Popliteal Angiosarcoma after Bypass with Autologous Saphenous Vein.

Angiosarcoma (AS) is a type of malignancy within sarcomas affecting soft tissue and bone. It presents a very heterogeneous distribution in the human body, and it can appear in multiple locations, from breast to liver or skin. The incidence of this type of tumor is low; however, it is aggressive and requires an early diagnosis to initiate the treatment as soon as possible. In the present article, we report the case of a male patient who was diagnosed with epithelioid AS of popliteal artery after an aneurysm popliteal surgery, as well as its treatment and evolution. This kind of tumors has controversial treatment and poor survival rates in the early years.

Angiosarcoma of a Thrombosed Arteriovenous Fistula in a Renal Transplant Recipient.

Angiosarcomas developing in nonfunctioning arteriovenous fistulas in renal transplant recipients are quite rare clinical entities with very poor prognosis. Herein we present a 60-year-old male who developed an angiosarcoma in a thrombosed radiocephalic fistula 6 years after renal transplantation. The patient presented with pain and swelling at the site of a previously asymptomatic fistula. The fistula was excised and diagnosis was made by histology. Despite radical surgery with an above-elbow amputation, the patient died of metastatic disease 6 months later.

Intravascular lymphoma forming massive aortic tumors complicated with sarcoidosis and focal segmental glomerulosclerosis: a case report and literature review.

BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by proliferation of B cells within small vessels. Herein, we report a case of a 77-year-old man who presented with IVLBCL and massive tumor formation on the aortic wall who was previously diagnosed with sarcoidosis and focal segmental glomerulosclerosis (FSGS). To our knowledge, this is the first reported case of an IVLBCL with aortic tumor formation. CASE PRESENTATION: A 77-year-old ambulatory man with sarcoidosis and FSGS had neurological symptoms for nine months. The patient presented to the emergency department with sudden left leg pain, and was diagnosed with acute femoral artery occlusion. Emergency thrombectomy was performed subsequently. Pathological evaluation of the thrombi revealed that its surface was filled with large atypical B cells. Bone marrow biopsy showed infiltration of large atypical B cells within the small vessels. IVLBCL was suspected and further examination was planned, but the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. Autopsy revealed intravascular tumors adherent to the aortic arch, left ventricle, and the abdominal aorta. All enlarged lymph nodes and the ventricular septum of the heart showed hyalinized lesions with granular formation consistent with sarcoidosis. The patient was diagnosed with IVLBCL with aortic tumor formation complicated with sarcoidosis and FSGS. CONCLUSIONS: IVLBCL may present with tumor formation on the aortic wall. Although the cause of its affinity to the aortic wall is yet unknown, autopsy findings imply that arteriosclerosis may have contributed to the tumor formation. The literature suggests that T-cell abnormalities could possibly be the common etiology of intravascular lymphoma, sarcoidosis, and FSGS.

A systematic review and meta-analysis of lymphovascular invasion in patients treated with radical cystectomy for bladder cancer.

PURPOSE: Lymphovascular invasion (LVI) is an important step in bladder cancer cell dissemination. We aimed to perform a systematic review and meta-analysis of the literature to assess the prognostic value of LVI in radical cystectomy (RC) specimens. PATIENTS AND METHODS: A systematic review and meta-analysis of the last 10 years was performed using the MEDLINE, EMBASE, and the Cochrane libraries in July 2017. The analyses were performed in accordance with the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement. RESULTS: We retrieved 65 studies (including 78,107 patients) evaluating the effect of LVI on oncologic outcomes in patients treated with RC. LVI was reported in 35.4% of patients. LVI was associated with disease recurrence (pooled hazard ratio [HR] = 1.57; 95% CI: 1.45-1.70) and cancer-specific mortality (CSM) (pooled HR = 1.59; 95% CI: 1.48-1.73) in all studies regardless of tumor stage and node status (pT1-4 pN0-2). LVI was associated with recurrence and CSM in patients with node-negative bladder cancer (BC). In patients with node-negative BC, LVI rate increased and was associated with worse oncologic outcome. LVI had a lower but still significant association with disease recurrence and CSM in node-positive BC. CONCLUSIONS: LVI is a strong prognostic factor of worse prognosis in patients treated with RC for bladder cancer. This association is strongest in node-negative BC, but it is also in node-positive BC. LVI should be part of all pathological reporting and could provide additional information for treatment-decision making regarding adjuvant therapy after RC.

Development of Angiosarcoma in a Saphenous Vein Graft after Femoral to Above-Knee Popliteal Artery Bypass.

Angiosarcoma is an exceedingly rare and aggressive neoplasm. Owing to early metastasis even in low-grade tumors, it is associated with poor survival. We report a late development of an angiosarcoma in a saphenous vein graft after a femoral artery to above-knee popliteal artery bypass performed for disabling claudication. Chronic inflammation, lymphatic disruption, and thrombosis of the graft may have contributed to the malignant transformation, similar to cases described as occurring after arteriovenous fistulae creation. This case illustrates that angiosarcoma may be a rare, late complication of autogenous vascular bypass.

Successful management of steroid-resistant vascular tumors associated with the Kasabach-Merritt phenomenon using sirolimus.

Vascular tumors associated with Kasabach-Merritt phenomenon (KMP) are life-threatening and the mortality is as high as 10-30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid-resistant cases. In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid-resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital. The time to initial response was 6.8 ± 2.7 days. The average stabilization time for the platelet count was 19.1 ± 8.5 days. At the time of publication, the average duration of sirolimus treatment was 14.1 ± 4.0 months, and the average time for sirolimus treatment as a single agent was 12.6 ± 4.2 months. The side-effects were tolerable and included oral ulcer, fever, pain, skin rash and transient ascension of serum transaminase and cholesterol. Our study indicated that sirolimus therapy is an effective and safe method for the treatment of corticosteroid resistant vascular tumors associated with KMP in infants.

Perigraft hygroma - a rare cause of post-EVAR aneurysm growth.

Development of perigraft hygromas following repair of abdominal aortic aneurysms is extremely rare. A case is presented of a patient who was found to have a large hygroma despite two re-interventions on a previous EVAR.

Epstein-Barr virus-associated smooth muscle tumor of the cavernous sinus: a delayed complication of allogenic peripheral blood stem cell transplantation: case report.

Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) have recently been associated with primary and secondary immunodeficiencies. They are broadly divided into 3 subgroups: HIV-related, posttransplant, and congenital immunodeficiency. Subsequent to organ transplantation and acquired immunosuppression, a few cases of EBV-associated SMTs have been described in the liver, respiratory tract, and gastrointestinal system. To the authors' knowledge, intracranial involvement after peripheral blood stem cell transplantation has never been reported previously. The authors describe the case of a 65-year-old woman who presented with recent-onset painful ophthalmoplegia. She had a prior history of acute myelogenous leukemia requiring allogenic peripheral blood stem cell transplantation 2 years earlier, but she was in a remission phase. Imaging revealed a T1/T2 isointense, homogeneously enhancing lesion of the left cavernous sinus. A presumptive diagnosis of Tolosa-Hunt syndrome was made, and she was treated with steroids; however, her symptoms progressed quickly and repeat imaging revealed that the lesion was growing. To rule out leukemic deposits, a minimally invasive lateral orbitotomy extradural transcavernous approach was performed for biopsy sampling and debulking of the lesion. The biopsied tumor tissue was found to be infiltrative, grayish, firm, and moderately vascular. The final pathology results indicated an EBV-associated SMT of the cavernous sinus. Subsequently, the patient's steroid treatment was stopped and she had obtained partial symptomatic relief at her last follow-up visit, 3 months after surgery. EBV-associated SMT should be included in the differential diagnosis for intracranial and dural-based central nervous system lesions, especially in immunocompromised patients. Paradoxical response to steroids with worsening of symptoms is a hallmark of EBV-associated SMTs.

Aortic Epithelioid Angiosarcoma after Endovascular Aneurysm Repair.

We report a case of epithelioid angiosarcoma of the abdominal aortic wall after endovascular treatment for abdominal aortic aneurysm (EVAR). A 60-year-old male, treated 7 years before with EVAR, presented with abdominal back pain, general fatigue, and fever. It was assumed to be a graft infection with periaortic tissue compatible with an inflammatory reaction. The endograft was therefore completely removed and a Dacron silver aorto-bisiliac graft was implanted. After a few days the patient worsened, the angio-computed tomography scan showed a progressive increase of the periaortic mass and numerous small nodules in the abdomen were also detected. The patient was again brought to surgery, an axillo-bifemoral bypass was performed, and the aorto-bisiliac graft was removed but the patient died after surgery. The histological examination showed an aortic epithelioid angiosarcoma with peritoneal metastasis.

Intravascular Myopericytoma of the Plantar Region: Case Report and Discussion of the Probable Origin From a Cutaneous Vascular Malformation.

Myopericytoma is a perivascular myoid neoplasm of skin and soft tissues characterized by numerous thin-walled blood vessels surrounded concentrically by round to spindle myoid tumor cells, which shows α-smooth muscle actin and h-caldesmon coexpression and commonly negativity for desmin. These tumors arise predominantly in extremities of adult patients with benign clinical course. Based on the architectural pattern, there are various histologic variants as classical-solid myopericytoma, hemangiopericytoma-like myopericytoma, angioleiomyoma-like myopericytoma, myofibroma-like myopericytoma, hipocelular fibroma-like myopericytoma, intravascular myopericytoma, cellular immature myopericytoma, and malignant myopericytoma. The authors report a case that fully satisfies the morphological and immunohistochemical criteria for intravascular myopericytoma, which plantar location is not previously described in the literature. In addition, the authors discuss about its possible development from a preexistent cutaneous vascular malformation.

"Caval Reconstruction for Lower-Extremity Sarcoma Metastasis Trapped within Inferior Vena Cava Filter".

This report describes the management of a hematogenously spread metastasis from a lower-extremity sarcoma found trapped within an inferior vena cava (IVC) filter. Although endovascular techniques for treating thrombosed IVC filters are successful in a majority of cases, the malignant nature of this lesion required a novel approach. In this unique case, the segment of infrarenal IVC with the thrombosed filter was resected and reconstruction performed with an interposition prosthetic graft. There were no early or late complications, and the patient remains clinically free of recurrence at 24-month follow-up.

CNS Masson Tumors: Frequent Association With Therapeutic Radiation.

Masson tumor (MT, papillary endothelial hyperplasia) is an exaggerated form of thrombus reorganization rarely occurring in the central nervous system (CNS), where it presents as a mass or hemorrhage in parenchyma, meninges, or venous sinuses. MT is subclassified as type 1 arising within a histologically normal vessel, type 2 associated with a ruptured vascular malformation, and extravascular. Limited reports of CNS MT after radiosurgery, or especially external radiation therapy, have emerged. We searched our databases for cases reported from 2008 to present. Nine cases were identified, 6 of which were associated with receipt of therapeutic radiation for known lesions, with intervals of 1 to 25+ years to MT development (4 neoplasms=external beam radiation; 1 neoplasm=external beam radiation+radiosurgery, 1 arteriovenous malformation=radiosurgery). MTs were coassociated with radiation-induced vascular malformations (1 cavernoma-like, 1 massive) only in 2 of 6 irradiated patients, whereas the other 4 had MTs only. The 3 MTs in nonirradiated patients were extravascular, with 1 spontaneously developing in a hemangioblastoma. Seven of 9 MTs were intracerebral, 1 was within the spinal cord, and 1 was subdural. Papillary MT architecture was best appreciated by CD31 or CD34 immunohistochemistry, although ERG verified the endothelial monolayer population. Most CNS MTs at our institution have arisen in patients who have received therapeutic cranial radiation, many of whom received only external beam radiation. Although MTs could conceivably represent early, severe phases in radiation-induced cavernoma development, most were not found coassociated with the latter. This study further extends our knowledge of types of radiation-induced CNS vascular abnormalities.

Systematic Review of Surgical Management of Nonmetastatic Renal Cell Carcinoma with Vena Caval Thrombus.

CONTEXT: Overall, 4-10% of patients with renal cell carcinoma (RCC) present with venous tumour thrombus. It is uncertain which surgical technique is best for these patients. Appraisal of outcomes with differing techniques would guide practice. OBJECTIVE: To systematically review relevant literature comparing the outcomes of different surgical therapies and approaches in treating vena caval thrombus (VCT) from nonmetastatic RCC. EVIDENCE ACQUISITION: Relevant databases (Medline, Embase, and the Cochrane Library) were searched to identify relevant comparative studies. Risk of bias and confounding assessments were performed. A narrative synthesis of the evidence was presented. EVIDENCE SYNTHESIS: The literature search identified 824 articles. Fourteen studies reporting on 2262 patients were included. No distinct surgical method was superior for the excision of VCT, although the method appeared to be dependent on tumour thrombus level. Minimal access techniques appeared to have better perioperative and recovery outcomes than traditional median sternotomy, but the impact on oncologic outcomes is unknown. Preoperative renal artery embolisation did not offer any oncologic benefits and instead resulted in significantly worse perioperative and recovery outcomes, including possibly higher perioperative mortality. The comparison of cardiopulmonary bypass versus no cardiopulmonary bypass showed no differences in oncologic outcomes. Overall, there were high risks of bias and confounding. CONCLUSIONS: The evidence base, although derived from retrospective case series and complemented by expert opinion, suggests that patients with nonmetastatic RCC and VCT and acceptable performance status should be considered for surgical intervention. Despite a robust review, the findings were associated with uncertainty due to the poor quality of primary studies available. The most efficacious surgical technique remains unclear. PATIENT SUMMARY: We examined the literature on the benefits of surgery to remove kidney cancers that have spread to neighbouring veins. The results suggest such surgery, although challenging and associated with high risk of complications, appears to be feasible and effective and should be contemplated for suitable patients if possible; however, many uncertainties remain due to the poor quality of the data.

Implication of microscopic and macroscopic vascular invasion for liver resection in patients with hepatocellular carcinoma.

BACKGROUND AND AIM: Long-term data after liver resection for hepatocellular carcinoma (HCC) with vascular invasion are rare for non-Asian patients. The aim of the present study was to analyze the long-term outcome of liver resection for HCC with vascular invasion and to compare the results of subgroups with micro- and macrovascular invasion. METHODS: From January 2000 to September 2010, 288 patients without extrahepatic metastases underwent liver resection for HCC. In 107 out of 288 patients (37%), vascular invasion was found in the final pathological analysis. The long-term outcome as well as the perioperative course of these patients was analyzed using a prospective database. RESULTS: The 1-, 3- and 5-year cumulative survival rate of HCC patients with vascular invasion was 64.3, 41.4 and 23.9%, respectively. The median survival was 19 months. In the multivariate analysis, the overall survival was not influenced by the type of vascular invasion (micro- vs. macrovascular invasion), however overall survival was significantly impaired in case of lymphatic vessel invasion, intraoperative blood transfusions, need of fresh frozen plasma application, prolonged ICU stay and elevated preoperative bilirubin levels. CONCLUSIONS: Acceptable survival rates can be achieved in selected patients with macrovascular invasion after surgical resection, which is not markedly different from those with microvascular invasion. In view of an otherwise poor prognosis, liver resection seems to be justified for selected HCC patients with macrovascular invasion, although this stands in contrast with the BCLC recommendations. However, it is in accordance for example with the guidelines of the Asia-Pacific Association for the Study of the Liver.

Angiosarcoma arising in an arteriovenous fistula in a patient without kidney transplant.

Angiosarcomas are relatively rare and account for only 1% of all sarcomas. They arise from endothelial cells of blood or lymph vessels. They are usually highly aggressive and long term outcome is poor with an overall 5-year survival rate of 10-20%. We report the case of a 80-year old man with an angiosarcoma arising in a non-functioning arteriovenous fistula. Angiosarcomas arising in an arteriovenous fistula are very rare and only eleven cases were found in the literature. In nine cases (82%) chronic immunosuppression, taken for renal transplant, was one of the causing factors. Our patient however did not receive a kidney transplant and was not on immunosuppressive therapy. Clinicians should be aware that an angiosarcoma can arise in an arteriovenous fistula even without chronic immunosuppression.

Ultrasound guided block of the saphenous neuroma following use of an AFO in a patient with paraplegia. A case report.

The saphenous nerve is the terminal branch of the femoral nerve and a pure sensory nerve that provide sensation to medial leg. Injury to saphanous nerve following trauma or surgery of the knee can result in formation of a painful neuroma along its distribution. We present a case of saphenous neuroma following use of an ankle-foot orthosis (AFO) in a patient with paraplegia. A 36-year-old patient with paraplegia who was capable of walking independently with his AFO presented to our department with a 3-month history of pain in his left calf. Examination revealed tenderness, paresthesias and positive Tinel sign over the anteromedial aspect of the calf. Ultrasonographic examination of the painful area showed a mass with heterogenous echogenity which was consistent with a saphenous neuroma at the site where fastener band of AFO compressed to skin. We performed a nerve block with steroid and local anesthetic injection under ultrasound guidance to the neuroma. The patient reported pain relief following injection. The use of the AFO may cause a painful saphenous neuroma which is an unusual cause of extremity pain in patients with paraplegia. Ultrasound may be a beneficial diagnostic tool and a guidance for the therapeutic interventions in this condition.