THE JOURNAL OF UROLOGY Copyright© 1977 by The Williams & Wilkins Co.

Vol. 118, November Printed in U.SA.




From the Department of Urology, Cleveland Clinic Foundation, Cleveland, Ohio


A case of a large pheochromocytoma weighing 1,150 gm. is reported. The surgical approach to this massive tumor is discussed. Early recognition of the various clinical and metabolic manifestations of the tumor are important objectives in the cure of this potentially lethal neoplasm. Pheochromocytoma was first described as a tumor in 1886 by Frankel. 1 However, it was not until 1912 when the nomenclature pheochromocytoma was established by Pick. 2 The first correct clinical diagnosis of paroxysmal hypertension was diagnosed by Vaquez and Donzelot in 1926. 3 During the last 4 decades the laboratory, biochemical and metabolic, as well as radiologic evaluation of the tumor has improved considerably. Nevertheless, the diagnosis is often overlooked because of the rarity of this condition; it is found in less than 1 per cent of hypertensive patients.

patients with hypertension pheochromocytoma was found in only 0.64 per cent, 4 yet it is calculated that 600 to 800 people a year in the United States die of undiagnosed and untreated pheochromocytomas. 5 In adults the clinical picture of paroxysmal hypertension is found in approximately 30 per cent of the individuals, persistent hypertension in 65 per cent, and headaches, sweating and palpitations in approximately 70 per cent. 6 Physical examination is often normal and in the absence of severe paroxysms of hypertension, headaches, sweating and palpitations the diagnosis of pheochromocytomas may be missed. CASE REPORT Large pheochromocytomas are often diagnosed on IVP by displacement of the upper pole of the kidney inferiorly and A 53-year-old white man was evaluated by his local medical laterally. Small tumors require adrenal angiography and doctor for obstructive voiding symptoms and a 5-year history venography for more accurate delineation. In essence only 3 of mild hypertension. A routine excretory urogram (IVP) per cent of the patients with pheochromocytomas will be revealed a right suprarenal mass and renal angiography missed by the combination of vanillyl mandelic acid and further delineated this as a 17 cm. adrenal mass (fig. 1). metanephrines, and even in these patients one or both of the The mild hypertension was under adequate control with tests will be in the upper range of normal.7 antihypertensive medication. Further studies included vaIn 1 review of 47 pheochromocytomas 15 silent tumors were nillyl mandelic acid and metanephrines, which were elevated. reported. There were no alarming symptoms present and However, the catecholamines and plasma cortisol levels were diagnosis was made at operation or at autopsy. The lack of within normal range. The effective thyroid ratio also was clinical symptoms is puzzling because patients with these normal as well as the creatinine clearance. Screening spiromsilent tumors often have high assays of catecholamines, which etry showed a moderate obstructed lung disease with a conare released only with operative manipulation. 8 tracted blood volume of 5,910 ml. Successful operation of pheochromocytomas is enhanced When the patient was hospitalized blood pressure, without greatly by special preoperative and intraoperative care. Intraantihypertensive medication, ranged between 195/100 to 180/ operative manipulation of the tumor will often result in 95. The patient admitted to several recent episodes of dizziness release of large amounts of catecholamines and the necessary and diaphoresis, especially when exposed to heat and exertion, precautions should be taken, that is alpha-receptor and betabut he denied having these symptoms while at rest. In the adrenergic blocking agents. Removal of the tumor is often absence of heart disease, with a contracted blood volume and followed by a hypotensive crisis owing to the elimination of with the suspicion of a pheochromocytoma the patient received chronic catecholamine stimulation of the peripheral arterioles a preoperative transfusion of 2 units. Exploration was done in a volume-depleted patient. 7 Preoperative transfusions can through a 9th intercostal thoracoabdominal incision that was correct the relative contracted blood volume and prevent a extended across the midline of the abdomen. A giant mass hypotensive crisis. 9 was seen on the right side, displacing the kidney medially and Wide exposure is necessary in patients with pheochromocyinferiorly (fig. 2). A nephroadrenalectomy was performed. The tomas. The thoracoabdominal approach appears to be ideally blood pressure remained stable throughout the procedure suited for massive upper pole tumors and for high retrocaval and convalescence was uneventful. The pheochromocytoma lesions. 10 This approach was first advocated in 1949 by Chute weighed 1,150 gm. (fig. 3). and associates for removal of large renal masses. 11 It is especially important since the possibility of carcinoma must DISCUSSION be considered and a wide exposure is mandatory to permit The incidence of pheochromocytoma as a cause of hyperten- radical en bloc non-manipulative resection of the tumor with sion varies between 0.4 to 2 per cent. In a series of 7,993 its ipsilateral kidney. It allows the surgeon to enter the chest and palpate the thoracic sympathetic chain in cases when the pheochromocytoma may be encountered. It also gives access Accepted for publication June 24, 1977. *Current address: 1643 BriarcliffRd., #4, Atlanta, Georgia 30306. to the abdominal cavity to rule out an extra-adrenal subdia840



FIG. 3. Cytoplasm is composed of fine granular jelly-like material. Caps~le is still intact with no microscopic evidence of vascular mvas10n. Some areas of dark cytoplasm and basophilic granular cell type are seen. Reduced from >

A case of a giant pheochromocytoma.

THE JOURNAL OF UROLOGY Copyright© 1977 by The Williams & Wilkins Co. Vol. 118, November Printed in U.SA. Single Case Reports A CASE OF A GIANT PHEOC...
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