The Journal of Integrated Health Sciences

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1 Available online at Extra adrenal phaeochromocytoma (paraganglioma) Case Report 1* 2 3 Piryani R M, Piryani S, Khakurel M P 1 Professor of Clinical Medicine & Medical Education, Nepal Police Hospital & Nepal Police Medical College, Maharajganj, Kathmandu, Nepal. 2 MPH trainee, Institute of Medicine, Kathmandu. 3 Professor of Surgery, Nepal Police Hospital & Nepal Police Medical College, Maharajganj, Kathmandu, Nepal. ABSTRACT JIHS The Journal of Integrated Health Sciences Extra-adrenal pheochromocytoma (EAP) or paraganglioma is a rare tumor. We report the patient having abdominal EAP. A 30 years age female presented initially with headache, sweating and hypertension. Hypertension remained controlled with drugs but had paroxysm of sweating on few occasions. Urinary VMA was raised. Renal artery CT angiography and intravenous urography revealed large soft tissue mass in left para aortic region, medial to mid and lower pole of left kidney getting arterial twigs from abdominal aorta and anterior division of left internal iliac artery and compressing the left proximal ureter resulting in gross hydronephrosis of left kidney. Tumor and non functional left kidney were removed through laparotomy. Patient is under follow-up. BP and 24 hours urinary VMA were normal range on each visit. This tumor falls to aorticosympathetic group of EAP as per Glenner and Grimley classification. Keywords: Aorticosympathetic group of EAP, Extra-adrenal pheochromocytoma, Paraganglioma INTRODUCTION Pheochromocytoma is derived from chromaffin cells of adrenal gland and other sites. It is a rare tumor. This tumor is called as an extra-adrenal pheochromocytoma, or paraganglioma, when arises from other sites. The World Health Organization uses the term pheochromocytoma for tumors arising from chromaffin cells in the adrenal medulla while tumors arising from extra-adrenal sympathetic and parasympathetic paraganglia are classified as extra- [1-3] adrenal paragangliomas. Here we present a case of extra adrenal phaeochromocytoma or paraganglioma. In this article we use the term extra adrenal phaeochromocytoma (EAP). CASE REPORT A 30 years lady attended outpatient department (OPD) of gynecology & obstetrics (OBGY) of KIST Medical College Teaching Hospital (KISTMCTH) Lalitpur, Nepal, on September 7, 2010 with complaint of pain in lower abdomen increasing *Correspondence: r_piryani@yahoo.com in severity over period of some months. Also she had discharge per vagina, burning, frequency and urgency of micturition for last few months. She was non-alcoholic, non-vegetarian, and non diabetic. Her menstrual cycle was normal with non significant past and family history. She quitted smoking 4 years back. She was found to have raised blood pressure four years back and was taking tab amlodipine 5 mg and atenolol 50 mg daily for last four years. With suspicion of pelvic inflammatory disease, consultant at OBGY OPD prescribed quadruple therapy regimen- tab cefixim 400 mg stat, tab fluconazole 150 mg stat, tab tinidazole 2 g stat and tab azithromycin 1 g stat. She was referred to medical OPD for the management of hypertension. On review at medical OPD she revealed that she was found to have raised blood pressure The Journal of Integrated Health Sciences Vol 2 Issue 1 June

2 4 years back at village. Initially she had headache and sweating for about one week; for which she consulted local doctor and received anti hypertensive medication- tab amlodipine 5mg and atenolol 50mg daily. She took medication regularly. On few occasions she developed sweating during last four years. resulting in gross hydronephrosis of left kidney. The findings were suggestive of extra adrenal phaeochromocytoma (paraganglioma) (Figure 3 a-b). She was thin built and anxious looking. At the time of presentation her BP was 100/80 mm Hg, pulse 78 beats per minute, Temp 98 F, respiratory rate 16 breaths per minute and rest of general physical and systemic examination was unremarkable. No other obvious abnormality detected on comprehensive medical checkup. She was advised to continue anti hypertensive medication. Her fasting blood sugar was 103mg/dl, urea 26 mg/dl, creatinine 0.6mg/dl, sodium 142meq/L, and potassium 4.3meq/L. Her routine urinalysis revealed albumin ++, sugar nil, WBC 4-5/HPF, RBC nil, epithelial cells 2-3/HPF, cast and crystals nil. Lipid profile showed cholesterol 215mg/dl, HDL 38 mg/dl, LDL 140 mg/dl, and triglyceride 185 mg/dl. Figure 1 Doppler study of renal arteries showed normal duplex study of abdominal aorta and right renal artery, gross hydronephrosis of left kidney with thin rim of parenchyma. Doppler study of left renal artery was not possible due to gross hydronephrosis Chest x-ray PA view was apparently normal. ECG showed LVH, flat T wave in lead II, III, and avf. Echocardiography revealed concentric hypertrophy of left ventricle, mild aortic regurgitation, normal chambers and valves, no intra cardiac mass, thrombus or pericardial effusion, and no regional wall motion abnormality (RWMA). Her left ventricle (LV) systolic function was good (Ejection Fraction-EF 81%). Doppler study of renal arteries showed normal duplex study of abdominal aorta and right renal artery, and gross hydronephrosis of left kidney with thin rim of parenchyma. Doppler study of left renal artery was not possible due to gross hydronephrosis (Figure 1). Intravenous urography showed Left hydroureteronephrosis (Figure 2). CT Renal artery angiogram showed large intensely enhancing soft tissue attenuation mass in left para-aortic region, medial to mid and lower pole of left kidney with small necrotic areas, getting arterial twigs from abdominal aorta and anterior division of left internal iliac artery and compressing the left proximal ureter Figure 2 Intravenous urography showed left hydroureteronephrosis Her 24hours urinary protein was 250mg and 24hours urinary vanillylmandelic acid (VMA) 15.5mg (normal value < 13 mg). She was hospitalized for surgical Intervention. Her preoperative BP was controlled with tab prazocin The Journal of Integrated Health Sciences Vol 2 Issue 1 June

3 5mg and Atenolol 25 mg OD. She was operated. Tumor and left kidney was removed through laprotomy. She developed Pneumonia in postoperative period and treated with antibiotics. She recovered and discharged after 10 days. She was put on tab prazocin 2.5 mg OD and advised to visit OPD with 24 hours VMA report. Figure 4 Macroscopic view of tumor Figure 3 Figure 3.CT Renal artery angiogram showed large intensely enhancing soft tissue attenuation mass in left para aortic region, medial to mid and lower pole of left kidney with small necrotic areas, getting arterial twigs from abdominal aorta and anterior Histopathology of tissue biopsy reported tumor cells arranged in clusters separated by endothelial lined spaces. The cells are of different sizes and shapes. They contain fine granular eosinophilic cytoplasm. The nuclei are of round or oval in shape with prominent nucleoli. Findings are consistent with paraganglioma tumor. Left kidney showed chronic obstructive pyelonephritis (Figure 4,5). Figure 5 Histopathology of tumor - Cells are arranged in clusters separated by endothelial lined spaces. The cells are of different sizes and shapes. They contain fine granular eosinophilic cytoplasm. The nuclei are of round or oval in shape with prominent nucleoli After thorough work up it was concluded the tumor belongs to aorticosympatheticgroup of extra adrenal phaeochromocytoma or paraganglioma as per Glenner and Grimley classification. Her BP and 24 hours urinary VMA were normal checked at 2 and 4weeks postoperatively and The Journal of Integrated Health Sciences Vol 2 Issue 1 June

4 six monthly intterval. The last follow up visit was in September DISCUSSION Extra-adrenal pheochromocytomas (EAPs) may originate from any part of the paraganglion system but most commonly found in the abdomen. The traditional teaching says 10% of all pheochromocytomas are extra-adrenal, but current urology reports (2007) reveled that 15% of adult and [4] 30% of childhood pheochromocytomas are EAPs. Glenner and Grimley have divided the tumors into four groups based on their location: branchiomeric, intravagal, aorticosympathetic, and visceroautonomic. The branchiomeric and intravagal tumors are seldom active, arise in the head and neck, and usually stain negative for chromaffin. The aorticosympathetic group is mostly functional, usually chromaffin positive, seen along the course of aorta, between the renal arteries, around the iliac bifurcation, and includes the organ of Zuckerkandl. The visceroautonomic group occurs in association with blood vessels or visceral organs like the bladder. This is also functional and generally chromaffin positive. As per mentioned classification, our patient's tumor falls into the aorticosympathetic [4] group. EAP is most commonly seen in age group of years. Our patient was female of 30 years age. Most commonly patient presents with hypertension either episodic and/or sustained, headaches, sweating, palpitations, anxiety, and [4,5] tremors. Initially our patient presented with headache, sweating and hypertension. Hypertension remained controlled with antihypertensive drugs but she developed paroxysm of sweating on few occasions. Patient may also develop symptoms due to compression of adjacent structures by tumor. Yadav et al reported case of an EAP presenting with unilateral [4,6] ureteral obstruction. Tumor in our patient obstructed the left proximal ureter resulting in gross hydronephrosis of left kidney. The elevated level of catecholamines and their metabolites in the blood and urine confirm the diagnosis of EAPs. Plasma-free metanephrines and urinary fractionated metanephrines are highly sensitive (sensitivity 99% and 97%, respectively) than plasma catecholamines, urinary catecholamines, total urinary metanephrines, and urinary vanillylmandelic acid (sensitivity 84%, 86%, 77%, and 64%, respectively). Urinary vanillylmandelic acid (95%) and urinary total metanephrines are highly specific (specificity 95% and 93% respectively) compared with plasma-free metanephrines, urinary catecholamines, plasma cathecholamines, and urinary fractionated metanephrines (specificity 89%, [4,7] 89%, 81% and 69% respectively). Urinary vanillylmandelic acid was found to be raised in our patient. Imaging studies are required to know the [4,8] location/s, extent, and presence of metastasis. For this purpose Doppler study of renal arteries, renal artery CT angiography and intravenous urography were done in our patient. These tests showed large soft tissue mass in left para aortic region, medial to mid and lower pole of left kidney with small necrotic areas, getting arterial twigs from abdominal aorta and anterior division of left internal iliac artery and compressing the left proximal ureter resulting in gross hydronephrosis of left kidney. The laparoscopic technique for resection of EAPs is preferred over the conventional treatment- open exploration and resection. The complicated tumors [4, 9] are still removed through laparotomy. Our patients' tumor and non functional left kidney were resected through laparotomy. There is limited data regarding recurrence and survival rates for EAPs. Some studies reported local recurrence rates ranging from 0% to 7%. The two important items biochemical markers and blood pressure must be checked postoperatively every 2 weeks during first year and then annually. If cathecholamines are found to be increased, then search for recurrence or metastasis at all possible sites [4,10] must be made. Our patient is remained under follow up. Till last visit in September 2013, BP of our patient was normal and 24 hours urinary VAM was within normal range. The Journal of Integrated Health Sciences Vol 2 Issue 1 June

5 REFERENCES 1. Tischler A S. Pheochromocytoma and Extraadrenal Paraganglioma Arch Pathol Lab Med 2008; 132: Chen et al. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of euroendocrine tumors- pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas August 2010; 39 (6): Pacak et al. Pheochromocytoma: recommendations for clinical practice from the First International Symposium. Nature Clinical Practice Endocrinology & Metabolism 2007; 3 (2): Disick GIS, Palese MA. Extra-adrenal pheochromocytoma: diagnosis and management. Current Urology Reports 2007; 8: Yadav S, Singh SK, Sharma SK, et al.: Extraadrenal pheochromocytoma: a cause of ureteral obstruction. J Uro 2000; 163: Lenders J, Pacak K, Walther W, et al.: Biochemical diagnosis of pheochromocytomas: which test is best? JAMA 2002; 287: Brink I, Hoegerle S, Klisch J, et al.: Imaging of peochromocytoma and paraganglioma. Fam Cancer 2005; 4: Mithani SK, Marohn MR, Freischlag JA, et al. Laparoscop resection of a paraganglioma of the organ of Zuckerkandin- a patient with a carotid body tumor. Am Surg 2006; 72: Edstrom E, Hjelm S, Hoog A, et al. The management of benign and malignant pheochromocytoma and abdominalparaganglioma. Eur J Surg Oncol 2003; 29: Somani SH, Bhawani JB, Chibber PJ. Extraadrenal pheochromocytoma - a case report. Indian J Urol 2001; 17: For further details : The Journal of Integrated Health Sciences Vol 2 Issue 1 June