Perioperative Management of Pheochromocytoma/Paraganglioma: Is There a State of the Art?

Mannelli, Massimo; Dralle, Henning; Lenders, Jwm

doi:10.1055/s-0032-1306275

Cited by 27 publications

(18 citation statements)

References 22 publications

(29 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Furthermore, pheochromocytomas can lead to life-threatening complications, especially in the context of medical interventions (surgery, biopsies etc.) (72,217,218). Additional hormonal work-up should depend on the stage of the extra-adrenal malignancy and life expectancy.…”

Section: Reasoningmentioning

confidence: 99%

Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors

Fassnacht

Arlt

Bancos

et al. 2016

European Journal of Endocrinology

1,339

1,571

View full text Add to dashboard Cite

By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions crucial for the management of adrenal incidentaloma patients, addressing these four with systematic literature searches: (A) How to assess risk of malignancy?; (B) How to define and manage low-level autonomous cortisol secretion, formerly called 'subclinical' Cushing's syndrome?; (C) Who should have surgical treatment and how should it be performed?; (D) What follow-up is indicated if the adrenal incidentaloma is not surgically removed? Selected recommendations: (i) At the time of initial detection of an adrenal mass establishing whether the mass is benign or malignant is an important aim to avoid cumbersome and expensive follow-up imaging in those with benign disease. (ii) To exclude cortisol excess, a 1 mg overnight dexamethasone suppression test should be performed (applying a cut-off value of serum cortisol ≤50 nmol/L (1.8 µg/dL)). (iii) For patients without clinical signs of overt Cushing's syndrome but serum cortisol levels post 1 mg dexamethasone >138 nmol/L (>5 µg/dL), we propose the term 'autonomous cortisol secretion'. (iv) All patients with '(possible) autonomous cortisol' secretion should be screened for hypertension and type 2 diabetes mellitus, to ensure these are appropriately treated. (v) Surgical treatment should be considered in an individualized approach in patients with 'autonomous cortisol secretion' who also have comorbidities that are potentially related to cortisol excess. (vi) In principle, the appropriateness of surgical intervention should be guided by the likelihood of malignancy, the presence and degree of hormone excess, age, general health and patient preference. (vii) Surgery is not usually indicated in patients www.eje-online.org European Journal of Endocrinology175:2 G2 Clinical Practice Guideline M Fassnacht and others ESE and ENSAT guideline on adrenal incidentalomawith an asymptomatic, nonfunctioning unilateral adrenal mass and obvious benign features on imaging studies. We provide guidance on which surgical approach should be considered for adrenal masses with radiological findings suspicious of malignancy. Furthermore, we offer recommendations for the follow-up of patients with adrenal incidentaloma who do not undergo adrenal surgery, for those with bilateral incidentalomas, for patients with extra-adrenal malignancy and adrenal masses and for young and elderly patients with adrenal inciden...

show abstract

Section: Reasoningmentioning

confidence: 99%

Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors

Fassnacht

Arlt

Bancos

et al. 2016

European Journal of Endocrinology

1,339

1,571

View full text Add to dashboard Cite

show abstract

“…Problems related to wound infection, recovery time, and image acquisition are more frequent in conventional methods as compared to laparoscopy in obese patients (8,10). In the conventional procedure; transperitoneal, retroperitoneal, lateral transperitoneal (Chevron incision), lateral (extraperitoneal), posterior (extraperitoneal), thoraco-abdominal (transpleural/ transperitoneal) approaches are being used, and it is reported that a midline incision is more appropriate for bilateral adrenalectomy (9,26,27).…”

Section: Discussionmentioning

confidence: 99%

Surgical approach in adrenal incidentalomas: Report of thirteen cases and review of the literature

Erdem

Çetinkünar

Kuyucu

et al. 2016

UCD

View full text Add to dashboard Cite

Objective: The rate of adrenal incidentalomas detected in routine diagnostic imaging techniques is approximately 4-7%. Although the lesions are generally benign, carcinoma and functional adenomas can be diagnosed with careful clinic and laboratory evaluation.Material and Methods: Data of 13 patients who underwent surgery for an adrenal mass between January 2010-June 2014 were analyzed retrospectively.Results: Seven (54%) patients were male, 6 (46%) were female, and the mean age was 38.2. The clinical diagnosis was pheochromacytoma in 5 patients (38.4%), non-functional adenoma in 5 (38.4), and metastatic lesion, Cushing syndrome, and adrenal carcinoma each in one patient (7.6%). Conventional open adrenalectomy was performed in 8 patients, while 5 patients underwent laparoscopic adrenalectomy. Conclusion:Adrenal incidentalomas should be carefully evaluated for hormonal activity even if asymptomatic, and non-functional lesions should be considered as suspicious-for-malignancy. Laparoscopic adrenalectomy has become the gold standard for patients with a mass less than 6 cm, and without infiltration to adjacent organs.

show abstract

“…Complete surgical resection with careful preoperative alpha-adrenergic blockade and subsequent beta-adrenergic blockade for any consequent tachyarrhythmia [26], in the presence of a functional pheochromocytoma, is the most appropriate therapy for CP with neuroblastoma. Prazosin is a commonly used selective alpha-1 adrenergic agonist, but requires frequent dosing due to a short half-life and its serum concentration may decrease to ineffective levels at the time of surgery if used preoperatively [27].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of a Composite Pheochromocytoma With Neuroblastoma

Steen

2014

J Endocrinol Metab

View full text Add to dashboard Cite

Composite pheochromocytoma (CP) is a rare tumor of the adrenal medulla, consisting of neuroendocrine and neural components. Despite similar neural crest origins, pheochromocytomas and neurogenic tumors are distinct entities. Symptoms may arise from hypersecretion of hormones by either component; however, not all patients present with classic symptoms. Moreover, various medical conditions and substances can confound screening tests and complicate the diagnosis. The patient, a 46-year-old male, was seen in the endocrine clinic regarding a 2-year history of paroxysmal headaches, palpitations and diaphoresis. His medical history was significant for hypothyroidism and substance misuse (nicotine, marijuana and cocaine). Family history was negative for pheochromocytoma, hyperparathyroidism or thyroid malignancies. He was found to be hypertensive in clinic, and two cafe-au-lait spots and bilateral axillary freckles were noted on dermatological examination. Pheochromocytoma was suspected and investigations revealed 24-h urinary catecholamines, metanephrines and vanillylmandelic acid levels that were substantially elevated. He was asked to repeat the urine collection while abstaining from cocaine, given the potential confounding with cocaine-induced elevations of catecholamines and their metabolites; however, the patient did not comply with this. His plasma free metanephrines and chromogranin A were later found to be elevated. Abdominal computed tomography revealed a 5.8 × 5 cm mass in the left adrenal gland, with an attenuation of 40 Hounsfield units. He underwent an uncomplicated laparoscopic left adrenalectomy, after which his blood pressure normalized. Histological features of the tumor revealed findings consistent with a typical pheochromocytoma with neuroblastomatous infiltrates comprising < 5% of the tumor. The mitosis karyorrhexis index was low and there was no ganglioneuromatous infiltrate evident. Medical oncology was consulted in view of the histopathological findings, and no adjuvant therapy was recommended. Unfortunately, he did not fully comply with repeat imaging or biochemical evaluation. Six months postoperatively, the patient remains completely asymptomatic and normotensive. Cocaine-induced catecholamine effects can clinically mimic pheochromocytoma, thereby complicating the diagnosis. In addition, the biologic behavior of CP with neuroblastoma is uncertain and warrants oncologic assessment and surveillance.

show abstract

Perioperative Management of Pheochromocytoma/Paraganglioma: Is There a State of the Art?

Cited by 27 publications

References 22 publications

Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors

Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors

Surgical approach in adrenal incidentalomas: Report of thirteen cases and review of the literature

An Unusual Case of a Composite Pheochromocytoma With Neuroblastoma

Contact Info

Product

Resources

About