Clinical experience with the treatment of retroperitoneal vascular leiomyosarcoma originating from large veins

Background Retroperitoneal vascular leiomyosarcoma (RVLMS) is an extremely rare disease in clinical practice, and it has poor prognosis. This article is to explore the diagnosis and treatment of RVLMS and present our experience. Methods Data of RVLMS patients were continuously collected in our hospital from August 2018 to February 2020: two males and two females with a median age of 56 (min–max = 33–61) years were included. Patients in whom paraganglioma could not be excluded were asked to take phenoxybenzamine before surgery. A multi-disciplinary team (MDT) meeting had been held and surgery was recommended. The operation procedures varied based on the tumor location, shape, and stage, and the core steps were "exposure of the retroperitoneum and tumor, identification of vital blood vessels, blocking the bloodstream, complete removal of the tumor and tumor thrombus, and release of blood flow". A Satinsky clamp was used to partially block the blood vessels. Follow-up was conveyed by revisits and phone calls. Results One patient underwent open surgery, and three patients underwent laparoscopic surgery, one of whom underwent conversion to open surgery. The procedures were finished successfully, with a median operative time of 314.5 (min–max = 224–467) mins. The median amount of intraoperative bleeding was 550 (min–max = 200–1500) ml, and three patients had transfusion during the operation. The mass was irregular in shape, with a median maximum size of 7.45 (min–max = 4.2–10.7) cm, and the pathological examination confirmed RVLMS, which has spindle-shape, high mitotic activity and atypia. One week after the operation, the median serum creatinine level was 85 (min–max = 70–99) µmol/L. The median follow-up time was 16 (min–max = 13–21) months, and 1 case reported asymptomatic recurrence. Conclusion Uncharacteristic manifestations and imaging features contribute to the problematic diagnosis of RVLMS. Comprehensive preoperative evaluation and careful surgical planning are essential. Multicenter research is needed in the future to reach a dominant consensus.

Chemoradiotherapy for Inoperable Carotid Body Leiomyosarcoma: A Case Report and Review of Literature

Vascular leiomyosarcoma is an extremely rare tumor and is associated with poor prognosis among leiomyosarcoma. Surgical resection remains the main treatment option. But outcome of definitive treatment with chemoradiotherapy in inoperable patients is not clear. Here, we report treatment and outcome of definitive chemoradiotherapy in a case of vascular leiomyosarcoma. A 64-year-old man with the initial presentation of pulsatile right neck mass was diagnosed with right carotid body leiomyosarcoma. He refused surgical intervention due to risk of carotid body injury and ischemic stroke. Successful tumor control was achieved with carboplatin-based concurrent chemoradiotherapy. Investigational liquid biopsy for circulating sarcoma cells was also performed to analyze drug sensitivity profile of this rare tumor. One year after treatment, the disease remained well controlled and there was no evidence of baroreflex failure or treatment-related late toxicities. To our best knowledge, this is the first case report of right carotid body leiomyosarcoma controlled with definitive concurrent chemoradiotherapy. The approach of personalized multi-modality treatment will be a focus of our future investigation.

Primary Leiomyosarcoma Of Inferior Vena Cava : An Uncommon Entity

Primary vascular leiomyosarcoma is a rare tumor that arises mainly from the inferior vena cava (IVC). It is usually a slow-growing progressive tumor. It shows mainly three type of growth patterns; 62% of cases demonstrate extraluminal pattern, 5% cases show intraluminal pattern, and 33% extra- and intraluminal growth patterns.

Vascular leiomyosarcoma of the femoral vein: an unusual cause of a femoral triangle mass

Lumps in the femoral triangle are common. The differential diagnosis includes lymphadenopathy and femoral hernias. Rarer causes of lumps are saphena varix, femoral artery aneurysm or a psoas abscess. We present a leiomyosarcoma of the femoral vein that presented as a femoral triangle mass.

A Rare Case of Vascular Leiomyosarcoma Originating from a Branch Vessel of the External Iliac Vein

Leiomyosarcoma arising from the external iliac vein is uncommon. This is a report of a 51-year-old Japanese man with venous leiomyosarcoma originating from a branch vessel of the left external iliac vein. The tumor was found during a medical examination, and the patient had no symptoms. Computed tomography showed a 72 × 49 mm mass adjacent to the left external iliac vein. The tumor was resected en-block along with ligation of the external iliac vein due to strong adhesion with the tumor. Histological examination showed venous leiomyosarcoma, and its origin was thought to be a branch vessel of the left external iliac vein. The patient has remained free from recurrence at 30 months after surgery.

Recurrence of Primary Vascular Leiomyosarcoma Five Years after Initial Diagnosis in the Lower Extremity

Primary leiomyosarcomas of vascular origin are rare tumors. They frequently arise within the inferior vena cava; however, the peripheral vein was also affected. To date, only a few hundred cases have been reported in the world literature. Although it is an extremely aggressive tumor, the symptoms may be unspecific, especially in the lower extremities. In this report, we present a case of primary vascular leiomyosarcoma, arising from the short saphenous vein, with symptoms mimicking thrombus in the initial diagnosis. The diagnosis of leiomyosarcomas was confirmed by standard H&E staining and immunohistochemical staining. Recurrence of the tumor has been observed five years after surgical treatment. Due to its rarity, experience in the management of this type of tumor is limited. The mainstay of treatment for these tumors is complete surgical resection. The purpose of the presented case is to discuss the clinicopathological features and management options of this tumor, under the light of the most recent literatures.

Primary leiomyosarcoma in the inferior vena cava- a case report

Vascular leiomyosarcoma of IVC is a rare tumor. Only about 200 cases have been reported worldwide. There is a strong predilection for females and middle segment of IVC is most commonly affected. The diagnosis is often challenging as patients present with non-specific complaints such as dyspnoea, abdominal discomfort, or back pain. A 43-year-old male presented with history of vague abdominal distention, discomfort, and dyspnoea in our hospital. Ultrasound of abdomen detected a mass in retro peritoneum with mixed echogenicity inseparable from IVC. CT Imaging examination of abdomen revealed a large elongated, heterogenous mass extending from right atrium to the confluence of common iliac veins. in IVC reaching up to inferior border of right atrium. Then USG guided biopsy done and Leiomyosarcoma of IVC was diagnosed by immunohistochemistry. Due to extensive involvement of intrahepatic segment and right atrium complete resection of mass was not possible, so radiotherapy followed by chemotherapy was started. Patient started deteriorating due to intolerability with poor response to radiotherapy and chemotherapy so he was put on supportive treatment.