Clinical Spectrum of Pheochromocytoma

Guerrero, Marlon A.; Schreinemakers, Jennifer M. J.; Vriens, Menno R.; Suh, Insoo; Hwang, Jimmy J.; Shen, Wen T.; Gosnell, Jessica E.; Clark, Orlo H.; Duh, Quan Yang

doi:10.1016/j.jamcollsurg.2009.09.022

Cited by 155 publications

(158 citation statements)

References 11 publications

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“…Metachronous metastases occurred on average 49.4 months (range, 5.9-194.8 months) postoperatively. The locations of recurrent disease were liver (6), bone (4), lung and pleura (4), and bladder (1). Patients were diagnosed with pheochromocytoma, either benign or malignant, within 0.02 months to 7.2 years (median, 1.8 years) from the onset of symptoms.…”

Section: Resultsmentioning

confidence: 99%

“…Pheochromocytoma is a catecholamine-secreting tumor that emerges from the chromaffin cells of the adrenal medulla, usually manifesting as a benign tumor but occasionally becoming malignant [1,2]. Because the long-term prognosis of patients with malignant pheochromocytoma is unclear or poor, identification of factors associated with malignancy could aid in modifying follow-up plans and could enhance disease-specific survival.…”

Section: Introductionmentioning

confidence: 99%

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Predictive Characteristics of Malignant Pheochromocytoma

Park¹,

Park²,

Song³

et al. 2009

Journal of Urology

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Purpose:The prognosis of patients with malignant pheochromocytoma is poor, but the predictive factors are not well understood. We aimed to identify the clinical characteristics predictive of malignancy after initial surgical removal in patients with pheochromocytoma. Materials and Methods:We retrospectively reviewed the records of 152 patients diagnosed with pheochromocytoma, including 5 (3.3%) with metastasis at the time of the initial surgical excision and 12 (7.9%) who developed metastasis during follow-up. To determine the factors predictive of malignancy, we compared clinical, radiographical, and urinary chemical findings between patients with benign and malignant disease. Mean follow-up was 41.5 months (range, 0.9-298 months) after surgery. Results: Malignant tumors were significantly larger than benign tumors (11.1±4.0 cm vs. 6.2±3.4 cm, p＜0.001), and postoperative persistence of arterial hypertension was more frequent after removal of malignant than benign tumors (p=0.001). Among the 147 patients without metastatic disease at diagnosis, those who developed metastasis had significantly lower concentrations of urinary catecholamine metabolites per unit of tumor, including vanillylmandelic acid (1.2 vs. 3.7 mg/day/cm, p=0.049), epinephrine (4.5 vs. 168.9 μg/day/cm, p=0.008), and norepinephrine (13.1 vs. 121.8 mg/day/cm, p ＜0.001). The overall 5-year metastasis-free survival rate was 84.4% and was significantly higher in patients with smaller tumors (≤5.5 vs. ＞5.5 cm; 90.6% vs. 81.2%, p=0.025) and higher 24-hour secretion of vanillylmandelic acid (＞2.1 vs. ≤2.1 mg/ day/cm; 94.9% vs. 70.9%, p=0.019). Conclusions: Large tumor size (＞5.5 cm) and minimally elevated 24-hour urinary vanillylmandelic acid (≤2.1 mg/day/cm) were significantly associated with a higher probability of a malignant pheochromocytoma portending a lower metastasis-free survival and mandating more rigorous follow-up after surgery.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Predictive Characteristics of Malignant Pheochromocytoma

Park¹,

Park²,

Song³

et al. 2009

Journal of Urology

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“…Pheochromocytoma symptoms result from the uncontrolled release of catecholamines: norepinephrine, epinephrine, and rarely dopamine (1)(2)(3)(4)(5). Compared with norepinephrine release, epinephrine has a predominant effect on b2-adrenoreceptors of the vessel muscles resulting in vasodilation and postural hypotension (1,2,5).…”

mentioning

confidence: 99%

“…Compared with norepinephrine release, epinephrine has a predominant effect on b2-adrenoreceptors of the vessel muscles resulting in vasodilation and postural hypotension (1,2,5). Norepinephrine release acts predominantly on b1-and a1-adrenoreceptors resulting in vasoconstriction and hypertension (1,2,5).…”

mentioning

confidence: 99%

“…Norepinephrine release acts predominantly on b1-and a1-adrenoreceptors resulting in vasoconstriction and hypertension (1,2,5). The release of both catecholamine types stimulates b1-adrenoreceptors resulting in an increased heart rate (1,2,5). Elevated blood glucose level is primarily due to stimulation of lipolysis and inhibition of glucose uptake in muscle cells and also to glycogenolysis and gluconeogenesis in the liver by epinephrine (1,6,7).…”

mentioning

confidence: 99%

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Point of controversy: perioperative care of patients undergoing pheochromocytoma removal–time for a reappraisal?

Lentschener¹,

Gaujoux²,

Tesnière³

et al. 2011

European Journal of Endocrinology

122

143

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Adrenalectomy for pheochromocytoma is reported with a mortality close to zero in recent studies. The dogma of preoperative fluid and hypotensive drug administrations is widely applied in patients scheduled for pheochromocytoma removal and is assumed to have a beneficial effect on operative outcomes. This paradigm is only based on historical studies of non-standardized practices and criteria for efficacy, with no control group. Pre-and intraoperative hypovolemia have never been demonstrated in patients scheduled for pheochromocytoma removal. Recent improvements in outcome of patients undergoing adrenalectomy for pheochromocytoma could also be the result of improvement in surgical techniques and refinement in anesthetic practices. Whether better knowledge of the disease, efficiency of available intravenous short-acting vasoactive drugs, and careful intraoperative handling of the tumor make it possible to omit preoperative preparation in most patients scheduled for pheochromocytoma removal is presently questionable. We reviewed available literature in this respect.

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