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.

Recently, the genetic background of pheochromocytomas/paragangliomas (PPGLs) has been rapidly revealed. These tumors have been referred to as the “ten percent tumor”; however, the frequency of genetic variants of PPGLs has turned out to be more common than expected. PPGLs are potentially hereditary tumors and appear clinically sporadic. Here, we report a case of bilateral pheochromocytoma (PCC) with a variant in the MYC-associated factor X (MAX) gene (c.295 + 1G > A). A male patient was diagnosed with adrenal pheochromocytoma (PCC) and underwent a left adrenalectomy at the age of 40. A new tumor in the right adrenal gland was detected at the age of 43. Urinary metanephrine and normetanephrine concentrations gradually increased. The size of the right adrenal PCC continued to increase one year after detection. Genetic testing of the peripheral blood revealed the presence of a pathogenic variant in MAX. The natural history of adrenal PCCs with the MAX variant has not yet been clarified, because the number of reported cases is not sufficient. Thus, clinicians should consider a MAX variant when they find bilateral or multiple PCCs.

Ectopic adrenocorticotropic hormone (ACTH) syndrome is usually caused by pulmonary and bronchial tumors and rarely by pheochromocytoma. To date, the majority of ACTH-secreting pheochromocytomas have been unilateral, with the exception of two cases. A 54-year-old male presented with hypertension and bilateral adrenal tumors. The patient did not report having classic cushingoid features or experience of paroxysmal headaches or sweating, but presented with a slight abdominal obesity. The patient was clinically and pathologically diagnosed with bilateral ectopic ACTH-secreting pheochromocytomas. Whole-exome sequencing demonstrated that the 19 pheochromocytoma-related genes were unmutated. The pheochromocytomas on the two sides exhibited negative ACTH staining, but the ACTH concentration was markedly higher in the tumor tissue homogenates than in those tumors of another 3 patients with non-ACTH secretion pheochromocytoma. Electron microscopy identified two types of neuroendocrine cells in the tumor tissues. Primary culture of the pheochromocytoma cells revealed that ACTH secretion was inhibited by a mechanistic target of rapamycin inhibitor, AZD8055.

BACKGROUND: von Hippel-Lindau disease (vHL disease) is a hereditary disease in which tumors and cysts develop in many organs, in association with central nervous system hemangioblastomas, pheochromocytomas, and pancreatic tumors. We herein report a case of vHL disease (type 2A) associated with bilateral pheochromocytomas, pancreatic neuroendocrine tumors (PNET), and cerebellar hemangioblastomas treated via pancreatectomy after adrenalectomy.
METHODS: A 51-year-old woman presented with a cerebellar tumor, bilateral hypernephroma, and pancreatic tumor detected during a medical checkup. 18F-fluorodeoxyglucose positron emission tomography-computed tomography revealed a bilateral adrenal gland tumor and a tumor in the head of the pancreas, while an abdominal computed tomography examination revealed a 30-mm tumor with strong enhancement in the head of the pancreas. Cranial magnetic resonance imaging showed a hemangioblastoma in the cerebellum. Therefore, a diagnosis of vHL disease (type 2A) was made. Her family medical history included renal cell carcinoma in her father and bilateral adrenal pheochromocytoma and spinal hemangioblastoma in her brother. A detailed examination of endocrine function showed that the adrenal mass was capable of producing catecholamine. Treatment of the pheochromocytoma was prioritized, and therefore, laparoscopic left adrenalectomy and subtotal resection of the right adrenal gland were performed. Once the postoperative steroid levels were replenished, subtotal stomach-preserving pancreatoduodenectomy was performed for the PNET. After a good postoperative course, the patient was discharged in remission on the 11th day following surgery. Histopathological examination findings indicated NET G2 (MIB-1 index 10-15%) pT3N0M0 Stage II A and microcystic serous cystadenoma throughout the resected specimen. The patient is scheduled to undergo treatment for the cerebellar hemangioblastoma.
CONCLUSIONS: A two-staged resection is a safe and effective treatment option for bilateral pheochromocytoma and PNET associated with vHL disease.

The 2017 Endocrine Society annual meeting included several communications and debates on the conservative adrenal surgery in bilateral hereditary pheochromocytomas (BHP), bilateral adrenal macronodular hyperplasia (BAMH) and primary hyperaldosteronism (PHA). The general principle is to preserve a part of the adrenal cortex to prevent the occurrence of a definitive adrenal insufficiency. In BHP, cortical sparing surgery allows more than 50% of patients to maintain normal corticotropic function at 10 years with a low recurrence rate (~ 10%). Since the adrenal medulla cannot be removed entirely, recurrence seems inevitable and long-term follow-up is essential. Individual risk of malignancy must be taken into account. In BAMH responsible for Cushing syndrome, unilateral adrenalectomy induces a normalization of urinary free cortisol in 92 to 100% of cases and even corticotropic insufficiency in 40 to 100% of cases. This is most often transient. Late recurrences of Cushing\'s syndrome may occur in 13 to 60% of cases. Prolonged patient monitoring is therefore essential. In PAH with lateralized aldosterone production, minimally invasive partial adrenal surgery, which consists of removing only the adrenal adenoma visualized at TDM, allows an improvement blood pressure in about 94% of patients. However, failure or recurrence may occur. Its place therefore remains marginal in the treatment of the lateralized PAHs.

The optimal operative approach in a patient with bilateral pheochromocytoma is controversial. Subtotal minimal invasive cortical sparing adrenalectomy is gaining interest in many centers. We describe a novel technique for single stage approach for cortical sparing adrenalectomy for bilateral pheochromocytoma using head docking in order to offer good exposure of bilateral upper peritoneum without requiring patient or robot repositioning.

A 66-year-old Lithuanian female patient with a history of hypertension was diagnosed with bilateral adrenal tumors during a routine sonoscopy. Scintigraphy with metaiodobenzylguanidine and computed tomography scan revealed right 130/116/93 mm and left 85/61/53 mm pheochromocytomas. The patient suffered from hypertension with blood pressure over 240/100 mm Hg and heartbeat disturbances. Blood adrenaline levels exceeded the norm 10-fold. After possible spread of tumors was rejected, laparoscopic transperitoneal adrenalectomy was planned in 2 stages, starting on the right then followed by the left side. After preoperative treatment with adrenoblockers, 2-stage bilateral laparoscopic adrenalectomy was performed. 13 cm × 12 cm × 9.5 cm right adrenal and, 3 months later, 8.5 cm × 8 cm × 6 cm left adrenal pheochromocytomas were removed. Histologically - radical extirpation, pheochromocytomas with possible malignant potential. Stable remission of hypertension was achieved postoperatively. Laparoscopic transperitoneal adrenalectomy is a safe and feasible method of treatment of large benign and possible malignant, but noninvasive pheochromocytomas.

Multiple endocrine syndromes are unusual familial disorders affecting multiple endocrine glands; they result from an autoimmune process, causing progressive inflammatory destruction of glandular tissue, leading to hormonal insufficiency. Neurologic manifestations result from endocrine failure and/or independent autoimmune neurologic disorders. Multiple endocrine neoplasia (MEN) results from oncogene mutations in cells derived from the neural crest with proliferation of neuroectodermal cells within endocrine glands and skin. Although most MEN-associated tumors are histologically benign, medullary carcinoma of the thyroid is invasive and potentially lethal. Neurologic symptoms may be caused by increased hormonal production and by the presence of other peripheral nervous system (PNS) and central nervous system (CNS) tumors.

Pheochromocytoma is a rare neuroendocrine tumor of childhood. We present a 14-year-old boy with bilateral pheochromocytoma, post nephrectomy in view of a non-functioning kidney presenting with severe hypertension and end organ damage. Diagnosis was confirmed with 24-hour urinary VMA, catechol amines, and CT scan. Preoperative blood pressure (BP) was controlled with prazosin, propranolol, nicardipine, and HCT-spironolactone. Anesthesia was given with general endotracheal anesthesia with epidural analgesia. Intraoperative BP rise was managed with infusion of NTG, MgSO4, esmolol, and dexmedetomidine which was especially challenging on account of bilateral tumor.