Cancer registry study of malignant hepatic vascular tumors: hepatic angiosarcomas and hepatic epithelioid hemangioendotheliomas.

BACKGROUND: Malignant vascular tumors (MVTs) are rare and often misdiagnosed due to wide range of clinical presentations, varied histology, and exquisite imagining features. We aim to characterize two different types of MVTs of the liver: hepatic angiosarcomas (HA) and hepatic epithelioid hemangioendotheliomas (HEHE). METHODS: Data on HA and HEHE between 1975 and 2016 were extracted from the SEER database and analyzed. RESULTS: A total of 366 patients with HA were identified where 64.2% were male and 79% of White race. The median age at diagnosis was 64 ± 16.2 years. Distant metastasis was found in 24% of patients, regional disease in 22.1%, and localized disease in 21.3%. The median overall survival for HA was 2 months. For HEHE, 120 cases were identified, 32.5% were male and 80% of White race. The median age of diagnosis was 51 ± 16.8 years. Distant metastasis was found in 37.5% of patients, regional disease in 27.5%, and localized disease in 20%. The median overall survival was 182 months. CONCLUSION: Patients' demographics such as race, age, and gender may assist in elucidating distinct subtypes of MVTs. HA is an aggressive tumor despite intervention. Patients with HEHE tumors have significantly better survival compared to patients with HA. Further studies are needed to deepen our knowledge about the histopathology of these tumors, the outcomes of liver transplantation as a therapeutic alternative, and available molecular targets for MVTs.

Neonatal Vascular Tumors.

Vascular tumors are a part of the vascular anomalies spectrum. Vascular malformations are congenital vascular lesions, originating from a mesenchymal stem cell defect and distinguished from vascular tumors by their low cell turnover and lack of invasiveness. They tend to grow in proportion to the child. Vascular tumors are proliferative and range from benign proliferation to malignant tumors. The appropriate differential diagnosis is imperative. Infantile hemangioma can be diagnosed clinically and rarely requires therapy; more rare tumors are difficult to diagnose and treat. This review provides an overview of vascular tumors seen in the neonatal period and summarizes treatment options.

Vascular Tumors of the Neck in Adults: 10-Year Experience in a Tertiary Center.

INTRODUCTION: The diagnosis and management of vascular lesions of the neck is a challenging task that requires a multidisciplinary approach. This retrospective study assesses the single center experience of vascular tumors of the neck. MATERIALS AND METHODS: Patients diagnosed with a vascular tumor and/or a mass in close proximity to the carotid artery were identified from our records over a 10-year period. The demographic characteristics, clinical features, surgical approach, and outcomes were reviewed. RESULTS: Surgical excision of 17 vascular lesions were performed in 16 patients with a mean age of 51.56 ± 17.35 years at the time of operation. Intra- and/or postoperative clinical and histological assessment revealed unilateral glomus caroticum (N = 11), glomus vagale (N = 2), bilateral glomus caroticum (N = 1), cavernous hemangioma (N = 1), and carotid sheath tumor (N = 1). In three patients, internal carotid artery, common carotid artery and vagal nerve were sacrificed to facilitate complete tumor excision. During the follow-up period, no tumor recurrences were observed, and the morbidity and mortality were minimal. CONCLUSION: Preoperative evaluation concerning the size, extent, and anatomical relationships of the tumor thoroughly should be investigated. Multidisciplinary approach involving vascular surgery, otolaryngology, and radiology is preferred to treat these patients for better outcomes. Preoperative embolization in selected cases may decrease estimated blood loss and operative time.

Survival Analysis of Patients with Osseous Malignant Vascular Tumors: Results of the Surveillance, Epidemiology, and End Results (SEER) Database from 1973 to 2015.

BACKGROUND Osseous malignant vascular tumors (OMVTs) are rare lesions. Moreover, the prognostic determinants of OMVTs have not been reported. This study aimed to present epidemiological data and analyze the prognostic factors of survival in OMVT patients. MATERIAL AND METHODS OMVT patients who were diagnosed between 1973 and 2015 were screened using the Surveillance, Epidemiology, and End Results (SEER) program database, with special attention paid to osseous hemangiosarcoma (OAS) and osseous hemangioendothelioma (OHE). We assessed the prognostic values of cancer-specific survival (CSS) and overall survival (OS) rates with a Cox proportional hazards regression model and univariate and multivariate analyses. OS and CSS curves were obtained using the Kaplan-Meier method. RESULTS A total of 202 cases were selected from the SEER database. The specific histopathological diagnoses were osseous hemangiosarcoma (n=127) and osseous hemangioendothelioma (n=75). Among OMVT patients, histology was an important factor in determining survival. Using multivariate analysis, old age, distant tumor stage, surgery, and low tumor grade were predictors of OS for OAS patients. Old age, surgery, and low tumor grade were predictors of CSS. Using multivariate analysis, old age and surgery were predictors of OS and CSS for OHE patients. CONCLUSIONS This study is the largest population-based study to show the demographic characteristics and analyze the prognosis of OMVT patients. Independent predictors of OS for patients with AS included old age, distant tumor stage, low tumor grade, and surgery. Old age, surgery, and low tumor grade were also predictors of CSS for patients with OAS. Independent predictors of CSS and OS for patients with OHE included old age and surgery.

Canine, feline, and equine corneal vascular neoplasia: A retrospective study (2007-2015).

Corneal vascular neoplasms (hemangioma and hemangiosarcoma) are rare in all species. Reported cases are single case reports in a single species. Archived cases of corneal hemangioma and hemangiosarcoma from dogs, cats, and horses were obtained from the Comparative Ocular Pathology Lab of Wisconsin (COPLOW, Madison, WI), tabulated, and examined. This retrospective study describes the breeds, ages, tumor types, and characteristics of vascular neoplasms that appeared to be primarily corneal in location, in feline, canine, and equine patients, with gross and histologic images. There is a discussion of predisposing factors and speculated association with chronic ocular surface disease.

Prognostic factors including lymphovascular invasion on survival for resected non-small cell lung cancer.

OBJECTIVES: The aim of this study was to report on the influence of tumor lymphovascular invasion on overall survival and in patients with resected non-small cell lung cancer and identify prognostic factors for survival. METHODS: This is a retrospective observational study of a consecutive series of patients who had surgical resection of non-small cell lung cancer in a single institution. The study covers a 3-year period. Overall survival was estimated by Kaplan-Meier method and multivariate Cox regression analysis was used to evaluate the relationship of lymphovascular invasion and other clinicopathologic variables. A multivariate regression was used to assess the relationship between tumor lymphovascular invasion and other clinical and pathologic characteristics. RESULTS: A total of 524 patients were identified and included in the study. Two hundred twenty-five patients (43%) had tumors with lymphovascular invasion. Patients with tumor lymphovascular invasion had a lower overall survival (P < .0001). Tumor lymphovascular invasion was independently associated with visceral pleural involvement (P < .0001). In a multivariable model, lymphovascular invasion (hazard ratio [HR], 2.58; 95% confidence interval [CI], 1.63-4.09; P < .0001), parietal pleural invasion (HR, 45.4; 95% CI, 2.08-990; P = .015), advanced age (HR, 1.028; 95% CI, 1.009-1.048; P = .004), and N2 lymph node involvement (HR, 1.837; 95% CI, 1.257-2.690; P = .002) were independent prognostic factors for lower overall survival. CONCLUSIONS: Lymphovascular invasion is associated with a worse overall survival in patients with resected non-small cell lung cancer regardless of tumor stage. Parietal pleural involvement, N2 nodal disease, and advanced age independently predict poor overall survival.

Expression profile of circular RNAs in infantile hemangioma detected by RNA-Seq.

BACKGROUND: Circular RNAs (circRNAs) have emerged as a novel class of widespread non-coding RNAs, and they play crucial roles in various biological processes. However, the characterization and function of circRNAs in infantile hemangioma (IH) remain elusive. METHODS: In this study, we used RNA-Seq and circRNA prediction to study and characterize the circRNAs in IH tissue and a matched normal skin control. Specific circRNAs were verified using real-time polymerase chain reaction. RESULTS AND CONCLUSION: We found that of the 9811 identified circRNAs, 249 candidates were differentially expressed, including 124 upregulated and 125 downregulated circRNAs in the IH group compared with the matched normal skin control group. A set of differentially expressed circRNAs (in particular, hsa_circRNA001885 and hsa_circRNA006612 expression) were confirmed using qRT-PCR. Gene ontology and pathway analysis revealed that compared to matched normal skin tissues, many processes that were over-represented in IH group were related to the binding, protein binding, gap junction, and focal adhesion. Specific circRNAs were associated with several micro-RNAs (miRNAs) predicted using miRanda. Altogether, our findings highlight the potential importance of circRNAs in the biology of IH and its response to treatment.

[Cutaneous infantile hemangiomas]. / Hémangiomes infantiles cutanés.

Superficial vascular anomalies are a heterogeneous group of diseases, developed from various types of vessels and differentiated by their clinical presentation, progression, and prognosis. Their classification was first published by Mulliken and Glowacki in 1982, then endorsed by the International society of the study of vascular anomalies (ISSVA) in 1996 and updated in 2014. It separates vascular anomalies into two categories: vascular tumors characterized by cellular proliferation and vascular anomalies that are structural anomalies of blood vessels. Infantile hemangiomas (IHs) are the most frequent childhood vascular tumor. In most cases, IHs are small, involute progressively with or without a few sequelae, and do not require any treatment. However, in particular localizations or in complicated hemangiomas, treatment is essential to limit tumor growth and accelerate their involution. Propranolol is now the first-line treatment. Its efficacy and safety have been clearly demonstrated.

Liver resection for hepatocellular carcinoma associated with hepatic vein invasion: A Japanese nationwide survey.

Because of the rarity of hepatic vein tumor thrombus (HVTT) compared with portal vein tumor thrombus (PVTT) in patients with hepatocellular carcinoma, little is known about this disease entity. The aim of this study was to evaluate the prognosis of each treatment modality for HVTT through an analysis of data collected in a Japanese nationwide survey. We analyzed data for 1,021 Child-Pugh A hepatocellular carcinoma patients with HVTT without inferior vena cava invasion registered between 2000 and 2007. Of these patients, 540 who underwent liver resection (LR) and 481 who received other treatments were compared. Propensity scores were calculated, and we successfully matched 223 patients (49.0% of the LR group). The median survival time in the LR group was 2.89 years longer than that in the non-LR group (4.47 versus 1.58 years, P < 0.001) and 1.61 years longer than that in the non-LR group (3.42 versus 1.81 years, P = 0.023) in a propensity score-matched cohort. After curative resection, median survival times were similar between patients with HVTT in the peripheral hepatic vein and those with HVTT in the major hepatic vein (4.85 versus 4.67 years, P = 0.974). In the LR group, the postoperative 90-day mortality rate was 3.4% (16 patients). In patients without PVTT, the median survival time was significantly better than that in patients with PVTT (5.67 versus 1.88 years, P < 0.001). CONCLUSION: LR is associated with a good prognosis in hepatocellular carcinoma patients with HVTT, especially in patients without PVTT. (Hepatology 2017;66:510-517).

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

Soft tissue sarcomas are very rare tumors, representing less than 1% of all cancers. Leiomyosarcomas are a rare group of them representing about 6% of soft tissue sarcomas and they involve smooth muscles. Less than 2% of all leiomyosarcomas involves large blood vessels. Leiomyosarcomas of vein tunica media are very rare (1/100,000 malignant cancers) and only 10% of these originate from the great saphenous vein. In this article, we report a clinical case that occurred in our institution and review all the literature available at now.

Epidemiology, diagnostics and treatment of vascular tumours and malformations.

Vascular tumours and vascular malformations are common vasculose anomalies characteristic for dissimilar clinical course, specific biological as well as immune cytological and histological properties. Vascular lesions classification system and their detailed division into groups and subgroups were elaborated and implemented in Rome, in 1996, during meeting of the International Society for the Study of Vascular Anomalies (ISSVA). It was based on modification of an earlier going division by Mullikien and Glowacki from 1982. Infantile hemangiomas are the most numerous group of benign tumours of mesenchymal origin. Vascular malformations appear definitely less often. They are composed of normal endothelium lined displastic vessels which originate from vascular tissue abnormal morphogenesis. In contrast, in hemangiomas, at the proliferation stage, increased, multiplication of endothelial cells is observed as well as of fibroblasts, mastocytes and macrophages. Infantile hemangiomas are usually not present at the moment of birth and white chloasma with superficial teleangiectasis appears which increases within 3-4 weeks and gets bright red colour and reveal very characteristic clinical course basing on intensive growth period and involution long process. Vascular malformations are observed most often at the delivery moment or they may appear at an early childhood. They enlarge proportionally along with the child's growth and their sudden expansion may be triggered by an infection, hormonal changes or trauma. Contrary to hemangiomas, they do not subside spontaneously and their abrupt increase may result in impairment or deformation of important anatomical structures. Infantile hemangiomas and vascular malformations require different and individual treatments which are often multi-stage procedures carried on in specialistic centres of plastic surgery, vascular surgery or maxillofacial surgery.

Role of CT in the assessment of muscular venous branch invasion in patients with renal cell carcinoma.

OBJECTIVE: The purpose of this article is to determine whether the relationship between a renal cell carcinoma and the renal sinus fat on contrast-enhanced CT could predict muscular venous branch invasion and the type of surgery needed. MATERIALS AND METHODS: A total of 115 consecutive patients underwent pre-operative contrast-enhanced CT between August 2011 and December 2011. Without access to histopathologic information, on nephrographic phase contrast-enhanced CT images, two radiologists independently determined whether the renal tumor was in contact with the renal sinus fat or separated from the renal sinus fat. Interreader agreements and performance characteristics of imaging tests were calculated, and histopathologic analysis served as the standard of reference. RESULTS: Histopathologic analysis identified 115 renal tumors, 90% (103/115) of which were renal cell carcinomas. Thirty-nine percent (31/80) of renal cell carcinomas that abutted the renal sinus fat on CT displayed muscular venous branch invasion on histopathologic analysis. Patients with renal cell carcinomas separated from the renal sinus fat were more likely to undergo partial nephrectomies (96% [22/23]; p = 0.013). Sensitivity and specificity for the identification of muscular venous branch invasion on CT were 94% (95% CI, 80-99%) and 30% (20-42%), respectively. Interreader agreement of visual assessment was excellent (κ = 0.87; 95% CI, 0.81-0.92). CONCLUSION: If a renal cell carcinoma was separated from the renal sinus fat on CT, the likelihood of muscular venous branch invasion being identified by histopathologic analysis was significantly decreased, and the patient was more likely to undergo a partial nephrectomy.

Clinical classification of cardiovascular tumors and tumor-like lesions, and its incidences.

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.

Microscopic Kaposiform hemangioendothelioma with extensive lymphangiomatosis: an extraordinary example of an unusual entity.

Kaposiform hemangioendothelioma (KHE) is presently classified as a vascular neoplasm of intermediate malignant potential. The clinical course of large, deep-seated tumors is frequently complicated by consumptive coagulopathy and life-threatening hemorrhage, while superficial tumors tend to behave in an indolent manner, with no known reports of distant metastasis. We describe an unusual example of KHE occurring as an incidental microscopic finding, within a background of extensive lymphangioma-like changes. The patient underwent 4 intralesional excisions over a period of 6 years, and the Kaposiform component accounts for less than 5% of the overall tissue excised. The patient remains clinically well with residual disease 5 years after conservative surgery, and there has been no evidence of regional or distant metastasis. Based on existing literature, it appears doubtful that KHE has any metastatic potential at all, which calls into question the appropriateness of its place in the spectrum of malignant vascular neoplasms.

Initiation and use of propranolol for infantile hemangioma: report of a consensus conference.

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.

Personality and hormone therapy use among postmenopausal women in the GAZEL cohort study.

OBJECTIVE: To examine with a prospective design the association of personality with hormone therapy (HT) use before and after the publication of the Women's Health Initiative (WHI) trial. DESIGN: Questionnaires were mailed to measure type A personality and hostility. Additional data from up to six mailed questionnaires every 3 years were used to determine the subsequent date of menopause and the use of HT. The association between personality and HT use was measured by the relative index of inequality computed through binary logistic regression. SETTING: The French GAZEL Cohort Study. PATIENT(S): Women (n = 1,803) who reached natural menopause during the study. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Hormone therapy use within 1 year after the date of menopause. RESULT(S): Hormone therapy use (916 users vs. 887 nonusers) was associated with type A personality (relative index of inequality = 1.88, 95% confidence interval = 1.35-2.63). This association remained significant after adjustment for all potential confounders (relative index of inequality = 1.84, 95% confidence interval = 1.23-2.74). There was no significant interaction between type A personality and the date of menopause as regards the WHI publication. CONCLUSION(S): Type A personality may be associated with HT use among postmenopausal women, regardless of other predictors, including vascular and breast cancer risk factors.

Neonatal soft tissue sarcomas.

Soft tissue tumors in very young children pose diagnostic and therapeutic challenges. Vascular tumors are the most prevalent soft tissue neoplasms in the neonatal period. They are generally benign tumors, but may exhibit aggressive behaviour and cause life-threatening complications. Fibroblastic tumors of intermediate prognosis, more prevalent in very young children (especially infantile fibrosarcoma), are locally aggressive. Since metastases are unusual in this group of tumors, complete surgical resection is generally curative. However, these tumors often present a therapeutic challenge because of the location which makes complete surgical resection difficult. Among the malignant soft tissue tumors, rhabdomyosarcoma is most frequent. It is an aggressive high-grade tumor, with local invasiveness and a propensity to metastasize. These tumors respond to chemotherapy and radiotherapy. Neonates with rhabdomyosarcoma seem to have a worse prognosis than in older age groups. This may be a result of inappropriate dosing of chemotherapeutic agents and decreased use of radiation therapy among other factors.

Intravenous leiomyomatosis: CT findings.

Intravenous leiomyomatosis (IVL) is a rare smooth muscle tumor. Although IVL is histologically benign, it might be aggressive in its behavior and can grow into pelvic veins and the inferior vena cava (IVC) extending into the heart chambers and pulmonary vasculature. Occasionally, it was found to have lung metastasis. We describe four cases of IVL in the IVC with a history of hysterectomy for uterine leiomyoma, one extending into the left renal vein and three growing into the right heart chamber. We report the computed tomography (CT) findings in the four cases and briefly discuss the CT features of IVL in order to help making accurately preoperative diagnosis and improve the rate of surgical resection and survival.

[Vascular tumors of the nasal cavities: a retrospective study of 10 cases]. / Les tumeurs vasculaires des cavités nasales: etude rétrospective a propos de 10 cas.

UNLABELLED: Vascular tumors of the sinus cavities are rarely documented in the literature. They are characterized by a histological diversity. They can be benign or malignant. The most common histologic type is the hemangioma. Their management is not well codified, it benefited from advances in modern imaging and endoscopic surgery. The objective of the work is to study anatomical and clinical characteristics, therapeutic and outcome. PATIENTS AND METHODS: The observations of 10 vascular tumors of the nasal cavities collected between January 2009 and July 2011 were studied retrospectively. The management of these tumors was based on nasal endoscopy, imaging, biopsy, and some angiography for embolization. The epidemiological parameters, histopathological, clinical, therapeutic and outcome were studied. RESULTS: The average age was 25.4 years, male was marked with a sex ratio of 4. Epistaxis was the revealing sign. All tumors were benign, with a predominance of the hemangioma (4 cases), followed by angiofibroma of the septum (3 cases) and nasopharyngeal angiofibroma (3 cases). All patients were operated, by endonasal technique in 7 cas/10 and transfacial road in 3 cases. Embolization was performed in 5 patients, there were two hemangiomas and 3 nasopharyngeal fibromas. No case of recurrence has been noted so far. CONCLUSION: The vascular tumors of the nasal cavities are difficult to treat, especially when they reach a large volume and at an extension to adjacent areas, which makes the surgical procedure difficult and bloody. The use of selective embolization in these cases is required.

Clinical analysis of vascular anomalies: a hospital-based retrospective study of 592 patients in southeast China.

BACKGROUND: Vascular anomalies are common and multidisciplinary involved diseases. The greatest impediment to their treatment in the past was their confusing terminology and clinical heterogeneities. This hospital-based retrospective study assessed some clinical characteristics, diagnosis, therapies and outcomes of patients with vascular anomalies in southeast China. METHODS: A total of 592 vascular anomalies patients (patients with intracranial tissues or viscera involved were excluded), admitted to the First Affiliated Hospital of Sun Yat-sen University from January 2006 to September 2009, were enrolled in the study. Data for clinical characteristics, diagnosis, therapies and outcomes were collected and analyzed. RESULTS: Of the 592 patients, the male:female ratios in the vascular tumor group (n = 187) and the vascular malformation group (n = 405) were 1:1.49 and 1:1.06 respectively, with no significant difference between them. The mean onset age of the vascular tumor group was significantly younger than that of the vascular malformation group (p < 0.001). The head and neck were the most commonly (31.4%) involved areas in vascular anomalies. A total of 23.8% of the patients with vascular anomalies had definite symptoms caused by the vascular lesions. In the vascular tumor group, 94.1% of them were infantile hemangiomas. Venous malformation was the most common (41.0%) subtype of vascular malformations. Surgical therapy was undertaken in 94.2% of the patients with vascular anomalies. Of the 519 patients available for the 16 - 58 month follow-up, 322 patients (62.0%) were cured, 108 patients (20.8%) were markedly improved, 57 patients (11.0%) were partially improved, and 32 patients (6.2%) were uncured. CONCLUSIONS: Vascular anomalies are clinically heterogeneous. While the outcome is generally favorable, further effort should be made to determine the appropriate terminology and management.