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Angiosarcomas represents a diverse group of aggressive high-grade vascular tumours with limited therapeutic options. We sought to determine the safety and efficacy of regorafenib, a small-molecule multikinase inhibitor, in the treatment of metastatic or locally advanced unresectable angiosarcoma. In this single-arm multicentre, open-label phase II clinical trial, 31 patients were enrolled and received regorafenib 160 mg PO daily for 21 days of a 28-day cycle. The primary endpoint for the study was progression-free survival at 4 months. Secondary endpoints included overall survival, response rate, and safety. Patients (≥18 years) with an Eastern Cooperative Oncology Group (ECOG) score of 0–1, a life expectancy of at least 4 months who had progressed on at least one but no more than 4 prior lines of therapy were eligible. Of the 23 patients evaluable for efficacy, 2 had a complete response (8.7%), and 2 had a partial response (8.7%), for a total overall response rate of 17.4%. Median PFS was 5.5 months, and 12/23 patients (52.2%) had a PFS of greater than 4 months. 10/31 (32.3%) patients evaluable for toxicity had a grade 3 or higher adverse events. Regorafenib is a safe and active treatment for refractory metastatic and unresectable angiosarcoma. Rates of adverse events were comparable to prior studies of regorafenib for other tumour types. Regorafenib, the single agent, could be considered as therapy for patients with metastatic or unresectable AS. • ORR for regorafenib in patients with relapsed/refractory AS was 17.4%. • 12/23 (52%) patients had a PFS of greater than 4 months. • For those who derived clinical benefits, the median PFS was 6.7 months. • 10/31 (32.3%) patients had grade 3 or higher adverse events. [ABSTRACT FROM AUTHOR]

Angiosarcoma is a rare mesenchymal neoplasm that may arise from vascular or lymphatic tissue. Angiosarcoma of bone is a rare high-grade malignant vascular tumour, representing less than 1% of all angiosarcomas. The most common locations of unifocal tumour are the long and short tubular bones, followed by the pelvis, and trunk. The literature regarding treatment and outcome of patients with this tumour is limited. We performed a retrospective study to analyse treatment and survival of four patients with angiosarcoma of bone. [ABSTRACT FROM AUTHOR]

Introduction: Terminal bleeding, a distressing symptom experience for patients, caregivers, and health professionals, occurs in a subset of patients in the palliative care setting. Terminal bleeding is often thought of as a large-volume catastrophically fatal event, but it can also occur for a longer period of time and still be the precipitating event for a patient's death. Case Report: We present the case of terminal bleeding in an 87-year-old patient with angiosarcoma, a rare aggressive vascular neoplasm that can occur anywhere in the body but tend to occur more frequently in the head and neck. Discussion: The patient's advanced age and aggressive disease presented challenges in managing the symptoms and precluded many of the conventional recommended interventions to manage bleeding. Conclusion: This case report speaks to the need for multidisciplinary planning that takes prognosis, performance status, previous therapies, and patient preferences into account when caring for patients with advanced cancer. [ABSTRACT FROM AUTHOR]

Background Angiosarcomas are rare and aggressive soft-tissue sarcomas. The only potential curative treatment is complete surgical excision. This study reports the outcome of isolated limb perfusion (ILP) with high-dose melphalan and tumour necrosis factor α for locally advanced angiosarcoma. Material and methods All patients who underwent an ILP for angiosarcomas between 1991 and 2016 in three tertiary referral centres were identified from prospectively maintained databases. Results A total of 39 patients were included, with a median follow-up of 18 months (interquartile range 6.1–60.8). Of these patients, 23 (58.9%) patients had a complete response (CR) after ILP, 10 (25.6%) had a partial response, 4 (10.3%) had stable disease and 2 (5.1%) patients had progressive disease immediately after ILP. A total of 22 patients developed local progression (56.4%), whereas nine (23.1%) developed distant metastases. The patients with CR had a significantly prolonged median local progression-free survival (PFS) (15.4 versus 7.3 months, p = 0.015) when compared with non-CR patients, and a trend towards better median overall survival (81.2 versus 14.5 months, p = 0.054). Six patients underwent multiple ILPs, whereby the CR rate of the first, second and third ILPs were 60%, 80% and 67%, respectively. Thirteen (33.3%) patients needed further surgical intervention, consisting of resection in eight patients (20.5%) and amputation in five patients (12.8%). Conclusion ILP is an effective treatment option for patients with locally advanced angiosarcoma in the extremities, resulting in a high number of CRs, a high limb salvage rate and prolonged local PFS. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioendothelioma is an uncommon vascular neoplasm. It is rare in the intracranial location. Its occurrence in the region of cavernous sinus is reported only once. Here, we report a case of 49-year-old man who presented with a headache, pain in the right eye, diplopia, and ptosis due to third and sixth cranial nerve involvement. Imaging showed an enhancing lesion in the region of cavernous sinus on the right side. Pterional craniotomy, combined extradural and intradural approach, and subtotal excision of the tumor was done. Histopathology and immunohistochemistry were suggestive of epithelioid hemangioendothelioma which is a rare tumor in this location. We report this case because of its rarity and uncommon location. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Though several case reports have discussed the imaging findings of KHE, larger comprehensive studies are lacking. The purpose of this study was to evaluate the imaging findings of KHE in children.Materials and Methods: A total of twelve cases of pathologically proven KHE were collected by searching our institution's pathology database for children diagnosed between January 2004 and April 2016 (6 male, 6 female; median age: 3 months; age range 7days - 18 years). CT (n=7) and MRI (n=9) findings were retrospectively evaluated. The location, morphology, enhancement, and growth pattern were analyzed.Results: KHEs involved various locations: superficial or deep soft tissue of the extremities (n=4); abdomen (n=3; 2 cases, pancreas; 1 case, small bowel), neck and mediastinum (n=1); chest wall, diaphragm, and pericardium (n=1); abdominal wall (n=1); and head (IAC, CP angle) (n=2). Eight of 10 cases (80%) with CT/MR findings were locally invasive and involved two or more adjacent organs. Three cases presented as well-defined solid masses, and seven were associated with infiltrative lesions of the surrounding areas with (n=4) or without definite solid regions (n=3). In nine patients with MRI, all KHEs were heterogeneous and hyperintense to muscles on T2-weighted images (T2WI), while four KHEs consisted of some regions that were nearly isointense to muscle. Eight of the 10 cases with imaging exhibited heterogeneous intense enhancement, while only one demonstrated mild enhancement. Signal voids (n=2), engorged vessels (n=1), calcification (n=3), hemorrhage (n=1), or bone changes (n=4) were infrequently observed. Four patients (33%) had Kasabach-Merritt phenomenon, and recurrence was observed in two cases.Conclusion: KHEs occurred in various locations, affected mostly infants, and generally exhibited intense heterogeneous enhancement. In more than half of the included cases, KHEs were highly infiltrative and locally invasive with ill-defined margins. Awareness of these features should prompt radiologists to include KHE in the differential diagnosis for pediatric masses. [ABSTRACT FROM AUTHOR]

Endobronchial ultrasound has been one of the success stories of the last decade, and the utility of the procedure continues to expand. Originally, it was developed for the staging and diagnosis of lung cancer, but its use rapidly expanded to other malignancies and even benign disease. We present the case of a patient originally referred with suspected endocar- ditis who was found to have a mass involving the right ventricle and the pulmonary outflow tract. Endobronchial ultrasound- guided biopsy was used to obtain a tissue diagnosis from the cardiac mass. [ABSTRACT FROM AUTHOR]

Echogenic breast lesions are generally thought to be benign. We herein report four cases of echogenic breast lesions that were seen at our centre over 38 months. One patient had a prior history of wide excision and radiotherapy for breast cancer and was imaged as part of routine cancer surveillance, while the other three were recalled for further assessment following an abnormal screening mammogram. All four patients were assessed on ultrasonography, which demonstrated an echogenic lesion in each patient. All four lesions underwent ultrasonography-guided core biopsy, followed by excision biopsy. The indications for biopsy were interval increase in the size of lesion or indeterminate features demonstrated in the lesion. Three lesions were benign, while the lesion from the patient who had received previous radiotherapy was angiosarcoma. Not all echogenic lesions are benign and lesions with suspicious features on ultrasonography should undergo biopsy. [ABSTRACT FROM AUTHOR]

We report an example of a cystic hepatic angiosarcoma that to our knowledge has not been previously described. The patient was a 70 year old woman who was admitted to the emergency room because of hypovolemic shock. A computed tomography showed four heterogeneous hepatic cystic masses varying from 2.5 to 11.2 cm; one of these with rupture and formation of a subcapsular hematoma. The cyst wall was lined by several layers of neoplastic epithelioid and spindle shaped endothelial cells that in some areas extended to the underlying stroma. They expressed CD31 and CD34, and were negative for cytokeratin. The patient is alive with residual hepatic cystic angiosarcoma. However, follow up is too short to be significant. [ABSTRACT FROM AUTHOR]

Background: Sutures plays an important role in wound repair by providing hemostasis and support for healing suture. Synthetic non-absorbable like polypropylene suture materials induce minimal tissue reaction. Polypropylene has not been associated to neoplasms in domestic animals. The aim this study is to describe the first case of polypropylene suture induced hemangiosarcoma in an abdominal wall of a feline. Case: A 13-year-old female domestic shorthair cat was presented with a ventral abdominal subcutaneous mass. Clinical examination revealed a firm, rough, and irregular, approximately 6 x 4 cm subcutaneous mass involving the caudal ventral abdomen. A complete blood (cell) count (CBC) was within normal limits. Leukemia and FIV tests were negative; and the results of a chemistry panel revealed elevated creatinine (valor: 2.0 reference range: 0.5-1.9). Abdominal ultrasound revealed a large mass of mixed echogenicity in the mid-ventral abdomen cranial to the bladder, measuring approximately 6 x 4 cm in the middle line with suture deeply embedded within mass suggesting foreign body reaction or neoplasm. There was evidence of sutures (hyperechoic lines) along the caudal abdominal wall extending into the mass. Records indicated that ovariohysterectomy procedure was performed 12 years previously at the same clinic and the closure of the abdominal wall was made with polypropylene sutures. No other abdominal surgery was performed in this cat. A fine-needle aspirate of the mass and contrast-enhanced computed tomography was not performed due to owner's finance restrains. The cat was referred to surgery and the mass was excised. No evidence of metastasis was noted during surgery. Histologically, the neoplastic cells were oval to round with granular cytoplasm and vesicular nucleus and exhibited moderate cellular and nuclear pleomorphism. A diagnosis of abdominal wall hemangiosarcoma was made with suture deeply embedded within mass. Surgical margins were clear. Chemotherapy was indicated, but the owner declined due to financial reasons. This is the first polypropylene suture mass associated hemangiosarcoma in cats. Discussion: Hemangiosarcoma is a malignant neoplasm of vascular endothelial cells origin and it may be associated with chronic inflammation and neoplastic transformation. It was reported in previous studies, and it could be a hypothesis for the presented clinical case. There are two cases described in the literature of abdominal wall tumor associated with foreign body and exuberant inflammatory response in cats using different types of suture; one case is a steel staple developing hemangiosarcoma and the other is polyester sutures developed fibrosarcoma. The present study shows a primary hemangiosarcoma diagnosed several years after closure of abdominal wall using polypropylene sutures in a female cat. Polypropylene is a monofilament suture that create less tissue-drag and induces less inflammation than multifilament sutures and is the preferred suture to close abdominal wall. Cats demonstrate a peculiar predisposition to neoplasms at the site of injury. Although the pathogenesis is still unclear, the introduction a "foreign body" may cause inflammatory process that act as a stimulus to neoplasia formation. We believe that polypropylene was the foreign material that may have played a role in tumor development in this case and it has not been reported before. Polypropylene sutures were found on gross examination of excised material. Any uncoated braided non-absorbable material located deeply in tissues may evoke a chronic inflammatory response (granuloma). A granuloma may evolve to malignancy in some cats. Despite polypropylene materials induce minimal tissue reaction, it may be associated to neoplasm. [ABSTRACT FROM AUTHOR]

Background: Primary angiosarcoma of breast (PAOB) is a rare and highly aggressive malignancy. There is no general agreement on optimal treatments or prognostic factors for this orphan disease. The objective of this study was to investigate the clinicopathologic features and management experiences of PAOB. Methods: We performed a retrospective review of medical and pathologic records of 17 consecutive patients diagnosed with PAOB between January 2000 and February 2014 at FuDan University Shanghai Cancer Center. We evaluated the clinical characteristics, multimodality treatments, and associated clinical outcomes. Results: A total of 16 patients were included in this retrospective study (median age at PAOB presentation 33.5 years, range: 19-56 years). Palpable tumor with or without breast skin ecchymosis presented as the most common initial symptom. All patients underwent surgery with curative intent. Median disease-free survival and overall survival (OS) were 9 months and 13.6 months, respectively. One-year and 3-year disease-free survival rates were 43.8% and 6.3%, with OS rates of 93.8% and 78.1%, respectively. High histologic grade indicated poorer OS by univariate analysis (P=0.01). However, neither adjuvant chemotherapy nor radiotherapy contributed to clinical outcomes in our series. Conclusion: PAOB is considered as an infrequent breast neoplasm with aggressive characteristics. Histologic grade and early metastasis (within 12 months after diagnosis) are associated with poor prognosis. Regardless of grade, additional benefit was not observed with adjuvant therapy. [ABSTRACT FROM AUTHOR]

Hepatic epithelioid hemangioendothelioma is a rare vascular neoplasm with an unpredictable malignant potential. Different therapeutic options are available, depending on the basis of disease extension and the patient's overall condition. A correct pathological diagnosis is necessary and is often based on scant material. Here, we report a case diagnosed on fine needle aspiration and on a small surgical biopsy. In addition, we will review the literature. The patient is a 54-year-old woman who presented with persistent pain in the right hypochondrium and suffered from weight loss. Ultrasound examination and CT scan showed several focal and confluent hepatic lesions. Thus, an ultrasound-guided fine-needle aspiration (US-FNA) was performed. A cytological diagnosis of vascular proliferation with epithelioid component was performed. Afterwards, a hepatic “small biopsy” (SB) was made. Histological and immunohistochemical data were consistent with a hepatic epithelioid hemangioendothelioma diagnosis. The patient, however, is in good general condition and is waiting for a hepatic transplantation; repeated total CT scan showed no signs of metastasis. The literature was reviewed in order to define the pathological features that were helpful in the cytological and histological diagnosis of hepatic epithelioid hemangioendothelioma, and to better understand if pathological data is prognostically useful. [ABSTRACT FROM AUTHOR]

Primary hepatic angiosarcoma is a rare, aggressive tumor; composed of spindle or pleomorphic cells that line, or grow into, the lumina of pre-existing vascular spaces like sinusoids and terminal hepatic venules; with only about 200 cases diagnosed annually worldwide but it is the most common primary malignant mesenchymal tumor of the liver in adults and accounts for 2% of all primary hepatic malignancies. HAS occurs in association with known chemical carcinogens, but 75% of the tumors have no known etiology. Patients present with vague symptoms like abdominal pain, weight loss, fatigue or an abdominal mass. Hepatic angiosarcoma is usually multicentric and involves both lobes, entire liver may also found to be involved. CD31 is the most reliable marker. These tumors lack specific features on imaging, so, pathological diagnosis is necessary. There are no established treatment guidelines because of low frequency and aggressive nature of tumor, chemotherapy is only palliative, liver resection is indicated for solitary mass and liver transplant is contraindicated. The aim of this article is to comprehensively review all the available literature and to present detailed information and an update on primary hepatic angiosarcoma. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma (KHE) is a vascular tumorwith poor prognosis. We present a child with progressive disability, extremepain, and autonomic dysfunction due to a retroperitoneal KHEwhere radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volumewas not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement. [ABSTRACT FROM AUTHOR]

Background Angiosarcoma is a rare and aggressive malignant neoplasm of endothelial cells. Recent studies have shown that the mTOR pathway is also aberrantly activated in cutaneous angiosarcoma. New therapeutic strategies are required because the prognosis of this disease is still poor. Objective The aim of the present study was to determine the driver gene of angiosarcoma useful for the screening of small molecule inhibitors. Methods We investigated the sensitivity of inhibitors for the PI3K/AKT/mTOR pathway in ISOS-1 and ISO-HAS cutaneous angiosarcoma cell lines. Flow cytometric analysis was conducted to evaluate cell-cycle progression and apoptosis. Western blot analyses were performed to elucidate the possible underlying mechanisms of growth inhibition. The colony formation assay was conducted to evaluate the clonogenic potential. We used the siRNA for PDK1 to examine the role of PDK1 on the growth of angiosarcoma cells. Results The PI3K inhibitor and mTOR inhibitor inhibited the growth of both cell lines in a dose-dependent manner. The PI3K inhibitor more effectively induced cell-cycle arrest at the G1 phase with the downregulated expression of cyclin D in ISOS-1 cells than the mTOR inhibitor. The PI3K inhibitor and mTOR inhibitor weakly but significantly induced G1 cell cycle arrest at the same degree in ISO-HAS cells. The expression of cyclin D was downregulated by the treatment with siRNA for PDK1, but not by the AKT inhibitor in ISOS-1 and ISO-HAS cells. The knock down of PDK1 with siRNA was more effective at reducing colony numbers than the mTOR inhibitor in ISOS-1 cells. Conclusion These data showed that PDK1 played a pivotal role in the growth of angiosarcoma cells. Therefore, inhibition of PDK1, but not AKT, may be a more appropriate strategy than that of mTORC1 for the treatment of cutaneous angiosarcoma; the PDK1 inhibitor is promising as a therapeutic agent. [ABSTRACT FROM AUTHOR]

Background: Classic Kaposi Sarcoma (KS) is vascular sarcoma, known to be more common in Mediterranean elderly men and characterized by an indolent clinical behavior. To our knowledge, this is the first evidence in literature, describing a spontaneous partial regression in a non-HIV, non-iatrogenic KS. Case presentation: A 68-years old woman, presenting with weight loss and respiratory symptoms, was diagnosed with a classic KS involving lungs and mediastinal lymph nodes. No skin or mucosal lesions were identified, HIV positivity was ruled out. Due to patient's choice, she was kept under surveillance with 3-monthly thorax-abdomen-pelvis computed tomography scan (TAP CT). A first reassessment proved progressive disease (PD) associated with symptoms worsening. A new TAP CT, performed at 5 months from the diagnosis, showed stable disease (SD), with a minor reduction in size of mediastinal lymphadenopathies. A further reassessment, performed 5 months later, resulted in a partial response (PR) despite the absence of anymedical treatment. Up to date, the disease is in remission, patient is asymptomatic and still on surveillance. Conclusion: Given the possible indolent behaviour of KS, we believe that close surveillance can represent a valuable approach in selected cases. [ABSTRACT FROM AUTHOR]

Benign lymphangioendothelioma is a rare lesion of controversial etiology and a histopathologic mimic of Kaposi sarcoma and so-called 'well-differentiated' angiosarcoma. Its most typical clinical presentation is as a slowly expanding, erythematous patch or plaque; it rarely presents as a large mass. We report the second case of a giant benign lymphangioendothelioma, which arose as a serpiginous mass involving most of the flank of an elderly male with no prior radiation exposure and with a remote history of herpes zoster infection. A biopsy revealed numerous anastomosing vascular channels extending from the superficial dermis to the subcutis that were dilated to progressively slit-like in architecture. The endothelial cells lacked cytologic atypia, hobnailing, or significant mitotic activity, and human herpesvirus-8 expression was absent. Positivity for podoplanin ( D2-40) was observed in the endothelial cells, supporting a lymphatic phenotype. Furthermore, the lesional cells lacked immunohistochemical expression of Wilms tumor 1, providing further support of a malformative - rather than neoplastic - pathogenesis. [ABSTRACT FROM AUTHOR]

We report here a case of a 69-year-old woman with a relapsing metastatic angiosarcoma treated with a combination of metronomic chemotherapy and propranolol. The beta blockers were added since the tumour was positive for betaadrenergic receptor. A complete response was quickly obtained and lasted for 20 months. With this case, the combination of metronomic chemotherapy and propranolol in angiosarcoma warrants additional studies and illustrates the potential of metronomics to generate innovative yet inexpensive targeted therapies for both high-income and low-/middle-income countries. [ABSTRACT FROM AUTHOR]

Primary cardiac angiosarcomas are rare tumors with unfavorable prognosis. Pathogenic driver mutations are largely unknown. We therefore analyzed a collection of cases for genomic aberrations using SNP arrays and targeted next-generation sequencing (tNGS) of oncogenes and tumor-suppressor genes. Recurrent gains of chromosome 1q and a small region of chromosome 4 encompassing KDR and KIT were identified by SNP array analysis. Repeatedly mutated genes identified by tNGS were KDR with different nonsynonymous mutations, MLL2 with different nonsense mutations, and PLCG1 with a recurrent nonsynonymous mutation (R707Q) in the highly conserved autoinhibitory SH2 domain in three of 10 cases. PLCγ1 is usually activated by Y783 phosphorylation and activates protein kinase C and Ca2+-dependent second messengers, with effects on cellular proliferation, migration, and invasiveness. Ectopic expression of the PLCγ1-R707Q mutant in endothelial cells revealed reduced PLCγ1-Y783 phosphorylation with concomitant increased c-RAF/MEK/ ERK1/2 phosphorylation, increased IP3 amounts, and increased Ca2+-dependent calcineurin activation compared with ectopic expressed PLCγ1-wild-type. Furthermore, cofilin, whose activation is associated with actin skeleton reorganization, showed decreased phosphorylation, and thus activation after expression of PLCγ1-R707Q compared with PLCγ1-wild-type. At the cellular level, expression of PLCγ1-R707Q in endothelial cells had no influence on proliferation rate, but increased apoptosis resistance and migration and invasiveness in in vitro assays. Together, these findings indicate that the PLCγ1-R707Q mutation causes constitutive activation of PLCγ1 and may represent an alternative way of activation of KDR/PLCγ1 signaling besides KDR activation in angiosarcomas, with implications for VEGF/KDR targeted therapies. [ABSTRACT FROM AUTHOR]

Objective. An increase in the incidence of prostate angiosarcoma may be expected owing to the exponential increase in the use of radiotherapy for prostate carcinoma in recent decades and the possible aetiology of radiation exposure on the development of angiosarcoma in general. The objective of this study was to give an overview of cases in the literature based on a case report of prostate angiosarcoma in a hospital in the Netherlands, and to discuss optimal treatment. Material and methods. All (related) articles In PubMed/Medline and Embase with possible cases of angiosarcoma were screened on title and abstract. A case of prostate angiosarcoma identified in the authors' institution was included. Results. The literature search yielded 13 cases of prostate angiosarcoma. The earliest six publications lack essential data. Four patients had a history of radiotherapy. The present patient developed angiosarcoma following brachytherapy for prostate cancer. Therapy consisted of radical surgery with or without chemotherapy in five cases. In eight cases curative therapy was not reported or not possible. Mean follow-up was only 1 year. Four patients died within 1 year of diagnosis, irrespective of treatment choice. One patient, treated with a combination of radical surgery and adjuvant chemotherapy, was still alive 36 months after therapy. Conclusions. The findings confirm that prostate angiosarcoma is mostly radiation induced. This patient is the first case of prostate angiosarcoma after primary brachytherapy. Angiosarcoma may occur more often in the future owing to widespread use of brachytherapy and radiotherapy of the prostate. Current guidelines on management of angiosarcoma suggest radical surgery in local disease as the primary treatment of choice. [ABSTRACT FROM AUTHOR]

BACKGROUND: Angiosarcomas are rare tumors that carry poor prognosis. Because of insidious growth rate, the diagnosis is often difficult and delayed. METHODS: Between 1990 and 2011, 72 (41 female, 31 male) patients were treated at our institution. Pathologic confirmation was obtained and multiple prognostic factors were evaluated for survival. RESULTS: Forty-four cases were sporadic and 28 cases were secondary. In the sporadic group, 16 (36%) patients had increased sun exposure, while in the secondary group, the majority (n = 23, 82%) of patients had prior exposure to radiation. The latent period between radiation exposure and diagnosis was predictive of survival (P = .037). Presentation was delayed by more than 3 months in 41% of patients. The majority of men developed head and neck angiosarcomas (n = 15, 48.5%), while women developed breast angiosarcomas (n = 21, 51%). Median survival was prolonged in patients treated initially with surgery. CONCLUSIONS: A delay in the diagnosis of angiosarcoma can affect survival. Clinical suspicion and prompt diagnosis are essential for successful multimodal therapy. Initial surgical resection with adjuvant chemotherapy provides survival advantage. [ABSTRACT FROM AUTHOR]

Angiosarcomas account for a mere 2-3% of adult soft tissue sarcomas, with an overall poor outcome. Depending on the primary site, angiosarcomas have distinct prognosis. Primary hepatic angiosarcomas ( PHAs) are much rare tumors, with worse prognosis compared with other angiosarcomas. PHA is reported to be associated with vinyl chloride, but the majority of patients were still with unknown etiology. As PHA lacks specific symptoms, signs, or images, pathological diagnosis is necessary. The review summarizes 25 articles published from January 2000 to December 2012, including 64 cases of PHA with detailed information. Survival curves are estimated using the Kaplan- Meier method by SPSS 21.0. We find that the median survival time is 5 months; local excision alone or combination with adjuvant therapy is the optimal choice, with median survival time of 17 months. In addition, liver transplant is abandoned for high recurrence rate; emergent transcatheter arterial embolization is thought to be an efficient method for controlling intra-abdominal bleeding; and transcatheter arterial chemoembolization and chemotherapy may be helpful in improving survival. [ABSTRACT FROM AUTHOR]

The article presents a case study of a woman who was diagnosed with a breast lump in February of 2012 and had a successful lumpectomy and presented to physicians in October of 2012 with fever and symptoms of pneumonia. A discussion of diagnostic testing which was conducted on the woman, and resulted in her receiving a diagnosis of cardiopulmonary thromboembolism of epithelioid angiosarcoma arising from malignant phyllodes tumour of the breast, is presented.

Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multi-ple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for loco-regional control of such a complex and extremely rare disease. [ABSTRACT FROM AUTHOR]

Background MYC amplification occurs in post-radiation and chronic lymphedema-associated secondary angiosarcoma and some primary angiosarcomas. In this study, we tested the ability of automated dual-color dual-hapten in situ hybridization ( DISH) staining to discriminate secondary angiosarcoma from radiation-associated atypical vascular lesions ( AVL), and to correlate with fluorescence in situ hybridization ( FISH) for MYC amplification. Methods Cases of secondary angiosarcoma, including 11 biopsies and 3 excisions from 11 patients, and 5 AVL biopsies from 5 patients, were examined by FISH and DISH. DISH staining was performed using the Dual Color Open Probe software on a Ventana Benchmark XT automated slide stainer. Metallic black silver ( MYC) and reference CHR8 red signals were qualitatively and semi-quantitatively enumerated for tumor nuclei. Small and large clusters of silver signals were recorded as 6 or 12 signals, respectively. MYC amplification was defined as MYC/ CHR8 ratio >2.0. Results Where tissue was available for both DISH and FISH, all secondary angiosarcoma cases showed MYC amplification (11/11 = 100%) by both DISH and FISH. All AVL were negative for MYC amplification by both techniques (0/5 = 0%). Conclusion In the current cohort, use of DISH identified all MYC amplified cases, and distinguished secondary angiosarcoma from AVL. DISH staining may be useful in distinguishing secondary angiosarcoma from AVL in challenging cases. [ABSTRACT FROM AUTHOR]

Vascular tumors of the breast are uncommon and include hemangioma, angiolipoma, and angiosarcoma. Among breast hemangiomas, capillary hemangiomas are relatively rare in contrast to cavernous hemangiomas. We report two rare cases of cavernous and capillary hemangiomas of the breast by mammography, sonography, and positron emission tomography/computed tomography. [ABSTRACT FROM AUTHOR]

Primary angiosarcomas following reconstructive surgery are thought to be attributable to radiotherapy or lymphoedema. We report the first case of a patient presenting with breast angiosarcoma demonstrating multiple morphologies (including the rare epithelioid subtype) arising after breast-conserving reconstruction for a previous adenocarcinoma, in the absence of these predisposing risk factors. Level of Evidence: Level V, diagnostic study [ABSTRACT FROM AUTHOR]

Fluid-fluid levels are a known feature of a number of osseous lesions, but have never before been described in primary osseous angiosarcoma, which is itself a rare malignancy. We report a case of humeral angiosarcoma presenting with fluid-fluid levels. Recognizing this entity as a possible etiology for fluid-fluid levels could help radiologists to avoid potential confusion in the differential diagnosis on imaging. [ABSTRACT FROM AUTHOR]

Angiosarcoma is a rare malignant neoplasm of mesenchymal origin. It is characterised by endothelial differentiation. Lesions are most commonly seen in the head and neck region, especially on the skin of the scalp in elderly men. Oral and intraosseous lesions are comparatively rare. We report an exceedingly rare case of primary intraosseous angiosarcoma in a 79-year-old male arising in the mandibular left retromolar region. [ABSTRACT FROM AUTHOR]

Abstract: Primary vascular neoplasms are rare entities. They were first described as arising spontaneously in the aorta and other vessels. However, in the past several decades, a number of systemic artery-derived vascular neoplasms, mostly sarcomas, have been reported as arising in intimate association with synthetic grafts. We describe two additional cases of intimal sarcoma seen at our institution. The first is an invasive intimal sarcoma detected in a thoracoabdominal aortic aneurysm at the time of surgical intervention. The second is a superficial spreading intimal sarcoma associated with a Dacron-coated graft, in place for 9 years, detected when the graft was replaced. When the patient died 3 months later, a metastatic subcutaneous sarcomatous lesion was detected at autopsy. In these cases, we studied selective molecular pathways that may be involved in the transformation of benign endothelium to malignant endothelium, with implications for possible therapeutic targets. These cases are presented in order to contribute additional data to the literature involving these vascular neoplasms and to potentially provide a spectrum of disease seen in the vasculature tissues that may arise spontaneously or after placement of a synthetic graft. [Copyright &y& Elsevier]

Objective: Primary hepatic angiosarcoma is a very rare and aggressive malignancy of vascular origin. We describe cross-sectional imaging findings of this entity with emphasis on dynamic contrast-enhanced (DCE) and diffusion-weighted (DWI) MR imaging. Methods: Seven cases of pathologically confirmed hepatic angiosarcoma were retrospectively reviewed (CT and MRI examinations were available in seven and six patients, respectively). Two radiologists evaluated lesion growth patterns, attenuation, signal intensity characteristics, contrast enhancement patterns, and apparent diffusion coefficients (ADCs). Results: Multifocal hepatic disease was present in six patients by means of a mixed pattern of large dominant masses and multiple small nodules; one patient had a solitary large mass. Unenhanced images depicted hemorrhagic areas and a markedly heterogeneous internal architecture within large tumors. Contrast-enhanced early phase images showed variable patterns including patchy peripheral or bizarre shaped intralesional foci of enhancement, peripheral rim enhancement, and small lesions without enhancement. On DCE images, the majority of lesions presented with varying degrees of progressive enhancement. Small nodules frequently displayed homogeneous enhancement on delayed phase images due to complete fill-in. DWI revealed a high interlesional variability of ADC values (range 0.57-2.41 × 10mm/s, mean 1.37 × 10mm/s). Conclusion: Cross-sectional imaging findings of hepatic angiosarcoma reflect the varied histopathological composition of the tumors. Multifocal disease, hemorrhage within large lesions, as well as progressive enhancement on DCE images are typical features of hepatic angiosarcoma. The mean ADC of lesions was found to be slightly elevated in comparison with other hepatic malignancies. [ABSTRACT FROM AUTHOR]

The article presents a study which discusses face and scalp angiosarcoma (AS) including its dermoscopic, histopathological, and clinical features. It says that biopsy specimens from infected patients were evaluated through dermoscopic devices, digital camera, and haematoxylin-eosin staining. Results show that AS exhibits a red, purple-blue color of vascular tumors due to the presence of epidermis which was thinned.

Objective: To describe the imaging and histopathology of pseudomyogenic hemangioendothelioma. Materials and methods: Five cases of pseudomyogenic hemangioendothelioma, which presented over the last 5 years, were retrieved from the files of the Royal National Orthopaedic Hospital. The imaging and histopathology were reviewed in all cases. Magnetic resonance imaging, which was available from all cases, was assessed for the following features: the number of lesions, location in soft tissue (superficial and or deep/subfascial) and bone, and the signal characteristics and morphology of individual lesions. Immunohistochemistry was performed in all cases to characterize the lesions. Results: Four of the five patients had multiple lesions involving a single limb. Bone was involved in 3 of the 5 individuals. All tumors diffusely expressed ERG and cytokeratins AE1/3, but not MNF116. CD31 was weakly positive in 4 cases. INI-1 expression was retained in all cases. Imaging features included ill-defined, infiltrative lesions in subcutaneous fat with extension to the adjacent skin, poor- to well-defined intramuscular nodules and predominantly intracortical focal bone lesions with rare medullary involvement. Conclusion: Pseudomyogenic hemangioendothelioma represents a distinct recently characterized tumor type presenting in young adults, with a tendency towards multicentric bone and soft tissue involvement. [ABSTRACT FROM AUTHOR]

Kaposiform haemangioendothelioma is a very rare vascular tumour of intermediate (borderline) malignancy, typically occurring in the skin and soft tissues of the extremities in infants and children. We report two morphologically and immunophenotypically classical cases occurring in the testicular parenchyma of old adults, review the literature on vascular tumours of the testis and discuss the differential diagnosis of these unusual cases. [ABSTRACT FROM AUTHOR]

Primary angiosarcoma of the breast is extremely rare. Radiologic findings are often non specific and may appear completely normal in one-third of cases with primary angiosarcoma. The prognosis is usually poor because of the high rates of local recurrence and early development of metastasis. Surgical removal followed by adjuvant chemotherapy seems improve the prognosis. We report a case of a 33- year-old woman with a highly vascular mass in her right breast which is suggestive of malignancy at radiology. Initial core needle biopsy showed a benign hemangioma. The patient underwent a mastectomy. The tumor histology showed papillary formations and vascular structures lined by atypical cells with hyperchromatic nucleus and eosinophilic cytoplasm with solid areas. The tumor cells expressed CD34 and CD31 but were negative for cytokeratin. The diagnosis of angiosarcoma grade III was made. The patient is now receiving chemotherapy. She is still alive. Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/ vs/1530481200889780. [ABSTRACT FROM AUTHOR]

The article describes the case of an eight-year old female domestic shorthair cat presented with a ventral abdominal subcutaneous hemangiosarcoma induced by a foreign body that appeared to be steel staples. Computed tomography showed a large soft tissue mass arising from the ventral abdominal wall. Pathologic findings revealed neoplastic cells with eosinophilic homogenous granular cytoplasm, as well as areas of coagulative necrosis, fibrin thrombi and hemorrhages scattered within the tumor.

Summary: Within the human medical literature, retiform haemangioendothelioma (RHE) is an established and well-recognized histopathological variant of endothelial tumours, but to date RHE has not been reported in animals. These tumours are characterized by the presence of elongate, arborizing vascular channels lined by neoplastic endothelium with prominent, often bulging (‘hobnail’) nuclei supported by a dense collagenous matrix and accompanied by abundant lymphoplasmacytic inflammation. Immunohistochemically, the neoplastic cells typically express endothelial markers such as von Willebrand factor and CD31. Human RHEs are categorized as low-grade malignancies. This report describes two canine vascular tumours with features consistent with RHE. In both cases there was suspected or known widespread tumour metastasis. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma (KH) is a rare tumor of vascular origin that commonly affects the cutaneous tissues of the extremities. It can, however, affect the abdomen, thorax, head, or neck. Compared with juvenile hemangiomas, which tend to regress, KH tumors are locally aggressive and usually persist. Associated morbidity and mortality rates range from 12 to 30 % and typically are related to either compressive effects on surrounding vital structures or effects of the Kasabach-Merritt phenomenon [, , ]. To our knowledge, this report is the first to describe KH presenting as a primary intracardiac tumor. [ABSTRACT FROM AUTHOR]

The classification of vascular bone tumors remains challenging, with considerable morphological overlap spanning across benign to malignant categories. The vast majority of both benign and malignant vascular tumors are readily diagnosed based on their characteristic histological features, such as the formation of vascular spaces and the expression of endothelial markers. However, some vascular tumors have atypical histological features, such as a solid growth pattern, epithelioid change, or spindle cell morphology, which complicates their diagnosis. Pathologically, these tumors are remarkably similar, which makes differentiating them from each other very difficult. For this rare subset of vascular bone tumors, there remains considerable controversy with regard to the terminology and the classification that should be used. Moreover, one of the most confusing issues related to vascular bone tumors is the myriad of names that are used to describe them. Because the clinical behavior and, consequently, treatment and prognosis of vascular bone tumors can vary significantly, it is important to effectively and accurately distinguish them from each other. Upon review of the nomenclature and the characteristic clinicopathological, radiographic and genetic features of vascular bone tumors, we propose a classification scheme that includes hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants. [ABSTRACT FROM AUTHOR]

Vascular tumors are the most common of the vascular anomalies affecting children. The management of these neoplasms has changed over the years as our knowledge of their pathophysiology has increased, available technology has evolved, and our clinical experience has widened. Medical, laser, and surgical therapies are used concurrently to treat these tumors. [ABSTRACT FROM AUTHOR]

Kaposiform hemangioendothelioma is a rare vascular tumor and has a high mortality rate in newborns when associated with Kasabach-Merritt syndrome. We present a case of a newborn with a huge lower limb Kaposiform hemangioendothelioma with a rapid tumor growth and a life-threatening Kasabach-Merritt syndrome, which was refractory to medical treatment. [ABSTRACT FROM AUTHOR]

Breast Angiosarcoma is a malignant vascular neoplasm rare in the breast with an incidence of less than 0.05% of all primary tumors in the breast that seat. Given its age of onset is from the third to fourth decade of life, it is disturbing to the occurrence of increasingly early ages. We describe the case of a patient 25 years old with a history of benign breast fibroid from the 17 years that degenerates into a breast angiosarcoma. [ABSTRACT FROM AUTHOR]

Hepatic epithelioid hemangioendothelioma (HEH) is a rare disease of unknown etiology for which a standard systemic treatment has not been established. The common expression of vascular endothelial growth factor (VEGF) and its receptor in HEH provide a rationale for the reported use of antiangiogenic drugs, including bevacizumab, lenalidomide and thalidomide. We report a case of a young male patient with HEH who was treated with sorafenib for almost 2 years. Sorafenib was used instead of other VEGF inhibitors due to its convenient oral route, its dual antiangiogenic and antiproliferative activity, and its favorable safety profile. Sorafenib therapy resulted in durable stabilization with progressive calcification of liver tumors and minor but stable response of lung lesions. [ABSTRACT FROM AUTHOR]

Vascular tumors are categorized into benign hemangiomas, frankly malignant angiosarcomas and tumors with intermediate biological behavior (hemangioendotheliomas). The latter group includes hemangioendotheliomas of the epithelioid, kaposiform, retiform and composite subtypes. Furthermore, a heterogeneous group of both benign and intermediate vascular tumors exhibits a peculiar hobnail cell morphology. This heterogeneous group encompasses hobnail hemangioma, retiform hemangioendothelioma, papillary intralymphatic angioendothelioma and a subset of angiosarcoma. We herein present a case of a cutaneous vascular neoplasm with hobnail morphology and unusual gross features. Wachter DL, Agaimy A. A cutaneous vascular neoplasm with hobnail microscopic morphology and unusual gross features. [ABSTRACT FROM AUTHOR]

Angiosarcomas are malignant endothelial cell tumors with few effective systemic treatments. Despite a unique endothelial origin, molecular candidates for targeted therapeutic intervention have been elusive. In this study, we explored the tunica internal endothelial cell kinase 2 (Tie2) receptor as a potential therapeutic target in angiosarcoma. Human angiosarcomas from diverse sites were shown to be universally immunoreactive for Tie2. Tie2 and vascular endothelial growth factor receptor (VEGFR) antagonists inhibited SVR and MS1-VEGF angiosarcoma cell survival in vitro. In the high-grade SVR cell line, Tie2 and VEGF antagonists inhibited cell survival synergistically, whereas effects were largely additive in the low-grade MS1-VEGF cell line. Xenograft modeling using these cell lines closely recapitulated the human disease. In vivo, Tie2 and VEGFR inhibition resulted in significant angiosarcoma growth delay. The combination proved more effective than either agent alone. Tie2 inhibition seemed to elicit tumor growth delay through increased tumor cell apoptosis, whereas VEGFR inhibition reduced tumor growth by lowering tumor cell proliferation. These data identify Tie2 antagonism as a potential novel, targeted therapy for angiosarcomas and provide a foundation for further investigation of Tie2 inhibition, alone and in combinations, in the management of this disease. [ABSTRACT FROM AUTHOR]

Summary: Epithelioid angiosarcoma of the parotid gland is rare, and may pose a great diagnostic challenge. We report a case of primary epithelioid angiosarcoma in a 64-year-old male without history of radiation. The histopathological findings demonstrated a high grade epithelioid neoplasm. Immunostaining showed that the tumor was positive for the pan-cytokeratin, p63, cytokeratin18, Vimentin and vascular markers CD31, and was negative for CD34, cytokeratin5/6, cytokeratin7, cytokeratin20, CD68, CD30, S-100, HMB45, desmin, α–SMA and CD45. The tumor was diagnosed as an epithelioid angiosarcoma. To our knowledge, this is the first reported case of angiosarcoma which showed common positivity for cytokeratin and p63. In addition to cytokeratin, p63 is considered a useful marker for carcinoma. The co-expression of cytokeratin and p63 in epithelioid angiosarcoma represents a diagnostic pitfall. Thus, using a panel of antibodies is essential for distinguishing this tumor from poorly differentiated carcinoma. [ABSTRACT FROM AUTHOR]

Abstract: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of uncertain biologic behavior. Most cases come out as a single lesion of the soft tissue but also may appear in the lung, liver, and other locations. Epithelioid hemangioendothelioma of bone is an extremely rare tumor and more prevalent in the second and third decades of life; its behavior is uncertain, it most commonly is unifocal, and it affects preferentially lower extremities. In this work, we present the clinical, radiologic, and pathologic findings of a 19-year-old man with a multicentric EHE of bone that involved 3 vertebrae and developed lung metastasis. [Copyright &y& Elsevier]