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Leiomyosarcoma of the infra-renal inferior vena cava
The American Journal of Surgery (2011) 201, e18 – e20
Clinical Image
Leiomyosarcoma of the infra-renal inferior vena cava Osama H. Al-Saif, M.D.a, Bodhisatwa Sengupta, M.B.B.S., F.R.C.S.a, Samir Amr, M.D.b, Abdul-Wahed Meshikhes, M.B. Ch.B., F.R.C.S.a,* a
Surgical Oncology Section, Department of Surgery, and bDepartment of Histopathology, King Fahad Specialist Hospital, Dammam 31444, Saudi Arabia KEYWORDS: Leiomyosarcoma; Inferior vena cava; Retroperitoneal tumor; Thrombosis
Abstract. Leiomyosarcoma of the inferior vena cava (IVC) is a rare slow-growing retroperitoneal tumor. Two percent of leiomyosarcomas are vascular in origin, and tumors of the IVC account for the majority of the cases. The diagnosis is frequently delayed, because affected patients remain asymptomatic for a long period. It has an extremely poor prognosis, with 5-year actuarial malignancy-free survival rates of 30% to 50% after a wide surgical resection. The authors present the case of a patient with IVC leiomyosarcoma who underwent en bloc resection of the tumor along with the involved segment of the infrarenal IVC without caval reconstruction. Complete surgical resection offers the only potential of long-term survival, but survival of unresected patients is generally measured in months. Palliative resections may temporarily improve symptoms but do not offer long-term survival. © 2011 Elsevier Inc. All rights reserved.
Case report A 66-year-old woman was referred with recurrent pain of the upper abdomen and both flanks of 6 months in duration. Ultrasound revealed a retroperitoneal mass suspected to be arising from the head of the pancreas. She underwent endoscopic ultrasound, which showed a large, rounded mass interposed between the pancreatic head and the inferior vena cava (IVC) measuring 39 ⫻ 45 mm (Fig. 1). Endoscopic ultrasound– guided tru-cut biopsy showed an atypical leiomyoma/smooth muscle tumor of unknown malignant potential. Computed tomography revealed a heterogeneous soft tissue mass originating from the infrarenal IVC about 6.3 ⫻ 5.1 ⫻ 4.8 cm in size and associated with an anterior exophytic component, causing a mass effect on the adjacent head of the pancreas without any obvious involvement. The infrarenal IVC was thrombosed, with multiple tortuous col* Corresponding author. Tel.: 966-3-843-1111 ext 6940; fax: 966-3855-1019. E-mail address: [email protected] Manuscript received February 18, 2010; revised manuscript March 9, 2010
0002-9610/$ - see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.amjsurg.2010.03.021
laterals (Fig. 2). The left gonadal vein was dilated, and the right gonadal vein was thrombosed as it drained into the tumor mass. Computed tomography was diagnostic for IVC leiomyosarcoma and inferior vena cavography revealed no filling of the IVC from the proximal right iliac vein to the infrarenal part. Retrograde filling of the suprarenal IVC through the gonadal veins and multiple tortuous pelvic collaterals was noted (Fig. 3). At laparotomy, a retroperitoneal mass was found arising from the infrarenal IVC, which was adjacent to the head of pancreas. After mobilization of the descending colon and adequate Kocher maneuver, the entire length of IVC was exposed. Resection of the infrarenal IVC along with the tumor and adjacent lymphadenectomy was performed. A thrombus was identified in the IVC lumen. Histopathology revealed cellular spindle cell neoplasm composed of long and short fascicles of mild to moderate pleomorphic spindle cells with cigar-shaped nuclei and a moderate amount of eosinophilic cytoplasm with brisk mitotic activity (⬎10/10 high-power field; Fig. 4). Focal areas of tumor cell necrosis were seen. Proximal, distal, and circumferential tumor margins were free. Findings were consistent with leiomyosarcoma. Five lymph nodes showed
O.H. Al-Saif et al.
Inferior vena caval leiomyosarcoma
e19
Figure 1 Endoscopic ultrasound picture showing a large rounded mass arising from the IVC with exophytic component interposed between the pancreatic head and the IVC.
reactive hyperplasia and were negative for metastasis. The patient developed postoperative deep vein thrombosis of the left lower limb, which resolved with anticoagulants. Repeat computed tomography showed no evidence of recurrence at 1-year follow-up.
Figure 3 Inferior vena cavography revealed absence of filling of the infrarenal IVC. Retrograde filling of the suprarenal IVC through the gonadal veins and multiple tortuous collaterals was noted.
Comments Leiomyosarcoma is a rare retroperitoneal tumor and was first reported in the German literature by Perl in 1871.1,2 It
Figure 2 Computed tomographic scan showing heterogeneous soft tissue mass originating from infrarenal IVC with associated with anterior exophytic component, causing a mass effect on the adjacent head of the pancreas.
Figure 4 Histopathologic micrograph of the resected tumor showing cellular spindle cell neoplasm composed of long and short fascicles of mild to moderate pleomorphic spindle cells with cigarshaped nuclei and a moderate amount of eosinophilic cytoplasm with brisk mitotic activity (hematoxylin and eosin staining; ⬎10/10 high-power field).
e20 is most often seen in women, and the diagnosis is often delayed because of the relative lack of symptoms.2 It is discovered incidentally in 10.5% of patients and at autopsy in 33%.3,4 It is estimated that 2% of leiomyosarcomas are vascular in origin, and tumors of the IVC account for a majority of cases.5 It has an extremely poor prognosis,3 with 5-year actuarial malignancy-free survival rates between 30% and 50%, after wide surgical resection.4,6,7 A true IVC sarcoma is believed to arise from the smooth muscle within the vessel, and its growth pattern may be both intraluminal and extraluminal and may involve neighboring structures.8 Tumor involvement is classified into 3 groups according to the level in the IVC: segment I, infrarenal (as in this case); segment II, interrenal and suprarenal up to but not including the main suprahepatic veins; and segment III, suprahepatic with possible intracardiac extension.3 The retrohepatic segment of the IVC between the renal and hepatic veins is the most common site of involvement.4,9 As in this case, 30% of patients with this tumor have IVC thrombosis.6 Ultrasound is the modality of choice for obtaining tissue for histologic diagnosis.10 Computed tomography or magnetic resonance imaging is essential in planning surgical treatment.11 Computed tomography is a sensitive tool in establishing the diagnosis and follow-up. The relationship of the mass, the extent of involvement, and opacification of the retroperitoneal vessels are seen on computed tomography. The typical leiomyosarcoma appearances are that of a solid lobulated retroperitoneal mass with cystic zones corresponding to areas of necrosis.10 Cavography shows a filling defect or compression and deviation of the IVC depending on whether growth is intraluminal or extraluminal. It also evaluates the collateral venous circulation and aids in the planning of venous reconstruction.12 Complete surgical resection offers the only potential of long-term survival. The survival of unresected patients is generally measured in months,4,7 and palliative resections may temporarily improve symptoms but do not offer longterm survival.4,6,7 Ligation of the infrarenal IVC is generally well tolerated in patients with preoperative caval occlusion and collateral formation.6,13 However, there is a risk for complications such as deep vein thrombosis, ascites, pedal edema, and renal dysfunction.6,11 Ligation of the suprarenal cava is avoided if possible to avoid venous hypertension to the kidneys with its detrimental effect on renal function.8,13 Small tumors that can be completely resected with a small caval defect may be closed primarily or with a saphenous vein patch.8 Reconstruction of the vena cava is most fre-
The American Journal of Surgery, Vol 201, No 2, February 2011 quently performed with a large-diameter polytetrafluoroethylene tube graft, although autologous tissue grafting from a variety of donor sites is also possible.3,6,8 The role of chemotherapy and radiation therapy for all retroperitoneal sarcomas in general, and IVC sarcoma in particular, is not clear.8 Kieffer et al3 considered radical resection followed by adjuvant chemotherapy the optimal therapeutic strategy for tumors without metastasis at the time of initial diagnosis. Surgery, whether performed alone or in combination with chemotherapy and possibly radiation therapy, is generally not curative, but it constitutes the only hope of prolonged survival. Therefore, we recommend aggressive surgical management using modern vascular and oncologic techniques.3
References 1. Perl L. Ein fall von sarkom der vena cava inferior. Virchows Arch 1871;53:378 – 83. 2. Crema E, Zanier-Gomes MG, Monteiro Ide O, et al. Leiomyosarcoma of the inferior vena cava: a case report. Angiology 2008;59:256 –9. 3. Kieffer E, Alaoui M, Piette JC, et al. Leiomyosarcoma of the inferior vena cava: experience in 22 cases. Ann Surg 2006;244:289 –95. 4. Mingoli A, Cavallaro A, Sapienza P, et al. International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. Anticancer Res 1996;16:3201–5. 5. Bower TC, Stanson A. Diagnosis and management of tumours of the inferior vena cava. In: Rutherford RB, ed. Vascular surgery. Philadelphia: W.B. Saunders; 2000:2077–92. 6. Hollenbeck ST, Grobmyer SR, Kent KC, et al. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. J Am Coll Surg 2003;197:375–9. 7. Hines OJ, Nelson S, Quinones-Baldrich WJ, et al. Leiomyosarcoma of the interior vena cava: prognosis and comparison with leiomyosarcoma of other anatomic sites. Cancer 1999;85:1077– 83. 8. Dew J, Hansen K, Hammon J, et al. Leiomyosarcoma of the inferior vena cava: surgical management and clinical results. Am Surg 2005; 71:497–501. 9. Kulaylat MN, Karakousis CP, Doerr RJ, et al. Leiomyosarcoma of the inferior vena cava: a clinicopathologic review and report of three cases. J Surg Oncol 1997;65:205–17. 10. Hemant D, Krantikumar R, Amita J, et al. Primary leiomyosarcoma of inferior vena cava, a rare entity: Imaging features. Australas Radiol 2001;45:448 –51. 11. Praseedom RK, Dhar P, Jamieson NV, et al. Leiomyosarcoma of the retrohepatic vena cava treated by excision and reconstruction with an aortic homograft: a case report and review of literature. Surg Innov 2007;14:287–91. 12. Bonura A, Saade C, Sharma P. Leiomyosarcoma of the inferior vena cava. Australas Radiol 2006;50:395–9. 13. Beck SDW, Lalka SG, Donohue JP, Long-term results after inferior vena caval resection during retroperitoneal lymphadencctomy for metastatic germ cell cancer. J Vase Surg 1998;28:808 –14.
Clinical Image
Leiomyosarcoma of the infra-renal inferior vena cava Osama H. Al-Saif, M.D.a, Bodhisatwa Sengupta, M.B.B.S., F.R.C.S.a, Samir Amr, M.D.b, Abdul-Wahed Meshikhes, M.B. Ch.B., F.R.C.S.a,* a
Surgical Oncology Section, Department of Surgery, and bDepartment of Histopathology, King Fahad Specialist Hospital, Dammam 31444, Saudi Arabia KEYWORDS: Leiomyosarcoma; Inferior vena cava; Retroperitoneal tumor; Thrombosis
Abstract. Leiomyosarcoma of the inferior vena cava (IVC) is a rare slow-growing retroperitoneal tumor. Two percent of leiomyosarcomas are vascular in origin, and tumors of the IVC account for the majority of the cases. The diagnosis is frequently delayed, because affected patients remain asymptomatic for a long period. It has an extremely poor prognosis, with 5-year actuarial malignancy-free survival rates of 30% to 50% after a wide surgical resection. The authors present the case of a patient with IVC leiomyosarcoma who underwent en bloc resection of the tumor along with the involved segment of the infrarenal IVC without caval reconstruction. Complete surgical resection offers the only potential of long-term survival, but survival of unresected patients is generally measured in months. Palliative resections may temporarily improve symptoms but do not offer long-term survival. © 2011 Elsevier Inc. All rights reserved.
Case report A 66-year-old woman was referred with recurrent pain of the upper abdomen and both flanks of 6 months in duration. Ultrasound revealed a retroperitoneal mass suspected to be arising from the head of the pancreas. She underwent endoscopic ultrasound, which showed a large, rounded mass interposed between the pancreatic head and the inferior vena cava (IVC) measuring 39 ⫻ 45 mm (Fig. 1). Endoscopic ultrasound– guided tru-cut biopsy showed an atypical leiomyoma/smooth muscle tumor of unknown malignant potential. Computed tomography revealed a heterogeneous soft tissue mass originating from the infrarenal IVC about 6.3 ⫻ 5.1 ⫻ 4.8 cm in size and associated with an anterior exophytic component, causing a mass effect on the adjacent head of the pancreas without any obvious involvement. The infrarenal IVC was thrombosed, with multiple tortuous col* Corresponding author. Tel.: 966-3-843-1111 ext 6940; fax: 966-3855-1019. E-mail address: [email protected] Manuscript received February 18, 2010; revised manuscript March 9, 2010
0002-9610/$ - see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.amjsurg.2010.03.021
laterals (Fig. 2). The left gonadal vein was dilated, and the right gonadal vein was thrombosed as it drained into the tumor mass. Computed tomography was diagnostic for IVC leiomyosarcoma and inferior vena cavography revealed no filling of the IVC from the proximal right iliac vein to the infrarenal part. Retrograde filling of the suprarenal IVC through the gonadal veins and multiple tortuous pelvic collaterals was noted (Fig. 3). At laparotomy, a retroperitoneal mass was found arising from the infrarenal IVC, which was adjacent to the head of pancreas. After mobilization of the descending colon and adequate Kocher maneuver, the entire length of IVC was exposed. Resection of the infrarenal IVC along with the tumor and adjacent lymphadenectomy was performed. A thrombus was identified in the IVC lumen. Histopathology revealed cellular spindle cell neoplasm composed of long and short fascicles of mild to moderate pleomorphic spindle cells with cigar-shaped nuclei and a moderate amount of eosinophilic cytoplasm with brisk mitotic activity (⬎10/10 high-power field; Fig. 4). Focal areas of tumor cell necrosis were seen. Proximal, distal, and circumferential tumor margins were free. Findings were consistent with leiomyosarcoma. Five lymph nodes showed
O.H. Al-Saif et al.
Inferior vena caval leiomyosarcoma
e19
Figure 1 Endoscopic ultrasound picture showing a large rounded mass arising from the IVC with exophytic component interposed between the pancreatic head and the IVC.
reactive hyperplasia and were negative for metastasis. The patient developed postoperative deep vein thrombosis of the left lower limb, which resolved with anticoagulants. Repeat computed tomography showed no evidence of recurrence at 1-year follow-up.
Figure 3 Inferior vena cavography revealed absence of filling of the infrarenal IVC. Retrograde filling of the suprarenal IVC through the gonadal veins and multiple tortuous collaterals was noted.
Comments Leiomyosarcoma is a rare retroperitoneal tumor and was first reported in the German literature by Perl in 1871.1,2 It
Figure 2 Computed tomographic scan showing heterogeneous soft tissue mass originating from infrarenal IVC with associated with anterior exophytic component, causing a mass effect on the adjacent head of the pancreas.
Figure 4 Histopathologic micrograph of the resected tumor showing cellular spindle cell neoplasm composed of long and short fascicles of mild to moderate pleomorphic spindle cells with cigarshaped nuclei and a moderate amount of eosinophilic cytoplasm with brisk mitotic activity (hematoxylin and eosin staining; ⬎10/10 high-power field).
e20 is most often seen in women, and the diagnosis is often delayed because of the relative lack of symptoms.2 It is discovered incidentally in 10.5% of patients and at autopsy in 33%.3,4 It is estimated that 2% of leiomyosarcomas are vascular in origin, and tumors of the IVC account for a majority of cases.5 It has an extremely poor prognosis,3 with 5-year actuarial malignancy-free survival rates between 30% and 50%, after wide surgical resection.4,6,7 A true IVC sarcoma is believed to arise from the smooth muscle within the vessel, and its growth pattern may be both intraluminal and extraluminal and may involve neighboring structures.8 Tumor involvement is classified into 3 groups according to the level in the IVC: segment I, infrarenal (as in this case); segment II, interrenal and suprarenal up to but not including the main suprahepatic veins; and segment III, suprahepatic with possible intracardiac extension.3 The retrohepatic segment of the IVC between the renal and hepatic veins is the most common site of involvement.4,9 As in this case, 30% of patients with this tumor have IVC thrombosis.6 Ultrasound is the modality of choice for obtaining tissue for histologic diagnosis.10 Computed tomography or magnetic resonance imaging is essential in planning surgical treatment.11 Computed tomography is a sensitive tool in establishing the diagnosis and follow-up. The relationship of the mass, the extent of involvement, and opacification of the retroperitoneal vessels are seen on computed tomography. The typical leiomyosarcoma appearances are that of a solid lobulated retroperitoneal mass with cystic zones corresponding to areas of necrosis.10 Cavography shows a filling defect or compression and deviation of the IVC depending on whether growth is intraluminal or extraluminal. It also evaluates the collateral venous circulation and aids in the planning of venous reconstruction.12 Complete surgical resection offers the only potential of long-term survival. The survival of unresected patients is generally measured in months,4,7 and palliative resections may temporarily improve symptoms but do not offer longterm survival.4,6,7 Ligation of the infrarenal IVC is generally well tolerated in patients with preoperative caval occlusion and collateral formation.6,13 However, there is a risk for complications such as deep vein thrombosis, ascites, pedal edema, and renal dysfunction.6,11 Ligation of the suprarenal cava is avoided if possible to avoid venous hypertension to the kidneys with its detrimental effect on renal function.8,13 Small tumors that can be completely resected with a small caval defect may be closed primarily or with a saphenous vein patch.8 Reconstruction of the vena cava is most fre-
The American Journal of Surgery, Vol 201, No 2, February 2011 quently performed with a large-diameter polytetrafluoroethylene tube graft, although autologous tissue grafting from a variety of donor sites is also possible.3,6,8 The role of chemotherapy and radiation therapy for all retroperitoneal sarcomas in general, and IVC sarcoma in particular, is not clear.8 Kieffer et al3 considered radical resection followed by adjuvant chemotherapy the optimal therapeutic strategy for tumors without metastasis at the time of initial diagnosis. Surgery, whether performed alone or in combination with chemotherapy and possibly radiation therapy, is generally not curative, but it constitutes the only hope of prolonged survival. Therefore, we recommend aggressive surgical management using modern vascular and oncologic techniques.3
References 1. Perl L. Ein fall von sarkom der vena cava inferior. Virchows Arch 1871;53:378 – 83. 2. Crema E, Zanier-Gomes MG, Monteiro Ide O, et al. Leiomyosarcoma of the inferior vena cava: a case report. Angiology 2008;59:256 –9. 3. Kieffer E, Alaoui M, Piette JC, et al. Leiomyosarcoma of the inferior vena cava: experience in 22 cases. Ann Surg 2006;244:289 –95. 4. Mingoli A, Cavallaro A, Sapienza P, et al. International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. Anticancer Res 1996;16:3201–5. 5. Bower TC, Stanson A. Diagnosis and management of tumours of the inferior vena cava. In: Rutherford RB, ed. Vascular surgery. Philadelphia: W.B. Saunders; 2000:2077–92. 6. Hollenbeck ST, Grobmyer SR, Kent KC, et al. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. J Am Coll Surg 2003;197:375–9. 7. Hines OJ, Nelson S, Quinones-Baldrich WJ, et al. Leiomyosarcoma of the interior vena cava: prognosis and comparison with leiomyosarcoma of other anatomic sites. Cancer 1999;85:1077– 83. 8. Dew J, Hansen K, Hammon J, et al. Leiomyosarcoma of the inferior vena cava: surgical management and clinical results. Am Surg 2005; 71:497–501. 9. Kulaylat MN, Karakousis CP, Doerr RJ, et al. Leiomyosarcoma of the inferior vena cava: a clinicopathologic review and report of three cases. J Surg Oncol 1997;65:205–17. 10. Hemant D, Krantikumar R, Amita J, et al. Primary leiomyosarcoma of inferior vena cava, a rare entity: Imaging features. Australas Radiol 2001;45:448 –51. 11. Praseedom RK, Dhar P, Jamieson NV, et al. Leiomyosarcoma of the retrohepatic vena cava treated by excision and reconstruction with an aortic homograft: a case report and review of literature. Surg Innov 2007;14:287–91. 12. Bonura A, Saade C, Sharma P. Leiomyosarcoma of the inferior vena cava. Australas Radiol 2006;50:395–9. 13. Beck SDW, Lalka SG, Donohue JP, Long-term results after inferior vena caval resection during retroperitoneal lymphadencctomy for metastatic germ cell cancer. J Vase Surg 1998;28:808 –14.