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Journal of Veterinary Internal Medicine 2023Pheochromocytoma (PCC) is rare in cats and plasma (PL) and urinary (U) metanephrines (metanephrine [MN]; normetanephrine [NMN]) measurement is rarely described in cats.
Plasma and urinary metanephrine and normetanephrine concentrations using liquid chromatography with tandem mass spectrometry in healthy cats and in a cat with pheochromocytoma.
BACKGROUND
Pheochromocytoma (PCC) is rare in cats and plasma (PL) and urinary (U) metanephrines (metanephrine [MN]; normetanephrine [NMN]) measurement is rarely described in cats.
OBJECTIVES
We evaluated the utility of PL and U MNs measurement in 10 healthy cats and a cat with a confirmed diagnosis of pheochromocytoma (PheoCat), using liquid chromatography with tandem mass spectrometry (LC-MS-MS).
METHODS
Urine and EDTA PL samples collected from each of the 10 cats and the PheoCat were promptly stored at -80°C and remained frozen until analysis. To evaluate U MNs stability, an additional urine sample collected from the healthy cats was refrigerated for 24 hours before freezing. Urinary creatinine concentration (Creat) was assessed using the same spot urine samples to calculate U MNs-to-creatinine ratios.
RESULTS
The PL-MN and PL-NMN median concentrations of the healthy cats were 2.73 and 7.02 nmol/L, respectively. The median U-MN/Creat and U-NMN/Creat ratios were 70 and 139 μg/g, respectively. The PheoCat had a PL-MN of 3.68 nmol/L, PL-NMN of 66.27 nmol/L, U-MN/Creat of 179 μg/g, and U-NMN/Creat of 1262 μg/g. The PheoCat had markedly increased concentrations of both PL and U MNs when compared to the healthy cats. No significant difference was found between U MNs measured in urine samples that underwent 24 hours of refrigeration in comparison to those that were frozen immediately.
CONCLUSIONS
We report preliminary reference intervals for PL and U MNs in cats using LC-MS-MS and the potential clinical applicability of these biomarkers for the diagnosis of PCC in cats.
Topics: Cats; Animals; Normetanephrine; Pheochromocytoma; Metanephrine; Tandem Mass Spectrometry; Creatinine; Chromatography, Liquid; Adrenal Gland Neoplasms; Cat Diseases
PubMed: 37057306
DOI: 10.1111/jvim.16648 -
Cureus May 2020Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare tumors originated in cells derived from the neural crest. The first ones are located in the adrenal medulla,... (Review)
Review
Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare tumors originated in cells derived from the neural crest. The first ones are located in the adrenal medulla, and the second ones in the sympathetic and parasympathetic nervous system. These kind of tumors may secrete excess catecholamines, including epinephrine, norepinephrine, dopamine and/or their metabolite metanephrine, normetanephrine and 3-methoxytyramine, respectively. Its clinical manifestations depend on the location, the secretory profile and the malignant potential of the tumor. These tumors are frequently benign in their presentation. Some arise in the context of familiar syndromes, accounting for up to one-third of the total of diagnosis. The metastatic form is the most common presentation of the tumors with familiar origin and due to their rarity, their diagnosis and management is often difficult. Over the years, our knowledge and perception of PHEO and PGL has greatly expanded and changed. This review article aims to focus on the genetic, clinical, diagnostic, therapeutic and prognostic approaches, to give the clinician knowledge of the most recent updates regarding these themes.
PubMed: 32523826
DOI: 10.7759/cureus.7969 -
Chronic Stress (Thousand Oaks, Calif.) 2023Social isolation (SI) and loneliness are major adult and adolescent health concerns, particularly in the coronavirus disease 2019 (COVID-19) era. Recent prospective...
Social isolation (SI) and loneliness are major adult and adolescent health concerns, particularly in the coronavirus disease 2019 (COVID-19) era. Recent prospective cohort studies indicate that older women who experienced both SI and loneliness had a significantly higher risk of cardiovascular disease (CVD). Hypertension, a well-established risk factor for CVD, is more prevalent in elderly women than men. Furthermore, a lack of social relationships is strongly associated with an increased risk of hypertension in middle-aged and elderly women compared to men. Although this has not been extensively studied, adolescents and young adults who experience loneliness or SI may also be at risk for CVD and depression. The purpose of this study was to examine the effect of SI on blood pressure and depression-like behavior in young male and female mice. Weaned C57BL/6 mice were randomly assigned ( = 6/group/sex) to either group housing (GH) or SI. Animals in the SI group were housed in individual cages for 8 weeks with no view of other animals. The cages were kept in ventilated racks to prevent pheromone exposure and socially isolated animals had no cage enrichment. SI increased systolic, diastolic, and mean arterial blood pressure in females and elevated heart rate in both sexes. Body weight gain was dramatically increased in socially isolated females but tended to decrease in socially isolated males. In the forced swim test, which detects depression-like behavior, there was no difference between groups in total immobility time. The latency to immobility, however, was significantly decreased in socially isolated females. Serum concentrations of corticosterone and metanephrine did not differ between socially isolated and group-housed females, but corticosterone levels were significantly reduced in socially isolated males. Our results indicate that 8 weeks of SI leads to significant changes in blood pressure and heart rate and mild changes in depression-like behavior in young mice, with females affected more than males.
PubMed: 37859939
DOI: 10.1177/24705470231207010 -
Endokrynologia Polska 2022Pheochromocytoma is a hormonally active tumour originating from neuroendocrine cells of the adrenal medulla. Chromogranin A (CgA) and peptide proSAAS belong to the...
INTRODUCTION
Pheochromocytoma is a hormonally active tumour originating from neuroendocrine cells of the adrenal medulla. Chromogranin A (CgA) and peptide proSAAS belong to the family of granins and are present in neuroendocrine cells of adrenal medulla, from where they are released to circulation, along with catecholamines. The aim of this study was to assess the usability of proSAAS peptide assay in patients with adrenal pheochromocytoma.
MATERIAL AND METHODS
23 patients (13 females and 10 males) with adrenal pheochromocytoma (benign in 18 patients and malignant in 5) confirmed by histopathology examination, and 35 blood donors as a control group. Plasma free metanephrines, CgA, and proSAAS peptide levels were measured in all participants.
RESULTS
CgA and proSAAS levels in the group of pheochromocytoma patients vs. the control were: 209 ng/mL and 0.8 ng/mL vs. 59 ng/mL and 0.3 ng/mL (p < 0.001), respectively. The following sensitivity and specificity indexes were obtained from ROC curves for CgA: 83% and 92%, respectively, and for the proSAAS peptide: 39% and 88%, respectively. The combination of 2 parameters: normetanephrine and proSAAS (96% and 100%) had a high diagnostic value, and the value of all determined parameters together (metanephrine, normetanephrine, CgA, and proSAAS) was 100%.
CONCLUSION
A single determination of the proSAAS peptide level is associated with a rather low diagnostic value. But collective determination of CgA and proSAAS may be an additional, valuable tool in biochemical diagnostics of pheochromocytoma.
Topics: Adrenal Gland Neoplasms; Chromogranin A; Chromogranins; Female; Humans; Male; Metanephrine; Normetanephrine; Peptides; Pheochromocytoma; Sensitivity and Specificity
PubMed: 35381093
DOI: 10.5603/EP.a2022.0017 -
BMJ Case Reports Jun 2020There are less than 150 cases of non-functioning retroperitoneal abdominal schwannoma (NRS) reported. Hormonal assay is crucial in confirming the diagnosis of NRS, as...
There are less than 150 cases of non-functioning retroperitoneal abdominal schwannoma (NRS) reported. Hormonal assay is crucial in confirming the diagnosis of NRS, as manipulation of a functional retroperitoneal paraganglioma will instigate an abrupt liberation of catecholamines, resulting in devastating consequences. We report the case of 42-year-old woman who presented with headache, night sweats and abdominal discomfort. Cross-sectional imaging demonstrated a retroperitoneal mass adherent to the aorta and inferior vena cava but biochemical testing of blood and urine was negative for metanephrines and normetanephrines. She underwent successful tumour resection via laparotomy, as location increased the complexity and risk of laparoscopic resection.
Topics: Abdominal Neoplasms; Adult; Female; Humans; Neurilemmoma; Retroperitoneal Neoplasms
PubMed: 32554461
DOI: 10.1136/bcr-2019-233371 -
The Pan African Medical Journal 2020Bladder Paraganglioma is a rare type of bladder tumor (0.06%). It is typically benign and the most common symptoms are hematuria, hypertension and headache. About 14% of...
Bladder Paraganglioma is a rare type of bladder tumor (0.06%). It is typically benign and the most common symptoms are hematuria, hypertension and headache. About 14% of these tumors are malign and consequently radio and chemoresistants. Therefore, surgery is the mainstay of treatment. As they are likely to recur and to metastize lifelong follow-up is required. The authors report a rare case of a 53 years old man with hematuria and a previous history of micturition syncope who was diagnosed with bladder lesion. During the transurethral ressection of bladder he became severely hypertensive. Plasma metanephrines, and urinary vanillylmandelic acid, were still high and the exams suggested residual tumor. The patient underwent radical cistoprostatectomy. After 4 years of follow-up the patient remains disease free.
Topics: Cystectomy; Humans; Male; Middle Aged; Paraganglioma; Syncope; Urethra; Urinary Bladder Neoplasms
PubMed: 33193992
DOI: 10.11604/pamj.2020.36.339.23086 -
Medicinski Glasnik : Official... Aug 2023Aim To determine the most common indications for measuring metanephrine and normetanephrine in plasma by gender and age and to compare the concentrations of metanephrine...
Aim To determine the most common indications for measuring metanephrine and normetanephrine in plasma by gender and age and to compare the concentrations of metanephrine and normetanephrine by indication, gender and age. Methods The study was conducted on 224 patients whose plasma metanephrine and normetanephrine concentrations were measured at the Clinical Institute for Laboratory Diagnostics at the University Hospital Centre Osijek for one year, until 1st January 2020. Results The most frequent indications for biochemical testing were adrenal incidentaloma, 138 (6.6 %), and symptoms of pheochromocytoma, 41 (18.3%). Metanephrine concentration was lower in females (p=0.009). No significant correlation was found between age and metanephrine concentration, while age and normetanephrine concentration were positively correlated (p=0.01). Of the 224 patients, only one patient was diagnosed with pheochromocytoma, whose indication for measurement of metanephrine and normetanephrine was adrenal incidentaloma. Conclusion Adrenal incidentalomas and symptoms suggestive of pheochromocytoma are very common in the general population, while the incidence of pheochromocytoma is extremely low. Clear guidelines for the referral of patients for biochemical testing are needed to avoid unnecessary costs and to identify the correct diagnosis promptly.
PubMed: 37300464
DOI: 10.17392/1588-23 -
Exploration of Targeted Anti-tumor... 2024Pheochromocytomas and paragangliomas (PPGLs) have emerged as one of the most common endocrine tumors. It epitomizes fascinating crossroads of genetic, metabolic, and... (Review)
Review
Pheochromocytomas and paragangliomas (PPGLs) have emerged as one of the most common endocrine tumors. It epitomizes fascinating crossroads of genetic, metabolic, and endocrine oncology, providing a canvas to explore the molecular intricacies of tumor biology. Predominantly rooted in the aberration of metabolic pathways, particularly the Krebs cycle and related enzymatic functionalities, PPGLs manifest an intriguing metabolic profile, highlighting elevated levels of oncometabolites like succinate and fumarate, and furthering cellular malignancy and genomic instability. This comprehensive review aims to delineate the multifaceted aspects of tumor metabolism in PPGLs, encapsulating genetic factors, oncometabolites, and potential therapeutic avenues, thereby providing a cohesive understanding of metabolic disturbances and their ramifications in tumorigenesis and disease progression. Initial investigations into PPGLs metabolomics unveiled a stark correlation between specific genetic mutations, notably in the succinate dehydrogenase complex () genes, and the accumulation of oncometabolites, establishing a pivotal role in epigenetic alterations and hypoxia-inducible pathways. By scrutinizing voluminous metabolic studies and exploiting technologies, novel insights into the metabolic and genetic aspects of PPGLs are perpetually being gathered elucidating complex interactions and molecular machinations. Additionally, the exploration of therapeutic strategies targeting metabolic abnormalities has burgeoned harboring potential for innovative and efficacious treatment modalities. This review encapsulates the profound metabolic complexities of PPGLs, aiming to foster an enriched understanding and pave the way for future investigations and therapeutic innovations in managing these metabolically unique tumors.
PubMed: 38745767
DOI: 10.37349/etat.2024.00222 -
Scientific Reports Jul 2021Diagnosis of pheochromocytomas and paragangliomas in patients receiving hemodialysis is troublesome. The aim of the study was to establish optimal conditions for blood... (Clinical Trial)
Clinical Trial
Diagnosis of pheochromocytomas and paragangliomas in patients receiving hemodialysis is troublesome. The aim of the study was to establish optimal conditions for blood sampling for mass spectrometric measurements of normetanephrine, metanephrine and 3-methoxytyramine in patients on hemodialysis and specific reference intervals for plasma metanephrines under the most optimal sampling conditions. Blood was sampled before and near the end of dialysis, including different sampling sites in 170 patients on hemodialysis. Plasma normetanephrine concentrations were lower (P < 0.0001) and metanephrine concentrations higher (P < 0.0001) in shunt than in venous blood, with no differences for 3-methoxytyramine. Normetanephrine, metanephrine and 3-methoxytyramine concentrations in shunt and venous blood were lower (P < 0.0001) near the end than before hemodialysis. Upper cut-offs for normetanephrine were 34% lower when the blood was drawn from the shunt and near the end of hemodialysis compared to blood drawn before hemodialysis. This study establishes optimal sampling conditions using blood from the dialysis shunt near the end of hemodialysis with optimal reference intervals for plasma metanephrines for the diagnosis of pheochromocytomas/paragangliomas among patients on hemodialysis.
Topics: Adrenal Gland Neoplasms; Aged; Aged, 80 and over; Blood Chemical Analysis; Blood Specimen Collection; Calibration; Dopamine; Female; Humans; Male; Metanephrine; Middle Aged; Paraganglioma; Pheochromocytoma; Poland; Pre-Analytical Phase; Reference Values; Renal Dialysis; Tandem Mass Spectrometry
PubMed: 34282182
DOI: 10.1038/s41598-021-94104-9 -
Journal of Obstetrics and Gynaecology... Aug 2022Pheochromocytoma during pregnancy is a rare cause of secondary hypertension with lethal consequences to both mother and fetus. As patients are young, the possibility of...
INTRODUCTION
Pheochromocytoma during pregnancy is a rare cause of secondary hypertension with lethal consequences to both mother and fetus. As patients are young, the possibility of syndromic associations like MEN-2, VHL, NF-1, etc., needs to be considered.
METHODOLOGY
Three primigravida were diagnosed before the 20th week of gestation when they presented with classical triad of pheochromocytoma.
RESULTS
Diagnosis of pheochromocytoma was confirmed by 24 h urinary metanephrine/normetanephrine or epinephrine/norepinephrine levels. Non-contrast MRI abdomen could localize the tumor. One patient had medullary thyroid carcinoma with hyperparathyroidism, indicative of MEN-2A. Another patient had brain stem hemangioblastoma, pancreatic cysts and family history of spinal hemangioblastoma, so diagnosed to have Von Hippel-Lindau (VHL) syndrome. Whereas, the third patient had sporadic pheochromocytoma. Preoperatively, they required antihypertensive medications including prazosin and metoprolol. They underwent laparoscopic/open adrenalectomy between 19th and 21st week of gestation without complication. Histopathology in all the three patients revealed low-grade pheochromocytoma by pheochromocytoma of the adrenal gland scaled score. None required antihypertensive medications after surgery. All the three newborns were small for gestational age, while one neonate expired due to intra-cardiac rhabdomyoma. So, the timely evaluation and surgical intervention for pheochromocytoma avoid lethal consequences.
CONCLUSIONS
Pregnancy leads to unmasking of pheochromocytoma as it is physiological stress. The syndromic association is more frequent as the population is younger. A poor fetal outcome like IUGR can be explained by endovascular changes in uterine vessel or due to the associated manifestations of MEN-2A, VHL syndromes. Family members should be screened for associated syndromic feature.
PubMed: 35928058
DOI: 10.1007/s13224-021-01532-8