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Pulmonary angiosarcoma is a rare and malignant disease of the blood vessels. Initially, it can be misdiagnosed as chronic thromboembolic hypertension (CTEPH). In CTEPH, there is increased pressure and resistance of the pulmonary arteries following persistent obstruction of pulmonary circulation from (recurrent) thromboembolism despite adequate anticoagulative treatment.A 76-year-old patient was referred to our centre for pulmonary hypertension after a central, left-sided, subacute pulmonary thromboembolism had been observed 7 months earlier. It was treated with apixaban, but the patient described persistent dyspnoea and cough. We observed severely reduced diffusion capacity, ineffective ventilation during cardiopulmonary exercise testing and right heart strain on echocardiograph, signs that are in agreement with suspected CTEPH. Computer tomography of the chest showed a persistent, size-constant obliteration of the left main pulmonary artery, and ventilation perfusion scan confirmed complete interruption of perfusion. We suspected malignancy; PET-CT scan confirmed metabolically active lesions. Histopathological examination of a sample obtained from the lesion by endobronchial ultrasound-guided needle aspiration showed a sarcomatous tumour with amplification of the MDM2-gene. We diagnosed an intimal angiosarcoma of the left pulmonary artery and referred the patient to pneumectomy.Angiosarcoma of the pulmonary arteries is a rare differential diagnosis of persistent thrombotic lesion and suspected CTEPH. In 2015 there were less than 300 cases described.Pulmonary angiosarcoma should be considered if: lesion occupies the entire lumen of pulmonary arteries with dilatation, contrast enhancement and infiltration of the wall in radiological examination, FDG-PET CT reveals metabolically active lesions, no pulmonary thromboembolism was documented in the anamnesis, increase in size is seen despite anticoagulation, patient presents with B symptoms.Diagnosis confirmed by biopsy, resection of tumour and removal of metastases is the therapeutic standard. Median survival remains poor. Further research is needed for improved diagnosis and treatment.Das Angiosarkom ist eine seltene, maligne Erkrankung, die von den Blutgefäßen ausgeht. Das pulmonale Angiosarkom kann sich diagnostisch initial wie eine chronisch thromboembolische pulmonale Hypertonie (CTEPH) darstellen. Die CTEPH ist eine Erhöhung des pulmonal-arteriellen Drucks und Widerstands als Folge einer persistierenden Obstruktion der Lungenstrombahn nach pulmonalen Thromboembolien trotz adäquater Antikoagulation.In unserer Ambulanz für pulmonale Hypertonie stellte sich ein 76-jähriger Patient mit anamnestisch vor 7 Monaten erlittener subakuter, links zentraler Lungenarterienembolie (LAE) vor, die seitdem mit Apixaban therapiert wurde. Bei persistierender Belastungsdyspnoe wurde der Verdacht auf eine CTEPH gestellt und es erfolgte weitere Diagnostik. Passend zur CTEPH zeigte sich spiroergometrisch eine ineffektive Ventilation und echokardiografisch eine Rechtsherzbelastung. Eine Verlaufs-CT des Thorax ergab einen persistierenden und größenkonstanten Füllungsdefekt der linken Pulmonalarterie mit szintigrafischem Nachweis einer primären Perfusionsstörung des gesamten linken Lungenflügels. Mit dem Verdacht auf ein malignes Geschehen veranlassten wir eine PET-CT, in welcher sich die Läsion der Pulmonalarterie stoffwechselaktiv darstellte. Eine unter endobronchialer Ultraschallkontrolle durchgeführte Punktion der Läsion ergab histologisch den Nachweis eines sarkomatoiden Tumors mit Amplifikation des MDM2-Gens. Wir stellten die Diagnose eines Intimasarkoms der linken Pulmonalarterie und führten den Patienten einer Pneumektomie zu.Die Intimasarkome der Pulmonalarterie sind selten und führen durch Wachstum und Thrombusneigung zur Stenose des Gefäßes. Die damit einhergehende klinische und echokardiografische Präsentation kann eine CTEPH imitieren und stellt somit eine wichtige Differenzialdiagnose zu dieser dar. 2015 wurden weniger als 300 Fallberichte beschrieben.Hinweise auf ein pulmonales Angiosarkom: Kontrastmittel aufnehmende, intraluminale Gewebsformation mit Dilatation der Pulmonalarterie und Infiltration der Gefäßwand, erhöhte Stoffwechselaktivität des Befundes in der FDG-PET CT, Größenprogredienz unter Antikoagulation, anamnestisch keine stattgehabte LAE, B-Symptome.Die Diagnosesicherung erfolgt durch Biopsie. Das mediane Überleben wird mit 1,5–17 Monaten beschrieben, welches mit therapeutischen Interventionen um einige Monate mit Symptomreduktion verlängert werden kann. Als therapeutischer Standard gilt die Resektion des Tumors mit ggf. Metastasenentfernung. Der Nutzen von Radio- und Chemotherapie ist Bestandteil aktueller Forschung. Eine Behandlung ist in erfahrenen Zentren anzustreben und es bedarf weiterer Forschung zur Verbesserung von Diagnostik und Therapie.

Whilst cutaneous angiosarcoma is rare tumour which primarily affects elderly patients, its management presents a significant therapeutic challenge. Indeed, complete surgical excision is often not possible due to the location and the diffuse and extensive nature of the tumour. Therefore, current treatment strategies often include chemo- and/or radiotherapy.We report our experience of combined chemo- and radiotherapy in the clinical course of 6 patients with cutaneous angiosarcoma who were treated between 2007 and 2018.All patients presented non-resectable tumours and were treated with radiotherapy in combination with the administration of liposomal, pegylated doxrubicin (25 mg/mCombined radio- and chemotherapy with liposomal, pegylated doxorubicin is a useful therapeutic option in the management of cutaneous angiosarcoma. Given the short-lived response rate, new treatment options are urgently required.

Upper gastrointestinal hemorrhage represents a common symptom in the internal medical practice. This Case Report depicts a severe gastrointestinal bleeding from an angiosarcoma that required multiple blood transfusion. As shown in this case, the actual cause may initially remain unclear. Repeated tissue sampling is mandatory to confirm a diagnosis and provide adequate treatment. Primary gastrointestinal angiosarcomas are extremely rare and highly aggressive malignant tumors that tend to grow multifocally and metastasise early. The only curative treatment option is radical surgery, thus an early stage diagnosis is crucial. Palliative care for metastatic angiosarkoma is currently a topic for clinical studies.

Mesenchymal tumors and tumor-like lesions of the urinary bladder are rare. They encompass a heterogeneous group of reactive pseudosarcomatous tumor-like changes and benign neoplastic lesions as well as malignant neoplasms (sarcomas) with variable biological behavior. The well-known differential diagnostic difficulties related to these conditions are mainly due to their rarity and thus limited experience and familiarity with their histological features and due to the significant morphological overlap between fully benign reactive conditions and aggressive malignant neoplasms. The distinction between them may on occasion represent a real challenge and is associated with several pitfalls. This overview summarizes the clinicopathological and differential diagnostic aspects of the most important lesions and discuss their differential diagnosis in the light of current knowledge.

Due to optimization of surgical techniques in surgical oncology and vascular surgery, the most modern approaches of anesthesia and intensive care medicine and effective multimodal therapeutic strategies, locally advanced malignant tumors are resected more frequently with a potentially curative intent. In the case of extensive tumors with infiltration of vital vascular structures or of structures which are crucial for extremity preservation, the necessary surgical procedure for complete tumor removal poses a major challenge for the surgeon and incorporates a high risk of perioperative morbidity for the patient. The decision to attempt tumor resection should therefore always be based on a concept considering all aspects of the malignant disease. The treating team should be highly experienced in this complex field of surgery, not only with respect to the surgical approach but also regarding the management of postoperative complications. In this article relevant aspects of decision making, surgical technique and postoperative outcome for malignant tumors involving vascular structures of the retroperitoneum and pelvis are presented.

This article reports a case of primary renal angiosarcoma, a very rare and aggressive malignancy, in a 59-year-old male patient. The mean overall survival time is limited to a few months if the diagnosis is made when clinical symptoms are present but chances of a cure can be increased with surgical resection of smaller incidental findings. Due to the lack of a standard therapy, systemic treatment is based on the therapy of other soft tissue sarcomas. The role of adjuvant medical treatment particularly in angiosarcoma remains poorly studied but using a doxorubicin-based chemotherapy regimen, a survival benefit can be achieved.

Cutaneous angiosarcoma is an aggressive endothelial cell neoplasm with a high associated mortality. Reliable and confident diagnosis is necessary but frequently challenging due to the wide morphological spectrum and broad differential diagnosis of the disease. Furthermore, the final diagnosis often relies on immunohistochemical evidence of endothelial cell differentiation which is further complicated by the lack of sensitivity and specificity of conventional endothelial cell markers. The following article outlines the clinical and histological spectrum of cutaneous angiosarcoma with emphasis on novel findings relating to immunohistochemistry and molecular genetics in the context of the relevant differential diagnosis.

Mammography for the detection of early stage breast cancer is widely established. Breast-conserving therapy followed by radiation therapy is the most common therapy for early stage breast cancer. Due to the increasing use of radiation therapy in breast cancer, secondary neoplasia are induced. The most common secondary neoplasia of the breast is the angiosarcoma. It occurs 4-7 years after radiation therapy. Genetic predisposition and a transformation of endothelial cells by radiation therapy are known to lead to angiosarcoma.The present paper outlines a concept for treatment on the basis of the current literature and the experience of the authors.Radical surgical resection of the tumour with a sufficient margin of safety is the treatment of choice for angiosarcoma. Tumour infiltration of the chest wall and other local structures are challenges for the surgeon performing a radical tumour resection. The replacement of the tissue is also demanding for the surgeon. After radiation therapy the reconstructive options are limited. Individual factors such as age, quality of local vessels and the patients demand for breast reconstruction should be considered. Although R0 resection is performed, the 5-year survival rates are 20-30%.

We report a case of a 62 year old woman who presented with Ogilvie's syndrome as a complication of mastectomy with free TRAM flap reconstruction due to angiosarcoma of the right breast. In the acute postoperative period, the patient expired as a result of Ogilvie's syndrome related complications. Several assumptions regarding the causes of Ogilvie's Syndrome as well as current theories about aetiology, diagnosis and therapy are discussed in this case presentation.

To evaluate the diagnostic value of peritoneal fluid (PF) cytology for clinical diagnosis of abdominal neoplasia in horses.Ten horses with histopathologically confirmed abdominal neoplasia, in which a PF analysis was performed, were included in this retrospective study. PF was analyzed for total protein concentration and a nucleated cell count was performed. Using cytological criteria of malignancy, the PF samples were evaluated regarding their probability of malignancy.Cytologic classification of cells according to criteria of malignancy allowed a positive cytologic diagnosis of neoplasia in 5 out of 10 peritoneal fluid samples. Malignant lymphoma was the most commonly diagnosed neoplasia (3/10) and could be identified by cytology in 2/3 cases. In 1/2 horses with plasma cell myeloma neoplastic cells were similarly found. Malignant melanoma (2/10) was diagnosed using cytology in one case (presence of melanin-containing cells). Cytological diagnosis of malignant neoplasia was established in the only horse with gastric squamous cell carcinoma, but the morphology of the identified tumour cells did not allow a specific diagnosis. Thus, a definitive diagnosis was achieved in 4/5 horses with proven abdominal neoplasia. The horses with adenocarcinoma (1/10) and haemangiosarcoma (1/10) had no evidence of neoplasia based on cytological findings. No relationship between total protein concentration or the nucleated cell count with the histolopathological diagnosis of abdominal neoplasia was found. Abnormal mitotic figures were considered of greater diagnostic value than the overall mitotic rate.The implementation of nuclear criteria of malignancy in the cytologic evaluation of PF samples allows the identification of neoplastic cells to an acceptable degree. For this purpose, the knowledge of the highly variable morphological features of mesothelial cells is essential. The absence of malignant cells does not rule out abdominal neoplasia.PF cytology should be considered as a valuable, minimally invasive, simple, and rapid diagnostic technique in horses with suspected abdominal neoplasia.

Splenic masses have a high prevalence and are more common than diffuse splenic enlargement in dogs. It was the aim of the present study to retrospectively describe clinical aspects and histopathologic characteristics of dogs with splenic masses.Records of patients with a histologically diagnosed splenic mass between January 2000 and March 2011 were reviewed.249 dogs met the inclusion criteria and could be included in the study. Splenic masses were diagnosed histologically as non-malignant disease (n=117; 47%) and malignant splenic disease (n=132; 53%). Hemangiosarcoma was the most common histological diagnosis (n=97; 73.5%). Other malignant tumors included sarcoma (n=14), fibrohistiocytic nodules (n=9) as well as lymphoma, blastoma and adenocarcinoma. The non-malignant masses consisted of nodular hyperplasia (n=60), splenic hematoma (n=41), and splenitis (n=6). Dogs with hemoperitoneum had a higher frequency of splenic neoplasia.The results corroborate previous findings that hemangiosarcoma is the most frequent neoplasm of the canine spleen. However, in approximately half of the cases benign lesions were histologically diagnosed.It is essential that a frank discussion is held with owners regarding the prognosis associated with the treatment of dogs with a splenic mass associated with hemoperitoneum.

A 76-year-old woman was admitted with a two-months history of pain in the right upper abdomen and nausea. There was no disease or premedication in her history.Labaratory tests revealed a normocytic anemia, elevated liver enzymes and signs of cholestasis. An MR of the abdomen showed a hyperperfused tumor in the liver segments IV, V and VIII, likely to be a hepatocellular carcinoma. However, tumor markers including AFP were not elevated. A liver biopsy revealed the final diagnosis of angiosarcoma.Because the tumor was not resectable transarterial chemoembolisation (TACE) with doxorubicin was performed with palliative intent. Six weeks later a CT scan revealed extensive tumor progression. Therefore no further causal tretament was performed. With best supportive care the patient died within 4 weeks after she had been discharged. Occupational history revealed that the woman had been exposed to polyvinylchloride for six years when she had worked in a factory producing varnish aerosol cans 44 years ago.Angiosarcomas of the liver are rare, highly malignant and diffuse infiltrating vascular tumors with rapid growth and poor prognosis. In patients who have been exposed to polyvinylchloride and present with an indistinct lesion of the liver an angiosarcoma should be considered.

Hemangiosarcomas of the breast represent a rare disease of the breast mainly occurring as secondary neoplasias with a latency of 5-10 years after primary treatment of breast cancer and are associated with an unfavourable prognosis. Radiation therapy, which is integrated within the concept of breast conserving therapy ranks as the main risk factor.In this report we describe the clinical course of 4 patients including their molecular genetic pattern and give a summary of the actual literature.Hemangiosarcomas occur as a secondary neoplasm with a latency of 5-10 years after primary treatment of breast cancer and have an unfavorable prognosis. A genetic predisposition is assumed, but we could not find a significant role of tumor suppressor genes BRCA1, BRCA2 or p53 in our patients.Due to limited data available for these tumors, recommendations for therapy include radical tumor resection achieving wide free margins and inconsistent regimens of chemo- and/or immunetherapy modalities. In the majority these are based on systemic therapy regimens for other cutaneous sarcomas, such as Kaposi's sarcoma. Efforts should be taken for a nation-wide systematic registration of all cases of post-irradiation hemangiosarcomas.

A 65-year-old man was admitted with history of claudication symptoms and painful skin lesions of the lower legs. Physical examination showed palpable purpura of the lower legs and livedo reticularis, most marked at the forefoot and toes.Computed tomography (CT) showed an aortic mass 2 cm above the bifurcation. This was treated after angiography with a covered stent. Biopsy of the skin lesions showed no sign of vasculitis and no cholesterol crystals.The patient was discharged and remained symptom-free for 9 months. He was readmitted at that time with recurrent complaints. CT revealed a subtotal stenosis of the aortic stent. A skin biopsy showed CD31-positive tumor cells in small arteries. Biopsy of a new osteolytic lesion in the ileum confirmed the diagnosis of angiosarcoma of the aorta. The patient decided in favor of palliative care and was discharged from the hospital.Primary tumors of the aorta, although they are rare, should be considered in the presence of an intravascular mass with stenosis to blood flow. A skin biopsy is easy to conduct and often leads to the final diagnosis.

Cardiac magnetic resonance imaging (MRI) is an important tool for the diagnosis of cardiac masses. Various cardiac tumors are predisposed to occurring in atrial structures. The aim of this review article is the description of atrial tumors and their morphological features in MRI. In general, cardiac tumors are rare: approximately 0.001 - 0.03 % in autopsy studies. About 75 % of them are benign. The most common cardiac tumor is the myxoma. They are predisposed to occur in the atria and show a characteristically strong hyperintense signal on T 2-wieghted images in MRI. In other sequences a heterogeneous pattern reflects its variable histological appearance. Lipomas exhibit a signal behavior identical to fatty tissue with a typical passive movement in cine imaging. Fibroelastomas are the most common tumors of the cardiac valves. Consisting of avascular fibrous tissue, they often present with hypointense signal intensities. Thrombi attached to their surface can cause severe emboli even in small tumors. Amongst primary cardiac malignancies, sarcomas are most common and favor the atria. Secondary malignancies of the heart are far more common than primary ones (20 - 40 times). In case of known malignancies, approximately 10 % of patients develop cardiac metastasis at the end of their disease. Lymphogenic metastases favor the pericardium, while hematogenic spread prefers the myocardium. Since they are not real atrial tumors, thrombi and anatomical structures of the atria have to be differentiated from other pathologies.

A 4.5-year-old Swiss Braunvieh cow was presented to the Department of Farm Animals, University of Zurich, because of severe haematuria. All other clinical findings were within normal ranges. Transrectal ultrasonography revealed a 1 cm x 1 cm echogenic, irregularly-shaped, raised mass in the wall of the urinary bladder. Endoscopy identified the mass as a proliferation, approximately 0.5 cm in diameter, which was bleeding continuously. Thermocautery of the bleeding site was carried out twice five days apart via endoscopy. Clinical signs resolved for the remainder of the cow's life; she was slaughtered 15 months later because of infertility. Histological examination of the mass revealed a haemangiosarcoma.

Angiosarcoma of the temporal bone is an extremely rare malignant tumor, which originates from vascular endothelium. We describe a case of a 57-year-old woman, initially presenting with progressive hearing loss, otorrhea as well as facial palsy diagnosed as a mastoiditis elsewhere. After subtotal mastoidectomy histological examination revealed an angiosarcoma of the mastoid. Since the tumor was not completely removed lateral petrosectomy and postoperative radiotherapy were performed. Afterwards the patient developed local recurrence with intracerebral tumor extension. During palliative polychemotherapy the patient developed a pneumonia and deceased. In this manuscript the morphology, imaging characteristics and current treatment options of angiosarcomas of the lateral skull base are reviewed and discussed.

Angiosarcomas are rare tumors accounting for 1-2% of all soft tissue sarcomas. Nonetheless a dermatologist needs to be knowledgeable about this tumor because the prognosis is particularly poor. Angiosarcoma favors superficial soft tissues and skin (60%) with a clear predilection for the head and neck region. The average age of the patients presenting with cutaneous angiosarcomas is around 70 years with a peak incidence in the 8th decade. However, some subtypes may occur in children and adolescents. Secondary angiosarcoma after tissue-conserving radiation therapy for carcinoma of the breast represents an increasing problem, both the differentiation between atypical vascular lesions and true aggressive angiosarcoma and the therapy are challenging. The prognosis for angiosarcoma patients is gloomy despite all therapeutic efforts. Only early therapy seems to influence the outcome at all. There are some established guidelines for the primary and palliative therapy. Interesting new options of biomodulatory and molecularly targeted therapy can be envisioned.

Neoplastic diseases were described very rarely in addax (Addax nasomaculatus). In this communication clinical signs, morphological and immunohistological findings in a 15-year-old, female addax with a primary cutaneous, re-occurring and metastasing haemangiosarcoma of a forelimb are reported.

Cutaneous angiosarcoma is a rare tumor of endothelial origin, often difficult to diagnose and with an unfavorable prognosis. A 85-year-old woman presented with an extensive angiosarcoma involving her right leg. The tumor was not clinically typical but the diagnosis was confirmed histologically. Because of her age and the extent of the tumor, we elected to treat primarily with cobalt-60 irradiation. The tumor unfortunately progressed during radiation therapy, so we decided to begin palliative chemotherapy with liposome-encapsulated doxorubicin. The patient received six cycles of doxorubicin (Caelyx, 15 mg/m(2) i.v. in four-week intervals) which was well-tolerated and led to complete regression of angiosarcoma which has lasted for 6 months. Chemotherapy with liposome-encapsulated doxorubicin may represent a well-tolerable therapeutic option in cases where surgery and irradiation are not possible or fail. Further studies are necessary to prove the efficacy of doxorubicin therapy in angiosarcoma.

Primary angiosarcoma of the aorta is a rare disease. The prognosis is poor, resulting of embolic complications and early metastatic disease, with a median survival of nine months. Diagnosis is difficult and often made post-mortem. We report the case of a 68-year-old woman referred for a thrombosis of the superior mesenteric artery, occurring a few weeks after resection of an angiosarcoma of the small intestine, disclosing a primary angiosarcoma of the aortic wall with metastatic disease.

An 84-year-old gentleman developed an angiosarcoma in the lateral temporal fossa 60 years after a World War II injury. The tumour resection confirmed the close spatial relationship of the tumour and a shell splinter. Analysis of the metallic composition showed no clear carcinogenic potential of the ingredients; an inflammation-related carcinogenesis seems more probable. A review of the literature revealed two similar cases.

The treatment of soft tissue sarcomas includes different modalities, but the complete excision of the tumor is the most important one. It is often difficult to resect the tumor completely and simultaneously restore a good function of the upper limb. Therefore a microsurgical reconstruction is often necessary as demonstrated here.

A clinico-pathologic study of typical symptoms of intra- and extraoral angiosarcomas and clinical course under therapy is presented as well as an analysis of the immunohistochemical differential diagnosis of the tumour specific formed spaces.Four male patients aged 63-78 years suffered from angiosarcomas of the maxillary sinus, the bucca (two patients) and the alveolar ridge of the lower jaw.For comparative analysis paraffin embedded tissue of the initial biopsies was available. The slides were stained with standardized HE, PAS and Gömörri. For standardized immunohistochemistry following primary antibodies were applied: monoclonal antibodies to pancytoceratin clones AE1/AE3, alpha-smooth-muscle-actin clone 1A4, CD31 clone JC/70A, factor-VIII-related antigen clone F/86, Fli-1 (polyclonal, Zymed, USA), tenascin-C: BC4 (Prof. L. Zardi), oncofetal glucosylated fibronectin clone FDC6 (ACCR), laminin-5: D4B5. Detection using AP-ChemMate and Autostainer (Dako, Denmark).While the benign appearance of the lesions resulted primarily in wrong diagnoses the histopathologic examination of the biopsies revealed the characteristic pattern of angiosarcomas. Wide surgical excision, radiotherapy and/or antiangiogenic chemotherapy could not prevent tumour progression and death within two and a half years after primary diagnosis. All angiosarcomas reacted partially positive for factor-VIII-related antigen and CD31. The tumour associated structural defect of vascular lamina with partial loss of pericytes/vascular smooth muscle cells was identified immunohistochemically by alpha-smooth-muscle-actin and for the first time by tenascin-C.(1.) The variable presentation and the benign appearance of oral and perioral angiosarcomas may often delay diagnosis. Oral and perioral angiosarcomas show poor prognosis despite of multimodal therapy. (2.) Cytoceratin and laminin-5-positivity as typical epithelial antigens don't exclude angiosarcoma. Factor-VIII-related antigen, CD31 as well as Fli-1 identify angiosarcoma. (3.) alpha-smooth-muscle-actin and the loss of the tenascin-C-matrix indicate immunohistochemically the characteristic sarcomatous defect of differentiation.

Angiosarcoma of the liver is a rare, highly malignant and sometimes diffusely infiltrating vessel tumor with rapid progression and poor prognosis.A 46-year-old male patient with rapidly progressive liver failure, initially regarded as decompensation of known alcoholic liver cirrhosis, is reported. The patient was referred to the authors' center for evaluation of liver transplantation, but a massive weight loss despite long absence of any alcohol intake raised the suspicion of a malignant disease. The abdominal ultrasound examination showed a massive hepatomegaly with an extremely inhomogeneous echo structure as well as moderate ascites. A following MRI demonstrated diffuse focal contrast enhancement in the entire liver parenchyma, confirming diffuse infiltration of the organ by a malignant tumor. In addition, MRI was suspicious of bone metastases. The usual tumor markers were normal. Sonographically guided percutaneous liver biopsy established the diagnosis of a malignant vascular tumor with diffuse infiltration of the liver parenchyma. 3 days later the patient died of uncontrollable bleeding from esophageal varices. Macroscopic examination of the liver during autopsy showed multiple lacunae filled with blood. Therefore, the differential diagnosis of a peliosis hepatis was raised. However, histology confirmed the diagnosis of a malignant angiosarcoma with diffuse osseous metastases.A diffuse infiltration of the liver by an angiosarcoma in the absence of any definite lesions may lead to a substantial delay of the diagnosis. The only relevant differential diagnosis in this case is the equally rare peliosis hepatis.

The Stewart-Treves syndrome (STS) is a lymphedema-associated angiosarcoma which usually develops in female patients after mastectomy and axillary lymph node dissection. A 55-year old woman developed STS in her lymphedematous left arm seven years after breast-preserving surgery with axillary lymph node dissection and radiotherapy. Various therapies which have been employed are radiotherapy and isolated limb perfusion with cytostatic agents. Since our patient had a huge lesion with additional chest wall involvement, neither approach represented a good option. Radiological staging showed no evidence of further lesions or metastases. We started infusion therapy with liposomal doxorubicin (20 mg/m2 body surface) fort six cycles at regular intervals of 14 days. The patient tolerated the therapy well. Palmar-plantar erythrodysesthesia, a well-known side effect of doxorubicin, did develop. Because the disease was stable, the therapy interval was increased to six weeks after the 6th cycle. The patient has shown no recurrence for eight months. STS is a very rare variant of an angiosarcoma with poor prognosis. The case report shows that liposomal doxorubicin provides an effective therapeutic option.

Transthoracic echocardiography is usually the initial diagnostic test in patients with a suspected cardiac mass. However, this technique is restricted by its small field of views and insufficient acoustic window in some patients. Magnetic resonance imaging (MRI) and, since its introduction, multislice spiral computed tomography allow for detailed delineation of intra and pericardiac tumors, their extent, and their influence on cardiac function. Primary benign and malignant cardiac tumors have several characteristic features in MR imaging. Assessment of such features may narrow down the differential diagnosis or even allow for reliable diagnosis in selected cases. Many such features can also be assessed using MSCT. This article provides an overview of examination protocols of MRI and CT for cases in which a cardiac mass is suspected and describes the appearance of primary and secondary cardiac masses as well as intracavitary thrombi.

Angiosarcomas are rare, aggressive tumors of vascular origin. They occur most often in areas of long term sun exposed skin in the elderly, in long standing lymphoedema or after radiation therapy. The prognosis is poor, radical surgery required, in addition radiation or chemo-therapy are considered to be therapeutical options. We report on a male patient, 70 years old with a slightly bleeding blueish tumor encompassing the complete nose. This patient treated with several antibiotics while continuous growth led to the described course. After 4 months a biopsy was taken revealing an angiosarcoma of the nose. Subsequently the patient was referred to our department and due to staging results a surgical therapy (ablatio nasi) followed. The final histological diagnosis was a low grade angiosarcoma of the cutaneous and subcutaneous tissue of the nose without infiltration of bone or cartilage, confirmed by immunohistological staining. Angiosarcomas of the head and neck region are extremely rare. Especially only few cases of nasal angiosarcoma infiltrating the complete nose are described in literature. Prognosis, therapy and differential diagnosis have to be discussed.

A 45-year old woman presented at our hospital with intermittent fever in the last three weeks and progressive exertional dyspnea. In addition she suffered from physical asthenia. Seven months ago the patient was treated for symptomatic pericardial effusion in our clinic.Physical examination revealed a pale skin color, epigastric pain on palpation and intense exertional dyspnea. Laboratory tests indicated microcytic anemia and high levels of LDH and CRP. The diagnosis of a large tumor of the right atrium and ventricle was based on the transthoracic echocardiogram and thoracic CT scan. The extent of the right atrial and ventricular mass of 9 x 8 cm was detected by trans-esophageal echocardiography. The thoracic and abdominal CT scan showed multiple nodular infiltrates in the basal lung fields and a cystic tumorous mass in the liver.The tumor developed in the short time of 7 months. Half a year ago, the transthoracic echocardiographic exam showed pericardial effusion with normal cardiac size, normal left ventricular function and no evidence of right ventricular outflow obstruction. No cardiac masses were observed. At that time pericardiocentesis was performed, but the pericardial fluid was cytologically negative for tumor cells. The diagnosis of angiosarcoma was made after surgical excision of the tumor and histological examination. There was only the possibility of a palliative therapy, because of the existence of pulmonary and liver metastases.Primary malignant cardiac tumors are rare and their prognosis is very poor. The heart angiosarcoma is often disseminated into the lungs and the liver at the time of clinical presentation. This case indicates that hemorrhagic nonspecific pericardial effusion, negative for tumor cells, can appear any time before a heart angiosarcoma is detectable by echocardiography.

A splenectomy was performed in a 37-year-old woman because of spontaneous rupture of the spleen. Primary angiosarcoma of the spleen was diagnosed postoperatively based on histology. Primary angiosarcoma of the spleen is a very rare neoplasm with disastrous prognosis. Immunohistochemical and ultrastructural examinations are necessary to verify the diagnosis because the histopathological picture of the tumor is variable. Due to the small number of reported cases, there are no guidelines for adjuvant or palliative therapy, and up to today all adjuvant means of radiotherapy and chemotherapy have not improved the prognosis of the patients.

The skin is the organ most commonly affected by malignancies. Various cancers of the skin show a dramatic increase in incidence over the last decades. Epithelial skin tumors are most frequently, e.g., basal cell carcinoma and the squamous cell carcinoma with its precursors, the actinic keratoses. Melanoma, which is extremely difficult to treat in advanced tumor stages, is dreaded. Besides that, there are other epithelial malignant diseases, e.g. Morbus Bowen and adnexal tumors originating from the skin appendices. Mesenchymal malignant neoplasias such as Morbus Kaposi, angiosarcomas and other dermal sarcomas, are rare. Since the majority of malignant neoplasms is removable and curable by a simple surgical intervention, the knowledge of the different skin tumors is essential for non-dermatologist.

A 68-year-old male patient presented with unspecific decline of general health, multiple tumours in the liver discovered by ultrasound examination and a focus suggestive of tumour on chest x-ray. He was hospitalised because of suspected metastatic lung carcinoma. Laboratory analysis revealed a distinct elevation of serum neuron-specific enolase (NSE, 26,1 micro g/l). A CT scan of the abdomen confirmed the presence of multiple tumour foci in both hepatic lobes, while the lung, however, was found to be free of tumour on CT-examination. Bronchoscopy, gastroduodenoscopy and colonoscopy were unremarkable. Subsequently, an ultrasound guided fine-needle biopsy of the liver was performed. Histological examination disclosed hemangiosarcoma with unusual, though unequivocal expression of NSE in tumour cells. As even follow-up examinations were unable to trace out any extrahepatic primary tumour, the hemangiosarcoma was concluded to be of hepatic origin. The patient succumbed to his disease 6 months later, an autopsy was not performed. This case report of a NSE-producing primary hemangiosarcoma of the liver clearly outlines that a histological confirmation of an allegedly clear clinical diagnosis should always be performed because malignant tumours are capable of (co-)expressing so called tumour markers independently of their histogenesis.