Pheochromocytoma
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This document discusses the diagnosis and treatment of pheochromocytoma, a catecholamine-secreting tumor of the adrenal medulla. It outlines the clinical presentation of hypertension, headache, and diaphoresis. It then describes a case of an incidentaloma found on CT scan and the workup to diagnose pheochromocytoma. Preoperative care involves beta-blockers and alpha-blockers. Surgical removal can be done laparoscopically using either a retroperitoneal or transabdominal approach, with different pros and cons for each. Postoperative care focuses on blood pressure management and long-term follow up to monitor for recurrence.
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Pheochromocytoma
- 1. Pheochromocytoma The zebra, not the horse
- 2. Outline • Epidemiology and clinical presentation • Surgical case • Preoperative care • Operative approaches • Intrapoerative considerations • Post-op care • Follow up
- 4. Presentation Hypertension Headache Diaphoresis Asymptomatic
- 5. Etiology Catecholamine secreting tumor from chromaffin and sympathetic ganglia of adrenal medulla • Sporadic • Familial • MEN-2 • Von-Hippel-Lindau
- 6. Case • 36M previously healthy, presents to ED with abdominal pains • ROS – some episodes of anxiety, chest pain, otherwise unremarkable • Investigations – Abdominal CT
- 7. Case
- 8. The incidentaloma Oshmyansky AR, et al. Serendipity in the diagnosis of pheochromocytoma. Journal of Computer Assissted Tomography. 2013 Sep;37(5):820-3. • 24h urine free cortisol • Fractionated urine catecholamines • Serum electrolytes • Plasma renin aldosterone Elevated urinary cortisol Hypokalemia, low-plasma renin, high plasma aldosterone High fractionated urine catecholamine levels Serum and urinary hormonal levels normal Cushing Syndrome Conn Syndrome Pheochromocytoma Nonfunctioning adrenal mass Endocrinology referral General Surgery referral No: Monitor growth with CT q6 months Adrenal mass found on imaging High attenuation with necrosis or calcification? Yes: Oncology referral
- 9. Pre-operative care • Beta-block • Propranolol • Alpha block • Phenoxybenzamine • Fluid correction/maintenance • High sodium diet • IV hydration
- 10. Operative approach Laparoscopic Open Retroperitoneal (RLA) Transabdominal (TLA) - Malignant disease - >8cm in diameter - Difficult access - Hypervascularity - Adhesiveness Pro Con Pro Con - No interference of intra-abdominal organs - Good approach for patients with abdominal adhesions 2˚ to prior surgeries - Restricted workspace - Limited trocar insertion space - Poor visibility - Good visibility and work cavity - Good mobility of instruments - Good anatomical landmarks - Risk of trauma to liver/pancreas Li, Li. Laparoscopic adrenalectomy in pheochromocytoma: Retroperitoneal approach versus transperitoneal approach. Journal of Endourology. 2010;24(9):1441-1445. Nigri, Rosman, Petrucciani, et al. Meta-analysis of trials comparing laparoscopic transperitoneal and retroperitoneal adrenalectomy. Surgery. 2013;153(1):111-119. RLA TLA
- 11. Intraoperative considerations Scholten A, Cisco R, Vriens M, Shen W, Duh Q. Variant adrenal venous anatomy in 546 laparoscopic adrenalectomies. JAMA Surgery. 2013;148(4):378-383 Clipping of adrenal vein: normal anatomy
- 12. Post-operative care • Discontinue antihypertensive meds • Manage postop hypotension/hypoglycemia via IV crystalloids +/- D5 infusion • 24h urine collection 2 weeks post op • Specimen to pathology • No histologic difference between benign vs. malignant
- 13. Outcomes • Recurrence risk is high, especially in familial syndromes or right sided tumors • Only 20% remain symptom free for at least 10 years • Long term follow up • 24h urine collection yearly for 10 years • Metastasis can present many years after resection
- 14. References • UpToDate – Clinical presentation and diagnosis of pheochromocytoma, May 2014 • Nigri, Rosman, Petrucciani, et al. Meta-analysis of trials comparing laparoscopic transperitoneal and retroperitoneal adrenalectomy. Surgery. 2013;153(1):111-119 • Scholten A, Cisco R, Vriens M, Shen W, Duh Q. Variant adrenal venous anatomy in 546 laparoscopic adrenalectomies. JAMA Surgery. 2013;148(4):378-383 • Li, Li. Laparoscopic adrenalectomy in pheochromocytoma: Retroperitoneal approach versus transperitoneal approach. Journal of Endourology. 2010;24(9):1441-1445
Editor's Notes
- HTN is the most common reason for visits to the GPs office, and about 20-30% of North Americas population is affected by HTN (>=140/90). The majority is diet, lifestyle and genetically caused, but there are about 0.2% of cases of HTN due to endocrine secreting tumours, such as pheochromocytoma catecholamine-secreting paragangliomas
- Classic triad of HTN, Headache, Diaphoresis Sustained or paroxysmal hypertension is the most common sign of pheochromocytoma, but approximately 5 to 15 percent of patients present with normal blood pressure. The frequency of normotension is higher in patients with adrenal incidentaloma or in those undergoing periodic screening for familial pheochromocytoma 5-15% can be normotensive Asymptomatic — With the more widespread use of computed imaging, an increasing number of pheochromocytoma patients have no symptoms and are diagnosed in the course of investigation of an adrenal incidentaloma or have one of the genetic forms of the disease (see "The adrenal incidentaloma"). At the Mayo Clinic, 15 of 150 patients with a pheochromocytoma were discovered serendipitously by abdominal computed tomography [7]. In a report of 41 patients, 20 (49 percent) had incidental imaging discovery of their pheochromocytoma [8]. At one institution, 19 of 33 patients (57.6 percent) with adrenal pheochromocytoma diagnosis from 1995 to 2002 were asymptomatic, and their adrenal tumors discovered incidentally on imaging done for other reasons [22]. Asymptomatic especially if familialy type of pheo such as in multiple endocrine neoplasia type 2 MEN-2
- Medullary thryroid carcinoma, pheo, hyperparathyroidism Signs and symptoms associated with VHL disease include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Conditions associated with VHL disease include angiomatosis, hemangioblastomas, pheochromocytoma, renal cell carcinoma, pancreatic cysts (pancreatic serous cystadenoma), endolymphatic sac tumor, and bilateral papillary cystadenomas of the epididymis (men) or broad ligament of the uterus (women).[5][6] Angiomatosis occurs in 37.2% of patients presenting with VHL disease and usually occurs in the retina. As a result, loss of vision is very common. However, other organs can be affected: strokes, heart attacks, and cardiovascular disease are common additional symptoms.[3] Approximately 40% of VHL disease presents with CNS hemangioblastomas and they are present in around 60-80%. Spinal hemangioblastomas are found in 13-59% of VHL disease and are specific because 80% are found in VHL disease.[7][8] Although all of these tumours are common in VHL disease, around half of cases present with only one tumour type.[8]
- 5x4cm mass
- RESULTS: In 40 (70.2%) of the 57 patients, an adrenal pheochromocytoma was detected in an imaging study performed without suspicion of an adrenal lesion. There were 13 chest computed tomography studies-8 to evaluate for possible pulmonary emboli. Other indications included abdominal pain or discomfort (n = 8), trauma (n = 3), abnormal liver function tests (n = 3), suspect renal artery stenosis (n = 3), hematuria (n = 2), colitis (n = 2), and 4 miscellaneous indications. If non resectable treat symptoms with beta block, alpha block, calcium channel block, chemotherapy Sensitivity was highest for plasma fractionated metanephrines (99 percent) followed by urinary fractionated metanephrines (97 percent), urinary catecholamines (86 percent), plasma catecholamines (84 percent), urinary total metanephrines (77 percent), and urinary VMA (64 percent). ● Specificity was highest for urinary VMA (95 percent) followed by urinary total metanephrines (93 percent), plasma fractionated metanephrines (89 percent), urinary catecholamines (88 percent), plasma catecholamines (81 percent), and lowest for urinary fractionated metanephrines (69 percent). ● Using receiver operating characteristic curves, sensitivity and specificity values at different upper reference limits were highest for plasma fractionated metanephrines. Despite the relatively low specificity, the authors concluded that plasma fractionated metanephrines were the best test for excluding or confirming pheochromocytoma and should be the diagnostic test of first choice.
- Propranolol to decrease HR, BP and contractions, alpha block to decrease BP Salt diet to counteract chatecolamin induced volume contraction increases free water excretion via alpha effect
- There are some single center studies that favour the retroperitoneal approach showing better outcomes, however a multicenter metanalysis shows no preferential outcomes
- This was via a single vein draining into the IVC on the right and draining into left renal vein on the left. Up to 20% of patients can have variant venous anatomy
- Adrenal hormones normalize in apprx. 1 week 10% can be malignant Postoperative hypotension can be avoided by adequate fluid replacement and hypoglycemia (which can occur in 10 to 15 percent of patients due to removal of catecholamine suppression of insulin secretion [50]) by glucose infusion. After tumor removal, catecholamine secretion should fall to normal in approximately one week.
- Surgical removal of a pheochromocytoma does not always lead to long-term cure of pheochromocytoma or hypertension, even in patients with a benign tumor. In one series of 176 patients, pheochromocytoma recurred in 29 (16 percent) and the recurrence was malignant in 15 of the 29 [51]. Recurrence was more likely in patients with familial pheochromocytoma or familial paraganglioma, right adrenal tumors, and extraadrenal tumors.