Not all cases of severe headaches originate in the head. Some patients may present with episodes of severe headaches associated with sweating and flushing. An endocrine cause must be suspected in these cases as it may diagnose a treatable condition as in this 56 years old gentleman!

He presented with severe unrelenting headache of abrupt onset with profuse sweating. Blood pressure at admission in ER was 230/140 mmHg. Serum Troponin was high and a cardiac emergency was suspected and ruled out after a coronary angiography. MR imaging of Brain was normal. MR imaging of abdomen confirmed a suprarenal mass suggestive of Phaeochromocytoma.

Phaeo-02

 

Phaeo-01

Phaeochromocytomas are rare tumours that occur most frequently in individuals in their fourth to sixth decades of life, and occur more often in females than in males.  Phaeochromocytoma is an interesting clinical entity with a wide spectrum of pathological and clinical manifestations.

Clinicians should be alert to the characteristic presentations of phaeochromocytoma, including typical or atypical paroxysmal episodes. The triad of hypertension, headaches and sweating is present in some patients. Hypertension is the clinical hallmark of phaeochromocytoma and is present in 90–100% of patients. When hypertension is absent from this triad of symptoms, the diagnosis of phaeochromocytoma is quite unlikely. Of all patients with phaeochromocytoma, sustained hypertension is seen in about half, paroxysmal hypertension in a third and normal blood pressure in less than a fifth of patients

The diagnosis of phaeochromocytoma is confirmed by chemical methods, and located using imaging techniques, with computed tomography, magnetic resonance imaging and 131I-meta-iodobenzyl guanidine radioisotope scanning being the most common.

Phaeochromocytoma may be either sporadic or part of a familial cancer syndrome. Mean age at presentation for sporadic phaeochromocytoma is a decade older in the fifth decade compared to familial phaeochromocytoma. The most common clinical feature and the presenting feature in cases of sporadic phaeochromocytoma is hypertension compared to only 35% presented with hypertension. Levels of adrenaline, noradrenaline, dopamine and metanephrines in a 24-hour urine connection confirm the diagnosis. Computed tomography located 100% of the sporadic and three- quarters of the familial phaeochromocytomas. All the MEN 2A phaeochromocytomas are bilateral compared with none of the sporadic phaeochromocytomas. All the patients with familial phaeochromocytoma require bilateral adrenalectomy. The patients with sporadic phaeochromocytoma are operated with removal of the affected gland or the tumour, if extraadrenal, is removed.