-
Life (Basel, Switzerland) Dec 2022Diabetes mellitus (DM) is a metabolic disorder whose prevalence has continuously increased worldwide and is associated with dysfunction of the autonomic nervous system...
Diabetes mellitus (DM) is a metabolic disorder whose prevalence has continuously increased worldwide and is associated with dysfunction of the autonomic nervous system and, in particular, that of the sympathetic nervous system (SNS). The objective of this study was to analyze the interaction of DM and the SNS, building a model of sympathectomized diabetic rats to determine alterations in the content of CA (catecholamines) in different intra-abdominal organs. Sympathectomy was conducted with guanethidine (GNT). Additionally, DM was induced with STZ (Streptozotocin). Treatment with GNT decreased norepinephrine (NE) content in all analyzed tissues, with significant differences found in the paraganglia, liver, pancreas, duodenum, and heart compared to the control group. With respect to epinephrine (E), which was only found in the liver, pancreas, and heart, presenting significant differences (p < 0.05) in the heart, a decrease in its concentration was observed for all of the experimental groups with respect to the control. The decrease in dopamine (DA) content due to the GNT−STZ treatment was 30.1% in the heart with respect to the diabetic (STZ) group. The amount of CA in the adrenal medulla indicates the effect of sympathectomy on the GNT group where there was a significant reduction (p < 0.05) of DA. These findings suggest that the elimination of the sympathetic nervous system in diabetic organisms contributed to a decrease in blood glucose; likewise, an alteration in the levels of CA was observed in the different selected organs, possibly attributed to the severity, duration, and pathogenesis of the complications of acute and chronic DM.
PubMed: 36556512
DOI: 10.3390/life12122147 -
Journal of Medical Case Reports Dec 2019Paragangliomas and pheochromocytomas are sympathetic or parasympathetic tumors derived from the paraganglia and the adrenal medulla, respectively. Paragangliomas and...
BACKGROUND
Paragangliomas and pheochromocytomas are sympathetic or parasympathetic tumors derived from the paraganglia and the adrenal medulla, respectively. Paragangliomas and pheochromocytomas can be sporadic or familial, the latter frequently being multifocal and possibly due to succinate dehydrogenase complex genes mutations. In addition, 12% of sporadic paragangliomas are related to covered succinate dehydrogenase complex mutations. The importance of identifying succinate dehydrogenase complex mutations is related to the risk for these patients of developing multiple tumors, including non-endocrine ones, showing an aggressive clinical presentation.
CASE PRESENTATION
We report the case of a 45-year-old Caucasian man with an indolent mass in his neck. Ultrasound of his neck, magnetic resonance imaging, and 1,4,7,10-tetraazacyclododecane-N(I),N(II),N(III),N(IIII)-tetraacetic acid(D)-Phe(1)-thy(3)-octreotide (Ga-DOTATOC) positron emission tomography-computed tomography and endocrine work-up were consistent with a carotid body paraganglioma with concomitant nodal enlargement in several body regions, which turned out to be a follicular lymphoma at histology. He was found to carry a germline Succinate dehydrogenase subunit B gene (SDHB) mutation.
CONCLUSION
It is crucial to look for a second malignancy in the case of a paraganglioma demonstrating succinate dehydrogenase complex germline mutations.
Topics: Adrenal Gland Neoplasms; Germ-Line Mutation; Head and Neck Neoplasms; Humans; Lymphoma, Follicular; Magnetic Resonance Imaging; Male; Middle Aged; Paraganglioma; Succinate Dehydrogenase; Treatment Outcome; Ultrasonography
PubMed: 31856921
DOI: 10.1186/s13256-019-2323-1 -
Cureus Jun 2022Arising from the autonomic paraganglia of the neuroendocrine system, paragangliomas are rare neoplasms that are derived from the embryonic neural crest. Primary...
Arising from the autonomic paraganglia of the neuroendocrine system, paragangliomas are rare neoplasms that are derived from the embryonic neural crest. Primary paragangliomas of the lung are exceedingly rare, with little known about their origin. Here we present a 47-year-old female presenting in 2021, one year after a COVID-19 infection, with symptoms of tachycardia, shortness of breath, and palpitations which she associated with presumed long COVID-19. An imaging workup noted a 1.5 cm nodule in the lingula of the left lung. She then had surgical resection of the nodule, which was found to be a primary lung paraganglioma. A follow-up dotatate positron emission tomography (PET) CT noted no residual disease, and genetic testing was negative for known mutations. This case demonstrates the need for close monitoring with follow-up for incidental findings in order to provide a timely and accurate diagnosis in accordance with guideline criteria.
PubMed: 35784994
DOI: 10.7759/cureus.25562 -
Frontiers in Surgery 2022Paraganglioma is a kind of neuroendocrine tumor that originates from paraganglia outside the adrenal gland. Gastrointestinal tract paraganglioma is very rare and only...
BACKGROUND
Paraganglioma is a kind of neuroendocrine tumor that originates from paraganglia outside the adrenal gland. Gastrointestinal tract paraganglioma is very rare and only four cases of paraganglioma originating in the colon have been reported.
CASE PRESENTATION
We report a case of metastatic paraganglioma originating in the colon, in which the differential diagnosis was established by comprehensively considering clinical information, histology, immunohistochemistry, and findings of fluorescence hybridization and next generation sequencing analyses. The patient has remained well for over 14 months after the treatment.
CONCLUSION
Since all paraganglioma have metastatic potential, we believe that radical surgical resection and regular follow-up are necessary. Genetic testing may be indicative of metastatic potential and prognosis. Because colonic paraganglioma is very rare, differential diagnosis is very important. Our report provides experience for the diagnosis and study of paraganglioma in rare sites.
PubMed: 36017518
DOI: 10.3389/fsurg.2022.961514 -
Surgical Case Reports Jun 2024Tumors arising from catecholamine-producing chromophil cells in paraganglia are termed paragangliomas (PGLs), which biologically resemble pheochromocytomas (PCCs) that...
BACKGROUND
Tumors arising from catecholamine-producing chromophil cells in paraganglia are termed paragangliomas (PGLs), which biologically resemble pheochromocytomas (PCCs) that arise from the adrenal glands. Spontaneous rupture of a PGL is rare and can be fatal. Although elective surgery for ruptured PCCs after transcatheter arterial embolization (TAE) has been shown to provide good outcomes, the efficacy of TAE pretreatment for ruptured PGL remains unknown.
CASE PRESENTATION
A 65-year-old female with hypertension and tachycardia was diagnosed with a 3-cm PGL located behind the inferior vena cava. The patient was scheduled to undergo an elective surgery with antihypertensive therapy. However, she presented with a chief complaint of abdominal pain and was diagnosed with intratumoral hemorrhage. Urgent TAE was performed that successfully achieved hemorrhage control. After TAE, serum levels of both epinephrine and norepinephrine were within the normal range. Abdominal computed tomography revealed resolving retroperitoneal hematoma. Elective open surgery was performed without significant intraoperative bleeding or fluctuations in blood pressure.
CONCLUSION
We report a case of successful preoperative TAE for functional PGL to control intraoperative blood pressure fluctuations and bleeding. Preoperative TAE could be a useful procedure for the surgical preparation of functional PGL, including unruptured cases.
PubMed: 38904868
DOI: 10.1186/s40792-024-01907-9 -
Veterinary Sciences May 2021Paraganglioma is a rare neuroendocrine neoplasm originating from paraganglia and consisting of neuroendocrine cells of the sympathetic and parasympathetic nervous...
Paraganglioma is a rare neuroendocrine neoplasm originating from paraganglia and consisting of neuroendocrine cells of the sympathetic and parasympathetic nervous system. Extra-adrenal paraganglioma occurs with a low incidence in both humans and animals. This report presents the first case of paraganglioma in a cat with orbital primary location. An 18-year-old spayed female European domestic shorthair cat of 3.60 kg body weight was evaluated in a private veterinary clinic in Perugia, Italy, for a pronounced exophthalmos of the right eye. The cat underwent surgery for the enucleation of the right eye and of the mass. The biopsy samples of the removed tissue were fixed in 10% buffered neutral formalin for histological and immunohistochemical evaluations. Therefore, specific markers were used for immunohistochemical investigations, such as anti-neuron specific enolase (NSE), anti-synaptophysin, anti-glial fibrillary acid protein, anti-cytokeratin and anti-chromogranin. The results of these investigations allowed establishing the final diagnosis of ocular extra-adrenal paraganglioma of the cat.
PubMed: 34068893
DOI: 10.3390/vetsci8050086 -
Cureus Apr 2024Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent tumors arising from chromaffin cells of the adrenal medulla and extra-adrenal sympathetic paraganglia,...
Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent tumors arising from chromaffin cells of the adrenal medulla and extra-adrenal sympathetic paraganglia, respectively. PCCs commonly produce one or more catecholamines (epinephrine, norepinephrine, and dopamine), but rarely are they biochemically silent. PGLs on the other hand, generally do not produce catecholamines. They have the highest heritability of all adrenal tumors and are known to be associated with genetic mutations. Patients with hereditary tumors typically present at a younger age and with multifocal disease when compared to sporadic disease. Specific genetic mutations have been well established with hereditary syndromes involving PCC/PGLs. Further research has aimed to identify other mutations and delineate specific phenotypes associated with these mutations. A 34-year-old woman presented for evaluation following a laparoscopic appendectomy that identified a 4-cm well-differentiated neuroendocrine tumor on final pathology. Further work-up included a repeat CT scan followed by a Dotatate PET CT scan which revealed a large (7.3 x 5.8 cm) periaortic mass related to the left adrenal gland. Functional adrenal work-up was negative and her Chromogranin A level was 679 ng/mL. She did report intermittent chest tightness and palpitations but was otherwise asymptomatic. The patient subsequently underwent an exploratory laparotomy with left adrenalectomy and adjacent tumor resection as well as completion of right hemicolectomy with ileocolonic anastomosis. Surgical pathology revealed two distinct masses consistent with multifocal PCC. No residual tumor was found in the colectomy specimen and 24 lymph nodes were negative. She had an uneventful recovery and genetic testing showed a variant of uncertain significance for the POLE and VHL genes. She has received genetic counseling and will be enrolled in an appropriate surveillance protocol.
PubMed: 38813302
DOI: 10.7759/cureus.59295 -
Cureus Sep 2023We report a rare case of a solitary paraganglioma arising from the small bowel mesentery, found in a 70-year-old female who presented with abdominal pain. Paragangliomas...
We report a rare case of a solitary paraganglioma arising from the small bowel mesentery, found in a 70-year-old female who presented with abdominal pain. Paragangliomas are rare neuroendocrine, neural crest-derived tumors, most commonly found in the adrenal medulla. While extra-adrenal paragangliomas arise from diverse locations, mesenteric origins are extremely rare. Our comprehensive review shows 35 previously documented cases and updates the epidemiology, clinical features, and outcomes of mesenteric paragangliomas.
PubMed: 37868495
DOI: 10.7759/cureus.45685 -
Hormones (Athens, Greece) Sep 2020Defective function of phenylalanine hydroxylase in phenylketonuria (PKU) results in the accumulation of phenylalanine (Phe) and the reduction of tyrosine (Tyr) in the...
PURPOSE
Defective function of phenylalanine hydroxylase in phenylketonuria (PKU) results in the accumulation of phenylalanine (Phe) and the reduction of tyrosine (Tyr) in the blood, interfering in the normal development and function of organs and tissues in the body. Tyr is the precursor of catecholamines, secreted in response to stress by the adrenal medulla and paraganglia. The aim of this study was to evaluate plasma catecholamine and amino acid response to an escalating series of sympathetic stress tests in PKU patients.
METHODS
Twelve males with classical PKU (aged 18-41 years) and ten healthy male controls were included in this study. The subjects were exposed to three different sympathetic stress stimulations: cold pressor, isometric handgrip, and peak treadmill tests to exhaustion. Physiological, metabolic, and hormonal changes were determined.
RESULTS
Aerobic capacity (VO) was significantly lower in the PKU group (p = 0.018); however, relative VO was similar in the two groups during the spiroergometric test. No significant differences in norepinephrine or in epinephrine response were found between the two groups during the different stimulation tests. Blood Phe increased significantly in the PKU group compared with controls (p = 0.027) during the spiroergometric test, while Tyr levels remained stable in both groups.
CONCLUSION
PKU itself might not influence stress-induced catecholamine changes. Only strenuous exercise increased blood Phe levels in PKU subjects.
Topics: Adolescent; Adult; Epinephrine; Humans; Male; Norepinephrine; Phenylalanine; Phenylketonurias; Physical Exertion; Stress, Physiological; Sympathetic Nervous System; Tyrosine; Young Adult
PubMed: 31993977
DOI: 10.1007/s42000-020-00176-z -
The Journal of International Medical... Mar 2021A paraganglioma is an extra-adrenal tumor of the paraganglia often found in association with sympathetic and parasympathetic nerves. A primary pulmonary paraganglioma...
A paraganglioma is an extra-adrenal tumor of the paraganglia often found in association with sympathetic and parasympathetic nerves. A primary pulmonary paraganglioma generally presents as multiple small tumors or a solitary mass; however, endobronchial involvement is extremely rare. A 49-year-old man was admitted to our hospital because of a chronic cough, intermittent dyspnea, and chest pain. Chest computed tomography revealed a rounded, high-density lesion in the left lower lung lobe. Fiberoptic bronchoscopy demonstrated an endobronchial mass characterized by smooth, hypervascularized mucosa. Transbronchial biopsy of the mass and immunohistochemistry results suggested a paraganglioma. The patient fully recovered after lobectomy and lymphadenectomy. Pulmonary paragangliomas are rarely reported. Complete surgical resection is considered the treatment of choice for pulmonary paragangliomas, and the long-term prognosis is generally good. However, life-long follow-up is mandatory because of the possibility of recurrence and metastasis. This case report adds valuable knowledge to the literature on pulmonary paragangliomas.
Topics: Bronchoscopy; Humans; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Recurrence, Local; Paraganglioma; Tomography, X-Ray Computed
PubMed: 33771069
DOI: 10.1177/03000605211003014