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Original Article |
1 Endocrine and Diabetes Unit, Department of Medicine, University of Wuerzburg, Josef-Schneider-Str. 2, D-97080 Wuerzburg, Germany
2 Department of Dermatology, University of Wuerzburg, Josef-Schneider-Str. 2, D-97080, Wuerzburg, Germany
3 Department of Urology, University of Wuerzburg, Josef-Schneider-Str. 2, D-97080, Wuerzburg, Germany
4 Department of Radiology, University of Wuerzburg, Josef-Schneider-Str. 2, D-97080, Wuerzburg, Germany
5 Department of General Surgery, University of Wuerzburg, Josef-Schneider-Str. 2, D-97080, Wuerzburg, Germany
6 Department of Anaesthesia, University of Wuerzburg, Josef-Schneider-Str. 2, D-97080, Wuerzburg, Germany
Correspondence: Address correspondence and reprint requests to: Bruno Allolio; E-mail: allolio_b{at}klinik.uni-wuerzburg.de
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ABSTRACT |
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 TOP ABSTRACT INTRODUCTIONPATIENTS AND METHODSDISCUSSIONREFERENCES |
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Patients and methods: We report a case series of four patients with established or suspected malignant disease (melanoma, transitional cell carcinoma and prostate carcinoma, thyroid carcinoma, colorectal carcinoma) harboring an adrenal mass. None of these patients showed clinical symptoms indicative for a pheochromocytoma.
Results: Surgery unrelated to the adrenal lesion (n = 3) or biopsy of the adrenal mass (n = 1) was performed without prior endocrine work-up. Pronounced hemodynamic instability including hypertensive crisis was observed during surgery in all patients. In contrast, in the same patients preoperative 
-blockade with phenoxybenzamine and an increased awareness of the potential risks led to improved hemodynamic stability following adrenalectomy for pheochromocytoma.
Conclusion: Our series is a strong reminder of the risks associated with surgery in patients harboring an unsuspected pheochromocytoma and underscores the need to exclude a pheochromocytoma in all patients with an adrenal mass and without a definitive diagnosis of the mass, especially when they are scheduled for surgery or adrenal biopsy. Otherwise, life-threatening hypertensive crisis can be precipitated even in the previously asymptomatic patient.
Key Words: Pheochromocytoma • Adrenal biopsy • Adrenal mass • Adrenalectomy • Hypertensive crisis
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONPATIENTS AND METHODSDISCUSSIONREFERENCES |
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Adrenal tumors belong to the most frequent types of human neoplasias. Although most of these tumors are benign adrenal adenomas, the adrenal gland is also a frequent site for metastasis. On total, only 19% of incidentally detected adrenal tumors are metastases.1 But the likelihood of an adrenal tumor to be a metastasis is clearly increased in patients with a known malignant disease, particularly in patients with lung cancer or melanoma, which frequently metastasize to the adrenal gland. However, even in patients with lung cancer 25% of adrenal masses are benign adrenal lesions.2 As up to 8% of incidentally detected adrenal masses are pheochromocytomas,1 it is therefore likely that a significant proportion of adrenal masses in patients with malignant disease consists of catecholamine-secreting tumors. While imaging procedures are very helpful to differentiate lipid-rich adrenocortical adenomas from adrenal metastases,3 it is well known that pheochromocytomas closely resemble malignant adrenal lesions in computed tomography (CT) or magnetic resonance imaging (MRI). Thus, in known malignant disease a lipid-poor adrenal lesion is often assumed to represent metastasized disease and no further diagnostic efforts are made to rule out a pheochromocytoma.
We here report a case series of four patients with established or suspected malignant disease and adrenal masses. Surgery unrelated to the adrenal lesion (n = 3) or biopsy of the adrenal mass (n = 1) was performed without prior diagnostic work-up for pheochromocytoma thereby creating a serious risk for the patients.
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PATIENTS AND METHODS |
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TOPABSTRACTINTRODUCTIONPATIENTS AND METHODSDISCUSSIONREFERENCES |
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). A pheochromocytoma was confirmed after surgery by elevated urinary catecholamine excretion (Table 2) and a positive I123-MIBG scintigraphy. After preoperative treatment with phenoxybenzamine (maximum dose: 60 mg/day) and bisoprolol, a right-sided open adrenalectomy and urethrectomy was performed safely in a second surgical approach with a maximum systolic blood pressure of 170 mmHg (Fig. 1). Pathohistological examination confirmed the diagnosis of a pheochromocytoma.
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Investigation after surgery revealed a pheochromocytoma with elevated urinary catecholamine excretion (Table 2
) and a high uptake of I123-MIBG in projection of the left adrenal tumor. The tumor in the right adrenal demonstrated high fat content in chemcial-shift MRI consistent with an adenoma and the further endocrine work-up was unremarkable (Table 2). Preoperative treatment with increasing doses of phenoxybenzamine (maximum dose: 30 mg/day) was begun and a left-sided transabdominal adrenalectomy was accomplished without complications. Maximum systolic blood pressure during surgery was 170 mmHg (Fig. 1). Pathohistological examination confirmed the diagnosis of a pheochromocytoma and the appearance of the right adrenal remained unchanged during follow-up.
Case 3
A 52-year-old man with goitre and cold nodules in thyroid scintigraphy was admitted for thyroidectomy because of suspicious fine-needle aspiration cytology. An abdominal ultrasound examination before surgery revealed bilateral adrenal masses (3.5 and 3.0 cm, respectively) but thyroid surgery was performed without any further endocrine investigation. Calcitonin was 3.7 pmol/L (<3.2) preoperatively.
During surgery systolic blood pressure rose to >360 mmHg. Pathohistological examination of the thyroid gland demonstrated adenomatous goitre with no evidence for a thyroid carcinoma or C-cell hyperplasia. The patient was admitted to our hospital for further investigation of the adrenal lesions. Urinary excretion of catecholamine metabolites was clearly increased (Table 2) and I123-MIBG-scintigra-phy demonstrated high uptake in the right adrenal mass with no accumulation of I123-MIBG elsewhere. The further endocrine work-up was unremarkable (Table 2) and the left lesion was suspected to be a hormonally inactive benign lesion according to its appearance in MRI studies. MEN II was excluded by mutational analysis of the RET-protooncogene. Therefore, right-sided unilateral laparoscopic adrenalectomy was performed after pretreatment with phenoxybenzamine (up to a dose of 95 mg/day) without major hypertensive or hypotensive episodes. After a first postoperative visit the patient was lost to follow-up.
Case 4
A 50-year-old normotensive man was seen for a scheduled follow-up 10 years after excision of a superficial spreading melanoma. Routine abdominal ultrasound examination revealed a left-sided adrenal mass (5 x 5 cm). Based on the assessment of CT scans, it was diagnosed as most likely malignant. Additionally, a CT scan of the thorax showed numerous small bilateral pulmonary nodules (<0.5 cm). To further establish the diagnosis of a metastasized melanoma a CT-assisted biopsy of the adrenal mass was performed (Fig. 2). All melanoma-specific markers were negative, but a definitive diagnosis could not be made based on the specimen. Only prior to a second biopsy a consultant endocrinologist was involved. He strongly recommended determination of urine catecholamine excretion prior to biopsy. Highly elevated urinary catecholamines (Table 2) established the diagnosis of a pheochromocytoma. An I123-MIBG scan was positive for the adrenal lesion, without evidence for pulmonary metastasis. After pretreatment with phenoxybenzamine (maximum dose 40 mg/day) a laparoscopic adrenalectomy was performed. The diagnosis was confirmed by histopathology.
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONPATIENTS AND METHODSDISCUSSIONREFERENCES |
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Platts et al.4 have reported that anesthesia and surgery were the cause of death in 16 out of 62 patients with occult pheochromocytoma. In addition, Primhak has described sudden death after minor abdominal trauma in a patient with occult pheochromocytoma.5 However, in our series the adrenal lesions had been known before surgery and a diagnostic work-up following generally accepted guidelines6–9 would have established the diagnosis. Thus even in case of a known malignancy it is mandatory to rule out a pheochromocytoma in the presence of an adrenal tumor, particularly prior to surgical procedures. As none of our patients had clinical symptoms suggestive for catecholamine excess, the latter recommendation obviously holds true also for asymptomatic patients with an adrenal mass. For analysis, measuring the excretion of catecholamines and meta- and normetanephrines in a 24-h urine sample or measuring plasma metanephrines in a blood sample10 are both established methods, but repeated measurements (e.g., up to three times) are recommended to rule out a pheochromocytoma. In this series, all pheochromocytomas were visualized by MIBG-scintigraphy. But the sensitivity of MIBG-scintigraphy is only 80–90%11,12 and, therefore, it is not possible to exclude a pheochromocytoma by a negative MIBG-scintigraphy. Before an adrenal mass is removed surgically, a complete hormonal evaluation is necessary including investigation of hypercortisolism in all patients and aldosterone excess in patients with hypertension or hypokalemia.13
Hemodynamic instability during surgery for pheochromocytoma is clearly higher than during surgery for adrenocortical tumors14 and is in most instances a result of sudden catecholamine release from the tumor. This is mainly caused by mechanical manipulation of the tumor, which may have occurred inadvertently in our patients, in particular in cases 1 and 2, as the pheochromocytoma was in the vicinity of the surgical area. However, catecholamine release can be precipitated also by other events during anesthesia and surgery like induction of anesthesia, intubation of the trachea and removal of the tracheal tube,15 which could have been the triggering factor for the hypertensive crisis at the end of the first surgery in patient 3 (Fig. 1, Table 3).
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Anaesthesia for pheochromocytoma follows established rules.15 Both -blocking agents (e.g., urapidil) for hypertensive episodes and ß-blockers (e.g., esmolol) for tachycardia should be readily available during surgery for pheochromocytoma, whereas the use of these agents may be delayed, if pheochromocytoma is not suspected.
Moreover, to improve hemodynamic stability during surgery for pheochromocytoma, pretreatment with -blocking agents or alternatively calcium antagonists16 is generally recommended and is missing, if a pheochromocytoma is not considered prior surgery.
In one of our cases biopsy of the pheochromocytoma was performed. While in this patient the biopsy was tolerated without major side effects, it may be associated with hypertensive crisis and even death.17,18 Thus biopsy of a pheochromocytoma is contraindicated and biopsy of a lipid-poor adrenal lesion before ruling out a pheochromocytoma is highly dangerous.
In summary, our series underscores the need to rule out a pheochromocytoma in all patients harboring an adrenal mass and without a definitive diagnosis of the mass, in particular in those scheduled for surgery or adrenal biopsy. Otherwise life-threatening hypertensive crisis may occur, even in the previously asymptomatic patient.
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ACKNOWLEDGMENTS |
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Received for publication May 19, 2006. Accepted for publication July 7, 2006.
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REFERENCES |
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This article has been cited by other articles:
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  J. T. Adler, G. Y. Meyer-Rochow, H. Chen, D. E. Benn, B. G. Robinson, R. S. Sippel, and S. B. Sidhu Pheochromocytoma: Current Approaches and Future Directions Oncologist, July 1, 2008; 13(7): 779 - 793. [Abstract] [Full Text] [PDF]
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 C. C. Solorzano, J. I. Lew, S. M. Wilhelm, W. Sumner, W. Huang, W. Wu, R. Montano, D. Sleeman, and R. A. Prinz Outcomes of Pheochromocytoma Management in the Laparoscopic Era Ann. Surg. Oncol., October 1, 2007; 14(10): 3004 - 3010. [Abstract] [Full Text] [PDF]
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