von Hippel-Lindau Syndrome and Secondary Hypertension: A Case Report.

von Hippel-Lindau (VHL) syndrome (OMIM #193300) is an autosomal dominant disorder with incomplete penetrance occurring due to a mutation in the VHL gene present on chromosome 3. We present the case of a 21-year-old male with a history of retinoblastoma presenting with intermittent headaches for one month. He was a known hypertensive and his blood pressure on presentation was 180/100 mmHg. A secondary cause for his hypertension was sought. Multiple cysts in his pancreas, both his kidneys, and a mass in the right suprarenal fossa were detected on an abdominal ultrasonogram and a subsequent computed tomography scan of the abdomen. VHL and a pheochromocytoma were suspected, and a positron emission tomography-computed tomography scan was done which collaborated with the above findings. The presence of multiple cystic lesions in the pancreas and kidneys, especially in an individual with a family history of VHL syndrome, should alert the physician to the possibility of VHL syndrome. The need for evaluation of causes for hypertension, especially in young individuals with resistant hypertension, is also highlighted.

Giant Cystic Pheochromocytoma Associated With Neurofibromatosis Type 1: A Case Report.

Pheochromocytomas are tumors that develop from the chromaffin cells of the adrenal medulla. More than 40% of cases of pheochromocytomas are associated with genetic conditions such as neurofibromatosis type 1 (NF1) or von Hippel-Lindau syndrome. Cystic pheochromocytomas are rare, generally asymptomatic, and thus of bigger size at the time of diagnosis. Surgical treatment is necessary to prevent cardiovascular morbidity and malignancy risk. We report the case of a 27-year-old patient admitted for further examination of a left adrenal mass that was discovered by an abdominal CT scan in the context of abdominal pain associated with hypertension evolving for three years. The clinical examination showed the presence of multiple café au lait spots, axillary and inguinal freckling with two dermal neurofibromas diagnosed clinically, as well as Lisch nodules on bilateral ophthalmic examination, thus meeting the clinical criteria for the diagnosis of NF1. The clinical laboratory investigation showed elevated urinary metanephrine and normetanephrine levels. CT scan examination showed a 10 cm left adrenal cystic mass on abdominal CT. This mass uptake of the radioligand in metaiodobenzylguanidine (MIBG) scintigraphy without secondary extra-adrenal localization allowed the diagnosis of a seemingly benign cystic pheochromocytoma to be made. The patient was put on presurgical drug preparation with volume expansion and then underwent left unilateral adrenalectomy. The histopathological study was in favor of a rather aggressive cystic pheochromocytoma with a pheochromocytoma of the adrenal gland scaled (PASS) score of 9. Blood pressure and urine catecholamines at seven days, three months, six months, and one year after surgery were normalized. Cystic pheochromocytoma is a rare tumor with a potentially poor prognosis. It is characterized by a more insidious evolution and a larger volume at diagnosis. It should be considered a diagnosis in patients with a cystic adrenal mass or an extra-adrenal mass with fluctuating blood pressure during surgery. This case illustrates the importance of both presurgical preparation and screening for pheochromocytoma in neurofibromatosis type 1.

Detection and Surgical Approach to Pheochromocytoma: A Case Report.

Pheochromocytomas are neuroendocrine tumors that produce, store, and secrete catecholamines. They are found in the chromaffin tissue of the adrenal medulla and manifest clinical symptoms by producing an excessive amount of one or more catecholamines, such as dopamine, adrenaline, and noradrenaline, as well as their inactive metabolites, such as metanephrine, normetanephrine, and 3-methoxytyramine. This paper is the case report of a 53-year-old male patient with diabetes and hypertension who has been experiencing symptoms such as night sweats, frequent colds, weight loss, reduced appetite, and generalized anxiety. The patient presented with pelvic pain and sought medical attention, leading to an abdominal MRI scan that revealed a right adrenal mass. The patient's plasma metanephrine levels were found to be four-fold higher than the normal range. A contrast CT scan of the abdomen and pelvis revealed a right adrenal gland with increased dimensions and well-defined edges. A diagnosis of right adrenal pheochromocytoma was made. The patient underwent a right laparoscopic adrenalectomy, which resulted in a reduction in metanephrine levels and normal blood pressure readings. The patient presented a favorable clinical evolution in the post-surgical period, for which it was decided to be discharged home.

A Double-Headed Physiologic Monster: A Case Report and Literature Review.

Adrenal incidentaloma (AI) is rare and found in approximately 2-4% of abdominal computed tomography scans. Up to 10% of patients with AI have autonomous secretion of adrenal hormones. If not quickly diagnosed and adequately treated, the outcome may be devastating to the patient. On very rare occasions, a pheochromocytoma may, in addition to the production of catecholamine, produce adrenocorticotropic hormone causing Cushing disease. We present a case of a patient with pheochromocytoma and Cushing syndrome.

Pheochromocytoma: A Troublesome Tumor.

This report presents the case of a 45-year-old man with a history of episodic headaches, palpitations, and sweating for the past six months. His blood pressure on admission was 170/100 mmHg. The patient was diagnosed with pheochromocytoma confirmed by elevated levels of plasma catecholamines and metanephrines. CT imaging revealed a 3 cm mass in the left adrenal gland with evidence of local invasion into the surrounding tissues. The patient underwent a laparoscopic adrenalectomy and was discharged on the third postoperative day with normal blood pressure. Histopathological examination confirmed the diagnosis of pheochromocytoma. The patient was followed for six months postoperatively with the resolution of symptoms and no evidence of tumor recurrence on imaging. Recurrence involves complex environment-gene interactions that are poorly understood. The diagnosis of pheochromocytoma could take several weeks to several years mainly because the symptoms are nonspecific and episodic. Although sudden death is rare, the debilitations associated with pheochromocytoma are often multisystemic with cardiovascular, emotional, and metabolic components. This case report highlights the importance of early diagnosis, appropriate management, and follow-up for pheochromocytoma.

Non-Functional Yet Terminal: A Case of Asymptomatic Retroperitoneal Paraganglioma With Distant Metastasis.

Retroperitoneal paraganglioma remains an extremely rare type of tumor that arises from either sympathetic or parasympathetic neural crest cells. It could be functional or non-functional. Non-functional paraganglioma may present as a diagnostic challenge since patients are usually asymptomatic and tend to present to the hospital with complications from the invasion of the tumor. Malignancy is usually determined by the degree of metastasis. The gold standard of diagnosis is biopsy and obtaining a sample for histological examination. This author presents a case of asymptomatic, non-functional retroperitoneal paraganglioma with distant metastasis.

Reverse Takotsubo Cardiomyopathy Triggered by Undiagnosed Right Adrenal Pheochromocytoma: A Rare Occurrence.

Takotsubo cardiomyopathy (TTC), also known as stress cardiomyopathy or broken heart syndrome, is a condition characterized by transient left ventricular dysfunction resembling myocardial infarction but without obstructive coronary artery disease. We present a rare case of a 59-year-old patient with cardiogenic shock (CS) caused by reverse TTC triggered by an undiagnosed right adrenal pheochromocytoma tumor. The patient initially presented with chronic headaches and difficulty breathing, and their condition rapidly deteriorated, necessitating intubation and inotropic support. Diagnostic tests confirmed the diagnosis of reverse TTC, and further investigation revealed an actively growing adrenal mass suggestive of a pheochromocytoma. The patient responded well to treatments, including the use of intra-aortic balloon pump support and subsequent weaning. A right adrenalectomy confirmed the presence of a pheochromocytoma. This case highlights the association between pheochromocytoma and reverse TTC, emphasizing the need to consider this rare etiology in patients presenting with CS. Long-term monitoring is crucial due to the risk of recurrence, even after tumor removal.

Adrenal Ganglioneuroma: Diagnosis, Presentation, and Management of a Rare Tumor.

Adrenal ganglioneuromas are rare tumors arising from sympathetic ganglion cells that may present similarly to other adrenal tumors, making preoperative diagnosis challenging. We present a case of a young woman with a history of Hashimoto's thyroiditis who presented with hypertension and headaches. An abdominal CT scan revealed a large left adrenal mass, and while laboratory tests for catecholamines and metanephrines were normal, the suspicion for pheochromocytoma remained high given the size of the mass and persistent hypertension. The patient was started on alpha-blockers and beta-blockers in preparation for surgical removal. Pathology revealed a mature ganglioneuroma without evidence of malignancy, and postoperative blood pressure was normalized. We hypothesize that vessel compression from the large mass created functional stenosis, resulting in persistent hypertension. This case highlights the importance of a thorough workup for hypertension in young adults and routine preventative care visits to avoid delayed management. Adrenalectomy with histopathological examination remains the gold standard for treatment and diagnosis, and patients have a good prognosis following resection, with minimal need for recurrent therapy.

Abandonment of intravenous volume expansion after preoperative receipt of α-blockers in patients with adrenal pheochromocytoma was not an independent risk factor for intraoperative hemodynamic instability.

Background: There is no consensus on whether intravenous rehydration must be added after preoperative phenoxybenzamine (PXB) administration for pheochromocytoma. The aim of this study is to investigate whether abandonment of intravenous volume expansion after PXB administration is associated with intraoperative hemodynamic instability. Methods: 83 Patients with pheochromocytoma received surgical treatment in the Department of Urology, Handan First Hospital, between October 2014 and July 2022. All patients were subclassified into either the hemodynamic stability group (HS group) or the hemodynamic instability group (HU group) according to whether intraoperative hemodynamic instability occurred, with 51 cases in HS group and 32 cases in HU group. Differences in data between the two groups were examined, and the risk factors for intraoperative hemodynamic instability were analyzed using logistic regression. Results: The results of the analysis showed no statistically significant differences in age, sex, location of the tumor, surgical method, body mass index (BMI) ≥ 24 kg/m2, blood and urine catecholamine test results, preoperative oral PXB followed by combined intravenous volume expansion, proportion of patients with hypertension or diabetes mellitus or coronary heart disease between the two groups (P>0.05). The size of the tumor in the HS group was smaller than that in the HU group (5.3 ± 1.9 cm vs 6.2 ± 2.4 cm P=0.010). Multivariate analyses demonstrated that abandonment of intravenous volume expansion after preoperative receipt of α-blockers in patients with adrenal pheochromocytoma was not an independent risk factor for intraoperative hemodynamic instability. Only the tumor size (P=0.025) was an independent risk factor for intraoperative hemodynamic instability. Conclusion: The purpose of general preoperative intravenous fluid expansion is to prevent hypotension after the tumor has been resected. In the current study, we indicated that preoperative management of pheochromocytomas using the α-blocker PXB in combination with intravenous volume expansion does not further reduce the risk of intraoperative hemodynamic instability or postoperative complications compared with oral PXB alone. Therefore, our study supports preoperative management of pheochromocytoma with a single α-blocker, PXB, as sufficient.

Robotic Adrenalectomy in a Patient With Neurofibromatosis Type 1 and Pheochromocytoma.

A 27-year-old female with a past medical history of neurofibromatosis type 1 (NF1) presented with obstructive hydrocephalus due to a thalamic tumor. The neurosurgery team attempted an operative intervention twice, but both times, the patient experienced a hypertensive emergency and unstable supraventricular tachycardia upon induction of anesthesia. After the second failed surgery, a pheochromocytoma was suspected and the workup demonstrated a left pheochromocytoma. Pheochromocytomas in patients with NF1 are known to be more dangerous and labile, requiring in-depth discussion and preparation by personnel in endocrinology, anesthesia, neurosurgery, and minimally invasive surgery. Once the patient was stable and deemed fit for surgery, a robotic adrenalectomy followed by ventriculoperitoneal shunt placement began. After induction of anesthesia, the patient went into hypertensive emergency again. However, the anesthesia team was prepared and quickly resolved this with medical therapy. Minimally invasive surgeons had the patient's live vitals displayed on their robotic monitors to increase their awareness of patient hemodynamics. This provided live feedback on the surgeons' effect as they removed the pheochromocytoma. Surgeons also performed vein clamping to preemptively see the effects of adrenalectomy. When vein clamping demonstrated safety to proceed, adrenalectomy was completed without complication. This case not only highlights the rare pathology of a woman with NF1 with pheochromocytoma, but it also demonstrates the importance of preparedness and communication among a multidisciplinary team in complex cases to ensure a successful outcome. Novel techniques were also used in performing a robotic-assisted adrenalectomy that can aid other adrenal surgeons.

Right adrenal giant cystic pheochromocytoma: A case report.

A 78-year-old woman was referred to our institution for evaluation and treatment of a mass on her right adrenal gland measuring 12 × 11 × 10 cm. Twenty-four-hour urine analysis revealed a total metanephrine level over 3 times the upper limit of normal. Scintigraphy using 123I-metaiodobenzylguanidine was positive. The mass was resected en bloc by laparotomy after a laparoscopic attempt was unsuccessful. Histopathologic examination revealed a pheochromocytoma of the right adrenal gland, weighing 576 g. The Grading System for Adrenal Pheochromocytoma and Paraganglioma score was 6, and the histology of the tumor was a moderately differentiated type.

CT Texture Analysis of Adrenal Pheochromocytomas: A Pilot Study.

Radiomics is a promising research field that combines big data analysis (from tissue texture analysis) with clinical questions. We studied the application of CT texture analysis in adrenal pheochromocytomas (PCCs) to define the correlation between the extracted features and the secretory pattern, the histopathological data, and the natural history of the disease. A total of 17 patients affected by surgically removed PCCs were retrospectively enrolled. Before surgery, all patients underwent contrast-enhanced CT and complete endocrine evaluation (catecholamine secretion and genetic evaluation). The pheochromocytoma adrenal gland scaled score (PASS) was determined upon histopathological examination. After a resampling of all CT images, the PCCs were delineated using LifeX software in all three phases (unenhanced, arterial, and venous), and 58 texture parameters were extracted for each volume of interest. Using the Mann-Whitney test, the correlations between the hormonal hypersecretion, the malignancy score of the lesion (PASS > 4), and texture parameters were studied. The parameters DISCRETIZED_HUpeak and GLZLM_GLNU in the unenhanced phase and GLZLM_SZE, CONVENTIONAL_HUmean, CONVENTIONAL_HUQ3, DISCRETIZED_HUmean, DISCRETIZED_AUC_CSH, GLRLM_HGRE, and GLZLM_SZHGE in the venous phase were able to differentiate secreting PCCs (p < 0.01), and the parameters GLZLM_GLNU in the unenhanced phase and GLRLM_GLNU and GLRLM_RLNU in the venous differentiated tumors with low and high PASS. CT texture analysis of adrenal PCCs can be a useful tool for the early identification of secreting or malignant tumors.

The Spectrum of Postoperative Complications and Outcomes After Open Adrenalectomy: An Experience From a Developing Country.

Background Open adrenalectomy is an invasive surgical procedure that is commonly performed for adrenal gland neoplasms in developing countries. Due to its complexity, the patients are predisposed to a number of complications and dismal outcomes. The objective of our study is to assess different characteristics of patients undergoing open adrenalectomy, including their histology, postoperative complications, and outcomes. Methods This retrospective cross-sectional study included 107 patients undergoing open adrenalectomy for primary adrenal gland neoplasms. Patients with bilateral involvement, metastatic disease, or unresectable tumors were excluded. Patients were evaluated for different features that included demographic data, tumor properties, postoperative outcomes, and complications. Results Out of 107 patients, 45 (42.1%) were females. The mean age of the patients was 47.53 ± 8.45 years. Abdominal pain and severe headaches were the most common presenting complaints. A total of 96 (89.7%) tumors were benign, while 11 (10.3%) were malignant. Upon the histopathological examination of the resected specimen, adrenal adenoma was present in 49 (45.8%) cases, while adrenal pheochromocytoma was present in 41 (38.3%) cases. A total of 51 patients developed different postoperative complications including surgical site infections (22.4%), atelectasis (11.2%), deep venous thrombosis (7.5%), and retroperitoneal hematoma (5.6%). In-hospital mortality occurred in three (2.8%) patients. Conclusion Surgical site infections, atelectasis, deep venous thrombosis, and retroperitoneal hematoma were frequent postoperative complications after open adrenalectomy. These complications increase morbidity and mortality, especially in developing countries. Improved surgical techniques, intraoperative hemostasis, and multidisciplinary approach can yield favorable postoperative outcomes.

Unusually large paraganglioma complicated with successive catecholamine crises: A case report and review of the literature.

Background: Paragangliomas are rare neuroendocrine tumors that could secret catecholamines. Hypertension and heart failure caused by the catecholamine crisis are fatal cardiovascular events. However, silent paragangliomas that lack typical symptoms of catecholamine pose a significant diagnostic challenge. Case summary: A 45-year-old woman who presented with more than 1-year history of abdominal discomfort was suspected of having a gastrointestinal stromal tumor by a local hospital since a vast metastatic mass occupied her left abdomen. Thus, she was recommended to our hospital. After completing the gastroscopy, she unexpectedly developed acute heart failure and was transferred to the Intensive Care Unit (ICU) where the initial diagnosis of paraganglioma was considered through path. However, a second catecholamine crisis due to constipation led to acute heart failure again. After anti-heart failure therapy and rigorous preoperative preparation, surgery was arranged to remove the tumor. Postoperative pathology confirmed the paraganglioma, and the patient was discharged from the hospital in good condition. Conclusion: We reported a rare case of huge retro-peritoneal paraganglioma with successive catecholamine crises and acute heart failure. This was probably the largest retro-peritoneal paraganglioma since the 1980s. Besides, we were the first to use surgical drawing to illustrate its complex anatomical adjacent relationship of retro-peritoneal paraganglioma. Our case emphasizes the inclusion of extra-adrenal paraganglioma in the differential diagnosis of retroperitoneal tumors. In suspected paragangliomas, catecholamine testing is preferable to invasive procedures including gastroscopy and biopsy to avoid triggering a catecholamine crisis. Surgical resection is the primary treatment. We highlight the priority of dealing with the venous reflux branches of the tumor to prevent the release of catecholamines into the blood. In particular, preoperative preparation plays a vital role in managing paraganglioma. Moreover, it is necessary to schedule genetic testing and clinical follow-up due to the metastatic potential of paragangliomas.

An Interesting Case of Treatment-Resistant Ventricular Tachycardia Secondary to Pheochromocytoma and Left Ventricular Non-compaction.

A 51-year-old patient was admitted with chest pain and broad complex ventricular tachycardia. He received three consecutive direct cardioversion (DC) shocks and was commenced on amiodarone infusion via a central venous catheter or central line (CVC). He responded to treatment and normal sinus rhythm (NSR) was achieved. He had elevated troponin I and underwent coronary angiogram which initially was thought to be responsible for his ventricular tachycardia. Coronary angiogram (CAG) showed unobstructed coronary arteries. He was recently diagnosed with pheochromocytoma and was commenced on Phenoxybenzamine 10 mg two months back. He developed ventricular tachycardia (VT) again the next day that did not respond to four consecutive direct cardioversion shocks (DC) and antiarrhythmic medications. He was intubated and ventilated to terminate his VT and was transferred to the intensive care unit (ICU). He remained intubated for 48 hours and he remained in NSR, after which he was extubated. He was commenced on bisoprolol and was later stepped down to the coronary care unit (CCU). Cardiac magnetic resonance imaging (CMR) showed left ventricular non-compaction (LVNC) or possibly myocarditis in view of patient's known history of pheochromocytoma. He was discussed with surgical team at another hospital for surgical resection of the adrenal tumor and had a few further runs of VT while he was waiting to be transferred. The patient finally underwent surgical resection of the tumor and was booked for implantable cardioverter defibrillator (ICD) in view of his VT. This was an interesting case of treatment-resistant VT driven by pheochromocytoma and LVNC, and it is important to be familiar with the fact that conventional therapies may fail in these patients and may require intubation and ventilation to terminate VT storms.

An Unusual Cause of Adrenal Mass in Neurofibromatosis Type 1: Malignant Peripheral Nerve Sheath Tumor.

A malignant peripheral nerve sheath tumor (MPNST) is an aggressive tumor that can arise from the malignant transformation of benign neurofibromas in patients with neurofibromatosis type 1 (NF1). MPNST occurs in 2% of patients with NF1, contributing to significant mortality in these patients. Here, we report the case of a 67-year-old female with a known history of neurofibromatosis type 1 who was referred to general surgery after the discovery of a large left-sided adrenal mass on CT imaging five months earlier. Lab workup revealed elevated urine catecholamines, concerning pheochromocytoma. As pheochromocytoma is also common in those with NF-1, appropriate medical management followed by surgical resection was performed. The final pathology report revealed an MPNST.

Major intraoperative bleeding and drastic change in circulatory dynamics in a pregnant patient with metastatic pheochromocytoma: a case report.

BACKGROUND: Metastatic pheochromocytoma in the spine is a very rare complication during pregnancy. We report anesthesia in a pregnant woman for resection of an undiagnosed spinal tumor, accompanied by remarkable hemodynamic changes and massive bleeding. CASE PRESENTATION: A 33-year-old woman at 17 weeks of gestation presented with the rapid progress of bilateral lower leg paralysis. A diagnosis of spinal tumor was made, and surgical resection was planned. Although the surgery was suspended because of remarkable hemodynamic changes and massive bleeding, fetal heart rate was stable. Postoperative examination revealed pheochromocytoma in the urinary bladder as a primary lesion with spinal metastasis. CONCLUSION: Although spinal pheochromocytoma is extremely rare in pregnant women, it should be suspected when abnormal hypertension is observed with accompanying neurological deficits. Preservation of maternal circulation and uteroplacental blood flow should be the first priority during anesthesia.

Diabetes Mellitus and Cardiomyopathy as Presenting Features of Occult Malignant Pheochromocytoma.

The concomitant occurrence of diabetes mellitus and cardiomyopathy secondary to occult malignant pheochromocytoma has rarely been reported. This case report describes the case of a 48-year-old female with a previous history of diabetes mellitus, hypertension, and cardiomyopathy who presented with fatigue and significant weight loss. Neither typical symptoms of pheochromocytoma nor metastatic symptoms were presented. Pheochromocytoma with extension to the liver was incidentally found from computed tomography of the abdomen and laboratory investigations during the work-up to identify the cause for the weight loss. Right adrenalectomy and a right hepatectomy were performed. Malignant pheochromocytoma was diagnosed based on pathology. All of her underlying conditions including diabetes mellitus, hypertension, and cardiomyopathy, were improved following the complete resection of the tumor. This case emphasizes the importance of early suspicion and diagnosis of malignant pheochromocytoma in individuals with atypical presentation of a chromaffin-secreting tumor.

Looking Deep Into the Palpitation - Pheochromocytoma Presenting as Sinus Node Dysfunction.

A 23-year-old male came to the outpatient department with a history of intermittent palpitations and headaches for the past six to eight months. He was diagnosed with hypertension and had a junctional rhythm on an electrocardiogram (ECG). On further workup for his hypertension, he was found to have elevated levels of serum metanephrines and computed tomography (CT) and positron emission tomography (PET) scan revealed pheochromocytoma. He was subsequently operated upon and his arrhythmia subsided after surgery. We discuss our approach to this scenario, which leads us to a rather rare cause of sinus node dysfunction.