|Year : 2013 | Volume
| Issue : 3 | Page : 241-243
Intra-renal paraganglioma: An unusual location and presentation
Santosh Kumar, Gautam Ram Choudhary, AS Sri Harsha, Manish Choudhary
Department of Urology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||28-Mar-2014|
Department of Urology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
Extra adrenal pheochromocytomas (Paragangliomas) are rare neuroendocrine tumors that may arise anywhere along the sympathetic and parasympathetic chain. Although these tumors are similar in origin to adrenal pheochromocytoma, the clinical manifestation, prognosis, and management differ. Surgical management of pheochromocytoma needs special perioperative requirements. Rarely patient may present with emergency symptoms may not allow to proceed evaluation, in these scenario clinician need to have high index of suspicion. We are presenting an atypical presentation of intra-renal location of paraganglioma which was diagnosed intraoperative period and could be managed without any complication.
Keywords: Hematuria, intrarenal, nephrectomy, paraganglioma
|How to cite this article:|
Kumar S, Choudhary GR, Sri Harsha A S, Choudhary M. Intra-renal paraganglioma: An unusual location and presentation. Arch Int Surg 2013;3:241-3
| Introduction|| |
Paragangliomas are extra-adrenal pheochromoctomas found along sympathetic and parasympathetic chain, many of them may not present with classical symptoms of excessive catecholamine secretion. They are very uncommonly present at an atypical site and with myriad of symptomatology and may not be diagnosed until the intra-operative period. This kind of presentation may have calamitous consequences. Intra-renal paraganglioma presenting with gross hematuria is a very rare presentation. We hereby present the diagnosis and treatment of extra-adrenal paragangioma in a 40-year-old woman.
| Case Report|| |
This was a case report of a 40-year female patient who was admitted to the emergency surgery department of our institute with gross hematuria and clot retention. She was having recurrent hematuria in the past 2 weeks and was evaluated elsewhere where ultrasound of the abdomen showed a large mass involving the left kidney. Magnetic resonance imaging (MRI) of abdomen done before she presented to us showed a mass of size 12 cm × 10 cm involving the upper pole of the left kidney with involvement of the pelvicalyceal system. Tumor was iso to hypo intense on T1 weighted images and heterogeneously hyper intense on T2 weighted images [Figure 1]a and b. She has been a diabetic and hypertensive for 10 years and had a cerebro-vascular accident (CVA) 1½ years back. Her blood pressure (BP) at presentation was 100/58 mmHg, pulse rate 98/min and she was pale. Abdominal examination revealed a firm non-tender mass measuring 10 cm × 10 cm in the left lumber region. The bladder and other abdominal viscera were not palpably enlarged. Her hemoglobin was 7.4 g%, whereas the white blood cells and platelet were normal. Serum creatinine was 0.9 mg/dL. Urine analysis showed red blood cells, pus cells 5-7/HPF, albumin + and sugar + . A provisional diagnosis of left renal cell carcinoma (RCC) was made. Following resuscitation she was taken for emergency nephrectomy because of continued gross hematuria. At operation, the mass was at the upper pole of the left kidney, with normal adrenal gland. During tumor manipulation, her BP shot up to 260/116 mmHg. A diagnosis of pheochromocytoma was considered and nitroglycerine infusion was started. Since the tumor was grossly in the kidney left nephrectomy was done along with ipsilateral adrenal gland. Cut section showed a well encapsulated yellow brown mass measuring 12 cm × 7 cm × 6 cm in the upper pole of left renal parenchyma and renal vein. Large areas of hemorrhage and necrosis within the tumor were also noted. Upper calyx and proximal part of renal vein was involved by the tumor [Figure 2]. Recovery was uneventful no hypotension occurred and BP normalized in the post-operative period and the patient was discharged on the 4 th post-operative day. Histopathology findings were, intra-renal tumor with peripherally compressed renal tissue observed mainly toward the renal hilum. There was no preserved renal parenchyma towards the upper pole. Tumor cells showed characteristic organoid pattern of arrangement with a moderate degree of anisonucleiosis [Figure 3], occasional mitosis and areas of necrosis. Intra-renal hilar fat was infiltrated by islands of tumor nodules. Renal vein showed tumor thrombus. Adrenal gland was grossly and microscopically of normal morphology.
|Figure 2: Gross left nephrectomy specimen showing the tumor involving the upper and mid pole, normal adrenal gland was being pointed with an arrow at the upper pole|
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|Figure 3: High power photomicrograph of the tumor to show a characteristic morphology of pheaochromocytoma (H and E, ×40)|
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| Discussion|| |
Among the renal masses, RCC is the most frequent. These tumors are usually hyper vascular and have a tendency to invade renal vein and inferior vena cava. Intra-renal pheochromocytoma with venous involvement have also been described in literature, but intra-renal location of paraganglioma presenting with hematuria secondary to PCS infiltration and renal vein thrombosis is very uncommon and difficult to diagnose pre-operatively without high index of suspicion along with hormonal evaluation and positron emission tomography (PET) imaging.  Most common cause of gross hematuria in the adult population is urothelial carcinoma, RCC and renal vein thrombosis.  The classic triad of pheochromocytoma presentation is episodic headache, sweating and palpitations. Persistent hypertension is frequently considered part of the presentation. The prevalence of an asymptomatic pheochromocytoma is estimated to be 21%. , Detailed retrospective interrogation along with direct questioning may sometime reveal the history of classical episodes of excessive catecholamine. Szumera et al. in their study have reported adrenal rests in kidney presenting as a simple renal cyst.  Simon et al. presented a rare case of intra-renal pheochromocytoma in a 27-year-old female with hypertension.  Our index patient exemplifies one of the rarest presentation of renal pheochromocytoma presenting with intermittent gross hematuria with fall in hemoglobin level necessitating an emergency nephrectomy. Because clinical examination and MRI located the tumor in the left kidney, a clinical diagnosis of RCC was made. However, on histological examination there ware neurosecretory granules, whose appearance is similar to those seen in other paraganglioma. This is probably the first case of intra-renal pheochromocytoma presenting with gross hematuria due to contiguous renal vein invasion along with PCS involvement.
Emergency presentation was one of the contributing factor, which led us to omit evaluation in the direction of upper tract urothelial malignancy and pheochromocytoma.
Nuclear imaging in combination with anatomic imaging may be required to fully delineate the extent of the paragangliomas, PET is a cornerstone in the evaluation of paragangliomas. The broad diversity of PET biomarkers enables assessment of different metabolic pathways that appear to be determined largely by the underlying genotypes and related tumor cell characteristics. Although 18F-fludeoxyglucose is the most accessible tracer several other agents have been used for PET scan including 82 rubidium, 11 C-hydroxyephedrine, 18 F-fluorodopamine and Ga-68DOTA-peptide. For metastatic work-up 123 I-metaiodobenzylguanidine ( 123 I-MIBG) is superior to 131 I-MIBG scintigraphy. Both have specificity of approximately 95%, but the sensitivity of 123 I-MIBG is higher (90% vs. 77%).
Pheochromocytoma surgery without preparation may be associated with a catastrophic outcome so any patient with a history of hypertension and upper polar renal mass should be viewed with suspicion and an adrenal lesion should be kept as a differential especially if there is history suggestive of hypertension, hyperglycemia and CVA.
| Conclusion|| |
An intra-renal mass in a hypertensive patient might be a pheochromocytoma. Appropriate perioperative precautions and preparation should be done to avoid catastrophic complications.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]