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Case Reports in Urology 2023Pheochromocytoma is a rare neoplasia arising from the adrenal medulla that secretes catecholamines. Those afflicted by this condition can present a wide range of...
INTRODUCTION
Pheochromocytoma is a rare neoplasia arising from the adrenal medulla that secretes catecholamines. Those afflicted by this condition can present a wide range of symptoms. One of the most common is paroxysmic hypertension. Interestingly, although rare, some patients present with shock. We describe two cases of pheochromocytoma in which the initial presentation was shock. . 49 year-old woman, with a history of resistant hypertension, presented to the emergency department with thoracic pain and fever. EKG, echocardiogram (ECC), and myocardial necrosis markers were compatible with Takotsubo syndrome (TS). CT demonstrated a staghorn calculus, hydronephrosis, and signs compatible with xanthogranulomatous pyelonephritis in the right kidney. Additionally, and incidentally, it revealed a 60 mm nodule on the right adrenal gland. Piperacillin/tazobactam was started immediately, and the patient was submitted to urgent upper urinary tract drainage. This procedure was complicated by a cardiorespiratory arrest that was treated with adrenaline administration. The patient was admitted to the ICU due to multifactorial shock and started alpha and, posteriorly, beta blockage. Biochemical adrenal incidentaloma endocrinologic study was negative (under hemodialysis). Multiorgan failure progressively improved. After 2 weeks, the patient was submitted to a laparoscopic transperitoneal right adrenalectomy. No complications were reported. Histological analysis revealed a pheochromocytoma. . 28-year-old woman presented to the emergency department with headaches and nausea. Vitals were compatible with shock. CT revealed an incidental 72 mm mass on the right adrenal. EKG, ECC, and myocardial necrosis markers were compatible with TS. The patient was started on alpha and, posteriorly, beta blockage. Adrenal incidentaloma endocrinological study demonstrated high urinary catecholamines. Right transperitoneal adrenalectomy was performed. No complications were noted. Histological analysis revealed a pheochromocytoma.
CONCLUSION
Pheochromocytoma can present with complex, enigmatic, and rare clinical pictures. Clinicians should be wary of the possibility of this diagnosis when managing adrenal masses.
PubMed: 38073711
DOI: 10.1155/2023/8901383 -
Journal of the European Academy of... Jun 2024Atopic dermatitis (AD) is a highly prevalent inflammatory skin disorder characterized by episodic exacerbations and remissions. Why the clinically healthy skin of AD...
BACKGROUND
Atopic dermatitis (AD) is a highly prevalent inflammatory skin disorder characterized by episodic exacerbations and remissions. Why the clinically healthy skin of AD patients becomes rapidly inflamed and very pruritic is poorly understood.
OBJECTIVE
To investigate cowhage- and histamine-induced itch and skin expression levels of their target receptors in lesional and non-lesional skin of AD, compared to the skin of patients with psoriasis, chronic spontaneous urticaria (CSU) and healthy subjects.
METHODS
Patients with AD, psoriasis and chronic spontaneous urticaria (CSU) as well as healthy control subjects (HC) (n = 20 each) were assessed for differences in itch parameters, neurogenic flare reaction and local blood flow responses to skin provocations with cowhage and histamine. Skin biopsies from 10 AD, 10 psoriasis,11 CSU and 12 HC were obtained to assess expression of protease-activated receptors 2 and 4 (PAR-2, PAR-4), histamine H1 and H4 receptors (H1R, H4R), and mast cells.
RESULTS
Provocation of non-lesional skin of AD patients with cowhage resulted in prolonged itch (p = 0.020), which was not observed in psoriasis and CSU. Significantly prolonged and more intense cowhage- and histamine-induced itch (for duration, peak and overall intensity) was also observed in lesional AD skin. Diminished neurogenic flare reaction and blood flow after histamine provocation were shown in AD and psoriasis patients. Non-lesional AD skin along with lesional AD and psoriasis skin showed an increased expression of PAR-2 and PAR-4, H1R and H4R. Mast cell number was higher in lesional AD and psoriasis skin (p = 0.006 and p = 0.006, respectively).
CONCLUSION
The non-lesional skin of AD patients markedly differs from healthy skin in cowhage-induced itch responses and the expression of receptors for proteases and histamine. Proactive therapeutic interventions that downregulate these receptors may prevent episodic exacerbation in AD.
Topics: Humans; Dermatitis, Atopic; Pruritus; Female; Adult; Male; Receptor, PAR-2; Histamine; Skin; Middle Aged; Receptors, Histamine H4; Psoriasis; Mast Cells; Receptors, Histamine H1; Mucuna; Chronic Urticaria; Case-Control Studies; Young Adult; Inflammation; Regional Blood Flow; Peptide Fragments
PubMed: 38063249
DOI: 10.1111/jdv.19694 -
Radiology Case Reports Jan 2024Pheochromocytoma is a tumor that originates from the chromaffin cells of the adrenal medulla and is responsible for the production of catecholamines. However, when it...
Pheochromocytoma is a tumor that originates from the chromaffin cells of the adrenal medulla and is responsible for the production of catecholamines. However, when it occurs outside the adrenal glands, it is called a paraganglioma and accounts for 10%-15% of cases. In this report, we present the case of a 27-year-old male patient with a history of hypertension, who presented hematuria and dizziness on urination with a diagnosis of bladder paraganglioma. Contrast-enhanced computed tomography revealed the presence of a bladder tumor. Bladder paraganglioma is a rare condition, and understanding possible imaging findings is crucial to raising suspicion of this diagnosis and expanding our knowledge of this rare disease.
PubMed: 38028301
DOI: 10.1016/j.radcr.2023.10.021 -
Cureus Oct 2023In this case, a Caucasian woman was incidentally found to have a left adrenal gland incidentaloma a decade ago. Initial tests indicated a non-functional lipid-poor...
In this case, a Caucasian woman was incidentally found to have a left adrenal gland incidentaloma a decade ago. Initial tests indicated a non-functional lipid-poor adenoma, but ongoing surveillance revealed irregularities in biochemical testing for pheochromocytoma. The patient was concurrently taking an SNRI, known to elevate biochemical markers artificially. Given the adenoma's growth and mild biochemical abnormalities, laparoscopic surgery was performed, and the tumor was found to be a 2.4 cm × 1.8 cm pheochromocytoma. Following the procedure, hormone levels normalized, and the patient experienced relief from symptoms. This case underscores the rarity of pheochromocytomas, emphasizing the importance of accurate diagnosis and effective management. Imaging techniques, notably computed tomography (CT) and magnetic resonance imaging (MRI), played a crucial role in localization, particularly through contrast-enhanced methods. Key characteristics like Hounsfield density, enhancement patterns, and washout behavior aided in distinguishing diverse adrenal masses. For cases where imaging had limitations, complementary techniques such as 23I-metaiodobenzylguanidine (MIBG) scintigraphy, specialized MR sequences, and GA-DOTATATE scans provided supplementary diagnostic insights, collectively contributing to a comprehensive clinical understanding. Despite advancements, challenges persist in differentiating specific adrenal tumors, highlighting the need for continued research and refined imaging methodologies.
PubMed: 38021916
DOI: 10.7759/cureus.47120 -
Cureus Oct 2023Pheochromocytomas and paragangliomas are catecholamine-secreting tumors that originate from chromaffin cells of the adrenal medulla and autonomic neural ganglia,...
Pheochromocytomas and paragangliomas are catecholamine-secreting tumors that originate from chromaffin cells of the adrenal medulla and autonomic neural ganglia, respectively. Patients with pheochromocytomas and paragangliomas typically present with paroxysmal headache, sweating, tachycardia, and hypertension. Although most pheochromocytoma cases are sporadic, many of the cases occur as part of a genetic disorder. Here, we report the case of a 14-year-old male patient who presented with hypertension. Laboratory tests showed elevated levels of serum and urinary catecholamines, metanephrines, and chromogranin. Abdominal ultrasound and computed tomography studies revealed bilateral solid adrenal masses and an isolated splenic mass. Further assessment identified an underlying Von Hippel-Lindau syndrome. The patient was initially treated medically and later surgically. This case highlights the importance of always considering pheochromocytomas and paragangliomas as rare differentials of secondary hypertension, especially in the presence of episodic headaches, sweating, and tachycardia. Furthermore, screening for underlying genetic disorders, such as in our case, should be considered in cases of bilateral tumors, onset at a young age, and presence of extra-adrenal tumors.
PubMed: 38021838
DOI: 10.7759/cureus.47787 -
Frontiers in Medicine 2023Pheochromocytoma is a rare catecholamine-producing neuroendocrine tumor originating from the adrenal medulla chromaffin cells. Hemodynamic instability can occur during...
INTRODUCTION
Pheochromocytoma is a rare catecholamine-producing neuroendocrine tumor originating from the adrenal medulla chromaffin cells. Hemodynamic instability can occur during the induction of anesthesia and surgical manipulation of the tumor. This study investigated the effects of intraoperative dexmedetomidine administration on hemodynamic stability in patients undergoing laparoscopic adrenalectomy for pheochromocytoma.
METHODS
Forty patients who underwent laparoscopic adrenalectomy for pheochromocytoma were randomly assigned to the dexmedetomidine ( = 20) or control ( = 20) group. The primary outcome of this study was intraoperative hemodynamic stability, and the secondary endpoint was the plasma catecholamine concentrations, specifically of epinephrine and norepinephrine.
RESULTS
The intraoperative maximum blood pressures were significantly lower in the dexmedetomidine group (control vs. dexmedetomidine group: 182 ± 31 vs. 161 ± 20, 102 ± 17 vs. 90 ± 10, and 128 ± 22 vs. 116 ± 12 [mean ± SD] mmHg and = 0.020, 0.015, and 0.040 for systolic, diastolic, and mean blood pressure, respectively). The maximum heart rate during surgery was 108 ± 15 bpm in the control group and 95 ± 12 bpm in the dexmedetomidine group ( = 0.010). Other parameters of hemodynamic instability were comparable between both groups. Plasma catecholamine concentrations did not differ between the groups.
CONCLUSION
Dexmedetomidine infusion following the induction of anesthesia at a rate of 0.5 μg/kg/h significantly attenuated the maximum intraoperative SBP, DBP, MBP, and HR, contributing to improved hemodynamic stability.
PubMed: 38020150
DOI: 10.3389/fmed.2023.1276535 -
Genes Nov 2023Knowledge of circadian rhythm clock gene expression outside the suprachiasmatic nucleus is increasing. The purpose of this study was to determine whether expression of...
Knowledge of circadian rhythm clock gene expression outside the suprachiasmatic nucleus is increasing. The purpose of this study was to determine whether expression of circadian clock genes differed within or among the bovine stress axis tissues (e.g., amygdala, hypothalamus, pituitary, adrenal cortex, and adrenal medulla). Tissues were obtained at an abattoir from eight mature nonpregnant Brahman cows that had been maintained in the same pasture and nutritional conditions. Sample tissues were stored in RNase-free sterile cryovials at -80 °C until the total RNA was extracted, quantified, assessed, and sequenced (NovaSeq 6000 system; paired-end 150 bp cycles). The trimmed reads were then mapped to a () reference genome (Umd3.1). Further analysis used the edgeR package. Raw gene count tables were read into RStudio, and low-expression genes were filtered out using the criteria of three minimum reads per gene in at least five samples. Normalization factors were then calculated using the trimmed mean of M values method to produce normalized gene counts within each sample tissue. The normalized gene counts important for a circadian rhythm were analyzed within and between each tissue of the stress axis using the GLM and CORR procedures of the Statistical Analysis System (SAS). The relative expression profiles of circadian clock genes differed ( < 0.01) within each tissue, with neuronal PAS domain protein 2 () having greater expression in the amygdala ( < 0.01) and period circadian regulator () having greater expression in all other tissues ( < 0.01). The expression among tissues also differed ( < 0.01) for individual circadian clock genes, with circadian locomotor output cycles protein kaput () expression being greater within the adrenal tissues and nuclear receptor subfamily 1 group D member 1 () expression being greater within the other tissues ( < 0.01). Overall, the results indicate that within each tissue, the various circadian clock genes were differentially expressed, in addition to being differentially expressed among the stress tissues of mature Brahman cows. Future use of these findings may assist in improving livestock husbandry and welfare by understanding interactions of the environment, stress responsiveness, and peripheral circadian rhythms.
Topics: Female; Cattle; Animals; Circadian Clocks; Period Circadian Proteins; Circadian Rhythm; Hypothalamus; Adrenal Glands
PubMed: 38003025
DOI: 10.3390/genes14112082 -
Archivos Argentinos de Pediatria Apr 2024Pheochromocytomas and paragangliomas are neuroendocrine tumors producing catecholamines. Pheochromocytomas occur in the adrenal medulla, while paragangliomas are those...
Pheochromocytomas and paragangliomas are neuroendocrine tumors producing catecholamines. Pheochromocytomas occur in the adrenal medulla, while paragangliomas are those that occur outside the adrenal gland. Here we describe a case series of children with a pathological diagnosis of pheochromocytoma or paraganglioma who consulted at a tertiary care children's hospital in Argentina. A total of 21 patients (14 males) were included; their median age was 11.4 years; 8 children had pheochromocytoma and 13, paraganglioma. Arterial hypertension was observed in 14/21. Most paragangliomas were para-aortic (9/13). Since they are a potentially curable cause of hypertension, clinical suspicion is very important. An early diagnosis and the initiation of an adequate antihypertensive treatment, which allows the patient to undergo surgery with normal blood pressure, ensure a cure in most cases if tumor resection is complete.
Topics: Humans; Male; Child; Pheochromocytoma; Argentina; Paraganglioma; Adrenal Gland Neoplasms; Hypertension; Hospitals
PubMed: 37999372
DOI: 10.5546/aap.2023-10099.eng -
Minerva Urology and Nephrology Apr 2024
Topics: Humans; Pheochromocytoma; Adrenalectomy; Adrenal Gland Neoplasms; Laparoscopy; Hyperplasia; Diagnosis, Differential; Female; Adrenal Medulla; Male; Surgery, Computer-Assisted; Middle Aged; Adult
PubMed: 37987635
DOI: 10.23736/S2724-6051.23.05607-0 -
Cureus Oct 2023Pheochromocytomas are rare tumors that arise from the sympathetic ganglia or adrenal medulla and secrete catecholamines that are known for the classic triad of...
Pheochromocytomas are rare tumors that arise from the sympathetic ganglia or adrenal medulla and secrete catecholamines that are known for the classic triad of headaches, profuse sweating, and paroxysmal hypertension. However, there have been instances of asymptomatic patients found to have a pheochromocytoma. Adrenal incidentalomas are accidentally discovered through radiologic imaging, and subsequent testing can confirm a pheochromocytoma. Here, we present a case of a 67-year-old female found to have an adrenal incidentaloma on kidney ultrasound (US) after presenting to the emergency room (ER) due to nausea. She had minimally elevated creatinine at the ER. At her follow-up with her primary care provider (PCP), a kidney US was ordered and showed a probable right suprarenal mass. Further abdominal computed tomography (CT) and abdominal magnetic resonance imaging (MRI) showed that the mass was indeed adrenal, but its etiology is considered indeterminant. Although asymptomatic, further biochemical tests showed elevated serum and urine metanephrines and normetanephrines. Together, these findings revealed that the adrenal mass was likely a silent pheochromocytoma. She underwent a successful right adrenalectomy with pathology confirming pheochromocytoma. This case adds to the literature on the existence of silent pheochromocytomas and highlights the importance of following up on any abnormal findings with a PCP. This patient, although asymptomatic from her pheochromocytoma, needed surgery to prevent possible pheochromocytoma crises, which could cause cardiovascular complications and even death.
PubMed: 37954727
DOI: 10.7759/cureus.46915