Graves' disease allied with multiple pheochromocytoma.

Pheochromocytoma is an uncommon cause of high blood pressure touching adults. The combination of severe hypertension in the triad of headache, sweating, and tachycardia should suggest this diagnosis; this clinical picture is similar to that of hyperthyroidism. We report the case of a 22-year-old patient with multiple pheochromocytoma associated with Graves' disease revealed by malignant hypertension and discussed the difficulties of the diagnosis and the treatment approach.

INTRODUCTION

Pheochromocytoma is usually revealed by paroxysmal or permanent arterial hypertension (AHT) associated with a symptomatic triad of headache, sweating, and palpitations. While the prognosis is very good when the cause of the AHT is determined and the tumor is removed, pheochromocytoma can nevertheless be the cause of serious, even fatal complications, consequences of severe vasospastic attacks; a source of ischemia and organ necrosis. A necrotic pheochromocytoma can also be dramatically revealed during surgery or any invasive procedure or in association with a pathology underlying an accumulation of adverse effects.[1]

We report on the case of an association of pheochromocytoma and Graves’ disease revealed simultaneously by severe cardiovascular symptomatology and discussed the difficulties of its management.

CASE REPORT

A female patient aged 22 years with no cardiovascular history was admitted to the hospital for ATH of 225/150 mmHg. The patient had suffered from intense headaches, palpitations, sweating, excessive nervousness, psychomotor agitation, and mood disturbances, as well as rapid weight loss 10 days before going to the hospital.

Evaluation at admission showed a regular sinus tachycardia at 195 bpm demonstrated by electrocardiogram, a fever of 38.5°, exophthalmia, and a firm, homogeneous diffuse goiter with thrill. A whole-body CT scan showed five tissue and hypervascular masses of 26–30 mm in diameter, two bilateral adrenal, and three retropancreatic para-aortic masses [Figures 1 and 2]. Thyroid scintigraphy showed homogeneous increased uptake involving the whole thyroid gland. The highly probable diagnosis of pheochromocytoma was confirmed by the assay of urinary metabolites: normetanephrines = 112,126 nmol/24H (normal range: 300 to 2200), metanephrines = 1602 (normal range: 200 to 1500), and 3 orthomethyldopamine = 176,00 (normal range: 200 to 1400). Biological hyperthyroidism was evident with TSHus < 0.05 μUI/ml (normal range: 0.25 to 5), T3 = 24.37 pmol/l (normal range: 4 to 8.3), and T4 > 70 pmol/l (normal range: 9 to 20). Moreover, the biological workup showed glycemia at 6.60 μmol/l, urea at 7.83 μmol/l, serum creatinine at 74 μmol/l, and serum potassium at 3.8 mmol/l.

Figure 1

CT scan showing a multiple tissue and hypervascular masse: 30 mm in diameter in right perirenal space and 25 mm in left perirenal space

Figure 2

CT scan showing a retroperitoneal pheochromocytoma measuring 10 mm in retroperitoneal left space

Treatment in intensive care consisted of the simultaneous administration of nicardipine via electric pump syringe in decreasing doses of 4 mg/h, then 3, 2, and 1 mg/h of propranolol in intravenous bolus injections of 0.1 mg using an iterative technique, intravenous methylprednisolone dosed at 15 mg/kg and synthetic antithyroid drugs (carbimazole) at the dose of 60 mg/day, but no preoperative treatment preparation by (iodine) have been performed. Surgical excision of the tumors was done by laparotomy after 7 days and normalization of blood pressure and cardiac rate. It was conducted under general anesthesia using propofol, sufentanil, and cistracurium with invasive monitoring of arterial blood pressure and central venous pressure. The surgery was marked by several bouts of ATH and tachycardia, treated with a bolus of nicardipine and propranolol at 1 and 0.1 mg, respectively.

The surgical procedure consisted of the excision of five tumoral masses without adrenalectomy. The postoperative period was uncomplicated, allowing the discharge of the patient on the 5th postoperative day with no treatment beyond the synthetic antithyroid drug. The anatomic pathology examination confirmed the diagnosis of benign multiple pheochromocytoma.

DISCUSSION

Pheochromocytoma can appear at the occurrence of serious events. Acute cardiac failure, myocardial infarction with healthy arteries, cardiac rhythm disorders, acute pulmonary edema, lesional or hemodynamic, ischemic cerebral vascular accident (CVA), and other organ necroses make up the bulk of these events.[12] They are due to a massive release of catecholamines, especially by necrosis of an adrenal tumor.[3] The most severe expression of Graves’ disease is the acute thyrotoxic crisis, which may be triggered particularly during surgery. This explains the need to achieve a euthyroid state before any surgical procedure, especially one performed on the thyroid gland itself. The association of pheochromocytoma and Graves’ disease is exceptional.[4] It raises a three-fold problem of diagnosis, treatment, and prognosis. On the diagnostic level, the overlapping of symptoms involved for the most part in the two pathologies of hypercathecholaminemia can mislead the diagnosis of one or another of the two pathologies, while the outcome of one or the other may be fatal during any invasive procedure. Although the exophthalmia and observation of a goiter typical of Graves’ disease strongly point at hyperthyroidism, the absence of AHT can, on the other hand, make it difficult to diagnose pheochromocytoma. On the therapeutic level,[5] pheochromocytoma calls for prompt surgical intervention, given its serious potential complications and thus raises the problem of the timing of surgery, considering the hyperthyroidism and the risk of an acute thyrotoxic crisis. The contribution of corticoids combined with beta blockers and synthetic antithyroid drugs, in the context of rapid preparation for surgery, is of interest in this sense.[6] Last, on the prognostic level the accumulation of the essentially cardiovascular effects of the two pathologies, the difficulties of their simultaneous treatment, and their inherent potential risks are likely to worsen the prognosis. Consequently, management in an intensive care unit with continuous invasive monitoring seems necessary to ensure supervision under optimal conditions and surgery at the opportune time.

CONCLUSION

The association of pheochromocytoma and Graves’ disease brings with it the risk of nonrecognition of one or another of two pathologies with serious consequences and raises the problem of medicosurgical management of pheochromocytoma due to the relatively urgent nature of the latter and the consequent need to rapidly achieve a euthyroid state. In our case report, observation of exophthalmia and the hypertensive condition of a young patient facilitated the diagnosis of both pathologies, and rapid treatment in an intensive care unit allowed a favorable outcome in a short period of time.