Tuesday, December 18, 2018

The clinical spectrum of pheochromocytoma

It may not be what you think. Most did not have the triad in this report.

From the article:

Of the 92 cases included 64% had presented as an incidentaloma, 32% as a suspected pheochromocytoma and 4% had been screened because of previously diagnosed MEN2A. Those screened were youngest while those with incidentalomas were oldest. The females were more common in the incidentaloma and the screening groups, and males in the suspected pheochromocytoma group. Measurements of noradrenaline/normetanephrine levels were highest in the suspected pheocromocytoma group and lowest in the screening group. Hypertension was present in 63% of the incidentalomas, 79% of suspected pheochromocytomas and in none of the screening group. Paroxysmal symptoms were present in almost all with suspected pheochromocytoma while only in half of the other groups. The suspected pheocromocytoma group had most symptoms and the screening group least. The classic triad was present in 14% of the incidentalomas, in 28% of the suspected and in none of the screening group, while no symptoms at all was present in 12%, 0% and 25%, respectively. Pheochromocytoma crisis occurred in 5%. There was a positive correlation between tumor size vs hormone levels, and catecholamine levels vs blood pressure…

This large modern study confirms the wide spectrum of presentations of pheochromocytomas but also that hypertension and typical symptoms may not be present at all. The predominant presentation was serendipitously in the workup of an incidentaloma. Even though the patients with incidentalomas had not sought medical attention for pheochromocytoma-related symptoms, in hindsight, 88% had symptoms, which could be related to this disorder. Those screened had very few symptoms while those found in the workup of suspected pheochromocytoma had most symptoms. The classic triad, i.e., headaches, sweating and palpitation, was only present in a minority of patients.

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