Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
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Drug
Enzyme
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Query: EC:1.3.5.1 (
succinate dehydrogenase
)
8,177
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
We report a case of familial, bilateral cervical paragangliomas (PGs) with lymph node metastasis. Patient I-1 is a 56-year-old man with a right carotid body tumor and a left vagal PG. Patient II-1 is a 29-year-old woman and the daughter of Patient I-1; she had a left carotid body tumor with regional lymph node metastasis. Histology of all the tumors showed the typical pattern of PGs, i.e., a zellballen pattern composed of chief cells positive for chromogranin A, and sustentacular cells positive for S100 protein. The Ki-67 labeling index was 1% to 3% in these PGs in both the primary and the metastatic tumors. Immunohistochemical analysis showed expression of
somatostatin receptor
(sstr) type 2A, but was negative for sstr type 5. Genomic mutation in
succinate dehydrogenase
type D was confirmed in both patients. Here, we present a case of familial PGs, and discuss the cases with special reference to pathologic diagnosis, genetics, and treatment.
...
PMID:Familial cervical paragangliomas with lymph node metastasis expressing somatostatin receptor type 2A. 1993 39
High
somatostatin receptor
expression on the cell membrane of
succinate dehydrogenase
mutation-related pheochromocytoma and paraganglioma provides a potential target for imaging and therapy. (68)Ga-DOTATATE positron emission PET/CT may represent a new gold standard for staging pheochromocytoma/paraganglioma and have future therapeutic implications.
...
PMID:Moving Beyond "Lumpology": PET/CT Imaging of Pheochromocytoma and Paraganglioma. 2587 86
Pheochromocytomas and paragangliomas (PPGLs) belong to the family of neural crest cell-derived neoplasms. In up to 70% of cases they are associated with germline and somatic mutations in 15 well-characterized PPGL driver or fusion genes. PPGLs can be grouped into three main clusters, where cluster 1 includes PPGLs characterized by a pseudohypoxic signature. Although cluster 1 tumors share several common features, they exhibit unique behaviors. We present here unique insights into the imaging phenotypes of cluster 1 PPGLs based on glucose uptake, catecholamine metabolism, and
somatostatin receptor
expression. Recent data suggest that succinate is a major player in the imaging phenotype of
succinate dehydrogenase
-deficient PPGLs. This review emphasizes the emerging stromal cell-succinate interaction and highlights new perspectives in PPGL theranostics.
...
PMID:New Insights into the Nuclear Imaging Phenotypes of Cluster 1 Pheochromocytoma and Paraganglioma. 2886 59
Primary bladder paraganglioma is rare. A 57-year old man presented with a mass in the inferior wall of the bladder identified on computerized tomography imaging. We investigated the mass with office cystoscopy followed by transurethral resection, which was found to be a nonfunctioning paraganglioma.
68
Ga-DOTA-conjugated
somatostatin receptor
-targeting peptide positron emission tomography revealed no other locations of the disease. The mass was then completely removed through robotic assisted partial cystectomy. Succinate dehydrogenase B immunohistochemistry was normal, arguing against a
succinate dehydrogenase
-deficient paraganglioma.
...
PMID:A rare case of non-functioning bladder paraganglioma treated with robotic assisted partial cystectomy. 3131 4
