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Ochsner Journal 2023Malignant pheochromocytomas are rare and aggressive tumors that arise from the adrenal medulla and secrete catecholamines. Patients exhibit episodic symptoms of...
Malignant pheochromocytomas are rare and aggressive tumors that arise from the adrenal medulla and secrete catecholamines. Patients exhibit episodic symptoms of hypertension, headaches, sweating, and palpitations. The diagnosis is supported by elevated levels of urinary metanephrines, and imaging is used to determine the stage. Treatment involves surgical resection when possible. A 57-year-old male presented with hematemesis and melena, and endoscopy revealed upper gastrointestinal bleeding. Imaging showed a malignant pheochromocytoma that had infiltrated the upper lobe of the right kidney and the right lobe of the liver, with a tumor thrombus extending into the hepatic inferior vena cava, the right atrium, and the right ventricle. The patient denied surgery and was treated with palliative medical therapy until he died 3 months later. Although rare, malignant pheochromocytomas may present with upper gastrointestinal bleeding. While metastasis to the liver is a typical manifestation of malignant pheochromocytomas, invasion of the inferior vena cava with infiltration to the right ventricle resulting in tricuspid valve malfunction is a rare finding.
PubMed: 38143552
DOI: 10.31486/toj.23.0049 -
International Journal of Molecular... Dec 2023The adrenal gland is paired peripheral end organs of the neuroendocrine system and is responsible for producing crucial stress hormones from its two functional...
The adrenal gland is paired peripheral end organs of the neuroendocrine system and is responsible for producing crucial stress hormones from its two functional compartments, the adrenal cortex, and the adrenal medulla under stimuli. Left-right asymmetry in vertebrates exists from the central nervous system to peripheral paired endocrine glands. The sided difference in the cerebral cortex is extensively investigated, while the knowledge of asymmetry of paired endocrine glands is still poor. The present study aims to investigate the asymmetries of bilateral adrenal glands, which play important roles in stress adaptation and energy homeostasis via steroid hormones produced from the distinct functional zones. Left and right adrenal glands from male C57BL/6J mice were initially histologically analyzed, and high-throughput RNA sequencing was then used to detect the gene transcriptional difference between left and right adrenal glands. Subsequently, the enrichment of functional pathways and ceRNA regulatory work was validated. The results demonstrated that the left adrenal gland had higher tissue mass and levels of energy expenditure, whereas the right adrenal gland appeared to be more potent in glucocorticoid secretion. Further analysis of adrenal stem/progenitor cell markers predicted that Shh signaling might play an important role in the left-right asymmetry of adrenal glands. Of the hub miRNAs, miRNA-466i-5p was identified in the left-right differential innervation of the adrenal glands. Therefore, the present study provides evidence that there are asymmetries between the left and right adrenal glands in glucocorticoid production and neural innervation, in which Shh signaling and miRNA-466i-5p play an important role.
Topics: Animals; Mice; Male; Glucocorticoids; Mice, Inbred C57BL; Adrenal Glands; Adrenal Cortex; MicroRNAs
PubMed: 38139285
DOI: 10.3390/ijms242417456 -
Virchows Archiv : An International... Feb 2024Tumors of the endocrine glands are common. Knowledge of their molecular pathology has greatly advanced in the recent past. This review covers the main molecular... (Review)
Review
Tumors of the endocrine glands are common. Knowledge of their molecular pathology has greatly advanced in the recent past. This review covers the main molecular alterations of tumors of the anterior pituitary, thyroid and parathyroid glands, adrenal cortex, and adrenal medulla and paraganglia. All endocrine gland tumors enjoy a robust correlation between genotype and phenotype. High-throughput molecular analysis demonstrates that endocrine gland tumors can be grouped into molecular groups that are relevant from both pathologic and clinical point of views. In this review, genetic alterations have been discussed and tabulated with respect to their molecular pathogenetic role and clinicopathologic implications, addressing the use of molecular biomarkers for the purpose of diagnosis and prognosis and predicting response to molecular therapy. Hereditary conditions that play a key role in determining predisposition to many types of endocrine tumors are also discussed.
Topics: Humans; Pathology, Molecular; Endocrine Gland Neoplasms; Mutation; Thyroid Gland; Adrenal Gland Neoplasms
PubMed: 38108848
DOI: 10.1007/s00428-023-03713-4 -
Indian Journal of Urology : IJU :... 2023Synchronous tumors comprising of cortical as well as medullary differentiation are rare in the adrenal gland. Collision tumors comprise of two independent neoplasms...
Synchronous tumors comprising of cortical as well as medullary differentiation are rare in the adrenal gland. Collision tumors comprise of two independent neoplasms arising from the cortex and the medulla without any histological admixture. Adrenal cortico-medullary mixed tumors are rare tumors comprising of adrenal cortical and chromaffin cells intermixed with each other. Ganglioneuroma is a rare benign tumor originating from the paravertebral sympathetic ganglia and is rarely seen in the adrenal medulla. The presence of a synchronous ganglioneuroma with an adrenal cortical adenoma in the ipsilateral adrenal gland is exceedingly rare. We report a collision tumor comprising of an adrenal cortical adenoma and a ganglioneuroma detected as an incidental finding in a pediatric patient who presented with hypertension and palpitations.
PubMed: 38077205
DOI: 10.4103/iju.iju_174_23 -
Cureus Nov 2023Phaeochromocytoma is indeed a rare and frequently misunderstood neuroendocrine tumor originating from chromaffin cells in the adrenal medulla. Its clinical presentation...
Phaeochromocytoma is indeed a rare and frequently misunderstood neuroendocrine tumor originating from chromaffin cells in the adrenal medulla. Its clinical presentation often includes paroxysmal hypertension, palpitations, headache, and diaphoresis, which can easily be mistaken for common medical conditions. Timely diagnosis and precise localization are paramount for ensuring the best possible outcomes for patients. In this case report, we describe an unusual presentation of phaeochromocytoma in a 36-year-old man who presented with acute myocarditis. This atypical manifestation underscores the diagnostic challenges associated with phaeochromocytoma, as its symptoms can mimic various other cardiac and non-cardiac conditions. Vigilant clinical evaluation and a multidisciplinary approach are essential for promptly recognizing and managing such cases, thus optimizing patient care and prognosis.
PubMed: 38073930
DOI: 10.7759/cureus.48554 -
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 -
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