A Large Vascular Leiomyoma Arising from the Superficial Femoral Artery: Case Report and Systematic Review.

OBJECTIVE: Vascular leiomyomas are rare begin tumor comprising mature vascular smooth muscles that originate in the tunica media of the blood vessels. Most of the tumors arise from the veins. Only a dozen cases of artery-arising vascular leiomyoma have been reported, most of which are presented as small nodules in the hand. METHODS: Here we report an interesting case of a large artery-arising vascular leiomyoma and perform a systematic review. RESULTS: A 55-year-old man complained a 6 × 3 cm firm, mobile, tender and pulseless mass in the medial region of his left thigh. The computed tomography showed a well-demarcated fusiform tumor encircling the superficial femoral artery and was enhanced significantly with contrast. The patency of the superficial femoral artery was intact. The magnetic resonance image exhibited slightly hypointense relative to skeletal muscle on T1-weighted images and a heterogeneous appearance on T2-weighted images. We performed the en bloc resection of the tumor and used a vascular graft to revascularize the artery through end-to-end anastomosis. After histopathological assessments, the tumor was diagnosed as an artery-arising vascular leiomyoma. We also performed a systematic review on artery-arising leiomyomas, discovering 21 cases. Most of the artery-arising vascular leiomyomas were small nodules (mean length: 2.4cm) and most of them were superficial solitary mass located in the hand (13 cases, 62%). Excision of the tumor was an effective treatment. The histological subtype of the artery-arising vascular leiomyoma in all cases was solid type. During the follow-up of each patient, there was no recurrence. CONCLUSIONS: Artery-arising vascular leiomyomas are extremely rare. Most of them are painless and locate in the hand. Their pathological subtype is solid type in all patients. Due to their begin nature, excision is a cure with little chance of recurrence.

Concomitant Aorto-Caval Reconstruction for Inferior Vena Cava Leiomyosarcoma.

BACKGROUND: Leiomyosarcoma of the inferior vena cava (IVC) is a rare smooth muscle neoplasm typically presenting in the fifth to sixth decades of life with both intraluminal and extraluminal growth patterns. Surgical resection remains the gold standard for nonmetastatic disease and often requires vascular reconstruction. We present an atypical case of leiomyosarcoma involving both the IVC and infrarenal abdominal aorta necessitating reconstruction with intraoperative veno-venous bypass. METHODS: A 63-year-old man initially presenting with back pain was found to have a large mass adjacent to the IVC on MRI, subsequently confirmed to be leiomyosarcoma by biopsy. After 6 months of neoadjuvant chemotherapy, the patient was taken for resection. However, intraoperatively the tumor was found to involve the aorta necessitating combined aorto-caval reconstruction. To facilitate en-bloc resection of the tumor, the aorta was reconstructed first followed by the inferior vena cava using veno-venous bypass. RESULTS: Postoperatively, the patient was taken to the intensive care unit for resuscitation and had an uncomplicated hospital course. He was discharged to rehab 6 days postoperatively and at one year remains free of significant tumor burden with patent aorto-caval bypass grafts. CONCLUSIONS: Primary leiomyosarcoma of the IVC is estimated to have aortic involvement in <10% of cases and concurrent aorto-caval reconstruction can be a well-tolerated option in good surgical candidates. Furthermore, veno-venous bypass can be a useful tool for accomplishing successful oncologic resections. With interdisciplinary collaboration between surgical oncologists, urologists, and vascular surgeons, difficult pathologies can be addressed with good patient outcomes.

Primary leiomyosarcoma is a rare cause of femoral triangle mass: A case report.

OBJECTIVES: Primary leiomyosarcoma in the femoral vein is an extremely rare vascular disorder and is often misdiagnosed. In order to improve the knowledge and treatment of this disease, a case of leiomyosarcoma in the femoral vein was reported. METHODS: We report the case of a 58-year-old woman with a leiomyosarcoma of the femoral vein and treated successfully by surgical resection. After surgery, no recurrence had been noted follow-up.Subsequently, we reviewed information derived from 31 previously published cases from 1954 to 2019. RESULTS: A total of 31 patients diagnosed with femoral vein leiomyosarcoma were presented in previous studies; in all of these patients, 14 (45.2%, 14/31) patients were men. Of these, the median age was 51.6 years old (ranging from 3 to 84). CONCLUSIONS: Complete resection with adjuvant chemotherapeutic was the main strategy to treat the disease. Prognosis remains poor if metastasis was present, especially pulmonary metastasis.

Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature.

Primary vaginal leiomyosarcoma is a rare gynecological malignancy. The clinical presentation is a benign looking well circumscribed mobile mass which might however occasionally present with distant metastases. Post treatment recurrence is common, and the clinical course is often unpredictable. Primary surgical management plus radiotherapy is the commonly practiced treatment of choice. We report a case of primary vaginal epithelioid leiomyosarcoma that recurred twice after local surgical resection. The patient subsequently underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a recurrent vaginal leiomyosarcoma. Histopathology of the vaginal mass revealed an epithelioid leiomyosarcoma of the vagina. She also received a course of adjuvant radiotherapy. The patient was free of recurrence at 3 year follow up. Vaginal mass must be evaluated with a high index of suspicion for malignancy. Local surgical resection alone is insufficient for primary vaginal leiomyosarcoma. Surgical resection with adjuvant radiotherapy offers better treatment outcomes with decreased risk of recurrence. Empirical oophorectomy in patients with completed family size might confer additional benefit in preventing disease recurrence in resource limited settings where testing for estrogen and progesterone receptor status of the tumour is unavailable. Due to the unpredictable course of the disease, lifelong patient follow-up is critical for better outcomes.

Successful Resection of a Retrohepatic Inferior Vena Cava Primary Leiomyosarcoma with Atrial Thrombus Extension in a 30-Week Pregnant Woman.

Leiomyosarcomas are an uncommon malignant subset of tumors accounting for approximately 20% of soft tissue sarcomas. Primary vascular leiomyosarcomas (PVLs) are a rare subset of leiomyosarcomas that may originate in the arterial or venous circulation but most commonly affect the inferior vena cava (IVC). PVLs more commonly affect women to men in a 2:1 ratio and most frequently occur in the fourth to sixth decades of life. Few reports have described this infrequent pathologic state in the setting of advanced pregnancy. Presented is a case of a 44-year-old 30-week pregnant woman who presented with a PVL of the retrohepatic IVC, which was complicated by occlusion of the IVC and tumor thrombus extension into the hepatic veins and right atrium. Herein, we describe our multidisciplinary management of this rare problem with successful surgical resection of her tumor and IVC reconstruction.

Leiomyosarcoma of the Great Saphenous Vein: Case Report and Literature Review.

Vascular leiomyosarcomas are extremely rare, and involve veins 5 times more often than arteries. In the lower extremity, the great saphenous vein is the most frequent site of origin. We report the case of a healthy 45-year-old patient who presented with a painless mass located proximally in the right thigh in line with the great saphenous vein. At surgery, the treatment consisted of wide local excision. Histopathological studies confirmed mass as leiomyosarcoma. In light of this report, we draw attention to this rare disease, though with a poor prognosis.

Resection of a Perirectal Leiomyosarcoma via a Posterior Transcoccygeal Approach.

INTRODUCTION: The management of perirectal tumors often requires rectal wall resection, and sometimes a complete proctectomy is required. Access to posterior perirectal masses via a posterior, transcoccygeal approach (Kraske procedure) avoids dissection of the intraperitoneal rectum. PATIENT: The patient was a 63-year-old male who presented to his primary care physician with debilitating perirectal pain of several months' duration. He did not respond to therapy with pain medications and topical agents, and underwent a lateral internal sphincterotomy for what was thought to be an anal fissure, without relief prior to referral. Diagnostic workup showed a low signal intensity mass on magnetic resonance imaging (MRI), and biopsy revealed high-grade leiomyosarcoma with myxoid features. Staging workup included a contrast-enhanced computed tomography chest, abdomen and pelvis, flexible sigmoidoscopy and endoscopic ultrasound. A lytic lesion in his left ilium on MRI was found to be avid on fluorodeoxyglucose-positron emission tomography scan and was therefore consistent with oligometastatic disease. He received six cycles of adriamycin, cyclophosphamide and dacarbazine, with good response. The metastatic lesion was treated with 24 Gy of radiotherapy, while the primary tumor was treated with 50 Gy of radiotherapy. The patient underwent the Kraske approach with radical resection of the perirectal mass. The rectal wall was closed with interrupted silk sutures, and layered closure of incision over a drain was performed. An R0 resection was achieved. A laparoscopic diverting loop ileostomy to protect the rectal repair was performed. CONCLUSION: The Kraske approach allows for adequate resection, while avoiding the morbidity of the transabdominal approach, and allowing the patient to maintain a continent rectum.

Leiomyosarcoma of the Innominate Vein: A Smooth Muscle Criminal.

Leiomyosarcomas of vascular origin are extremely rare malignant tumors that can present a diagnostic challenge when they present as a mediastinal mass. Although they portend a poor prognosis with both aggressive local symptoms and metastatic disease, we present a rare case in which surgical en bloc resection of the tumor was curative.

Inferior vena cava reconstruction for leiomyosarcoma of Zone I-III requiring complete hepatectomy and bilateral nephrectomy with autotransplantation.

The inferior vena cava (IVC) is the most common site of leiomyosarcomas arising from a vascular origin. Leiomyosarcomas of the IVC are categorized by anatomical location. Zone I refers to the infrarenal portion of the IVC, Zone II from the hepatic veins to the renal veins, and Zone III from the right atrium to the hepatic veins. This is a rare presentation of a Zone I-III leiomyosarcoma. Fifty-two-years-old female with a medical history significant only for HTN was admitted to the hospital with bilateral lower extremity edema and dyspnea. Two-dimensional echo demonstrated a right atrial thrombus, extending into the IVC. On subsequent CT and MRI, a 15 cm mass was noted that began in the right atrium and extended into the IVC, with continuation below the renal veins to above the level of the confluence of the common iliac veins. The patient underwent a complete resection of the mass, replacement of the IVC with Dacron graft, total hepatectomy and bilateral nephrectomy, with liver and kidney autotransplantation. Pathology was consistent with a high grade spindle cell sarcoma of vena cava origin. Patient was readmitted approximately 4 weeks postoperatively to begin adjuvant chemotherapy. This case represents a zone I-III IVC leiomyosarcoma treated with surgical R0 resection. This included a hepatectomy, bilateral nephrectomy, and hepatic and left renal autotransplantation. These complex tumors should be treated with surgical resection, and require a multidisciplinary approach.

Leiomyosarcoma of the thoracic aorta in a patient with hypertrophic cardiomyopathy.

We report a case of leiomyosarcoma of the thoracic aorta in a 49-year-old male patient with history of hypertrophic cardiomyopathy. The only presenting symptom was back pain localized under the left scapula with the frequency and severity of the pain increasing with time. Imaging studies detected the presence of an aortic tumor. The tumor was excised en bloc, and an interposition graft was implanted. The histology showed a fully excised grade 3 leiomyosarcoma. This article discusses features of this rare condition.

Spiral saphenous vein graft for major pelvic vessel reconstruction during exenteration surgery.

This article describes a great saphenous vein spiral graft technique for reconstruction of iliac vessels after en bloc resection during pelvic exenteration. Use of different size syringes as a scaffold allows the surgeon to construct autologous vascular interposition conduits of variable diameter to match the luminal size of the vessel requiring reconstruction. Autologous vascular grafts are preferred in exenteration surgery in which the operative field is commonly contaminated by concomitant bowel resection, which carries an increased risk of graft infection.

Surgical management of leiomyosarcoma of the inferior vena cava.

The optimal surgical management of patients with leiomyosarcoma of inferior vena cava remains a controversy. From 1975 and 2009, five patients with leiomyosarcoma of inferior vena cava were treated at the Chinese PLA General Hospital and Beijing Shijitan Hospital. The age ranged 39-61 years and the duration of symptoms ranged from 18 to 36 months. Abdominal and back pain are the most common complaints. A combination of various imaging modalities is essential for treatment planning. R0, R1, R2, and biopsy only were accomplished in 2, 1, 1, and 1 case, respectively. Combined resections included inferior vena cava, right kidney, adrenal gland, psoas, colon, duodenal, gallbladder, liver, and/or aorta, without inferior vena cava reconstruction. No inferior vena cava-related postoperative complication was seen in our series.

Efficacy and safety of trabectedin as an early treatment for advanced or metastatic liposarcoma and leiomyosarcoma.

AIMS: We aimed to evaluate the effect of prior chemotherapies on the outcomes of patients with liposarcoma and leiomyosarcoma treated with trabectedin as a 24-h infusion every 3 weeks. PATIENTS & METHODS: Data from 129 patients who received trabectedin as second-line treatment following failure with an anthracycline/ifosfamide and those who had received at least two lines of prior chemotherapy were analyzed. RESULTS: Forty seven patients received one prior regimen (group A) and 82 patients received at least two lines of chemotherapy (group B). A favorable trend in median time to progression (4.4 vs 3.0 months), progression-free survival (4.4 vs 2.6 months) and overall survival (17.4 vs 13.3 months) was found in group A. A trend toward higher overall response rate (6.4 vs 4.9%) and disease control rate (34.0 vs 26.8%) also favored group A. Both groups had equivalent safety profiles. CONCLUSION: All efficacy outcomes were better in patients who received trabectedin as second-line treatment compared with patients with more extensive prior therapy.

Rectus abdominis muscle resection and fascial reconstruction for the treatment of uterine leiomyosarcoma invading the abdominal wall: a case report.

The authors present a case of intra-abdominal recurrent leiomyosarcoma invading a large area of the abdominal wall. The patient underwent cytoreductive surgery, including resection of the rectus abdominis muscle, followed by reconstruction of the defect using synthetic mesh. The tumor was surgically removed by en bloc resection, including most of the rectus abdominis muscle and ileum. The abdominal wall defect was repaired using synthetic mesh. The patient underwent radiotherapy and chemotherapy after the surgery and was healthy one year later.

Left atrial leiomyosarcoma extending into the posterior mediastinum and mimicking a left atrial myxoma.

BACKGROUND: Intracardiac malignancies are extremely rare and hard to detect or differentiate preoperatively. CASE REPORT: We present a 48-year-old female patient who was diagnosed primarily with left atrial myxoma and taken into emergency surgery. The tumor extended into the pulmonary veins and infiltrated the atrial endocardium, and the histopathologic diagnosis was leiomyosarcoma. The left atrial endocardium was successfully peeled off with the tumor and complete resection was achieved. CONCLUSION: The possible malignant nature of intracardiac masses should be kept in mind, especially in middle-aged patients. The extent of the tumor must be determined in elective cases to establish the proper strategy for complete resection, which is the only chance of successful treatment for this lethal disease entity. Endocardial peeling is warranted for successful removal of the tumor mass in leiomyosarcoma.

Leiomyosarcoma of the splenic vein.

Leiomyosarcomas are smooth muscle-derived tumors generally found intra-abdominally in the retoperitoneum, mesentery, or omentum. Only approximately 5% of these tumors originate from vessel wall smooth muscle. Those derived from the splenic vein are exceedingly rare, with only one previously published case in the literature. We present a second case of leiomyosarcoma of the splenic vein in a 58-year-old woman with 2 months of epigastric pain. A distal pancreatectomy was performed to include the tumor found centered in the splenic vein at the splenic and portal vein confluence and growing into the pancreas in the body on the posterior aspect. A saphenous vein patch was used for reconstruction.

Technical considerations for radical resection of a primary leiomyosarcoma of the vena cava.

BACKGROUND: Radical resection provides the best hope for cure in leiomyosarcoma of the inferior vena cava (IVC). Multi-visceral resection is often indicated by extensive tumour involvement. This report describes the technical challenges encountered during resection of a retrohepatic IVC leiomyosarcoma. METHODS: Computed tomography showed an IVC leiomyosarcoma measuring 7.8 × 10.0 × 19.3 cm in a 41-year-old patient. The tumour reached the confluence of the hepatic veins, displacing the caudate lobe anteriorly and extending towards the IVC bifurcation inferiorly. En bloc resection of the IVC tumour with a right hepatic and caudate lobectomy, and a right nephrectomy was performed. RESULTS: Subsequent to a Cattel manoeuvre, the operative procedures carried out can be broadly categorized in four major steps: (i) mobilization of the infrahepatic IVC and tumour; (ii) mobilization of the suprahepatic IVC from diaphragmatic attachments; (iii) right hepatectomy with complete caudate lobe resection, and (iv) en bloc resection of the IVC tumour. This approach allowed the entire length of tumour-bearing IVC to be freed from the retroperitoneum and avoided the risk for iatrogenic tumour rupture during dissection at the retrohepatic IVC. Reconstruction of the IVC was not performed in the presence of venous collaterals. CONCLUSIONS: Experience in liver resection and transplantation, and appreciation of the hepatocaval anatomy facilitate the safe and radical resection of retrohepatic IVC leiomyosarcoma.

Prognostic factors and outcomes in 28 cases of uterine leiomyosarcoma.

OBJECTIVES: It was the aim of this study to evaluate clinicopathological characteristics and prognostic factors of uterine leiomyosarcomas (LMS). METHODS: Twenty-eight patients with uterine LMS were evaluated in this retrospective study. Their features and survival were analyzed by Kaplan-Meier and log-rank tests. RESULTS: The median age of the patients was 52 years (range 25-74). Nine patients had a disease with a mitotic count <10/10 high-power fields. Twenty-one patients presented with stage I disease, 1 with stage II and 6 with stage IV. Twelve patients underwent total hysterectomy and bilateral salpingo-oophorectomy, 2 simple hysterectomy, 5 myomectomy and 9 more comprehensive surgical treatments. Adjuvant chemotherapy was administered to 16 patients, whereas chemoradiation was given only to 2 patients. Fifty percent presented with recurrence of the disease. The median overall survival was 46 months. Age, mitotic count, type of surgery, adjuvant therapy, recurrence and clinical response to chemotherapy were not found to affect survival, while the menopausal status and FIGO (International Federation of Gynecology and Obstetrics) stage were found to be prognostic factors. CONCLUSION: In our series, the menopausal state and FIGO stage were found to be prognostic factors related to survival.

Leiomyosarcoma of the renal vein.

A 43-year-old woman was referred to our clinic for evaluation of a left retroperitoneal mass. She presented to our internal medicine department complaining of back pain. Computed tomography (CT) scan revealed a left retroperitoneal mass 55 mm in size in the hilum of the left kidney. Enhanced CT scan and magnetic resonance imaging (MRI) disclosed a poorly staining mass. Metaiodobenzylguanidine scintigraphy demonstrated no accumulation in the mass; moreover, endocrinologic examination was normal. Laparoscopic resection of the left retroperitoneal tumor was attempted; however, strong adhesion between the tumor and the left renal vein was encountered. Thus, left nephrectomy after open conversion was performed. Histological findings indicated leiomyosarcoma originating from the left renal vein. The postoperative course has been uneventful; neither recurrence nor metastasis is evident 2 years postsurgery.