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.

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.

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.

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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.

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.

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.

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 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.

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.