Case Report
Primary leiomyosarcoma of saphenous vein presenting as deep venous thrombosis
Vascular 2014, Vol. 22(6) 450–453 ! The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1708538113516446 vas.sagepub.com
Daniel I Fremed, Peter L Faries, Harry R Schanzer, Michael L Marin and Windsor Ting
Abstract Only a small number of venous leiomyosarcomas have been previously reported. Of these tumors, those of saphenous origin comprise a minority of cases. A 59-year-old man presented with symptoms of deep vein thrombosis and was eventually diagnosed with primary leiomyosarcoma of great saphenous vein origin. The tumor was treated with primary resection and femoral vein reconstruction with autologous patch. Although extremely rare, saphenous leiomyosarcoma can present as deep vein thrombosis. Vascular tumors should be included in the differential diagnosis of atypical extremity swelling refractory to conventional deep vein thrombosis management.
Keywords Leiomyosarcoma, saphenous leiomyosarcoma, vascular tumor, venous sarcoma, deep vein thrombosis
Introduction Primary vascular sarcomas are extremely rare. Soft tissue sarcomas represent less than 1% of all malignancies, of which leiomyosarcomas comprise 6%. Of these tumors, leiomyosarcomas of vascular origin constitute less than 2%.1 To our knowledge, only 32 cases of leiomyosarcoma of saphenous origin have been reported since first described by Aurfrecht in 1868.2 We present a case of saphenous leiomyosarcoma located in the sapheno-femoral junction presenting as deep vein thrombosis (DVT). The tumor was treated with primary resection and reconstruction of the femoral/common femoral vein using an autologous vein patch. Perioperative imaging of the tumor will be presented along with a discussion of current literature regarding saphenous leiomyosarcoma.
Case report A 59-year-old man with a 30-pack-year smoking history presented to the emergency department with a complaint of right lower extremity swelling. He reported progressive swelling and sensation of heaviness after injuring his right leg in the bath tub 1 week prior to admission. The patient had no personal or family history of venous thrombosis or malignancy.
Clinical examination revealed severe edema involving the entire right lower extremity from his toes to proximal thigh. There were no palpable cords or groin masses. Lower extremity pulses were palpable. DVT was diagnosed on an outside ultrasonography revealing a non-compressible common femoral vein, proximal femoral vein, and profunda vein. Occlusive thrombus was noted in the right external iliac vein as well as proximal great saphenous vein. Treatment with intravenous heparin was initiated. On hospital day 2 the patient developed right foot pain and the edema did not change. Because of this, the patient was brought to the operating room for venography and catheter-directed pharmaco-mechanical thrombolysis. Intraoperative venography revealed a 4-cm filling defect in the right common femoral vein. The distal femoral vein, profunda femoral vein, external iliac vein, and vena cava appeared to be patent. Pharmaco-mechanical thrombectomy was performed Division of Vascular Surgery, The Mount Sinai Medical Center, New York, NY, USA Corresponding author: Daniel I Fremed, Division of Vascular Surgery, The Mount Sinai Medical Center, 5 East 98th Street, Box 1273, New York, NY 10029, USA. Email: [email protected]
Downloaded from vas.sagepub.com at UQ Library on June 17, 2015
Fremed et al.
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using Angiojet with recombinant tissue plasminogen activator. Subsequent venography showed persistence of the thrombus. Although a guide wire was able to pass around the thrombus, attempts at transluminal angioplasty of the common femoral vein were unsuccessful. The procedure was terminated and conservative management with compression and anticoagulation was resumed. The patient’s symptoms failed to improve. The following day, T1 weighted magnetic resonance imaging (MRI) was completed and revealed a 3.4 2.1 3.0 cm3 soft tissue mass in the subcutaneous tissues of the right groin encasing the common femoral
Figure 1. Magnetic resonance imaging in coronal view demonstrating contrast filling defect in right femoral vein (asterik) with soft tissue density below.
vessels. The proximal great saphenous vein including the saphenofemoral junction and the common femoral vein appeared thrombosed (Figure 1). Because of these findings, the patient was taken back to the operating room on hospital day 9 for surgical exploration. The femoral artery and the femoral vein were dissected free from surrounding tissues. Proximal and distal control of the femoral vein was established. The common femoral vein was opened exposing a lobular mass approximately 4 cm in length within its lumen (Figure 2). The mass did not appear to involve the wall of the common femoral vein. Further exploration showed the mass in fact extended through the saphenofemoral junction and originated from within the proximal great saphenous vein. The tumor was excised en bloc and sent to pathology for frozen section. This biopsy revealed spindle shaped cells, characteristic of leiomyosarcoma. Two centimeter margins were excised from distal great saphenous vein to common femoral vein. Several local lymph nodes were excised and sent to pathology. After ligation of the great saphenous vein, attention was directed towards a dilated segment of proximal common femoral vein. This segment provided an adequately sized vein patch for reconstruction of the saphenofemoral junction while leaving sufficient residual common femoral vein diameter to close primarily with 6-0 Prolene suture. At the end of the procedure, the patient was transferred to recovery in stable condition and discharged home on postoperative day 4 with low molecular weight heparin and warfarin. His postoperative course was complicated by a lymphocutaneous fistula which closed within 30 days with a vacuum-assisted wound dressing. On final pathological examination, the vein mass stained positive for vimentin, smooth muscle actin and desmin, consistent with leiomyosarcoma.
Figure 2. (Left) Tumor (a) extending from GSV (b) to CFV (c). Additional vessel loops on femoral vein (d) and common femoral artery (e). (Right) Mass removed from GSV.
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The tumor appeared to arise from the wall of the great saphenous vein. Tissue margins were negative. Five reactive lymph nodes dissected intraoperatively were negative for malignancy. On his first month of follow up, the patient’s swelling completely resolved. Metastatic workup was unremarkable. He was referred to our oncology team to discuss adjuvant treatment, but the patient declined further work up. At 6-month follow up repeat ultrasound showed no evidence of DVT in the right lower extremity. Repeat MRI was unremarkable.
Discussion Leiomyosarcomas are malignant smooth muscle tumors, accounting for 6% of all soft tissue sarcomas. Vascular leiomyosarcoma is an extremely rare subgroup, accounting for less than 2%.1 Tumors of veins are primarily leiomyosarcomas which arise from smooth muscle cells of the media within the vein wall. Approximately 60% of leiomyosarcoma are located in the vena cava but venous leiomyosarcomas have been reported in many locations, their symptoms location specific and treatments individualized.3 The most complete review of vascular leiomyosarcomas was a series of 86 cases described by Kevorkian and Cento in 1973.4 Of these cases, 33/86 (38.4%) were of inferior vena cava origin. 10/86 (11.6%) were of saphenous origin. Of those 10, only 3 presented with extremity pain and edema, similar to our patient. The size of these tumors ranged from 2.5 cm to 6 cm, with no correlation between size of the tumor and clinical presentation. Saphenous leiomyosarcomas develop in an intraluminal to extraluminal fashion.5 Typically these tumors develop a pseudocapsule which we were able to identify on surgical excision. Histologically, leiomyosarcomas are identified by spindle cells with pinocytotic vesicles. Immunohistochemistry assays stain positive for vimentin, smooth muscle cell actin, and desmin. Tumors with greater than 5 mitoses per 10 high-powered field should be considered malignant.6 Our specimen contained 24 mitoses per 10 high-powered field. Staging is based on histological grade, size and depth, regional nodal involvement, and distant metastases. Grading is based on the degree of cellularity, differentiation, pleophorphism, necrosis, and number of mitoses. In the case presented, the venous tumor was staged at T2b (high grade, deep tumor,
Primary leiomyosarcoma of saphenous vein presenting as deep venous thrombosis
Vascular 2014, Vol. 22(6) 450–453 ! The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1708538113516446 vas.sagepub.com
Daniel I Fremed, Peter L Faries, Harry R Schanzer, Michael L Marin and Windsor Ting
Abstract Only a small number of venous leiomyosarcomas have been previously reported. Of these tumors, those of saphenous origin comprise a minority of cases. A 59-year-old man presented with symptoms of deep vein thrombosis and was eventually diagnosed with primary leiomyosarcoma of great saphenous vein origin. The tumor was treated with primary resection and femoral vein reconstruction with autologous patch. Although extremely rare, saphenous leiomyosarcoma can present as deep vein thrombosis. Vascular tumors should be included in the differential diagnosis of atypical extremity swelling refractory to conventional deep vein thrombosis management.
Keywords Leiomyosarcoma, saphenous leiomyosarcoma, vascular tumor, venous sarcoma, deep vein thrombosis
Introduction Primary vascular sarcomas are extremely rare. Soft tissue sarcomas represent less than 1% of all malignancies, of which leiomyosarcomas comprise 6%. Of these tumors, leiomyosarcomas of vascular origin constitute less than 2%.1 To our knowledge, only 32 cases of leiomyosarcoma of saphenous origin have been reported since first described by Aurfrecht in 1868.2 We present a case of saphenous leiomyosarcoma located in the sapheno-femoral junction presenting as deep vein thrombosis (DVT). The tumor was treated with primary resection and reconstruction of the femoral/common femoral vein using an autologous vein patch. Perioperative imaging of the tumor will be presented along with a discussion of current literature regarding saphenous leiomyosarcoma.
Case report A 59-year-old man with a 30-pack-year smoking history presented to the emergency department with a complaint of right lower extremity swelling. He reported progressive swelling and sensation of heaviness after injuring his right leg in the bath tub 1 week prior to admission. The patient had no personal or family history of venous thrombosis or malignancy.
Clinical examination revealed severe edema involving the entire right lower extremity from his toes to proximal thigh. There were no palpable cords or groin masses. Lower extremity pulses were palpable. DVT was diagnosed on an outside ultrasonography revealing a non-compressible common femoral vein, proximal femoral vein, and profunda vein. Occlusive thrombus was noted in the right external iliac vein as well as proximal great saphenous vein. Treatment with intravenous heparin was initiated. On hospital day 2 the patient developed right foot pain and the edema did not change. Because of this, the patient was brought to the operating room for venography and catheter-directed pharmaco-mechanical thrombolysis. Intraoperative venography revealed a 4-cm filling defect in the right common femoral vein. The distal femoral vein, profunda femoral vein, external iliac vein, and vena cava appeared to be patent. Pharmaco-mechanical thrombectomy was performed Division of Vascular Surgery, The Mount Sinai Medical Center, New York, NY, USA Corresponding author: Daniel I Fremed, Division of Vascular Surgery, The Mount Sinai Medical Center, 5 East 98th Street, Box 1273, New York, NY 10029, USA. Email: [email protected]
Downloaded from vas.sagepub.com at UQ Library on June 17, 2015
Fremed et al.
451
using Angiojet with recombinant tissue plasminogen activator. Subsequent venography showed persistence of the thrombus. Although a guide wire was able to pass around the thrombus, attempts at transluminal angioplasty of the common femoral vein were unsuccessful. The procedure was terminated and conservative management with compression and anticoagulation was resumed. The patient’s symptoms failed to improve. The following day, T1 weighted magnetic resonance imaging (MRI) was completed and revealed a 3.4 2.1 3.0 cm3 soft tissue mass in the subcutaneous tissues of the right groin encasing the common femoral
Figure 1. Magnetic resonance imaging in coronal view demonstrating contrast filling defect in right femoral vein (asterik) with soft tissue density below.
vessels. The proximal great saphenous vein including the saphenofemoral junction and the common femoral vein appeared thrombosed (Figure 1). Because of these findings, the patient was taken back to the operating room on hospital day 9 for surgical exploration. The femoral artery and the femoral vein were dissected free from surrounding tissues. Proximal and distal control of the femoral vein was established. The common femoral vein was opened exposing a lobular mass approximately 4 cm in length within its lumen (Figure 2). The mass did not appear to involve the wall of the common femoral vein. Further exploration showed the mass in fact extended through the saphenofemoral junction and originated from within the proximal great saphenous vein. The tumor was excised en bloc and sent to pathology for frozen section. This biopsy revealed spindle shaped cells, characteristic of leiomyosarcoma. Two centimeter margins were excised from distal great saphenous vein to common femoral vein. Several local lymph nodes were excised and sent to pathology. After ligation of the great saphenous vein, attention was directed towards a dilated segment of proximal common femoral vein. This segment provided an adequately sized vein patch for reconstruction of the saphenofemoral junction while leaving sufficient residual common femoral vein diameter to close primarily with 6-0 Prolene suture. At the end of the procedure, the patient was transferred to recovery in stable condition and discharged home on postoperative day 4 with low molecular weight heparin and warfarin. His postoperative course was complicated by a lymphocutaneous fistula which closed within 30 days with a vacuum-assisted wound dressing. On final pathological examination, the vein mass stained positive for vimentin, smooth muscle actin and desmin, consistent with leiomyosarcoma.
Figure 2. (Left) Tumor (a) extending from GSV (b) to CFV (c). Additional vessel loops on femoral vein (d) and common femoral artery (e). (Right) Mass removed from GSV.
Downloaded from vas.sagepub.com at UQ Library on June 17, 2015
452
Vascular 22(6)
The tumor appeared to arise from the wall of the great saphenous vein. Tissue margins were negative. Five reactive lymph nodes dissected intraoperatively were negative for malignancy. On his first month of follow up, the patient’s swelling completely resolved. Metastatic workup was unremarkable. He was referred to our oncology team to discuss adjuvant treatment, but the patient declined further work up. At 6-month follow up repeat ultrasound showed no evidence of DVT in the right lower extremity. Repeat MRI was unremarkable.
Discussion Leiomyosarcomas are malignant smooth muscle tumors, accounting for 6% of all soft tissue sarcomas. Vascular leiomyosarcoma is an extremely rare subgroup, accounting for less than 2%.1 Tumors of veins are primarily leiomyosarcomas which arise from smooth muscle cells of the media within the vein wall. Approximately 60% of leiomyosarcoma are located in the vena cava but venous leiomyosarcomas have been reported in many locations, their symptoms location specific and treatments individualized.3 The most complete review of vascular leiomyosarcomas was a series of 86 cases described by Kevorkian and Cento in 1973.4 Of these cases, 33/86 (38.4%) were of inferior vena cava origin. 10/86 (11.6%) were of saphenous origin. Of those 10, only 3 presented with extremity pain and edema, similar to our patient. The size of these tumors ranged from 2.5 cm to 6 cm, with no correlation between size of the tumor and clinical presentation. Saphenous leiomyosarcomas develop in an intraluminal to extraluminal fashion.5 Typically these tumors develop a pseudocapsule which we were able to identify on surgical excision. Histologically, leiomyosarcomas are identified by spindle cells with pinocytotic vesicles. Immunohistochemistry assays stain positive for vimentin, smooth muscle cell actin, and desmin. Tumors with greater than 5 mitoses per 10 high-powered field should be considered malignant.6 Our specimen contained 24 mitoses per 10 high-powered field. Staging is based on histological grade, size and depth, regional nodal involvement, and distant metastases. Grading is based on the degree of cellularity, differentiation, pleophorphism, necrosis, and number of mitoses. In the case presented, the venous tumor was staged at T2b (high grade, deep tumor,
