Malignant pheochromocytoma: pain, palpitation, perspiration and perplexities

Background

Pheochromocytoma is a rare disease, which is mainly sporadic but also associated with some familial disorders such as Von Hippel-Lindau syndrome, Von Recklinghausen’s disease, Sturge-Weber syndrome, Multiple Endocrine Neoplasia type 2A and 2B. The first case of pheochromocytoma was reported by Felix Frenkel in a woman who suffered from intermittent attacks of palpitation, anxiety, headaches and vertigo in 1886. Autopsy of the adrenal glands revealed brown colour on being treated with chromium salts. Hence, the name pheochromocytoma (Greek phaios, dusky coloured tumour). Malignancy accounts for only 10% of the cases approximately. They arise from the chromaffin cells of the adrenal medulla. They are also known as tumour of 10 because 10% are bilateral, 10% are malignant, 10% are extra adrenal, 10% are familial and 10% occur in paediatric population. Most pheochromocytomas produce both epinephrine and norepinephrine. Increased production of dopamine and homovanillic acid is usually seen in malignant lesions.1 People with germline succinate dehydrogenase complex subunit B mutation appear to have higher propensity for extra adrenal and malignant lesions.2 To date, there is no definitive criteria to diagnose malignant pheochromocytoma. The main treatment is surgery. Other treatment options suggested are chemotherapy and radionuclide therapy. Here, we share our experience with one such rare case alongside the challenges that we faced at each level.

Case presentation

A 60-year-old man presented to our casualty with headache, giddiness, palpitation and lower abdominal pain for 2 days. Patient was evaluated outside for the same and identified to have high blood pressure (BP), which did not settle with antihypertensive medication. ECG showed non-ST elevation myocardial infarction changes for which he underwent a coronary angiogram and it was reported to be normal. On examination, patient had flushing of his face associated with increased sweating. His pulse was 110 beats/min and BP was 160/90 mm Hg. His general systemic examination was normal.

Investigations

The routine blood investigations revealed elevated blood sugars, blood urea nitrogen, creatinine and troponin T. CT of the abdomen showed a well-defined lobulated iso-hyperdense heterogeneous mass lesion of size 7.1×5.6×8.2 cm (AP×TRANS×CC) involving the left suprarenal region with focal area of calcification, perilesional fat stranding and loss of fat plane with the left kidney (figure 1). CT of the chest showed no evidence of any other mass. Twenty-four-hour serum metanephrine, urine metanephrines and normetanephrines were sent for confirmation of the diagnosis and were reported as serum metanephrines >3600 pg/ml (normal value <65 pg/ml), urine metanephrines 3.3 mg/24 hours (normal value upto 1 mg/24 hours) and urine normetanephrines 5854.8 mcg/24 hours (normal value <600 mcg/24 hours), respectively.

Treatment

In the ward, the patient recorded a BP of 220/120 mm Hg, pulse of 120 beats/min following which he had an episode of seizure-like activity and became unconscious. In view of pheochromocytoma crisis, the patient was shifted to the intensive care unit (ICU), where he had an episode of postural hypotension recording a BP of 80/52 mm Hg (figure 2). Initially, the patient was resuscitated with fluids and inotropes for the hypotension. Once the BPs plummeted, tab prazosin 5 mg was started. The patient’s BP stabilised over 2 weeks and later he was taken up for laparoscopic adrenalectomy. Intraoperatively initially, there was severe hypertension, which was managed with nitroglycerin (0.05 mcg—5 mcg/min) infusion and boluses of diltiazem (0.25 mg/kg), metoprolol (2.5–5 mg) and esmolol (0.5–1 mg/kg). After the adrenal vein (figure 3) was clipped, there was severe hypotension, which was managed using norepinephrine infusion (0.05–0.5 mcg/kg/min).

The specimen was sent for histopathology. The gross specimen received weighed 189 g measuring 9.5×6×2.5 cm with attached fibro fatty tissue measuring 6×3.5×2 cm (figure 4A). The cut surface was gray-brown, well circumscribed and homogeneous (figure 4B). The encapsulated lesion measured 9×6×2.5 cm with adjacent normal parenchyma measuring 0.5×0.5×0.5 cm. No capsular breech was identified. Histopathological examination was suggestive of a malignant pheochromocytoma (figure 5A–C) with a Pheochromocytoma Adrenal Scaled Score (PASS) of 10/20 (table 1).

Outcome and follow-up

Postoperatively, the patient was shifted to the ICU and slowly weaned off the inotropes. His surgical scars healed well by primary intension and blood pressures were stable. In view of the suggested malignancy, iodine 131 labelled metaiodobenzylguanidine (MIBG) whole body scan was done and it showed no evidence of residual tumour. The patient is currently fine and in follow-up.