Professor and Head Professor Associate Professor Assistant Professor Postgraduate Dept. of Medicine Sree Balaji Medical College and Hospital Chennai Tamil Nadu Abstract A young individual presented as hypertensive emergency due to a rare cause a tumor arising from autonomic nervous system. Screening test done in this patient revealed a case of extra adrenal pheochromocytoma which was confirmed by imaging studies. Pheochromocytoma usually manifests itself with paroxysmal symptoms and can be easily missed therefore all young individuals with secondary hypertension must be screened for pheochromocytoma. This case presented clinically as recurrent cerebrovascular disease CVD . Keywords Hypertensive emergency pheochromocytoma secondary hypertension recurrent cerebrovascular disease Pheochromocytoma is a tumor of the sympathetic or parasympathetic nervous system and is one of the causes of secondary hypertension which is refractory to antihypertensive therapy. This is a curable cause of hypertension and its early detection and diagnosis is equally important. Case Report Chief Complaints Mr Suresh a 22 year old male was admitted to the hospital with chief complaints of a Inability to use both his left upper and lower limbs for 3 days b inability to speak c deviation of angle of mouth to the right side d double vision and e recurrent episodes of headache palpitation and sweating. There was history of similar episode 2 years ago. He also had history of one episode of seizure 7 years before. On examination his blood pressure BP was 220 120 mmHg and pulse rate was 120 min. Cardiovascular system respiratory rate and per abdomen examination revealed no abnormalities. On neurological examination he had left sided hemiplegia horizontal nystagmus with lateral gaze left sided 3rd nerve palsy right sided 6th nerve palsy and right sided lower motor neuron type of facial palsy and cerebellar signs. Investigations Blood Investigations Hemoglobin 13.2 g dL total leukocyte count 5 000 cells mm3 differential leukocyte count P62L34E4 erythrocyte sedimentation rate 16 mm hour. Urea 35 mg dL creatinine 1 mg dL Na 134.1 mEq L Cl 85.2 mEq dL K 3.1 mEq dL. Total cholesterol 138 mg dL triglycerides 119 mg dL high density lipoprotein 38 mg dL low density lipoprotein 76 mg dL very low density lipoprotein 24 mg dL. Cholesterol ratio 3.6. Antiphospholipid antibody 6.67 U mL. 24 hour urinary vanillylmandelic acid VMA 18.88 mg 24 hours normal value 6 8 mg dL in adults . ECG Sinus tachycardia electrical left atrial enlargement left ventricular hypertrophy. Chest X ray Normal MRI Abdomen Multiple relatively defined discrete lesions of varying sizes noted in the bilateral para aortic region extraadrenal pheochromocytoma shrunken right kidney. CT Brain Multiple infarcts. Course in Hospital He was admitted to the intensive care unit ICU with a BP of 220 120 mmHg and nitroglycerin NTG infusion was started to control his BP. There was a very irregular response. NTG infusion was gradually tapered and he was put on prazosin calcium channel blockers and angiotensin converting enzyme ACE inhibitors. Later he was put on phenoxybenzamine starting with 10 mg b.i.d. and increased to 20 mg t.i.d. After that a b blocker was added to control the tachycardia. He was advised low salt diet and adequate hydration. His BP was gradually reduced from 180 130 150 90 mmHg with episodes of wide fluctuations. After his BP was consistently below 160 90 mmHg for 2 weeks he was taken up for surgery. Laparotomy was done and multiple tumors were excised from the bilateral para aortic region. There was an intraoperative rise in the BP associated with handling of the tumors which was brought to control by NTG infusion. A small portion of the tumor near the left renal vein was left behind because of difficulty in removing it without damaging the left renal artery. Postoperatively the BP increased to 220 130 mmHg which was managed by NTG infusion and magnesium sulfate regime. His BP was stabilized to 130 100 mmHg within a week and was discharged with methyldopa 250 mg b.i.d. and vitamins. During follow up his BP was found to be within normal limits. Histopathological Examination Specimen Extra adrenal pheochromocytoma. Gross Several fragmented dark brown masses in aggregates of size 7 4 3 cm. The cut surface showed a dark yellow to orange nodular lesion with a thin rim at the periphery. Microscopy Revealed small nesting growth pattern zellballen appearance . Tumor cells were fairly uniform with finely granular cytoplasm. Diagnosis Extra adrenal mass excision pheochromocytoma. Discussion The patient was a young hypertensive who developed crossed hemiplegia due to brainstem lesion. Computed tomography CT brain showed multiple infarcts which were secondary to hypertension. Urinary VMA was elevated and magnetic resonance imaging MRI abdomen showed multiple extra adrenal masses. BP was refractory to antihypertensive therapy. Patient was put on a and b blockers ACE inhibitor methyldopa. Inspite of these drugs his BP did not come under control. Metaiodobenzylguanidine MIBG scan could not be done because the patient could not afford it. Bilateral multiple extra adrenal paraganglion masses were excised. In the postoperative period BP started to come down and on follow up his BP was normal. About 10 pheochromocytomas are extra adrenal and most of them are 10 cm in diameter. It manifests as hypertension which responds paradoxically to antihypertensive therapy. Patients have paroxysms during which they develop headache sweating and palpitations. Cardiac manifestations are supraventricular tachycardia ventricular arrhythmias angina and cardiomyopathy. Some patients present with acute respiratory distress syndrome. There are reports of pheochromocytomas presenting as panic attacks pre eclampsia impaired glucose tolerance and pulmonary edema. Biochemical assessment are done for urinary VMA homovanillic acid fractionated metanephrines and catecholamines. Plasma catecholamines dopamine free metanephrines most sensitive and chromogranin A may also be assayed. MRI CT of the abdomen and MIBG scan help to localize the tumor. Patients should also be evaluated for other associated syndromes like multiple endocrine neoplasia 2A 2B Von Hippel Landau syndrome neurofibromatosis familial paraganglioma familial pheochromocytoma with islet cell tumor Sturge Weber syndrome ataxia telangiectasia tuberous sclerosis and Carney s triad. Conclusion This case is being presented as a young hypertensive with recurrent cerebrovascular accident CVA and seizures who had resistant hypertension not amenable to antihypertensive therapy given in high dosage in different combinations. Urinary VMA was positive and further investigations confirmed pheochromocytoma. Surgical removal brought complete cure to the patient. Pheochromocytoma presenting as recurrent CVA is a very rare and unusual manifestation of the tumor. Suggested Reading 1. Stein PP Black HR. A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution s experience. Medicine Baltimore . 1991 70 1 46 66. 2. Adler JT Meyer Rochow GY Chen H Benn DE Robinson BG Sippal RS et al. Pheochromocytoma current approaches and future directions. Oncologist. 2008 13 7 779 93. 3. Luo A Guo X Ren H Huang Y Ye T. Clinical features and anesthetic management of multiple endocrine neoplasia associated with pheochromocytoma. Chin Med J Engl . 2003 116 2 208 11. 4. Pacak K Linehan WM Eisenhofer G Walther MM Goldstein DS. Recent advances in genetics diagnosis localization and treatment of pheochromocytoma. Ann Intern Med. 2001 134 4 315 29. 5. Menger WM Gifford RW Jr Eds. . Clinical and Experimental Pheochromocytoma. 2nd Edition. Cambridge MA Blackwell Science 1996. 6. Sandur S Dasgupta A Shapiro JL Arroliga AC Mehta AC. Thoracic involvement with pheochromocytoma a review. Chest. 1999 115 2 511 21. 7. Whalen RK Althausen AF Daniels GH. Extra adrenal pheochromocytoma. J Urol. 1992 147 1 1 10. 8. Plouin PF Chatellier G Fofol I Corvol P. Tumor recurrence and hypertension persistence after successful pheochromocytoma operation. Hypertension. 1997 29 5 1133 9. 9. Scott HW Jr Halter SA. Oncologic aspects of pheochromocytoma the importance of follow up. Surgery. 1984 96 6 1061 6. 10. d Herbomez M Gouze V Huglo D Nocaudie M Pattou F Proye C et al. Chromogranin A assay and 131 I MIBG scintigraphy for diagnosis and follow up of pheochromocytoma. J Nucl Med. 2001 42 7 993 7.