Kaohsiung Journal of Medical Sciences (2014) 30, 319e320
Available online at www.sciencedirect.com
ScienceDirect journal homepage: http://www.kjms-online.com
LETTER TO THE EDITOR
Paraganglioma in the renal pelvis
Dear Editor, Paraganglioma is a rare tumor originating from the primitive neural crest, and can develop at many locations, such as the abdomen, aortic bifurcation, neck, pelvis, and thorax. However, the renal pelvis is an extremely rare location for paragangliomas to occur. Here, we report a case initially suspected to be a renal mass in a 59-year-old patient with paroxysmal hypertension, heart palpitation, and sweating. However, the mass was pathologically confirmed to be a paraganglioma in the renal pelvis. This report describes its rarity as well as its clinical and therapeutic characteristics. The patient, a 59-year-old Chinese woman, had a 2-month history of paroxysmal hypertension, heart palpitation, and sweating. Her blood pressure measured 137/77 mmHg, and the remaining physical examination results were normal upon admission. Her blood metanephrine and normetanephrine levels were 322 ng/L and 632 ng/L, respectively, and other laboratory test results were normal. The left renal mass, measuring 4 cm in diameter, was detected by a computed tomographic (CT) scan without border differentiation, and enhanced CT results showed images of enhanced kidney only but not the mass. It was difficult, however, to determine the origin of the mass. The patient then underwent a preoperative preparation with a and b blockers for 2 weeks. During the surgery, we determined that both the mass and the left kidney had the same origin; therefore, radical excision of the left kidney and the tumor was performed. Paraganglioma in the renal pelvis was diagnosed pathologically, with Pan Cytokeratin (PCK) being negative, whereas synaptophysin, chromogranin A, and S-100 were positive immunohistochemically (Fig. 1). The patient’s postoperative recovery was without complications. During 3 months of follow-up, her
symptoms were controlled and blood catecholamine levels were within normal limits. Paragangliomas can be classified as either functional or nonfunctional, depending on catecholamine levels. Functional paragangliomas may be detected early if clinical presentations or excess catecholamine is recognized, whereas nonfunctional paragangliomas are difficult to identify preoperatively until they cause symptoms [1]. Preoperative a and b blockers are therefore necessary when paragangliomas are suspected. The primary symptoms of paragangliomas are headaches, hypertension, palpitation, and sweating. Catecholamine test results are required, whereas CT, magnetic resonance imaging, and ultrasonography can detect tumor locations and metastasis. Iodine-131-meta-iodobenzylguanidine scanning can also detect and treat paragangliomas. There are a few English language reports on renal hilar paragangliomas. Chandra et al. [2] reported one case of renal hilar paraganglioma complicated with renal artery stenosis. Ahallal et al. [3] reported a 58-year-old woman with renal hilar pheochromocytoma; and Bhandarkar et al. [4] reported two patients with renal hilar pheochromocytomas treated with laparoscopic resection successfully. Melegh et al. [5] reported a case of giant cystic pheochromocytoma in the renal hilum; however, there are few reports on renal pelvic paraganglioma. Radical removal of the kidney or tumor is the optimal surgical procedure to treat paragangliomas in the renal pelvis. The criteria of malignancy cannot be easily distinguished histologically, but a metastasis in follow-up is a sign of malignancy [3]. In conclusion, paraganglioma in the renal pelvis is extremely rare, and mass with hypertension or increased catecholamine levels should lead to suspicion of paraganglioma. We presented this case to demonstrate the
1607-551X/$36 Copyright ª 2013, Kaohsiung Medical University. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.kjms.2013.04.007
320
Letter to the Editor
Figure 1. (A) Hematoxylin and eosin staining (350); (B) immunohistostaining of chromogranin A (350); (C) immunohistostaining of S-100 (350); and (D) immunohistostaining of synaptophysin (350).
clinical and therapeutic characteristics of this extremely rare tumor.
[5] Melegh Z, Re ´nyi-Va ´mos F, Tanyay Z, Ko ¨ves I, Orosz Z. Giant cystic pheochromocytoma located in the renal hilus. Pathol Res Pract 2002;198:103e6.
References [1] Rossi G, Oleari G, Botti C, Torricelli P, Baccarini P, Barbolini G. Cystic paraganglioma of the renal capsule. J Urol 2001;165:511e2. [2] Chandra V, Thompson GB, Bower TC, Taler SJ. Renal artery stenosis and a functioning hilar paraganglioma: a rare cause of renovascular hypertensionda case report. Vasc Endovascular Surg 2004;38:385e90. [3] Ahallal Y, Tazi MF, Elfatemi H, Znati K, Tazi E, Amarti A, et al. Renal hilar pheochromocytoma: a case report. Cases J 2009;2: 6416. [4] Bhandarkar D, Katara A, Mittal G, Chauhan P, Chadha M. Laparoscopic management of renal hilar pheochromocytomas. Surg Laparosc Endosc Percutan Tech 2012;22:e148e51.
Hua-Wei Liu Liang-Ren Liu De-Hong Cao Qiang Wei* Department of Urology, West China Hospital, Sichuan University, Sichuan, PR China *Corresponding author. Department of Urology, West China Hospital, Sichuan University, Number 37, Guo Xue Xiang, Chengdu, Sichuan 610041, PR China. E-mail address: [email protected] (Q. Wei)
Available online at www.sciencedirect.com
ScienceDirect journal homepage: http://www.kjms-online.com
LETTER TO THE EDITOR
Paraganglioma in the renal pelvis
Dear Editor, Paraganglioma is a rare tumor originating from the primitive neural crest, and can develop at many locations, such as the abdomen, aortic bifurcation, neck, pelvis, and thorax. However, the renal pelvis is an extremely rare location for paragangliomas to occur. Here, we report a case initially suspected to be a renal mass in a 59-year-old patient with paroxysmal hypertension, heart palpitation, and sweating. However, the mass was pathologically confirmed to be a paraganglioma in the renal pelvis. This report describes its rarity as well as its clinical and therapeutic characteristics. The patient, a 59-year-old Chinese woman, had a 2-month history of paroxysmal hypertension, heart palpitation, and sweating. Her blood pressure measured 137/77 mmHg, and the remaining physical examination results were normal upon admission. Her blood metanephrine and normetanephrine levels were 322 ng/L and 632 ng/L, respectively, and other laboratory test results were normal. The left renal mass, measuring 4 cm in diameter, was detected by a computed tomographic (CT) scan without border differentiation, and enhanced CT results showed images of enhanced kidney only but not the mass. It was difficult, however, to determine the origin of the mass. The patient then underwent a preoperative preparation with a and b blockers for 2 weeks. During the surgery, we determined that both the mass and the left kidney had the same origin; therefore, radical excision of the left kidney and the tumor was performed. Paraganglioma in the renal pelvis was diagnosed pathologically, with Pan Cytokeratin (PCK) being negative, whereas synaptophysin, chromogranin A, and S-100 were positive immunohistochemically (Fig. 1). The patient’s postoperative recovery was without complications. During 3 months of follow-up, her
symptoms were controlled and blood catecholamine levels were within normal limits. Paragangliomas can be classified as either functional or nonfunctional, depending on catecholamine levels. Functional paragangliomas may be detected early if clinical presentations or excess catecholamine is recognized, whereas nonfunctional paragangliomas are difficult to identify preoperatively until they cause symptoms [1]. Preoperative a and b blockers are therefore necessary when paragangliomas are suspected. The primary symptoms of paragangliomas are headaches, hypertension, palpitation, and sweating. Catecholamine test results are required, whereas CT, magnetic resonance imaging, and ultrasonography can detect tumor locations and metastasis. Iodine-131-meta-iodobenzylguanidine scanning can also detect and treat paragangliomas. There are a few English language reports on renal hilar paragangliomas. Chandra et al. [2] reported one case of renal hilar paraganglioma complicated with renal artery stenosis. Ahallal et al. [3] reported a 58-year-old woman with renal hilar pheochromocytoma; and Bhandarkar et al. [4] reported two patients with renal hilar pheochromocytomas treated with laparoscopic resection successfully. Melegh et al. [5] reported a case of giant cystic pheochromocytoma in the renal hilum; however, there are few reports on renal pelvic paraganglioma. Radical removal of the kidney or tumor is the optimal surgical procedure to treat paragangliomas in the renal pelvis. The criteria of malignancy cannot be easily distinguished histologically, but a metastasis in follow-up is a sign of malignancy [3]. In conclusion, paraganglioma in the renal pelvis is extremely rare, and mass with hypertension or increased catecholamine levels should lead to suspicion of paraganglioma. We presented this case to demonstrate the
1607-551X/$36 Copyright ª 2013, Kaohsiung Medical University. Published by Elsevier Taiwan LLC. All rights reserved. http://dx.doi.org/10.1016/j.kjms.2013.04.007
320
Letter to the Editor
Figure 1. (A) Hematoxylin and eosin staining (350); (B) immunohistostaining of chromogranin A (350); (C) immunohistostaining of S-100 (350); and (D) immunohistostaining of synaptophysin (350).
clinical and therapeutic characteristics of this extremely rare tumor.
[5] Melegh Z, Re ´nyi-Va ´mos F, Tanyay Z, Ko ¨ves I, Orosz Z. Giant cystic pheochromocytoma located in the renal hilus. Pathol Res Pract 2002;198:103e6.
References [1] Rossi G, Oleari G, Botti C, Torricelli P, Baccarini P, Barbolini G. Cystic paraganglioma of the renal capsule. J Urol 2001;165:511e2. [2] Chandra V, Thompson GB, Bower TC, Taler SJ. Renal artery stenosis and a functioning hilar paraganglioma: a rare cause of renovascular hypertensionda case report. Vasc Endovascular Surg 2004;38:385e90. [3] Ahallal Y, Tazi MF, Elfatemi H, Znati K, Tazi E, Amarti A, et al. Renal hilar pheochromocytoma: a case report. Cases J 2009;2: 6416. [4] Bhandarkar D, Katara A, Mittal G, Chauhan P, Chadha M. Laparoscopic management of renal hilar pheochromocytomas. Surg Laparosc Endosc Percutan Tech 2012;22:e148e51.
Hua-Wei Liu Liang-Ren Liu De-Hong Cao Qiang Wei* Department of Urology, West China Hospital, Sichuan University, Sichuan, PR China *Corresponding author. Department of Urology, West China Hospital, Sichuan University, Number 37, Guo Xue Xiang, Chengdu, Sichuan 610041, PR China. E-mail address: [email protected] (Q. Wei)
