Paragangliomas represent a group of neuroendocrine tumours which occur in various localizations. Most of them produce catecholamines, and in advanced cases present with typical symptoms and signs such as palpitations, headache and hypertension. The only curative treatment is radical resection. About one-quarter of paragangliomas are malignant, defined by the presence of distant metastases. There are multiple treatment options for unresectable metastatic tumours. They include radionuclid therapy, chemotherapy, and radiotherapy, although none of them are curative. Cytoreductive surgery can also be considered, especially when the goal is to decrease symptoms related to advanced disease. We present a rare case of a large paraganglioma of the left retroperitoneum. Despite radical surgery, early recurrence of the disease was observed.

• Klíčová slova
• multiple organ resection, neuroendocrine tumours, oncology, paraganglioma, surgery,
• MeSH
• lidé MeSH
• paragangliom * diagnostické zobrazování   chirurgie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• Klíčová slova
• PARAGANGLIOMA/diagnosis *,
• MeSH
• lidé MeSH
• paragangliom diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Adrenaline-producing tumors are mostly characterized by a sudden release of catecholamines with episodic symptoms. Noradrenergic ones are usually less symptomatic and characterized by a continuous overproduction of catecholamines that are released into the bloodstream. Their effects on the cardiovascular system can thus be different. The aim of this study was to determine the prevalence of cardiovascular complications by catecholamine phenotype. METHODS: We retrospectively analyzed data on the prevalence of cardiovascular events in 341 consecutive patients with pheochromocytoma and paraganglioma treated from 1995 to 2023. Biochemical catecholamine phenotype was determined based on plasma or urinary catecholamines and metanephrines. RESULTS: According to the phenotype, 153 patients had noradrenergic pheochromocytoma and paraganglioma and 188 had adrenergic pheochromocytoma and paraganglioma. In the whole sample, the incidence of serious cardiovascular complications was 28% (95 patients), with no difference between the phenotypes or sexes. The noradrenergic phenotype had significantly more atherosclerotic complications (composite end point of type 1 myocardial infarction and symptomatic peripheral artery disease; odds ratio, 3.58 [95% CI, 1.59-8.83]; P=0.003), while the adrenergic phenotype more often had type 2 myocardial infarction and takotsubo-like cardiomyopathy (OR, 0.24 [95% CI, 0.09-0.57]; P=0.002). These changes remained even after adjustment for conventional risk factors of atherosclerosis. CONCLUSIONS: We found a 28% incidence of cardiovascular complications in a consecutive group of patients with pheochromocytoma and paraganglioma. Patients presenting with a noradrenergic phenotype have a higher incidence of atherosclerotic complications, while the adrenergic phenotype is associated with a higher incidence of acute myocardial damage due to takotsubo-like cardiomyopathy.

• Klíčová slova
• blood pressure, catecholamines, myocardial infarction, paraganglioma, pheochromocytoma,
• MeSH
• adrenergní látky MeSH
• ateroskleróza * komplikace   MeSH
• fenotyp MeSH
• feochromocytom * diagnóza   MeSH
• infarkt myokardu * MeSH
• kardiomyopatie * MeSH
• katecholaminy MeSH
• lidé MeSH
• metanefrin MeSH
• nádory nadledvin * patologie   MeSH
• paragangliom * komplikace   MeSH
• retrospektivní studie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• adrenergní látky MeSH
• katecholaminy MeSH
• metanefrin MeSH

Extra-adrenal paragangliomas constitute 10 % or less of phaeochromocytomas/paragangliomas. Even rarer is the occurrence of paragangliomas outside the usual distribution of paraganglionic tissue. We report a case of extra-adrenal paraganglioma occurring in the small intestine mesentery in a 65-year-old man. To our knowledge, there are only seven case reports of paraganglioma occurring in this non-typical site. Computed tomography showed a solid expansive non-homogenously enhancing mesenteric mass, measuring 10 x 8 cm with peripheral cystic component. Histologically, the tumour had a typical organoid "zellballen" pattern, showed immunohistochemical positivity for synaptophysin, neuron specific enolase, CD-56, chromogranin, and focally vimentin, and was cytokeratin and EMA negative. S-100 protein stained few sustentacular cells. The patient was free from recurrence or metastasis three months after tumour resection. Although rare, paraganglioma should be included in the preoperative differential diagnosis of solid mesenteric tumours, to prevent any potential life-threatening event peroperatively in the case of a catecholamines-producing tumour.

• MeSH
• lidé MeSH
• mezenterium * MeSH
• paragangliom patologie   MeSH
• peritoneální nádory patologie   MeSH
• senioři MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

We report a case of a pigmented composite paraganglioma-ganglioneuroma of the urinary bladder in a 70-year-old female. Grossly, the tumor measured 6.5 cm in diameter and had arisen from the base of the urinary bladder. Histologically, the tumor was composed of approximately equal components of paraganglioma and ganglioneuroma, which were partly separated and partly mixed, and intermingled with each other. There were foci of ample dark brown pigmentation in the cytoplasm of chromaffin paraganglioma cells. The pigment was Masson-Fontana-positive and had been bleached by hydrogen peroxide (H2O2). Electron microscopy showed large, abundant, pleomorphic electron-dense granules consistent with neuromelanin. In addition, there were numerous electron-dense neurosecretory-type granules. Neuromelanin, melanin or lipofuscin are occasionally observed in paragangliomas, although the occurrence of these pigments has never been described in a composite tumor originating from either adrenal medulla or extraadrenal paraganglia. To the best of our knowledge, our report represents the first case of pigmented composite paraganglioma-ganglioneuroma and expands the morphological spectrum of these unusual tumors.

• MeSH
• elektronová mikroskopie MeSH
• ganglioneurom metabolismus   patologie   MeSH
• imunohistochemie MeSH
• lidé MeSH
• nádory močového měchýře metabolismus   patologie   MeSH
• paragangliom metabolismus   patologie   MeSH
• senioři MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH

• Klíčová slova
• PARAGANGLIOMA *, TEMPORAL BONE/neoplasms *,
• MeSH
• lidé MeSH
• nádory * MeSH
• nechromafinní paragangliom * MeSH
• paragangliom * MeSH
• spánková kost * MeSH
• tumor karotického glomu * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

• MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory duodena diagnóza   terapie   MeSH
• paragangliom diagnóza   terapie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH

• Klíčová slova
• EAR, MIDDLE/neoplasms *, PARAGANGLIOMA *,
• MeSH
• lidé MeSH
• nádory * MeSH
• paragangliom * MeSH
• střední ucho * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

We report a case of paraganglioma of the cauda equina and review the MRI features in 11 previously published cases with MRI description. On T1-weighted images, paraganglioma is isointense relative to spinal cord and shows moderate to intense enhancement after Gd-DTPA application, usually with heterogeneous texture. On T2-weighted images, different findings were found. MRI features do not allow to distinguish paraganglioma of the cauda equina from majority of other tumors in this region.

• MeSH
• cauda equina * MeSH
• lidé středního věku MeSH
• lidé MeSH
• magnetická rezonanční tomografie * MeSH
• nádory periferního nervového systému diagnóza   MeSH
• paragangliom diagnóza   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH

Warburg's metabolic hypothesis is based on the assumption that a cancer cell's respiration must be under attack, leading to its damage, in order to obtain increased glycolysis. Although this may not apply to all cancers, there is some evidence proving that primarily abnormally functioning mitochondrial complexes are indeed related to cancer development. Thus, mutations in complex II (succinate dehydrogenase (SDH)) lead to the formation of pheochromocytoma (PHEO)/paraganglioma (PGL). Mutations in one of the SDH genes (SDHx mutations) lead to succinate accumulation associated with very low fumarate levels, increased glutaminolysis, the generation of reactive oxygen species, and pseudohypoxia. This results in significant changes in signaling pathways (many of them dependent on the stabilization of hypoxia-inducible factor), including oxidative phosphorylation, glycolysis, specific expression profiles, as well as genomic instability and increased mutability resulting in tumor development. Although there is currently no very effective therapy for SDHx-related metastatic PHEOs/PGLs, targeting their fundamental metabolic abnormalities may provide a unique opportunity for the development of novel and more effective forms of therapy for these tumors.

• Klíčová slova
• SDHx, Warburg effect, gastrointestinal stromal tumor, glycolysis, hypoxia, paraganglioma, pheochromocytoma, pseudohypoxia, reactive oxygen species, renal cell carcinoma, succinate dehydrogenase,
• MeSH
• feochromocytom genetika   metabolismus   MeSH
• fyziologie buňky MeSH
• glykolýza MeSH
• lidé MeSH
• mutace genetika   MeSH
• nádory nadledvin genetika   metabolismus   MeSH
• paragangliom genetika   metabolismus   MeSH
• sukcinátdehydrogenasa genetika   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Research Support, N.I.H., Intramural MeSH
• Názvy látek
• sukcinátdehydrogenasa MeSH