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Pseudomyogenic hemangioendothelioma (PMH) is a rare vascular tumor that occurs in young mostly male patients. Seventy percent of PMH cases are multifocal and 25% involve bones. PMH is an indolent tumor with mild local aggressiveness and an unclear pathology. Only two cases of spontaneous regressive bone PMH have been reported. Here, we report the case of a 17-year-old boy with a multifocal bone PMH diagnosed from a chronic pain in his left knee. The PMH affected the right scapula, both humeri, the right olecranon, the second metacarpal bone, the second and fourth right ribs, the thoracic and lumbar spine, the pelvic ring, the left and right femoral neck, and the left patella. Every lesion presented with a lobulated, lytic pattern, sometimes with a peripheral sclerotic rim. MRI showed a tissue lesion with a low intensity on T1-weighted sequences and high intensity on T2-weighted sequences. Enhancement of T1 gadolinium fat-saturated sequences was bright. After discussion, a national specialized board decided to actively monitor the patient and start general chemotherapy in the case of progression. The disease was stable at 3 and 6 months and showed signs of regression at 1 year, which was further confirmed at 2 years. CT scan and MRI highlighted a progressive filling of the tumor with cancellous bone and a regression of the tissue contingent. This case report highlights to a new therapeutic approach for indolent PMH that does not prevent further treatment in the case of progression.

Anastomosing hemangioma (AH) is a rare, benign vascular neoplasm with distinctive histopathology and characteristic tumor distribution. AHs show marked proclivity to involve the kidneys, gonads and the retroperitoneal soft tissues; kidney is the most common target site often in the context of end stage renal disease. Recent studies have identified activating mutations of GNA genes that drive the molecular pathogenesis of AHs. AH appears as a solitary, well-circumscribed, hypervascular tumor that charters a benign course with an excellent prognosis. The purpose of this article is to provide a current update on clinical, pathological and imaging features of anastomotic hemangioma.

Retroperitoneal tumours arising from the inferior vena cava (IVC) are rare tumours often requiring large vessel resection for complete surgical excision. Limited exposure to such tumours often discourages surgeons from offering surgical resection to these patients, depriving them of the only potentially curative modality. We present here the surgical technique for resection of a large IVC sarcoma without IVC reconstruction.A 53-year-old lady presented with a large retroperitoneal sarcoma encasing the infra-hepatic IVC with tumour thrombus extension into the hepatic cloaca as well as the left renal vein. Surgical resection was planned as the disease remained stable after 2 cycles of neoadjuvant chemotherapy with adriamycin and ifosfamide.Complete surgical excision of the tumour was achieved by performing a resection of the entire length of infra-hepatic IVC and right kidney, without IVC reconstruction. Left renal vein was divided after careful preservation of a draining collateral. Tumour thrombus was extracted from the hepatic cloaca, and proximal IVC stump closure was achieved with preservation of right hepatic vein insertion. Total blood loss during the procedure was 2300 mL, and the patient recovered without compromise of renal function or development of lower limb oedema.IVC resection without reconstruction can be safely performed for large retroperitoneal sarcomas involving major vascular structures. Familiarity with the retroperitoneal, retro-hepatic and supra-hepatic anatomy is paramount to achieving good surgical outcomes.

Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign non-neoplastic vascular lesion. A typical presentation consists of a subcutaneous nodule that may simulate other clinical entities. Presentation in the oral cavity is uncommon. It is thought to develop as an abnormal proliferative reaction of endothelial cells in a process of impaired thrombogenesis. When endothelial proliferation occurs, a differential diagnosis with a soft tissue sarcoma, in particular an angiosarcoma, should be performed. We report a case of a 68-year-old female patient who presented with a lesion on the upper lip of 3 months' duration. Surgical resection revealed an IPEH. 1 year later, the patient showed a local recurrence requiring excision with clear margins. Pathological and immunohistochemical features can help us distinguish these lesions from those requiring more aggressive treatment. The gold standard is surgical resection with clear margins. Accurate preoperative diagnosis is essential to avoid overtreatment. Emphasis should be placed on clinical, radiological and histological studies.

Inferior vena cava (IVC) leiomyosarcomas (LMS) are a rare group of retroperitoneal tumors. R0 surgical resection is the only curative modality of treatment. IVC resection for retroperitoneal sarcoma is a complex surgery with no definitive guidelines for reconstruction.Retrospective review of all patients who underwent surgical resection of primary leiomyosarcoma of the IVC requiring resection from 2010 to 2020 at our tertiary care center was performed.Among 24 patients who required IVC resection for LMS, only 7 (29%) required reconstruction of IVC. According to Clavien-Dindo classification, there was one grade 3 or more morbidity and 1 post-operative mortality. Seventeen patients underwent R0 resection whereas 7 patients had R1 resection on final histopathology. At a median follow-up of 25 months (range 8-91 months), the median OS was 40 months with median DFS of 28 months. Two patients presented with local recurrence while 13 patients developed systemic recurrence on follow-up.Careful preoperative multidisciplinary planning can make IVC resection without reconstruction feasible with acceptable perioperative morbidity, mortality, and oncological outcomes for IVC LMS.

Epithelioid hemangioma is a rare, histologically benign but locally aggressive primary vascular neoplasm that can rarely arise in bone. Mainstay treatment is surgical resection or curettage with bone grafting. We report a novel multidisciplinary, joint-sparing treatment approach for an epithelioid hemangioma of bone arising in the acetabulum causing severe thinning of the subchondral bone plate. After 4 sessions of transarterial bland particle and ethanol embolization, the resultant increased ossification of the tumor allowed preservation of the articular surface during surgical resection. Imaging follow-up 14 months after surgical resection showed no evidence of recurrence and continued ossification of the portions of the lesion treated only with embolization.

Background Intravascular papillary endothelial hyperplasia (IPEH) is a vascular tumor characterized by the proliferation of endothelial cells with papillary formation. It is a rare benign condition affecting the head and neck. Currently, no cases of IPEH of the spleen have been reported. Here, we report a case of IPEH of the spleen in a child and discuss its clinical manifestations, imaging features, and surgical treatment. Case presentation A 5-year-old female presented with a 4-month-old tumor in the left upper abdomen, abdominal pain, and constipation. She underwent radiography, barium enema, US, and MRI. A solid space-occupying mass was found in the left abdominal cavity on preoperative imaging, and it was diagnosed as angiosarcoma. The lesion was surgically resected. Histopathological analysis was consistent with IPEH. Conclusion Clinicians should consider the possibility of IPEH in patients presenting with tumors in the spleen, which is curable by surgical resection. Malignant vascular tumors must be excluded in the differential diagnosis of IPEH to prevent misdiagnosis and inappropriate overtreatment.

Background Malignant peritoneal mesothelioma (MPM) is a rare malignant tumor with a high mortality rate and extremely poor prognosis. In-depth pathological analysis is essential to assess tumor biological behaviors and explore potential therapeutic targets of MPM. Nucleoplasmin 2 (NPM2) is a molecular chaperone that binds histones and may play a key role in the development and progression of tumors. This study aimed to analyze the correlation between the expression level of NPM2 and the main clinicopathological characteristics and prognosis of MPM. Methods Ninety-two postoperative specimens from MPM patients following cytoreductive surgery were collected. Postoperative specimens were stained with immunohistochemistry. The expression level of NPM2 was quantitatively analyzed by QuPath-0.3.2 software. Univariate and multivariate analyses were conducted to investigate the correlation between NPM2 expression and other conventional clinicopathological characteristics. Results Among the 92 MPM patients, there were 47 males (48.9%) and 45 females (51.1%), with a median age of 56 (range: 24–73). There were 70 (76.0%) cases with loss of NPM2 protein expression, 11 (12.0%) cases with low expression, and 11 (12.0%) cases with high expression. Univariate analysis showed that NPM2 protein expression level (negative vs. low expression vs. high expression) was negatively correlated with the following three clinicopathological factors: completeness of cytoreduction (CC) score, vascular tumor emboli, and serious adverse events (SAEs) (all P < 0.05). Multivariate analysis showed that NPM2 protein expression level (negative vs. low expression vs. high expression) was independently negatively correlated with the following two clinicopathological factors: CC score [odds ratio (OR) = 0.317, 95% CI: 0.317–0.959, P = 0.042] and vascular tumor emboli (OR = 0.092, 95% CI = 0.011–0.770, P = 0.028). Survival analysis showed that loss of NPM2 protein expression (negative vs. positive) was associated with poor prognosis of MPM. Conclusions Loss of NPM2 expression is a potential immunohistochemical marker for MPM.

Although central nervous system (CNS) metastases are common in advanced cancer, CNS involvement solely by intravascular tumor cells, known as intravascular carcinomatosis, is extremely rare. We report two cases of brain metastasis in which tumor cells were restricted to the vascular lumina without parenchymal involvement, resulting in ischemic lesions. The first patient is a previously healthy young woman who presented with symptoms of community-acquired pneumonia and progressed to respiratory failure. Computed tomography of the brain showed infarcts of differing ages. At autopsy, she was found to have widely metastatic cervical squamous cell carcinoma and cerebral tumor emboli with multifocal infarcts, mainly microinfarcts. The second patient is an elderly man with cognitive impairment and mild Parkinsonism who presented with symptoms of a urinary tract infection. Magnetic resonance imaging of the brain showed atrophy and changes suggestive of chronic microvascular ischemic disease. Postmortem examination demonstrated prostatic adenocarcinoma and cerebral tumor emboli with multifocal infarcts. These cases illustrate that this pattern of intracranial metastasis may rarely be a cause of cerebral ischemic lesions and emphasize the importance of thorough pathologic examination of the brain.

Whether adjuvant chemotherapy (AC) for patients with upper tract urothelial carcinoma (UTUC) offers survival benefit is still controversial. To explore the impact of AC on overall survival (OS) of cN0M0 UTUC patients, we conducted a propensity score-matched analysis using the regression model, including pathologic features such as lymphatic and vascular invasion.A multi-institutional cohort of 413 UTUC patient record was used. Propensity score matching was performed to reduce bias by potential confounding factors for survival, including pathologic features from the specimen of radical nephroureterectomy (RNU), RESULTS: Ninety-eight patients were identified as pair-matched groups (49 patients in RNU and 49 patients in RNU + AC). Kaplan-Meier curves demonstrated that a 5-year OS rate of 72.7% for patients treated with RNU + AC was significantly higher than 51.6% for those treated with RNU (p = 0.0156). On multivariate analysis, pathologic vascular invasion (HR 3.41, 95% CI 1.24-10.66, p = 0.0166) and administration of AC (HR 0.45, 95% CI 0.19-0.98, p = 0.0438) still remained as the significant predictors for OS. In patients with pathologic vascular invasion (51 of 98 patients), a significantly longer OS in RNU + AC groups was observed (median OS of 30 and 70 months in RNU and RNU + AC groups, respectively: p = 0.0432), whereas there was no significant difference in the OS between RNU (median OS: not reached) and RNU + AC (median OS: not reached) groups in patients without the invasion (p = 0.4549).The result indicates a significant benefit for OS by the administration of AC, and pathologic vascular invasion in the specimen of RNU could help the patient selection to better predict the effect of AC.

Contains fulltext : 219900.pdf (Publisher’s version ) (Open Access) Perivascular and vascular neoplasms of the head and neck are a rare group of tumors comprising a spectrum of clinical/biologic and histological features. They are frequently diagnostically challenging, due to their morphologic and immunohistochemical overlap. In this review, we summarize the pathology of these neoplasms, discussing morphology, immunohistochemistry, associated genetic findings, and the differential diagnoses.

The purpose of this article is to provide an up-to-date overview on imaging of paediatric vascular soft tissue masses, including both neoplastic and non-neoplastic lesions. We describe the-often challenging-imaging diagnosis-mainly performed by ultrasound (and secondarily by MRI) and differential diagnosis of vascular soft tissue lesions in children. We underline how important it is to determine whether a vascular anomaly has a regional vascular origin, or if there are other entities, ranging from benign to malignant lesions, which have flow-signal or blood degradation products. Even though clinical examination and patient's history are the first and indispensable steps in the initial diagnosis, the role of imaging is crucial, not only to determine whether a mass represents a true tumour/pseudo-tumour, but also to achieve a more correct diagnosis and determine the extension of the tumour/pseudo-tumour and its relation with the nearby anatomic structures.

There are few reports on microvascular invasion (MVI) located intra- or extratumorally and prognosis of hepatocellular carcinoma (HCC). The aim of this study was to evaluate patient outcome according to the location of MVI, and to build a nomogram predicting extratumoral MVI. We included 681 consecutive patients who underwent hepatic resection (HR) or liver transplantation (LT) for HCC from January 1994 to June 2012, and evaluated patient outcome according to the degree of vascular invasion (VI). A nomogram for predicting extratumoral MVI was created using 637 patients, excluding 44 patients with macrovascular invasion, and was validated using an internal (n = 273) and external patient cohort (n = 256). The 681 patients were classified into four groups based on pathological examination (148 no VI, 33 intratumoral MVI, 84 extratumoral MVI, and 29 macrovascular invasion in patients who underwent HR; 238 no VI, 50 intratumoral MVI, 84 extratumoral MVI, and 15 macrovascular invasion in patients who underwent LT). Multivariate analysis revealed that extratumoral MVI was an independent risk factor for overall survival in patients who underwent HR (hazard ratio 2.62, p

In view of a recent meta-analysis reporting increased mortality following angioplasty with paclitaxel-coated devices in peripheral arteries, we performed a patient-level 2-year mortality analysis based on pooled original data of four randomized controlled trials (THUNDER, FEMPAC, PACIFIER and CONSEQUENT). Clinical data of four randomized controlled trial were pooled to assess 2-year mortality following paclitaxel-coated balloon (PCB) angioplasty compared to angioplasty without paclitaxel (control group). A logistic regression model was applied to identify potential predictors of mortality. At two years, 13 of 185 (7.0%) patients had died in the control group and 16/184 (8.7%) in the PBC group, p = 0.55. Kaplan–Meier analysis revealed no significant difference from all-cause death at 2 years (log rank p = 0.54). Causes of death were well balanced between the groups with no pattern or trend in favour of any specific causes in the PBC group. Logistic regression revealed that treatment groups (controls or PBC) were not a predictor of 2-year mortality. The only predictor for mortality was patient age ≥ 75 years. The delivered paclitaxel doses per patient were not significantly different in patients that died and those who did not die during the 24-month follow-up (5.300 ± 4.224 μg vs. 6.248 ± 4.629 μg, p = 0.433). Based on original patient-level data of four pooled randomized controlled trials, we found no increase in 2-year mortality in patients treated with PCB compared to control patients treated with uncoated balloons. Causes of death were well balanced between PCB and control patients.

Primary pulmonary artery sarcoma is a rare tumor originating from the pulmonary artery tree. Given the low incidence, few centers have reported on more than a handful of cases. Because of its rarity it is also commonly misdiagnosed as pulmonary embolism. Multi-modality diagnostic imaging and recognition of specific imaging characteristics along with a high index of suspicion is required to make the correct diagnosis and expedite treatment. The primary imaging modality for most cardiac tumors such as primary pulmonary artery sarcoma is now MRI. It provides superb spatial resolution as well as functional assessment of the heart and pulmonary circulation. CT imaging also is part of routing imaging and remains as the most pertinent imaging modality to evaluate the lung parenchyma and presence of metastatic disease. Here we review the pertinent imaging modalities and tissue characteristics that facilitate recognition of primary pulmonary artery sarcoma. We also provide a short overview of surgical resection and reconstruction, which is the mainstay therapy, for this rare tumor.

The aim of this review is to describe the preoperative evaluation, surgical techniques, and postoperative management of patients with renal cell carcinoma (RCC) undergoing radical nephrectomy (RN) and inferior vena cava (IVC) thrombectomy. RN and IVC thrombectomy remains the standard management option in non-metastatic RCC patients with IVC thrombus. A comprehensive preoperative workup, including high-quality imaging, blood works, and appropriate consultations are required for all patients. The aim of the surgery is complete resection of all tumor burden, which requires a skillful surgical team for such a challenging procedure and is inherently associated with a high rate of perioperative morbidity and mortality. Preoperative CT or MRI is essential for surgical planning. The surgical approach is mainly determined by the level of the tumor thrombus. The open approach has been the standard, though minimally invasive and robotic techniques are emerging in selected cases by experienced surgeons.

Background We aimed to explore the causal analysis, clinical characteristics and treatment strategies of laparoscopic conversion to open approach (LCTOA) in radical nephrectomy and tumor thrombectomy. Methods We included all patients with Mayo level I–III renal tumors with inferior vena cava (IVC) tumor thrombus who underwent laparoscopic radical nephrectomy and tumor thrombectomy as the first choice from May 2015 to July 2019. Results There were 70 cases of renal tumor with IVC tumor thrombus treated with a laparoscopic approach as the first choice; 31 Mayo level I, 30 Mayo level II, and 9 Mayo level III. A completely laparoscopic approach was performed in 51 cases (72.9%), and 19 cases (27.1%) underwent active or passive LCTOA. The LCTOA group had higher median preoperative serum creatinine (110.0 μmol/L vs 92.0 μmol/L; P = 0.026), longer postoperative hospital stay (9 days vs 7 days; P = 0.008), longer median operation time (374 min vs 311 min; P = 0.017), higher median intraoperative hemorrhage volume (1300 vs 600 ml; P = 0.020), and higher proportion of male patients (94.7% vs 66.7%; P = 0.016) vs the completely laparoscopic group, respectively. Although preoperative serum creatinine and gender were risk factors in the univariate analysis, multivariate analysis revealed no independent risk factors for LCTOA. We divided the reasons for LCTOA into active conversion and passive conversion; 4 (21.1%) cases underwent active conversion, and 15 (78.9%) underwent passive conversion. Most of the patients undergoing passive conversion had multiple concurrent risk factors, among which perirenal adhesion (30.9%), organ invasion (16.4%), and IVC adhesion (25.5%) were the most common. Fourteen (73.7%) cases underwent renal treatment, and 5 (26.3%) cases underwent tumor thrombus treatment. Conclusions The LCTOA group had a higher median preoperative serum creatinine concentration, longer hospital stay, longer median operation time, and higher median intraoperative hemorrhage volume. However, none of the predictors in our study was an independent risk factor for LCTOA. Perirenal adhesion, organ invasion, and IVC adhesion were the most common causes of LCTOA. Considering the limitations of this study, studies with large sample sizes are required to validate our conclusions.

We encounter patients with mediastinal tumors invading the left innominate vein (LIV), and there is no evidence confirming whether the LIV should simply be ligated or reconstructed. The need for postoperative anticoagulant therapy after ligation of LIV is also controversial. 3209 patients with thoracic malignant tumors underwent surgical resection between 1994 and 2014 in our institute. Nineteen (0.6%) patients had mediastinal malignant tumors invading the LIV and underwent LIV resection. Of these patients, only 3 underwent reconstruction of LIV. We did not start anticoagulant therapy routinely after resection of LIV. The patients were divided into 2 groups: group A showed at least 50% patency of LIV by preoperative contrast-enhanced computed tomography (CECT) and group B showed less than 50%. We investigated the safety of resecting LIV and the need for postoperative anticoagulant therapy. The 30-day and 90-day mortalities were zero in both groups. Thrombosis of the LIV stump and increased edema in the left neck and upper limb were observed in 2 (10.5%) patients only in group A. After initiating the anticoagulant therapy, the embolisms disappeared and weaning the patients off warfarin could be done in less than 1 year. In this study, there was no case of mortality or severe morbidity among the patients with LIV resection. Moreover, there was no need to initiate routine anticoagulant therapy after the LIV division as the frequency of embolism in the LIV stump was low and was expected to disappear prior to starting anticoagulant therapy.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor affecting infants and young children. Although benign, it can be associated with an aggressive locally growing tumor and/or a life-threatening Kasabach–Merritt phenomenon (KMP). To date, only reviews of limited cases have been performed. We, therefore, conducted a comprehensive literature search to collect relevant data and make recommendations for future treatment trials. Review of the available literature between 1993 and 2017 revealed a total of 105 publications involving 215 patients of less than 21 years of age. To this, we added 12 from our department and 4 from the Cooperative Weichteilsarkomstudie database. We found that KMP was present in 79% of the infants, in 47% of the 1–5-year olds, in 43% of the 6–12-year olds, and in 10% of the 13–21-year-old patients. KMP was present in nearly all (94%) patients with retroperitoneal tumors and in all patients with extra-regional tumors. The median size of a KHE without KMP was 12 cm2 as compared to 49 cm2 when associated with a KMP. With complete (not further classifiable if R0 or R1) resection, all patients were cured. If inoperable, response regarding KMP/regression of tumor size was seen in 29/28% with steroid-, 47/39% with vincristine-, 44/43% with interferon alpha-, 65/61% with anti-platelet agents-, and in 97/100% with sirolimus-containing therapies. Patients with progressive KHE should undergo resection whenever it is considered a safe option. If inoperable, sirolimus should be the first choice for treating KMP and reducing tumor size.

Intracranial malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. Previously reported cases of intracranial MPNST have been associated with development within a prominent cranial nerve. This is the first report of an MPNST with both nerve sheath and vascular phenotype that follows the neurovascular bundle, without arising in a major cranial nerve or in the setting of neurofibromatosis type 1 (NF1). The patient is a 14-year-old boy with a history of worsening headaches for the past several months, left-sided hearing loss, nausea, vomiting, and vertigo. MRI was performed that demonstrated a large extra-axial tumor compressing the left infratemporal posterior temporal region. The tumor was associated with significant destruction of the superior portion of the petrous bone and extension through the petrous into the upper posterior fossa, immediately below the tentorium. The patient underwent surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide. Pathology demonstrated a variant malignant peripheral nerve sheath tumor with both nerve sheath and vascular phenotype by immunostains. The patient’s symptoms improved following treatment. We present the first reported case of an intracranial MPNST variant that developed along the neurovascular bundle as a sarcoma with both nerve sheath and vascular phenotype through the petrous bone and not associated with a major cranial nerve or with stigmata of neurofibromatosis type 1 (NF1). Although this is an extremely unusual presentation due to location and lack of prominent cranial nerves in that location, it is not unusual for benign nerve sheath tumors to follow the neurovascular bundle through foramen of cortical long bone or pelvis. This case suggests that physicians should incorporate intracranial MPNST variant into their differential diagnosis in the cranium, even when tumor is not located near a prominent cranial nerve. Surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide has led to improvement in patient symptoms.

The proximal jejunal vein which branches from the dorsal side of the superior mesenteric vein (SMV) usually drains the inferior pancreatoduodenal veins (IPDVs) and contacts the uncinate process of the pancreas. We focused on this vein, termed the proximal dorsal jejunal vein (PDJV), and evaluated the anatomical classification of the PDJV and surgical outcomes in patients with pancreatic ductal adenocarcinoma (PDAC) with PDJV involvement (PDJVI). The jejunal veins that branch from the dorsal side of the SMV above the inferior border of the duodenum are defined as PDJVs. We investigated 121 patients who underwent upfront pancreaticoduodenectomy for PDAC between 2011 and 2017; PDJVs were resected in all patients. The anatomical classification of PDJV was evaluated using multidetector computed tomography. Surgical and prognostic outcomes of pancreticoduodenectomy for PDAC with PDJVI were evaluated. The PDJVs were classified into seven types depending on the position of the first and second jejunal veins relative to the superior mesenteric artery. In all patients, the morbidity and mortality rates were 15.7 and 0.8%, respectively. The rates for parameters including SMV resection, presence of pathological T3–4, R0 resection, and 3-year survival were 46.2, 92.3, 92.3, and 61.1%, respectively, when there was PDJVI (n = 13). When there was no PDJVI (n = 108), the rates were 60.2, 93.5, 86.1, and 58.3%, respectively. Overall, there were no significant differences. Pancreaticoduodenectomy with PDJV resection is feasible for PDAC with PDJVI and satisfactory overall survival rates are achievable. It may be necessary to reconsider the resectability of PDAC with PDJVI.

Treatment of malignancies invading the hepatic veins/inferior vena cava is a surgical challenge. An ante situm technique allows luxation of the liver in front of the situs to perform tumor resection. Usually, cold perfusion and veno-venous bypass are applied. Our experience with modified ante situm resection relying only on total vascular occlusion is reported.Retrospective analysis on an almost 15-year experience with ante situm resection without application of cold perfusion or veno-venous bypass RESULTS: The ante situm technique was applied on eight patients. Five individuals were treated due to intrahepatic cholangiocellular cancer and one case each for mixed cholangio-/hepatocellular carcinoma, colorectal liver metastasis, and pheochromocytoma. Trisectorectomy (n = 4), left hemihepatectomy, right hepatectomy, atypical resection, or mesohepatectomy (each n = 1) were performed, combined with dissection of suprahepatic/retrohepatic vena cava/hepatic veins. Venous reconstruction was achieved by reimplantation of hepatic veins with/without vascular replacement using allogeneic donor veins or PTFE grafts. Median total vascular occlusion of the liver was 23 min. Severe morbidity occurred in three patients (Dindo-Clavien 3A). R0 status was achieved in six cases with a median overall survival of 33.5 months.Ante situm liver resection can be applied without cold perfusion nor veno-venous bypass with acceptable morbidity and mortality. However, this procedure remains challenging even for the experienced hepato-pancreato-biliary surgeon.

An inflammatory myofibroblastic tumor originating from the aorta is extremely rare. Here, we report a case involving a 41-year-old female patient with an aortic inflammatory myofibroblastic tumor. Although preoperative imaging showed a mediastinal mass indicative of invasive thymoma, surgical findings revealed that this mass originated from the aorta. The patient underwent partial resection of the aorta, including the mass with patch angioplasty. Based on the postoperative histological findings, the patient was diagnosed with an aortic inflammatory myofibroblastic tumor and is currently under outpatient follow-up.

A 67-year-old man consulted our department with gross hematuria. Computed tomography (CT) revealed a huge renal tumor with a paracaval metastatic lymph node (mLN). Right total nephrectomy was planned for the renal tumor diagnosed as renal cell carcinoma preoperatively. Just before the resection of the renal vein and artery, intraoperative ultrasonography revealed an inferior vena caval tumor thrombus (IVCTT) extending from the mLN. Ultrasonography clearly and dynamically demonstrated a rhythmic flapping movement of the IVCTT with blood flow in the inferior vena cava. Following right radical nephrectomy, IVCTT resection with the vessel wall was performed to reduce the risk of pulmonary tumor thrombus. Histopathological diagnosis of the renal tumor was clear cell renal cell carcinoma, and the resected IVCTT was confirmed histopathologically as tumor involvement from the mLN of the renal cell carcinoma. Intraoperative ultrasonography can detect IVCTT extending from the mLN, whereas CT cannot.

To evaluate the efficacy and safety of percutaneous endovascular biopsy (PEB) in intravascular filling-defect lesions (IVLs) of the great vessels. We retrospectively reviewed 19 patients (age 65 ± 12 years), 11 men and eight women, who underwent PEB for IVLs, between March 2004 and November 2014. All PEBs were performed for early diagnosis and/or characterization of the IVL, or in case of reasonable doubt about the IVL nature. Pre-intervention imaging work-up included CT, MRI and/or PET-CT. PEBs were obtained with a 7F biopsy forceps device. Clinical profile, procedure technical success and safety, and clinical success were evaluated. PEB was technically successful in all patients (mean of two samples per IVL). No intra- or post-procedural complications were reported. Histopathological analysis provided a diagnosis in all PEBs with a clinical success of 100%. Of the 19 IVLs, 14 were malignant (74%). The most frequent malignant lesion observed was leiomyosarcoma (29%). Benign lesions (26%) included three thrombi (pulmonary artery) and two myxomas. PEB is a safe and efficient procedure providing the most effective technique to obtain a tissue sample of high diagnostic quality, which serves to establish early diagnosis in patients with suspected malignant lesions. • Intravascular filling-defect lesions are related to both benign conditions and malignant tumours. • Endovascular biopsy is indicated in case of doubt about intravascular lesion nature. • Percutaneous endovascular biopsy is a safe technique. • Endovascular biopsy provides tissue samples leading to correct histopathological analysis. • Percutaneous endovascular biopsy provides early diagnosis of malignant intravascular lesions.

To evaluate vertebral artery (VA) dominancy and the risk of brain infarction in T4 lung cancer patients with tumor invasion into the subclavian artery. We reconstructed the subclavian artery in 10 patients with T4 non-small cell lung cancer. The histological stages were IIIA in eight patients and IIIB in two patients. We evaluated the VA dominancy by performing a four-vessel study preoperatively and investigated the relationship between the methods of VA treatment and postoperative brain complications, retrospectively. Seven patients had a superior sulcus tumor (SST) and three had direct invasion into the mediastinum. Based on the tumor location, a transmanublial approach was used in five patients and a posterolateral hook incision was used in the other five. All subclavian artery (SA) reconstructions were done using an artificial woven graft. Preoperative angiography of the VA revealed poor development of the contralateral side in two patients. One of these patients suffered a severe brain infarction on postoperative day 2, which proved fatal. In the other patient, the VA was connected to the left SA graft by a side-to-end anastomosis and there was no postoperative brain complication. Preoperative SA and VA angiography is mandatory for identifying the need for VA reconstruction in lung cancer patients with major arterial invasion.

Sonography can be used in the management of pediatric soft-tissue vascular anomalies for diagnosing, for assessing lesion extent and for evaluating complications and response to therapy. The sonographic technique includes a combination of gray-scale imaging with color and spectral Doppler techniques. However the interpretation of the sonographic findings requires correlation with the clinical findings, some of which can be easily obtained at the time of scanning. This has to be combined with the use of appropriate nomenclature and the most updated classification in order to categorize these children into the appropriate management pathway. In this article, which is part 1 of a two-part series, the authors review the current classification of vascular anomalies, provide a clinical and a sonographic approach to these lesions, and review the most relevant clinical and sonographic features of vascular tumors including infantile and congenital hemangiomas, tufted angioma, kaposiform hemangioendothelioma, pyogenic granuloma, intramuscular capillary-type hemangioma and angiosarcoma.

Glomangiomatosis is a benign vascular variant of a glomus tumor. The lesion represents only 5% of glomus tumors with unusual or atypical features and even fewer glomus tumors with typical features. The lesions are most commonly located in the distal extremities and are multiple, deep, extensive, and often pain producing. They develop from small arteriovenous anastamoses and are most often identified in young adults. The lesions may recur. We present a case of a 33-year-old male who presented clinically with multiple slowly enlarging masses of the leg over a 5-year period, of which one caused significant pain. One of the lesions was reported to be present at birth. Imaging studies were performed and were suggestive of neurofibromatosis. Biopsies from multiple lesions led to an eventual diagnosis of multiple glomangiomatosis.

Intravenous leiomyomatosis has an unusual growth pattern characterized by proliferation of smooth muscle in uterine and systemic veins. Although histologically benign, this condition could eventually have a clinically aggressive course. At an early stage, the disease is often misdiagnosed on preoperative imaging because of its low prevalence, non-specific initial clinical manifestation, and poorly known radiological characteristics. An early, accurate diagnosis is needed for appropriate surgical management that could result in a good prognosis, reducing the risk of recurrence and morbidity. Magnetic resonance imaging is a particularly valuable technique for assessing intravenous leiomyomatosis preoperatively.

Tumors of the hand comprise a vast array of lesions involving skin, soft tissue and bone. The majority of tumors in the hand are benign. Malignant tumors, although rare, do occur and frequently have unique characteristics in this specific anatomic location. Careful staging, histological diagnosis and treatment are essential to optimize clinical outcome. However, straightforward most of the time, hand tumor management does have pitfalls; caution is advised, as a missed or delayed diagnosis or an improperly executed biopsy may have devastating consequences. This article reviews the clinical spectrum of the most common benign and malignant bone and soft tissue tumors of the hand and discusses the clinicopathological findings, imaging features and current concepts in treatment for these tumors.

Combretastatin A4-phosphate (CA4P) is an anti-vascular agent which selectively shuts down blood supply in tumours, resulting in extensive tumour necrosis. The aim of this study was to assess in vivo, non-invasive ultrasound techniques for the early evaluation of tumour perfusion following CA4P treatment of spontaneous tumours. Eight dogs that bore spontaneous tumours were enrolled and were subsequently treated with a single dose of intravenous CA4P. Perfusion of tumours was evaluated by power Doppler ultrasound (PDUS) pre-treatment (0 h), during the injection (10 min, 20 min, 30 min) and after CA4P infusion (24 and 72 h). Vascularity index (VI) of the tumour tissue was quantitatively analysed and accuracy was verified by correlation analysis with the results of immunohistochemical evaluation of microvessel density (MVD). Central and peripheral perfusion was evaluated by contrast-enhanced ultrasound (CEUS) pre-treatment and at 72 h post-treatment. Post-treatment, PDUS demonstrated a significant decrease in VI within 10 min of CA4P infusion. CEUS parameters demonstrated a significant decrease in blood velocity and volume in the central aspect of the tumour. Histology revealed a 4.4-fold reduction (p 2 = 0.957, p

Intravascular large B-cell lymphoma (IVLBCL) is a distinct disease entity with the peculiar characteristic that tumor cells proliferate within vessels. Despite recent advances in understanding the disease from clinical aspects, the underlying pathogenesis remains unknown. Here we demonstrate analyses of IVLBCL biology using four xenograft mouse models established from primary IVLBCL samples. In all four models, the main characteristic of IVLBCL tumor cell proliferation within vessels was retained. Time-lapse engraftment analyses revealed that the tumor cells initially engrafted and proliferated in the sinusoids and vessels in the liver and then engrafted and proliferated in multiple organs. Intriguingly, serial passage of tumor cells from the adrenal gland of a transplanted mouse developed from primary patient bone marrow cells into a second mouse showed that the tumor cells mainly distributed into the adrenal gland in the second mouse, implying the existence of clonal selection and/or evolution at engraftment of a specific organ. Gene expression profiling analyses demonstrated that the gene set associated with cell migration was enriched for normal peripheral blood B cells, indicating that inhibition of cell migration might be involved in IVLBCL pathogenesis. In conclusion, the mouse xenograft models described here are essential tools for uncovering IVLBCL biology.

Microvascular invasion (mVI) is known to be a risk factor of hepatocellular carcinoma (HCC) recurrence. Several factors such as the tumor grade, tumor size, tumor margin status on imaging studies, fluorine-18 fluorodeoxyglucose-positron emission tomography ((18)F-FDG-PET) results, and tumor markers have been proposed to predict mVI of HCC. However, the values of these factors have not yet been validated.Among the patients evaluated using enhanced CT/MRI, (18)F-FDG-PET, and tumor markers prior to hepatectomy from 2007 to 2012, 79 HCC patients without apparent macrovascular invasion in preoperative imaging were enrolled in this study. The image tumor margin status (smooth/non-smooth), (18)F-FDG-PET, and tumor markers, which were previously described as predictors for mVI, were evaluated.Fifteen patients had mVI (mVI+ group) and 64 patients had no evidence of mVI (mVI- group) on pathological examinations. A univariate analysis showed that the mVI+ group had a higher SUV and TNR (5.2 vs 3.8, p = 0.02 and 1.8 vs 1.3, p = 0.02, respectively) and a higher portion of non-smooth tumor margin (87 vs 27 %, p = 0.0001). There was no significant difference in the tumor markers. A multivariate analysis showed that non-smooth tumor margin alone could independently predict mVI (odds ratio 18.3, 95 % CI 3.27-102.6, p = 0.0009).A non-smooth tumor margin on preoperative imaging predicts microvascular invasion of HCC.

This study was designed to determine whether perineural invasion (PNI) and lymphovascular invasion (LVI) are independent predictors for biochemical recurrence (BCR) of prostate cancer (PCa) following radical prostatectomy (RP) in the Asian population. The study population comprised 2394 PCa patients undergoing RP at our institution in Korea. After excluding 360 patients, we compared the baseline characteristics between the groups according to the presence of PNI or LVI and estimated BCR-free survival using the Kaplan–Meier survival. Multivariate Cox regression model was adopted to identify significant predictive factors of BCR following RP. Among 2034 patients, PNI and LVI were detected in 69.3 and 12.4 % patients, respectively. Patients with PNI or LVI had higher rates of advanced biopsy and pathological Gleason score (≥7), and higher proportions of advanced clinical and pathological T stage ≥3, extraprostatic extension, seminal vesicle invasion, and surgical margin positivity. Notably, BCR-free survival was lower in patients with PNI or LVI compared with that in patients without these markers and lower in patients with both markers compared with that in other populations of patients. Moreover, PNI (hazard ratio [HR] = 2.11) and LVI (HR = 1.57) were significant predictors of BCR. The presence of the two markers was associated with a higher risk of BCR (HR = 4.60) compared with the presence of either marker alone (HR = 3.47). PNI and LVI are adverse pathologic parameters and independent predictors for BCR, and the concurrent presence of PNI and LVI resulted in poorer outcomes for BCR in PCa patients who underwent RP.

Background CD5-positive diffuse large B-cell lymphoma (DLBCL) and intravascular large B-cell lymphoma (IVL) are recognized as rare subsets of large B-cell lymphoma with poor prognosis. These two categories have similar clinicopathological features suggesting that they might overlap. Case presentation We present a case of a 72-year-old man with submental tumors. Positron emission tomography/computed tomography (PET/CT) showed tumors in the nasal and paranasal region and multiple submental and jugular swollen lymph nodes with abnormal uptake of 18F-fluorodeoxyglucose (FDG). Histology of biopsy from nasal tumors showed diffuse infiltration of large lymphoid cells, which showed positive expressions for CD20, CD79a, CD5 and negative for CD3 on immunohistochemistry. Thus, a CD5-positive DLBCL was diagnosed. After administration of 8 cycles of R-THPCOP (rituximab, pirarubicin, cyclophosphamide, vincristine and prednisolone), complete remission was achieved. Eight months after the first chemotherapy dose, local recurrence occurred. After salvage chemotherapy, nasal and paranasal tumors and lymph node swelling disappeared on PET/CT images, although the patient suffered from respiratory disturbance. A random skin biopsy revealed IVL, which was consistent with intravascular recurrence of CD5-positive DLBCL. Bone marrow smears showed hemophagocytosis. Conclusion We present a rare case of primary CD5-positive DLBCL that relapsed as pure IVL after chemotherapy. Our case suggests that CD5-positive DLBCL is closely related to IVL.

Although hematogenous spread of osteosarcoma is well known, the imaging findings of cardiovascular involvement by osteosarcoma are seldom reported and can be difficult to recognize. The enhanced resolution of modern CT and MRI scanners may lead to better detection of cardiovascular involvement. To describe the key imaging findings and clinical behavior of cardiovascular involvement by osteosarcoma. We retrospectively reviewed the imaging findings and clinical characteristics of 20 patients with cardiovascular involvement by osteosarcoma identified by two pediatric radiologists from a review of imaging studies at our institution from 2007 to 2013. At initial diagnosis, the median age of the patients was 15.1 years (range 4.8–24.6 years), and 7 (35%) patients had detectable metastases. Median time to detection of cardiovascular metastases was 1.8 years (range 0–7.3 years). Sixteen patients died of disease; 4 have survived a median of 7.4 years since initial diagnosis. The sites of cardiovascular involvement were the systemic veins draining the primary and metastatic osteosarcoma, pulmonary arteries, pulmonary veins draining the pulmonary metastases, and heart. A dilated and mineralized terminal pulmonary arteriole is an early sign of metastatic osteosarcoma in the lung. Unfamiliarity with the imaging features resulted in under-recognition and misinterpretation of intravascular tumor thrombus as bland thrombus. Knowledge of imaging findings in the era of modern imaging modalities has enhanced our ability to detect cardiovascular involvement and lung metastases early and avoid misinterpreting tumor thrombus in draining systemic veins or pulmonary arteries as bland thrombus.

To evaluate the outcomes of preoperative transarterial chemoembolization (TACE) for resectable hepatocellular carcinoma (HCC) with portal vein invasion. From February 2006 to July 2011, 320 patients initially diagnosed with resectable HCC and portal vein invasion were prospectively non-randomized into two arms. In the immediate resection arm (Arm 1, n = 205) patients received immediate surgical resection. 115 patients were included in the preoperative TACE arm (Arm 2), and eventually 85 patients underwent TACE followed by surgical resection. The 1-, 3- and 5-year overall survival rates were 48.3 %, 18.7 % and 13.9 % for Arm 1 and 61.2 %, 31.7 % and 25.3 % for Arm 2 (P = 0.001), respectively. In the subgroup analysis of types I and II portal vein tumour thrombus (PVTT), the preoperative TACE arm demonstrated significantly better survival rates than the immediate resection arm (P I = 0.001, P II = 0.036). However, no significant difference was found for patients with type III PVTT (P III = 0.684). No significant difference was found between the two arms in terms of complications and mortality. Preoperative TACE seems to confer a survival benefit for resectable HCC with PVTT, especially for types I and II PVTT, and preoperative TACE should therefore be recommended as a routine procedure. • Preoperative TACE improves the clinical outcomes for patients with PVTT • Preoperative TACE could significantly improve the rate of en bloc thrombectomy • Preoperative TACE does not increase the related adverse events

The common sites of breast cancer metastases include bones, lung, brain, and liver. Renal metastasis from the breast is rare. We report a case of breast cancer metastatic to the kidney with extension into the renal vein. A 40-year-old woman had undergone left mastectomy for breast cancer at the age of 38. A gastric tumor, which was later proved to be metastasis from breast cancer, was detected by endoscopy. Computed tomography performed for further examination of the gastric tumor revealed a large left renal tumor with extension into the left renal vein. It mimicked a primary renal tumor. Percutaneous biopsy of the renal tumor confirmed metastasis from breast cancer. Surgical intervention of the stomach and the kidney was avoided, and she was treated with systemic chemotherapy. Breast cancer metastatic to the kidney may present a solitary renal mass with extension into the renal vein, which mimics a primary renal tumor.

Background The serine/threonine protein kinases ROCK1 and 2 are key RhoA-mediated regulators of cell shape and cytoskeletal dynamics. These proteins perform multiple functions in vascular endothelial cell physiology and are attractive targets for cancer therapy based on their roles as oncogenes and metastatic promoters. Given their critical functions in both of these processes, we hypothesized that molecular targeting of ROCK proteins would be exceedingly effective against vascular tumors such as hemangiomas and angiosarcomas, which are neoplasms composed of aberrant endothelial cells. Methods In this study, we compared ROCK1 and 2 protein expression in a large panel of benign and malignant vascular tumors to that of normal vasculature. We then utilized shRNA technology to knockdown the expression of ROCK1 and 2 in SVR tumor-forming vascular cells, and evaluated tumor size and proliferation rate in a xenograft model. Finally, we employed proteomics and metabolomics to assess how knockdown of the ROCK paralogs induced alterations in protein expression/phosphorylation and metabolite concentrations in the xenograft tumors. Results Our findings revealed that ROCK1 was overexpressed in malignant vascular tumors such as hemangioendotheliomas and angiosarcomas, and ROCK2 was overexpressed in both benign and malignant vascular tumors including hemangiomas, hemangioendotheliomas, hemangiopericytomas, and angiosarcomas. shRNA-mediated knockdown of ROCK2, but not ROCK1, in xenograft vascular tumors significantly reduced tumor size and proliferative index compared to control tumors. Proteomics and metabolomics analysis of the xenograft tumors revealed both overlapping as well as unique roles for the ROCK paralogs in regulating signal transduction and metabolite concentrations. Conclusions Collectively, these data indicate that ROCK proteins are overexpressed in diverse vascular tumors and suggest that specific targeting of ROCK2 proteins may show efficacy against malignant vascular tumors. Electronic supplementary material The online version of this article (doi:10.1186/s12885-017-3470-7) contains supplementary material, which is available to authorized users.

Soft tissue tumors arising in deep veins of the extremities are uncommon, although a few cases of synovial sarcoma or leiomyosarcoma arising in the femoral vein have been documented. However, to the best of our knowledge, an extraskeletal myxoid chondrosarcoma (EMC) arising in the femoral vein has not been reported in the English literature. We report a case of EMC arising in the femoral vein of a 70-year-old man who presented with right leg edema and was diagnosed with a deep venous thrombosis (DVT) by computed tomography (CT). Magnetic resonance imaging (MRI) revealed a mass in the right proximal thigh that was diagnosed as myxomatous sarcoma by aspiration cytology, and anticoagulant therapy was initiated. The mass was surgically resected en bloc, including the femoral vein and surrounding soft tissue, and the femoral artery was preserved. The femoral vein was not reconstructed. The histologic diagnosis was an extraskeletal myxoid chondrosarcoma. The patient received postoperative local radiation treatment, with a total dose of 60 Gy, and is currently doing well with no evidence of local recurrence or metastasis at 8 months after surgery. In summary, this case report shows that EMC can arise in the femoral vein, and that reconstruction of the femoral vein is not always necessary during surgery for soft tissue tumors.

Histiocytic sarcoma is a rare malignant neoplasm of hematopoietic origin composed of cells showing morphologic and immunophenotypic evidence of histiocytic differentiation. We describe the 2nd case of primary histiocytic sarcoma of the cavernous sinus/Meckel's cave, and the 8th case involving the CNS. A 61-year-old Caucasian man presented with numbness on the entire left side of his face, shooting pain in the left frontal region, and headaches. Imaging revealed an enhancing extra axial soft tissue mass located in the left cavernous sinus and left Meckel's cave. Diagnosis was established through open biopsy, after failed attempts via CT-guided trans-foramen ovale fine-needle aspiration biopsy and keyhole biopsy. The tumor was composed of large non-cohesive epithelioid cells invading nerves and ganglion cells. Tumor cells were immunopositive for CD68, CD163, and immunonegative for the anaplastic large cell lymphoma marker ALK-1 as well as other lymphoid, myeloid, and dendritic cell markers. Histiocytic sarcoma has strong potential for systemic spread; early diagnosis and treatment are important. Our patient was initially treated with radiation therapy but subsequently developed metastases.

Cardiovascular disease and cancer incidence and prevalence have risen over the past few decades to become the leading causes of death. On the one hand, cancer patients will be treated with cardiotoxic chemotherapies; on the other, cardiovascular patients will receive a new diagnosis of cancer and will have to face treatments that may worsen their disease. Moreover, venous thromboembolism can commonly complicate the natural course of patients with cancer in an apparently spontaneous manner or can be triggered by a clinical event such as surgery, invasive procedures, a course of chemotherapy or radiotherapy and is known to be the second cause of death in these patients who also may need to be treated for pre-existing medical conditions or comorbidities. Thus, we introduce the concept of cardiovascular oncology (in the place of cardiooncology) to underline that the problems in this field are not limited to cardiotoxicity of chemotherapies and to the interaction between cardiologists and oncologists, and we focus on the role of the Internist, the only health care giver able to face the multiple problems that cancer patients may undergo.

Intravenous leiomyomatosis is a rare disorder characterized by benign smooth-muscle tumours, termed leiomyomas, which originate from uterine leiomyomas or pelvic veins. Tumours may extend into the right-sided heart chambers, termed intracardiac leiomyomatosis (ICLM), and may be potentially life-threatening due to mechanical interference with cardiac structures or pulmonary arteries. While surgical excision is the optimal therapy, incomplete retrieval of a tumour or fatal retroperitoneal hemorrhage may occur. We present a case where intraoperative transesophageal ultrasound (TEU) guided complete removal of an intracardiac leiomyoma in a single-stage surgery solely through the right atrium without vein injury. A 46-yr-old female patient presented with a two-week history of exertional dyspnea, palpitations, and syncope. Preoperative imaging modalities revealed a continuous solid mass extending from the inferior vena cava (IVC) into the right atrium, and the patient subsequently underwent open heart surgery for tumour removal and definitive diagnosis. A systematic intraoperative TEU examination performed before resection showed that the serpentine tumour was free from any attachment to the IVC and the heart. Furthermore, the diameter of the intracardiac end of the tumour was wider than that of the IVC. Given these findings, the surgeons carefully drew the cord-like tumour out of the right atrium under close TEU monitoring without vein injury. Post-extraction TEU examination showed complete removal of the tumour. Microscopic examination of the specimen confirmed the diagnosis of intravenous leiomyomatosis. For cases with ICLM, intraoperative TEU plays a significant role in helping to plan the surgical approach, monitor the movement of the tumour and the IVC during the extraction, and assess the completeness of tumour resection.

Tumors of the heart and the great vessels are very rare disease, and there are many disorders such as tumors originated from the heart and great vessels, metastatic tumors, and tumor-like lesions which do not fit into the usual concept of tumor or neoplasm; thus, it is very difficult to classify these tumors. We proposed a new classification of cardiovascular tumors for clinical use based on the accumulated biological analyses and clinical data of the reported literatures and our own study as benign tumors, malignant tumors, ectopic hyperplasia/ectopic tumors/others, and tumors of great vessels, with reference to the series of Atlas of tumor pathology of the Armed Forces Institute of Pathology and the recent World Health Organization classification of cardiac tumors issued in 2004. More than 50 disorders have been reported as tumors originated from the cardiovascular system, and various metastatic tumors from nearby organs, distant lesions, and intravascular extension tumors to the heart were reported. Based on the new classification, we reviewed epidemiology and incidence of cardiovascular tumors. Metastatic tumors are more frequent than tumors originated from the heart and great vessels, and cardiac myxoma is the most frequent tumors in all cardiac tumors.

Leiomyosarcoma of the inferior vena cava is a rare tumor that is usually fatal. The tumor may grow very slowly or occasionally very rapidly, shows extensive local invasion, and metastasizes more frequently than previously believed. Complete surgical resection remains the only potential curative therapeutic option. The aim of this study was to report the clinical experience in the management of a patient with leiomyosarcoma. A 65-year-old woman with a history of vague abdominal pain and leg swelling underwent computed tomography which demonstrated an occlusion of the inferior vena cava. The patient received a complete excision of the tumor without reconstruction and histological analysis confirmed the diagnosis of leiomyosarcoma type 1. At 3 years, the patient is still doing well with minimal leg edema and a contrast-enhanced CT demonstrates no evidence of recurrence locally or in distant sites. Leiomyosarcoma is a rare and aggressive tumor that presents with non-specific symptoms. Computerized tomography with 3-D reconstruction is a useful tool to define the presence and entity of the collateral circulation and therefore to decide on the surgical strategy. Resection probably offers the best opportunity for long-term survival.