORC2 and Hsp90 Leads to a Distinctly Effective Therapeutic Strategy in Malignant Pheochromocytoma. 61
30727894 2019
32
The Identification of Differentially Expressed Genes Showing Aberrant Methylation Patterns in Pheochromocytoma by Integrated Bioinformatics Analysis. 61
31803246 2019
33
Transcriptome Analysis Reveals Significant Differences in Gene Expression of Malignant Pheochromocytoma or Paraganglioma. 61
31205467 2019
34
Apogossypolone acts as a metastasis inhibitor via up-regulation of E-cadherin dependent on the GSK-3/AKT complex. 61
30787981 2019
35
Single-Agent Thalidomide for Treatment of Malignant Paraganglioma of the Organ of Zuckerkandl. 61
31582983 2019
36
Malignant Pheochromocytoma with Widespread Bony and Pulmonary Metastases. 61
30483454 2018
37
A clinical prediction model to estimate the metastatic potential of pheochromocytoma/paraganglioma: ASES score. 61
29929757 2018
38
Successful treatment of malignant pheochromocytoma with sacrum metastases: A case report. 61
30170467 2018
39
[Germline gene testing of the RET, VHL, SDHD and SDHB genes in patients with pheochromocytoma/paraganglioma]. 61
30122763 2018
40
Antitumor effects of radionuclide treatment using α-emitting meta-211At-astato-benzylguanidine in a PC12 pheochromocytoma model. 61
29350258 2018
41
Giant malignant pheochromocytoma in an elderly patient: A case report. 61
29742697 2018
42
Propofol Prevents the Progression of Malignant Pheochromocytoma In Vitro and In Vivo. 61
29565198 2018
43
What determines mortality in malignant pheochromocytoma? - Report of a case with eighteen-year survival and review of the literature. 61
29768630 2018
44
Prevention of serious skeletal-related events by interventional radiology techniques in patients with malignant paraganglioma and pheochromocytoma. 61
29305799 2018
45
Paraganglioma as a risk factor for bone metastasis. 61
29187703 2018
46
Treatment for Patients With Malignant Pheochromocytomas and Paragangliomas: A Perspective From the Hallmarks of Cancer. 61
29892268 2018
47
Temozolomide treatment of a malignant pheochromocytoma and an unresectable MAX-related paraganglioma. 61
29099418 2018
48
131-I MIBG therapy of malignant pheochromocytoma and paraganglioma tumours - a single-centre study. 61
29645065 2018
49
Unusual presentation of pheochromocytoma: thirteen years of anxiety requiring psychiatric treatment. 61
29644077 2018
50
Recent advances in the management of malignant pheochromocytoma and paraganglioma: focus on tyrosine kinase and hypoxia-inducible factor inhibitors. 61
30109021 2018

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 11.47 VHL ERBB2 ENO2
3 11.24 TERT NME1 MAX
4 11.03 TH SYP NCAM1

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 neuron projection GO:0043005 9.35 TH SYP SLC6A2 NCAM1 CALCA
2 terminal bouton GO:0043195 8.8 TH SYP CALCA

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.56 VHL TERT NME1 MAX
2 response to drug GO:0042493 9.46 TH SST SLC6A2 NME1
3 response to pain GO:0048265 9.32 SLC6A2 CALCA
4 catecholamine biosynthetic process GO:0042423 8.96 TH PNMT
5 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 TH TERT SYP NME1 NCAM1 MAX

Sources for Malignant Pheochromocytoma

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 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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