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Radical nephrectomy with en bloc resection of liver, diaphragm, and lung for locally invasive sarcomatoid renal cell carcinoma
CASE REPORT
RADICAL NEPHRECTOMY WITH EN BLOC RESECTION OF LIVER, DIAPHRAGM, AND LUNG FOR LOCALLY INVASIVE SARCOMATOID RENAL CELL CARCINOMA JAIME A. WONG, THOMAS WHELAN,
AND
MICHAEL MORSE
ABSTRACT Despite the widespread use of abdominal imaging, some patients with renal cell carcinoma still present with advanced disease. We report 1 case of locally invasive sarcomatoid renal cell carcinoma requiring resection of the kidney, adrenal glands, liver, diaphragm, and lung, with diaphragmatic reconstruction with a polytetrafluoroethylene patch. The patient was alive and well 5 years postoperatively. However, we acknowledge that the success experienced with this case does not represent the typical outcome for a patient with such advanced disease. UROLOGY 68: 890.e1–890.e4, 2006. © 2006 Elsevier Inc.
M
any renal tumors are being diagnosed at earlier stages with the more common use of abdominal imaging studies to evaluate other medical conditions.1 However, some patients still present with advanced disease. Renal cell carcinoma (RCC) can be an aggressive regionally invading tumor. Invasion into adjacent organs increases the pathologic stage, decreasing the chance for surgical cure and is, thus, a poor prognostic sign.2 Infrequently, adjacent tissue and organs require resection to completely remove the mass and provide the patient with the best opportunity for cure. CASE REPORT A 35-year-old man presented with a 6-month history of right upper quadrant and right-sided chest wall pain and an associated 15-lb weight loss. He did not have gross hematuria or flank pain. The pain had become increasingly severe, such that it necessitated the use of parenteral narcotics and required him to be off work receiving disability. The patient had no other significant past medical history. An abdominal ultrasound scan revealed a right renal mass. Additional investigations, including computed tomography (CT) and magnetic resonance im-
From the Department of Urology, Dalhousie University, QEII Health Sciences Centre, Halifax, Canada Address for correspondence: Jaime Alan Wong, M.D., Department of Urology, Dalhousie University, QEII Health Sciences Centre, VG Site, Room 294, 5 Victoria, 1278 Tower Road, Halifax NS B3H 2Y9, Canada. E-mail: [email protected] Submitted: January 5, 2006, accepted (with revisions): April 3, 2006 © 2006 ELSEVIER INC. ALL RIGHTS RESERVED
aging confirmed the presence of a large right renal mass that was invading into the liver, through the diaphragm, and into the lung (Fig. 1). A metastatic survey, including chest x-ray and bone scan, revealed no evidence of metastatic disease. Screening investigations to rule out a functional adrenal lesion were negative. The liver function tests were normal. Despite the local invasion of the renal mass, the severe incapacitating pain and the patient’s young age mandated an aggressive surgical approach. The patient underwent a 10th intercostal thoracoabdominal incision. Right radical nephrectomy was performed, along with right segmental hepatic resection, diaphragm resection, and right lower lobectomy. Gross tumor infiltration was used to guide the excision. Specimens for frozen section analysis of the margins were not sent intraoperatively. The diaphragmatic defect was approximately 10 cm in diameter. Because the defect was too large for primary repair, it was closed with a polytetrafluoroethylene (PTFE) graft using a running 2-0 braided polyester nonabsorbable suture. The patient had an uneventful course in hospital, his chest tubes were removed without difficulty, and he was discharged home on the seventh postoperative day. Surgical pathologic examination confirmed an 11-cm renal tumor with direct invasion into the adrenal, liver, and lower lobe of the right lung. The surgical margins were negative for malignancy on the final histologic review. Histologic examination revealed spindle-shaped cells consistent with poorly 0090-4295/06/$32.00 doi:10.1016/j.urology.2006.04.004 890.e1
FIGURE 1. (A) Coronal view of T2-weighted magnetic resonance image demonstrating large heterogeneous mass with its epicenter in upper pole of kidney infiltrating into liver, beyond diaphragm, and into lung. (B) Sagittal view of T1-weighted magnetic resonance image with gadolinium enhancement demonstrating large heterogeneous mass with its epicenter in upper pole of kidney, infiltrating into liver, beyond diaphragm, and into lung.
FIGURE 2. (A) Hematoxylin-eosin stain at 100⫻ magnification demonstrating poorly differentiated tumor composed of spindle cells with moderate cellular pleomorphism, hyperchromatic nuclei, and irregular mitotic figures. (B) Vimentin immunohistochemical stain at 100⫻ magnification demonstrating positive staining for vimentin.
COMMENT differentiated sarcomatoid RCC (Fig. 2A). Immunohistochemistry revealed that the tumor cells were positive for keratin, CK-7, and vimentin (Fig. 2B). Epithelial membrane antigen was negative. The tumor infiltrated the liver (Fig. 3A), beyond the diaphragm (Fig. 3B), and into the lung. The lymph nodes were negative for malignancy. Six months postoperatively, two nodules were identified on CT in the renal fossa, measuring 2.5 and 2.6 cm in the largest diameter, respectively. The patient elected to undergo radiotherapy. Five years later, he had no evidence of recurrent disease on imaging studies and only intermittent minor discomfort requiring no analgesics. He had no respiratory symptoms and had an excellent quality of life. 890.e2
A paucity of data is available regarding the best therapeutic option for those patients with RCC and suspected involvement of adjacent organs. The 3-year survival rate for patients with adjacent visceral organ invasion is less than 5%.3 Despite the advances in adjunctive therapy for RCC, surgical extirpation remains the best option for long-term survival.4 Aggressive surgical resection may be indicated in a select group of patients with locally advanced disease and may provide the only option for cure.3,5 The urologist should be prepared to perform combined resection of adjacent organs when local extension is found at surgery.5 Longterm survival was obtained in 2 patients with RCC and direct hepatic extension by radical nephrectomy and en bloc partial resection of the liver.5 UROLOGY 68 (4), 2006
FIGURE 3. (A) Hematoxylin-eosin stain at 100⫻ magnification demonstrating tumor infiltrating liver. L ⫽ liver; T ⫽ tumor. (B) Hematoxylin-eosin stain at 100⫻ magnification demonstrating tumor infiltrating diaphragm. D ⫽ diaphragm; T ⫽ tumor.
These 2 patients survived at least 57 and 100 months postoperatively.5 Long-term survival was also reported for a 35-year-old woman who had undergone en bloc resection of the kidney, adrenal gland, hepatic lobe, and inferior vena cava for sarcomatoid RCC.6 She remained recurrence free at 4 years postoperatively.6 Of 4 patients requiring partial hepatic resection for RCC, 2 were disease free for 21 and 32 months.3 The other 2 patients demonstrated sarcomatoid features on pathologic examination. However, these 2 patients had disease progression within 6 months and both patients survived less than 14 months.3 It is well known that the sarcomatoid variant of RCC usually portends a poorer prognosis.2 Pautler et al.7 described the intentional resection of the diaphragm during cytoreductive laparoscopic radical nephrectomy in 3 patients with metastatic RCC. The defect was repaired primarily by UROLOGY 68 (4), 2006
laparoscopic intracorporeal suturing techniques in 2 of the 3 patients.7 Rehman et al.8 also described the intentional resection of the diaphragm during laparoscopic radical nephrectomy for metastatic ovarian cancer to the kidney. The defect was also closed primarily using intracorporeal suturing techniques.8 In contrast to renal cancer, it is more common for the diaphragm to require resection in patients with non-small cell lung cancer. However, even lung cancer involving the diaphragm is a rare entity.9 The involvement of the diaphragm in lung cancer is reported to occur in 0.17% to 0.4% of cases.9,10 In most cases requiring diaphragmatic resection, the defect is closed primarily.9 However, the use of prosthetic material to repair diaphragmatic defects has been used successfully in thoracic surgery when primary closure of the defect is not possible.10,11 The material used has included PTFE, fluorinated polyester, Gore-Tex, and polypropylene mesh.8 PTFE is the most commonly used material owing to concern about the possible erosion of the prosthesis into adjacent structures.11 To the best of our knowledge, this is the first case reported in the English-language literature describing the resection of the kidney, adrenal gland, liver, diaphragm, and lung with diaphragmatic reconstruction by a PTFE patch for locally invasive sarcomatoid RCC. It is also interesting that despite the locally advanced nature of his disease and sarcomatoid features demonstrated on pathologic examination, this patient was alive and well 5 years postoperatively. However, we acknowledge that the success experienced with this case does not represent the typical outcome for a patient with such advanced disease. The aggressive surgical management in this case was thought justified because of this patient’s young age and significant symptoms, including narcotic dependence. REFERENCES 1. Guillaume M-P, Baldassarre S, Takeh H, et al: Localized renal cell carcinoma of an unusually large size: case report. Acta Chir Belg 103: 321–323, 2003. 2. Novick AC, and Campbell SC: Renal tumors, in Walsh PC, Retik AB, Vaughan ED, et al (Eds): Campbell’s Urology. Philadelphia, WB Saunders, 2002, pp 2702–2704. 3. Bennett BC, Selby R, and Bahnson RR: Surgical resection for management of renal cancer with hepatic involvement. J Urol 154: 972–974, 1995. 4. Crotty KL, and Macaluso JN: Partial colectomy required for resection of renal cell carcinoma: a case report and review of treatment options for locally advanced disease. J La State Med Soc 152: 119 –123, 2000. 5. Johnin K, Nakai O, Kataoka A, et al: Surgical management of renal cell carcinoma invading into the liver: radical nephrectomy en bloc with right hepatic lateral sector. Urology 57: 975x–975xii, 2001. 6. Sakaguchi S, Hishiki S, Nakamura S, et al: Extension incision for renal carcinoma including invaded vena cava and right lobe of liver. Urology 39: 285–288, 1992. 890.e3
7. Pautler SE, Richards C, Libutti SK, et al: Intentional resection of the diaphragm during cytoreductive laparoscopic radical nephrectomy. J Urol 167: 48 –50, 2002. 8. Rehman J, Landman, Kerbl K, et al: Laparoscopic repair of diaphragmatic defect by total intracorporeal suturing: clinical and technical considerations. JSLS 5: 287–291, 2001. 9. Yokoi K, Tsuchiya R, Mori T, et al: Results of surgical treatment of lung cancer involving the diaphragm: results of
890.e4
surgical treatment of lung cancer involving the diaphragm. J Thorac Cardiovasc Surg 120: 799 – 805, 2000. 10. Weksler B, Bains M, Burt M, et al: Resection of lung cancer invading the diaphragm. J Thorac Cardiovasc Surg 114: 500 –501, 1997. 11. Slim K, Bousquet J, and Chipponi J: Laparoscopic repair of missed blunt diaphragmatic rupture using a prosthesis. Surg Endosc 12: 1358 –1360, 1998.
UROLOGY 68 (4), 2006
RADICAL NEPHRECTOMY WITH EN BLOC RESECTION OF LIVER, DIAPHRAGM, AND LUNG FOR LOCALLY INVASIVE SARCOMATOID RENAL CELL CARCINOMA JAIME A. WONG, THOMAS WHELAN,
AND
MICHAEL MORSE
ABSTRACT Despite the widespread use of abdominal imaging, some patients with renal cell carcinoma still present with advanced disease. We report 1 case of locally invasive sarcomatoid renal cell carcinoma requiring resection of the kidney, adrenal glands, liver, diaphragm, and lung, with diaphragmatic reconstruction with a polytetrafluoroethylene patch. The patient was alive and well 5 years postoperatively. However, we acknowledge that the success experienced with this case does not represent the typical outcome for a patient with such advanced disease. UROLOGY 68: 890.e1–890.e4, 2006. © 2006 Elsevier Inc.
M
any renal tumors are being diagnosed at earlier stages with the more common use of abdominal imaging studies to evaluate other medical conditions.1 However, some patients still present with advanced disease. Renal cell carcinoma (RCC) can be an aggressive regionally invading tumor. Invasion into adjacent organs increases the pathologic stage, decreasing the chance for surgical cure and is, thus, a poor prognostic sign.2 Infrequently, adjacent tissue and organs require resection to completely remove the mass and provide the patient with the best opportunity for cure. CASE REPORT A 35-year-old man presented with a 6-month history of right upper quadrant and right-sided chest wall pain and an associated 15-lb weight loss. He did not have gross hematuria or flank pain. The pain had become increasingly severe, such that it necessitated the use of parenteral narcotics and required him to be off work receiving disability. The patient had no other significant past medical history. An abdominal ultrasound scan revealed a right renal mass. Additional investigations, including computed tomography (CT) and magnetic resonance im-
From the Department of Urology, Dalhousie University, QEII Health Sciences Centre, Halifax, Canada Address for correspondence: Jaime Alan Wong, M.D., Department of Urology, Dalhousie University, QEII Health Sciences Centre, VG Site, Room 294, 5 Victoria, 1278 Tower Road, Halifax NS B3H 2Y9, Canada. E-mail: [email protected] Submitted: January 5, 2006, accepted (with revisions): April 3, 2006 © 2006 ELSEVIER INC. ALL RIGHTS RESERVED
aging confirmed the presence of a large right renal mass that was invading into the liver, through the diaphragm, and into the lung (Fig. 1). A metastatic survey, including chest x-ray and bone scan, revealed no evidence of metastatic disease. Screening investigations to rule out a functional adrenal lesion were negative. The liver function tests were normal. Despite the local invasion of the renal mass, the severe incapacitating pain and the patient’s young age mandated an aggressive surgical approach. The patient underwent a 10th intercostal thoracoabdominal incision. Right radical nephrectomy was performed, along with right segmental hepatic resection, diaphragm resection, and right lower lobectomy. Gross tumor infiltration was used to guide the excision. Specimens for frozen section analysis of the margins were not sent intraoperatively. The diaphragmatic defect was approximately 10 cm in diameter. Because the defect was too large for primary repair, it was closed with a polytetrafluoroethylene (PTFE) graft using a running 2-0 braided polyester nonabsorbable suture. The patient had an uneventful course in hospital, his chest tubes were removed without difficulty, and he was discharged home on the seventh postoperative day. Surgical pathologic examination confirmed an 11-cm renal tumor with direct invasion into the adrenal, liver, and lower lobe of the right lung. The surgical margins were negative for malignancy on the final histologic review. Histologic examination revealed spindle-shaped cells consistent with poorly 0090-4295/06/$32.00 doi:10.1016/j.urology.2006.04.004 890.e1
FIGURE 1. (A) Coronal view of T2-weighted magnetic resonance image demonstrating large heterogeneous mass with its epicenter in upper pole of kidney infiltrating into liver, beyond diaphragm, and into lung. (B) Sagittal view of T1-weighted magnetic resonance image with gadolinium enhancement demonstrating large heterogeneous mass with its epicenter in upper pole of kidney, infiltrating into liver, beyond diaphragm, and into lung.
FIGURE 2. (A) Hematoxylin-eosin stain at 100⫻ magnification demonstrating poorly differentiated tumor composed of spindle cells with moderate cellular pleomorphism, hyperchromatic nuclei, and irregular mitotic figures. (B) Vimentin immunohistochemical stain at 100⫻ magnification demonstrating positive staining for vimentin.
COMMENT differentiated sarcomatoid RCC (Fig. 2A). Immunohistochemistry revealed that the tumor cells were positive for keratin, CK-7, and vimentin (Fig. 2B). Epithelial membrane antigen was negative. The tumor infiltrated the liver (Fig. 3A), beyond the diaphragm (Fig. 3B), and into the lung. The lymph nodes were negative for malignancy. Six months postoperatively, two nodules were identified on CT in the renal fossa, measuring 2.5 and 2.6 cm in the largest diameter, respectively. The patient elected to undergo radiotherapy. Five years later, he had no evidence of recurrent disease on imaging studies and only intermittent minor discomfort requiring no analgesics. He had no respiratory symptoms and had an excellent quality of life. 890.e2
A paucity of data is available regarding the best therapeutic option for those patients with RCC and suspected involvement of adjacent organs. The 3-year survival rate for patients with adjacent visceral organ invasion is less than 5%.3 Despite the advances in adjunctive therapy for RCC, surgical extirpation remains the best option for long-term survival.4 Aggressive surgical resection may be indicated in a select group of patients with locally advanced disease and may provide the only option for cure.3,5 The urologist should be prepared to perform combined resection of adjacent organs when local extension is found at surgery.5 Longterm survival was obtained in 2 patients with RCC and direct hepatic extension by radical nephrectomy and en bloc partial resection of the liver.5 UROLOGY 68 (4), 2006
FIGURE 3. (A) Hematoxylin-eosin stain at 100⫻ magnification demonstrating tumor infiltrating liver. L ⫽ liver; T ⫽ tumor. (B) Hematoxylin-eosin stain at 100⫻ magnification demonstrating tumor infiltrating diaphragm. D ⫽ diaphragm; T ⫽ tumor.
These 2 patients survived at least 57 and 100 months postoperatively.5 Long-term survival was also reported for a 35-year-old woman who had undergone en bloc resection of the kidney, adrenal gland, hepatic lobe, and inferior vena cava for sarcomatoid RCC.6 She remained recurrence free at 4 years postoperatively.6 Of 4 patients requiring partial hepatic resection for RCC, 2 were disease free for 21 and 32 months.3 The other 2 patients demonstrated sarcomatoid features on pathologic examination. However, these 2 patients had disease progression within 6 months and both patients survived less than 14 months.3 It is well known that the sarcomatoid variant of RCC usually portends a poorer prognosis.2 Pautler et al.7 described the intentional resection of the diaphragm during cytoreductive laparoscopic radical nephrectomy in 3 patients with metastatic RCC. The defect was repaired primarily by UROLOGY 68 (4), 2006
laparoscopic intracorporeal suturing techniques in 2 of the 3 patients.7 Rehman et al.8 also described the intentional resection of the diaphragm during laparoscopic radical nephrectomy for metastatic ovarian cancer to the kidney. The defect was also closed primarily using intracorporeal suturing techniques.8 In contrast to renal cancer, it is more common for the diaphragm to require resection in patients with non-small cell lung cancer. However, even lung cancer involving the diaphragm is a rare entity.9 The involvement of the diaphragm in lung cancer is reported to occur in 0.17% to 0.4% of cases.9,10 In most cases requiring diaphragmatic resection, the defect is closed primarily.9 However, the use of prosthetic material to repair diaphragmatic defects has been used successfully in thoracic surgery when primary closure of the defect is not possible.10,11 The material used has included PTFE, fluorinated polyester, Gore-Tex, and polypropylene mesh.8 PTFE is the most commonly used material owing to concern about the possible erosion of the prosthesis into adjacent structures.11 To the best of our knowledge, this is the first case reported in the English-language literature describing the resection of the kidney, adrenal gland, liver, diaphragm, and lung with diaphragmatic reconstruction by a PTFE patch for locally invasive sarcomatoid RCC. It is also interesting that despite the locally advanced nature of his disease and sarcomatoid features demonstrated on pathologic examination, this patient was alive and well 5 years postoperatively. However, we acknowledge that the success experienced with this case does not represent the typical outcome for a patient with such advanced disease. The aggressive surgical management in this case was thought justified because of this patient’s young age and significant symptoms, including narcotic dependence. REFERENCES 1. Guillaume M-P, Baldassarre S, Takeh H, et al: Localized renal cell carcinoma of an unusually large size: case report. Acta Chir Belg 103: 321–323, 2003. 2. Novick AC, and Campbell SC: Renal tumors, in Walsh PC, Retik AB, Vaughan ED, et al (Eds): Campbell’s Urology. Philadelphia, WB Saunders, 2002, pp 2702–2704. 3. Bennett BC, Selby R, and Bahnson RR: Surgical resection for management of renal cancer with hepatic involvement. J Urol 154: 972–974, 1995. 4. Crotty KL, and Macaluso JN: Partial colectomy required for resection of renal cell carcinoma: a case report and review of treatment options for locally advanced disease. J La State Med Soc 152: 119 –123, 2000. 5. Johnin K, Nakai O, Kataoka A, et al: Surgical management of renal cell carcinoma invading into the liver: radical nephrectomy en bloc with right hepatic lateral sector. Urology 57: 975x–975xii, 2001. 6. Sakaguchi S, Hishiki S, Nakamura S, et al: Extension incision for renal carcinoma including invaded vena cava and right lobe of liver. Urology 39: 285–288, 1992. 890.e3
7. Pautler SE, Richards C, Libutti SK, et al: Intentional resection of the diaphragm during cytoreductive laparoscopic radical nephrectomy. J Urol 167: 48 –50, 2002. 8. Rehman J, Landman, Kerbl K, et al: Laparoscopic repair of diaphragmatic defect by total intracorporeal suturing: clinical and technical considerations. JSLS 5: 287–291, 2001. 9. Yokoi K, Tsuchiya R, Mori T, et al: Results of surgical treatment of lung cancer involving the diaphragm: results of
890.e4
surgical treatment of lung cancer involving the diaphragm. J Thorac Cardiovasc Surg 120: 799 – 805, 2000. 10. Weksler B, Bains M, Burt M, et al: Resection of lung cancer invading the diaphragm. J Thorac Cardiovasc Surg 114: 500 –501, 1997. 11. Slim K, Bousquet J, and Chipponi J: Laparoscopic repair of missed blunt diaphragmatic rupture using a prosthesis. Surg Endosc 12: 1358 –1360, 1998.
UROLOGY 68 (4), 2006