Cross-Femoral Venous By-Pass (Palma’s Procedure) to Relieve Venous Hypertension Due to Retroperitoneal Leiomyosarcoma: A Case Report

Retroperitoneal leiomyosarcoma arising in, or involving, the inferior vena cava or the iliac vein are infrequent tumours. They are usually treated by “en-block” surgery. Venous reconstruction is usually not needed, since collateral circulation can adequately offset the main vessel flow. However, in rare cases, collateral circulation may be insufficient. The authors report a case of leiomyosarcoma infiltrating the left iliac vein. One week after radical resection surgery, the patient developed left leg compartmental syndrome due to venous hypertension.

The patient was successfully treated by thrombectomy of the left femoral vein and cross-femoral venous bypass (Palma’s Procedure) by means of an 8 mm diameter polytetrafluoroethylene (PTFE) prosthesis. To improve the flow inside the graft, an arterio-venous fistula (AVF) was performed. The choice of a prosthetic conduit, its diameter, and the AVF are discussed.     

Primary venous leiomyosarcoma: a rare but lethal disease.

Primary venous leiomyosarcoma is rare, only 197 patients have been reported. To examine progress in diagnosis, treatment, and clinical outcome, we retrospectively reviewed data of 13 patients, 12 women and one man (mean age, 55 years; range, 19 to 75 years), who in the last 35 years underwent surgical treatment for primary venous leiomyosarcoma at our institution. The tumor arose from the inferior vena cava in eight, iliac vein in two, ovarian vein in one, and greater saphenous vein in two patients. Primary venous leiomyosarcoma was detected by physical examination in nine patients, symptoms were present in six. The 13 patients underwent 16 surgical procedures to resect primary (12), recurrent (2), or metastatic (2) tumors. Local excision alone was performed in seven, and local excision with reconstruction of the inferior vena cava or iliac vein was performed in six patients. The tumor was greater than 10 cm in eight patients. The perioperative mortality rate was 15% (2 of 13). Median survival of the 11 early survivors was 3.5 years (range, 6 months to 17 years). Currently five patients are alive (four of them free of known tumor), with a median survival of 3 years (6 months to 17 years) after surgery. Of the eight deceased patients, primary venous leiomyosarcoma recurred after resection in six. Tumor recurrence was not affected by tumor grade, size, or adjuvant treatment. Although early detection with modern imaging techniques could potentially be of benefit, wide local excision with selective venous reconstruction affords the only hope for prolonged survival.     

Sequelae after limb-sparing surgery with major vascular resection for tumor of the lower extremity

PURPOSE: Limb-sparing procedures have recently replaced amputations as the treatment for tumors invading major vessels of the lower extremity. Major arteries must be reconstructed for limb salvage. The veins are not usually reconstructed. This study was undertaken to investigate the sequelae such as chronic venous disease after venous resection for tumors. METHODS: Ten patients who underwent limb-sparing surgery for a tumor of the lower extremity or retroperitoneum that required major vascular resection were studied. The median follow-up period was 48 months. After combined resection of a major artery and vein, arterial reconstruction was performed. The veins were not reconstructed. The resected veins included the inferior vena cava (n = 2), the external iliac and common femoral veins (n = 3), the superficial femoral vein (n = 3), and the popliteal vein (n = 2). The main outcome measures were clinical classification of chronic venous disease in 10 patients and air plethysmography in seven patients. RESULTS: Clinical classification was C(0A) in 6 patients, C(3A) in 1 patient, C(3S) in 2 patients, and C(4S) in 1 patient. Venous claudication with uncontrollable edema was observed in two patients with C(3S) disease. Pain and itching with inflammatory skin changes were observed in one patient with C(4S) disease. These three patients had undergone resection of the femoral vein, including the deep femoral vein along with proximal adductor muscles. Air plethysmography revealed that the ejection fraction was significantly lower and the residual volume fraction was significantly higher in the three patients with symptoms than in symptom-free patients. CONCLUSIONS: Significant chronic venous disease was observed in the patients who underwent combined resection of the femoral vein, the deep femoral vein, and the adductor muscles for a tumor.     

Primary superficial femoral vein leiomyosarcoma: Report of a case

The majority of leiomyosarcomas occur in the muscular layers of the gastrointestinal tract and uterus. Primary leiomyosarcomas rarely arise in the veins of the lower extremities. Primary leiomyosarcoma of the superficial femoral vein is extremely uncommon. We herein present a case of a 69-year-old man with a primary leiomyosarcoma of the superficial femoral vein of his left thigh, which manifested as an anteromedial palpable painless mass in the middle third of his left thigh. Duplex ultrasonography revealed a 4 × 8-cm mass attached to the left superficial femoral vein. The mass was removed surgically en bloc. Histological examination showed a low-grade leiomyosarcoma. Seven months after the resection, a local recurrence of the tumor was observed, and the patient underwent another surgery. Five years after the second operation the patient remains free of illness.     

Primary leiomyosarcoma of the innominate vein

Primary venous leiomyosarcoma is rare. We report the case of a primary leiomyosarcoma of the left innominate vein, with neoplastic thrombus extending into the left jugular and subclavian veins. The tumor was curatively resected en bloc with anterior mediastinal and laterocervical lymphatics, through a median sternotomy prolonged into left cervicotomy. Primary venous sarcomas may be associated with prolonged survival in individual cases, with curative resection recommended as the standard treatment, in the absence of distant spread.     

Early Vein Reconstruction and Right-to-Left Dissection for Left-Sided Pancreatic Tumors with Portal Vein Occlusion

Large left-sided pancreatic tumors are frequently associated with portal vein (PV) and/or superior mesenteric vein (SMV) occlusion. Traditionally, vein reconstruction is deferred until after removal of the tumor. However, division of venous collaterals, as is done in a typical left-to-right fashion, leads to progressive portal hypertension and increased risk of variceal hemorrhage during the dissection. Conversely, early PV/SMV resection and reconstruction restores mesenteric-portal flow and decompresses varices, thereby enabling a safer and easier right-to-left pancreatic resection. This “How I Do It” report describes the technique and advantages of a “reconstruction-first” approach for large left-sided pancreatic tumors with venous involvement and left-sided portal hypertension.     

Primary great saphenous vein leiomyosarcoma: Report of a case

Leiomyosarcomas rarely arise in primary veins, especially the great saphenous vein. We have found only 20 case reports of leiomyosarcoma arising in the great saphenous vein, most of which manifested as nonspecific symptoms of advanced disease, such as a palpable mass, swelling, and back or abdominal pain. We report the case of greater saphenous vein leiomyosarcoma diagnosed in a 48-year-old man with a 4-month history of an inguinal mass. Ultrasonography and computed tomography showed a 6-cm mass attached to the right superficial femoral vein. Fine-needle aspiration biopsy confirmed that it was a vascular sarcoma. At the time of surgery there was no evidence of distant metastasis; therefore, we removed the tumor en bloc along with the sartorius muscle, inguinal lymph nodes, and 10 cm of the common femoral vein, and replaced the femoral vein with a polytetrafluoroethylene graft. A pathological examination revealed poorly differentiated leiomyosarcoma of the great saphenous vein, involving the deep femoral vein, without lymph node involvement. During follow-up, a thrombosis of the prosthesis developed, followed by proximal stenosis, which was treated successfully with percutaneous transluminal angioplasty. The patient was found to have lung metastases 25 months after surgery and he died about 5 months later.     

Complex venous and arterial reconstruction with deep vein after pelvic exenterative surgery: a case report

Effective local regional control for solid tumor malignancies is dependent upon prerequisite surgical excision with negative margins. Invasion of contiguous adjacent structures, particularly in anatomical spaces of limited size, may preclude the surgical goal of histologically negative margins. From a historical perspective, the invasion of vascular structures in the pelvis has proved to be a significant limitation in achieving local regional disease control with surgical resection. In recent years, advances in the field of vascular surgery have caused us to reassess the historical criteria of resectability when blood vessels are focally invaded by malignancy. En bloc resection of adjacent vessels has been reported primarily with head and neck extirpations and increasingly with hepatobiliary and pancreatic lesions. In many cases, where the local vasculature is invaded or impinged by the tumor, venous structures are often ligated while arteries are bypassed with an appropriate conduit. In pelvic exenterative surgery, significant morbidity from chronic limb edema and deep venous thrombosis may result from the ligation of larger veins. This is especially the case when multiple major venous structures such as the common and the external iliac vein, as well as the hypogastric vein, are all simultaneously interrupted. To the best of our knowledge, there is no prior report examining venous reconstruction using the femoral vein for pelvic exenterative surgery requiring major iliac artery and venous resection. Herein we describe a case of a complex vascular reconstruction after pelvic exenterative surgery in a patient with recurrent rectal cancer invading multiple adjacent contiguous structures, including the iliac vessels.     

Leiomyosarcoma of the external iliac vein

Leiomyosarcoma of the iliac vein is an uncommon tumor. We report a case of a 63-year-old Japanese woman with leiomyosarcoma of the right external iliac vein. The patient complained of right inguinal pain and swelling. Computed tomography demonstrated a mass surrounding the right external iliac artery and vein. Metastases in the lungs and liver were found. Complete resection of the tumor along with the involved vessels was performed. Polytetrafluoroethylene grafts were used to reconstruct the vessels. Pathological examination revealed leiomyosarcoma of the external iliac vein. Although the prognosis of leiomyosarcoma is poor, en bloc tumor resection is the treatment of choice.     

腔静脉内肿瘤六例诊治分析

目的 探讨腔静脉内肿瘤的诊断和手术治疗. 方法回顾性研究近年来收治的6例腔静脉内肿瘤患者的临床资料,包括患者首发症状、主要临床表现、手术治疗过程和预后,对比研究B超、CTA、MRA等诊断方法.结果 6例患者术前均行B超检查;2例经CTA、4例经MRA确诊;累及心脏3例.均采取手术治疗,根据肿瘤生长范围,3例采取胸腹联合切口,于心包外阻断下腔静脉;3例体外循环下腹部正中切口和正中开胸切口,打开右心房;所有病例的肿瘤均完整切除且发现双瘤体1例.病理报告平滑肌瘤4例,平滑肌肉瘤2例.1例平滑肌肉瘤患者因术后肝功能衰竭死亡,其余术后均恢复良好.平均随访51个月未发现复发. 结论 B超、CTA、MRA对腔静脉内肿瘤均有诊断价值,MRA能提供多方位更清晰的解剖关系,有助于手术方法的选择.外科手术是腔静脉内肿瘤主要治疗方法,术中应探查腔静脉及髂总静脉的主要属支,盆腔来源肿瘤可以结扎髂内静脉,以预防复发.     

Iliofemoral venous obstruction without thrombosis

Nonthrombotic iliofemoral venous obstruction, masquerading as deep vein thrombosis, was diagnosed in four patients. In each instance the patient was hospitalized and intravenous heparin therapy was started. Phlebography demonstrated venous outflow obstruction without thrombosis; subsequent CT scanning revealed an obstructing lesion in each case. At surgical exploration, (1) endoaneurysmorrhaphy of a hypogastric artery aneurysm decompressed an obstructed right iliac vein; (2) a primary iliac vein leiomyosarcoma was extirpated; (3) a synovial cyst arising from the right hip joint, which obstructed the femoral vein, was excised; and, (4) a postherniorrhaphy inflammatory mass obstructing the left iliofemoral vein junction was confirmed with biopsy results. Improved diagnostic accuracy with its attendant specific therapy is achieved in suspected cases of iliofemoral vein thrombosis if, in addition to noninvasive venous studies or phlebography, CT scanning of the abdomen and pelvis is performed.     

Robot-assisted laparoscopic retroperitoneal leiomyosarcoma resection with inferior vena cava graft replacement: a case report

The aim of the present study was to report the initial clinical experience of robot-assisted laparoscopic retroperitoneal leiomyosarcoma resection with inferior vena cava graft replacement. The patient was a 45-year-old female with abdominal pain. She was referred to our hospital and found to be with a retroperitoneal mass (46 mm × 45 mm). The inferior vena cava and the distal part of left renal vein were invaded by the tumor and compression was obviously seen from magnetic resonance imaging. The serum level of potassium, epinephrine, norepinephrine, cortisol, adrenocorticotropic hormone and renin angiotensin aldosterone system were all in normal ranges before the surgery. The operation was performed via a six port, robot assisted, transperitoneal laparoscopic approach. The tumor was completely resected and adherent part of inferior vena cava (approximately 5 cm) was dissected. Considering severe impairment of the great vessel, we decided to replace excised caval segment with an extended polytetrafluoroethylene graft and undertook the inferior vena cava reconstruction. The patient was discharged 11 days postoperatively with embolus in the graft. Anticoagulants were routinely administrated and the thrombus seemed to be smaller 3 months after operation. Abdominal pain was resolved and pathological examination finally confirmed that the tumor was leiomyosarcoma with negative margins free from tumor. Leiomyosarcoma of inferior vena cava present a technical challenge to surgeons. Comprehensive preparation should be made preoperatively to facilitate tumor resection and vascular management. In specific cases, robotic resection of leiomyosarcoma from great vessels and vascular repairment might be feasible options in experienced hands.     

Inferior venacaval reconstruction with bovine pericardium, re-implantation of renal veins and right hepatectomy in a patient with recurrent venous leiomyosarcoma

Management of patients with venous leiomyosarcomas is difficult because of multiple reasons. In most patients symptoms occur late, adjuvant therapy is hardly effective, and surgical resection with caval replacement is a major operation with unpredictable long-term benefit. Our patient with type II leiomyosarcoma underwent Inferior Vena Cava (IVC) resection and reconstruction with a “neo IVC” fashioned out of bovine pericardial patch. In addition, she required reimplantation of both renal veins to the neo IVC, right hepatectomy and cholecystectomy. The specific venous perfusion strategies employed because of the juxta renal location of tumor and the use of bovine pericardium for IVC reconstruction are discussed.     

Two cases of pulmonary leiomyosarcoma

Pulmonary sarcoma is a rare tumor of the lung. There are 81 cases in the literature for review, including our two cases. Our first case who was a thirty-seven year old male who was admitted to our hospital with complaints of left back pain. His chest X-ray showed an 11 X 14 cm tumor shadow in the lower left lung area. A left upper lobectomy was performed, and the histological diagnosis was leiomyosarcoma of the pulmonary vein. He died due to mediastinal recurrence 29 months later. Our other case was a fifty-six year old female. Her chest X-ray showed an abnormal shadow that was a tumor shadow in the right central lung area of 4 cm in diameter. As the diagnosis of the transcutaneous lung biopsy was benign tumor, partial resection of lung was performed. The histological diagnosis was leiomyosarcoma of the bronchus, and she is alive and well at 29 months after the operation. As the rate of diagnosis in leiomyosarcoma detected by transcutaneous lung biopsy is low, the most acceptable method of diagnosis is a total examination, including cytology of sputum, bronchoscopic findings, and roentgenoscopy. Probe thoracotomy would also be necessary if malignancy could not ruled out. The treatment of lobectomy should include lymph node resection.     

Leiomyosarcoma of the renal vein: case report and review of the literature

Leiomyosarcomas of vascular origin is a rare and aggressive pathology, its presentation on the renal vein is infrequent, of which 29 cases have been reported world-wide. We describe a case of a 76 year-old woman, who presented with intermittent left flank pain, irradiated to the lumbar region of 6 months duration. A left radical nephrectomy with en-bloc resection of a para-aortic tumor was performed. Microscopic examination of the specimen reported a moderately differentiated leiomyosarcoma, originated in the wall of the renal vein. The patient received adjuvant chemotherapy and radiation therapy. The tumor recurred in the liver. After 24 months from the primary surgery, the patient is alive with evidence of multiple metastatic spread to the liver. She is now under palliative care.     

Primary smooth muscle tumors of venous origin.

Vein tumors are rare, difficult to diagnose, and usually malignant. We have encountered three: a leiomyoma of the jugular vein and leiomyosarcomas of the saphenous vein and inferior vena cava (IVC). The leiomyoma was lost to follow-up, the saphenous vein leiomyosarcoma survived nine years, and the leiomyosarcoma of the IVC is six months without recurrence. Half of venous leiomyosarcomas arise in the IVC, predominately in women over 50 years of age. Surgical excision is the treatment of choice since malignant or benign status cannot be determined operatively. Resection should include a segment of the original vessel. This poses problems in the IVC when the renal veins require sacrifice. Right renal vein interruption mandates nephrectomy. Edema following IVC resection is evaluated. The incidence is lower than anticipated when resection is for tumor if there is no history of phlebitis. The IVC was reconstructed with a composite autograft but this is not now recommended. Despite significant local recurrences or distal metastases, cure or long-term palliation can often be achieved. Radiation and chemotherapy do not improve survival or prevent recurrence.     

Percutaneous transvenous catheterization and embolization of vein of galen aneurysms

Seven cases of vein of Galen aneurysms treated by percutaneous transvenous endovascular occlusion of the aneurysmal vein are presented. In one case, the approach was via the femoral vein, and in the other six cases, by the jugular vein. All of the malformations were multipedicular and, additionally, in six of the seven there was an intervening arterial-arterial network between the posterior thalamoperforating arteries and the wall of the venous aneurysm. This fistulous network was interpreted as purely arterial and not as an associated arteriovenous malformation. For this reason, the transvenous approach was considered justified, and was performed without risk of hemorrhage caused by retrograde venous hypertension. Measurement of intra-aneurysmal pressure during the course of treatment allowed better understanding of the hemodynamics of the lesions, guided the amount of occlusion to be accomplished during each treatment session, and thus may have prevented the phenomenon of normal perfusion pressure breakthrough. The percutaneous transvenous approach offers all the advantages of the transtorcular approach but avoids surgery. Because of our excellent angiographic and clinical results--five complete and two partial occlusions, with favorable outcomes and no major complications--we believe that this technique is better for the treatment of multipedicular vein of Galen aneurysms than transarterial embolization or surgery.     

Gastric Venous Reconstruction After Radical Pancreatic Surgery: Case Report and Review of the Literature

Vascular resection during surgery for adenocarcinoma of the pancreas is being performed with increasing frequency in order to achieve an R0 resection. With increasingly radical operations come challenges for reconstruction. Generally, these are related to reconstruction of the portal vein; this is particularly true of long-segment vein involvement by the tumor, in which venous outflow from dependent organs can become compromised. We report the first case of left gastric vein to inferior mesenteric vein bypass during a radical total pancreatectomy with long-segment portal vein resection for pancreatic adenocarcinoma, performed to relieve severe gastric venous congestion.     

Postoperative radiographic findings following acoustic neuroma removal

This paper assesses the radiographic findings seen on early postoperative CT following acoustic neuroma resection. CT head scans were routinely obtained from 86 patients within 24 hours of tumor resection via a translabyrinthine or retrosigmoid approach. Repeat CT scans were performed in those patients with abnormal clinical symptoms. The abnormalities seen on postoperative CT included cerebellar hematoma (nine patients), cerebral and/or cerebellar infarction (six patients), CSF leak at the incision (two patients), subdural hematoma (two patients), hydrocephalus (one patient), and residual acoustic neuroma (two patients). An unexpected CT finding was ipsilateral temporal lobe lucency, suggesting venous edema, ischemia, and/or infarction in 16% (14/86) of patients. Overall, the clinical complication rate was 8%, and subclinical CT abnormalities were seen in 17% of patients. Temporal lobe venous edema, ischemia, or infarction is a complication of translabyrinthine resection of acoustic neuroma and is thought to be due to obstruction of an inferior temporal lobe draining vein. Some cases may be related to intraoperative interruption of the superior petrosal sinus or petrosal vein, and/or coagulation of the sigmoid sinus dural margins, interruption of an inferior temporal vein, or venous hypotension. Care in dealing with the superior petrosal and sigmoid sinuses at surgery is needed.     

Postoperative Radiographic Findings Following Acoustic Neuroma Removal

This paper assesses the radiographic findings seen on early postoperative CT following acoustic neuroma resection. CT head scans were routinely obtained from 86 patients within 24 hours of tumor resection via a translabyrinthine or retrosigmoid approach. Repeat CT scans were performed in those patients with abnormal clinical symptoms. The abnormalities seen on postoperative CT included cerebellar hematoma (nine patients), cerebral and/or cerebellar infarction (six patients), CSF leak at the incision (two patients), subdural hematoma (two patients), hydrocephalus (one patient), and residual acoustic neuroma (two patients). An unexpected CT finding was ipsilateral temporal lobe lucency, suggesting venous edema, ischemia, and/or infarction in 16% (14/86) of patients. Overall, the clinical complication rate was 8%, and subclinical CT abnormalities were seen in 17% of patients. Temporal lobe venous edema, ischemia, or infarction is a complication of translabyrinthine resection of acoustic neuroma and is thought to be due to obstruction of an inferior temporal lobe draining vein. Some cases may be related to intraoperative interruption of the superior petrosal sinus or petrosal vein, and/or coagulation of the sigmoid sinus dural margins, interruption of an inferior temporal vein, or venous hypotension. Care in dealing with the superior petrosal and sigmoid sinuses at surgery is needed.