Lower Extremity Angiosarcoma: A Life-Threatening Complication of Lymphedema.

ABSTRACT: When angiosarcoma, a rare and aggressive tumor of the soft tissue, develops in the setting of chronic lymphedema, it is referred to as Stewart-Treves syndrome. It is usually seen in chronic lymphedema of the upper limbs postmastectomy. Angiosarcoma developing in the lower limb in the setting of chronic lymphedema is rare and has a poor outcome. The presentation of angiosarcoma can vary, ranging from a bleeding papule to a plaque or a subcutaneous mass, which can later progress to ulceration or necrosis. Treatment for Stewart-Treves syndrome is aggressive because of its poor prognosis and usually requires a multidisciplinary approach of surgery, radiation, and chemotherapy. Several theories have been put forth to explain the mechanism of Stewart-Treves syndrome, but it remains ambiguous. The current literature regarding angiosarcoma developing in the setting of chronic lymphedema in the lower limb is limited to single case reports. Herein, the authors report a series of six cases of biopsy-proven angiosarcoma in the setting of lower extremity lymphedema. Providers should include angiosarcoma in the differential diagnosis of ulcerative or vascular tumors arising in the context of lower extremity lymphedema.

Obstructive cardiac myxosarcoma of the right ventricular outflow tract with pulmonary embolism and concurrent right atrial hemangiosarcoma in a dog.

A 13-year-old spayed female rottweiler crossbreed dog was presented with an 8-day history of abnormal gait and collapse associated with excitement or physical activity. A cardiac gallop was noticed on thoracic auscultation, and a 1st-degree atrioventricular block and sinus tachycardia were noted on an electrocardiogram. Echocardiography identified a hypoechoic, irregularly marginated luminal mass in the right ventricle at the level of the pulmonic valves. Postmortem gross examination confirmed the presence of a soft, polypoid, and botryoid mass (9 × 3 × 3 cm) with a smooth and glistening surface attached to the endocardium of the right ventricular outflow tract and extending to the pulmonary artery. The histological findings were consistent with the diagnosis of myxosarcoma with pulmonary embolism. In addition, the dog in this report had a right atrial hemangiosarcoma and a cutaneous hemangioma unrelated to her clinical findings. Key clinical message: Cardiac myxosarcomas are very rare neoplasms in dogs and concomitant primary heart tumors of different histogenesis are even rarer in dogs. To the authors' knowledge, this is the first report of coexistent myxosarcoma and hemangiosarcoma in the heart of a dog. Cardiac myxosarcomas should be considered in the differential diagnosis of intracavitary heart masses associated with signs of cardiac obstruction and failure.

Myxosarcome cardiaque obstructif de la voie d'éjection du ventricule droit avec embolie pulmonaire et hémangiosarcome auriculaire droit concomitant chez un chien. Une chienne croisée rottweiler stérilisée âgée de 13 ans a été présentée avec une histoire de démarche anormale et d'effondrement associés à l'excitation ou à l'activité physique depuis 8 jours. Un galop cardiaque a été noté à l'auscultation thoracique, un bloc auriculo-ventriculaire du 1er degré et une tachycardie sinusale ont été notés à l'électrocardiogramme. L'échocardiographie a permis d'identifier une masse luminale hypoéchogène et irrégulièrement marginalisée dans le ventricule droit au niveau des valvules pulmonaires. L'examen macroscopique post-mortem a confirmé la présence d'une masse molle, polypoïde et botryoïde (9 × 3 × 3 cm) avec une surface lisse et brillante attachée à l'endocarde de la voie d'éjection du ventricule droit et s'étendant jusqu'à l'artère pulmonaire. Les résultats histologiques concordaient avec le diagnostic de myxosarcome avec embolie pulmonaire. De plus, la chienne dans ce rapport présentait un hémangiosarcome auriculaire droit et un hémangiome cutané sans rapport avec ses résultats cliniques.Message clinique clé :Les myxosarcomes cardiaques sont des néoplasmes très rares chez le chien et les tumeurs cardiaques primaires concomitantes d'histogenèse différente sont encore plus rares chez le chien. À la connaissance des auteurs, il s'agit du premier rapport de myxosarcome et d'hémangiosarcome coexistant dans le cœur d'un chien. Les myxosarcomes cardiaques doivent être pris en compte dans le diagnostic différentiel des masses cardiaques intracavitaires associées à des signes d'obstruction et d'insuffisance cardiaque.(Traduit par Dr Serge Messier).

Recurrent metastatic angiosarcoma presenting as Kasabach-Merritt syndrome.

Angiosarcoma is an incredibly rare type of malignancy, accounting for only 1%-2% of all soft-tissue sarcomas globally. It is clinically, pathologically and radiologically difficult to diagnose angiosarcoma owing to its varied presentation with little or no well-defined imaging findings.Kasabach-Merritt syndrome is also a lesser-heard entity which carries extremely poor prognosis. It is primarily seen in infants with vascular malformations and in kaposiform haemangioendothelioma. It is a condition of consumptive coagulopathy and only few of the cases have been reported so far in the adults with a background of angiosarcoma.This report presents the case of a male in his 70s who was diagnosed with metastatic angiosarcoma and experienced a complicated disease course due to Kasabach-Merritt syndrome.

Bilateral pneumothorax in a patient with angiosarcoma of the scalp: a case report.

BACKGROUND: Angiosarcoma is a rare, vascular malignancy that arises from endothelial cells of blood vessels. This case report aims to create the awareness of its existence in the region and its mode of presentation. CASE PRESENTATION: A 63-year-old Pakistani man presented to the emergency department with sudden bilateral chest pain and shortness of breath for 2 days. On examination, a scalp lesion was seen which had been increasing in size over the last 6 weeks. The lesion was 8 × 10 cm in size with an irregular border, non-tender, violet and dome-shaped in elevation on the right occipito-parietal lobe of the skull. Chest computed tomography (CT) showed multiple cystic lesions on both lungs, patchy areas of ground-glass opacities, nodules of variable sizes and bilateral pneumothorax. Bilateral tube thoracostomy was performed which provided symptomatic relief for shortness of breath. His bronchoalveolar lavage (BAL) was negative for infection. He underwent biopsy of scalp lesion which was positive for aggressive angiosarcoma. CONCLUSION: Bilateral spontaneous pneumothorax can be the initial manifestation of aggressive cutaneous angiosarcoma and frequently leads to respiratory failure. Early recognition is essential to prevent delay in diagnosis and management.

A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.

BACKGROUND: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. OBJECTIVES: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. METHODS: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. RESULTS: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. STUDY LIMITATIONS: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. CONCLUSIONS: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

A rare coexistence: Poland's syndrome and cardiac angiosarcoma.

Poland's syndrome, a rare genetic disorder that accompanies malignancies, musculoskeletal disorders, cardiac and genitourinary syndromes. There is no study that represents the association between cardiac angiosarcoma and Poland's syndrome. A 24-year-old female patient previously diagnosed with Poland's syndrome was admitted to our hospital complaining of dyspnea. Diagnostic imaging showed an irregular mass in the right atrial cavity. After successful surgery, she was discharged uneventfully and the 3rd month oncologic follow-up reveals none of residual mass. The coexistence has not been diagnosed and treated in a cardiac surgery department before. With this presentation, we aimed to contribute to the literature with this presentation, for the right and early diagnosis and management of possible new cases in the future can be diagnosed and treated correctly and early.

[Face to a bilateral pneumothorax, just scratch your head!] / Devant un pneumothorax bilatéral, il faut bien se gratter la tête !

Head and neck skin angiosarcoma is a rare and aggressive tumor (1 % of head and neck tumors). Prognosis remains poor, with a 5-year survival rate ranging from 10 to 54%, depending on the initial stage. Metastatic disease markedly worsens the prognosis. Metastatic lung involvement is classic and can take on several forms. The cystic form is responsible for numerous complications, particularly pneumothorax. In this case, an 83-year-old patient was diagnosed with bilateral pneumothorax complicating cystic interstitial lung disease, which was revealed by hemoptoic sputum. Skin examination revealed two large necrotic lesions of the calvaria. Anatomo-pathological examination confirmed cutaneous angiosarcoma on both skin biopsy and lung resection. At a metastatic stage, only systemic treatment with paclitaxel can be proposed. The clinical course was unfavorable, leading to death before any specific treatment. This observation highlights the importance of a complete clinical skin examination in the assessment of pulmonary cystic lesions.

Skull angiosarcoma associated with a calcified chronic subdural hematoma.

Angiosarcoma is an uncommon, aggressive endothelial-cell tumor that usually affects the skin, and involvement of the skull is rare. Here, we describe a case of skull angiosarcoma associated with a calcified chronic subdural hematoma (CSDH). HIGHLIGHTSA very rare case of skull angiosarcoma associated with a calcified chronic subdural hematoma is presented.An asymptomatic subdural hematoma with an atypical history and radiological features should prompt further investigation.Contrast MRI images should to be obtained early to differentiate a subdural hematoma from other pathologies.

Suspicious Plantar Lesion-A Case Report of Angiosarcoma.

Diabetic foot is one of the most common complications of diabetic mellitus (DM). This DM patient was admitted to our hospital presented with a 2-month history of plantar lesion. Shortly afterward, the patient appeared hemoptysis, respiratory failure, and multiple purpuric papules on his limbs. Biopsy of left plantar lesions demonstrated angiosarcoma. Therefore, it is suggested that tissue biopsy should be taken as early as possible for DM patients with prolonged nonhealing wounds.

Hemoptoe, thin-walled lung cysts, and spontaneous pneumothorax are features of metastatic cutaneous angiosarcoma : A case report.

We present a case of bilateral cystic lung metastases originating from cutaneous angiosarcoma (cAS) of the scalp in a 73-year-old man. He presented with hemoptysis and recurrent bilateral pneumothorax. The clinical, radiological, and histological features and a potential pathophysiological mechanism of pulmonary changes in cutaneous angiosarcoma are discussed.

[Metastatic lesion of the bone marrow caused by primary angiosarcoma of the spleen]. / Metastaticheskoe porazhenie kostnogo mozga pri pervichnoi angiosarkome selezenki.

The article presents a rare case of bone marrow metastasis of the spleen angiosarcoma. The observation is of particular interest due to the fact that secondary bone marrow damage in angiosarcoma in the vast majority of cases is due by primary tumor growth in the spleen. Clinically, such cases may resemble the course of blood diseases with hematological disorders and splenomegaly. Patients come into the field of view of a hematologist, and the final diagnosis is unexpected to the attending physician. Detection of angiosarcoma growth in a bone marrow trephine biopsy during morphological examination can be a rare finding for a pathologist. In this regard, the presented case is of interest not only for pathologists, but also for doctors of clinical specialties.

Case report: angiosarcoma of the pulmonary artery diagnosed as pulmonary artery emboli.

BACKGROUND: Angiosarcomas are the most common malignant tumors of the heart and great vessels. Late onset and unspecific symptoms are reasons why a diagnosis is made rather late at a time when most tumors have already metastasized. We report a rare case of an angiosarcoma presenting as pulmonary artery emboli. CASE PRESENTATION: A 66-year-old patient was initially admitted to the hospital with a STEMI. Days later a successful reanimation due to ventricular fibrillation followed. An emboli of the pulmonary artery was diagnosed as the cause and after a lysis therapy the patient was discharged. A few weeks later the patient suffered an episode of absolute arrhythmia. TTE as well as CT-Scan showed an emboli of the pulmonary artery and a pericardial effusion with compression to the right ventricle. Intraoperative findings, showed an infiltrating tumor of the pulmonary artery, the pulmonary valve, the RVOT, the LA and LV. A resection of the tumor from the pulmonary artery, valve and RVOT was carried out. A new pulmonary valve was implanted with the reconstruction of the RVOT and pulmonary artery. Due to LV infiltration, only a palliative surgical approach was possible. Despite an uncomplicated postoperative course, the patient died at home two months later. CONCLUSION: Although a rarity, a tumor of the pulmonary artery should be taken into consideration as a differential diagnosis to pulmonary artery emboli. Development of better diagnostic tools (specific tumor markers) and more effective chemotherapeutic agents is necessary to improve the prognosis of these patients.

Evaluation of the validity of the double two-thirds rule for diagnosing hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass: a systematic review.

OBJECTIVE: To evaluate the validity of the double two-thirds rule for a diagnosis of splenic hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass. SAMPLE: Systematic literature review. PROCEDURES: 3 databases (PubMed, CAB abstracts, and World of Science) were searched in November 2020. Articles were included if data on dogs with nontraumatic hemoperitoneum due to a splenic mass were included and subsequent pathologic diagnosis could be determined. RESULTS: In total, 2,390 unique articles were identified, with 66 articles meeting the criteria for full-text review and 14 articles included for analysis. A total of 1,150 dogs were evaluated, with 73.0% (840/1,150) of dogs being diagnosed with a malignant splenic lesion and 27.0% (310/1,150) being diagnosed with a benign splenic lesion. Of the malignancies, 87.3% (733/840) were hemangiosarcoma. Levels of evidence were low, and bias was high as most included studies were retrospective case series. CLINICAL RELEVANCE: The double two-thirds rule should be refined when evaluating dogs with nontraumatic hemoperitoneum from a ruptured splenic mass, with more dogs being diagnosed with a malignancy and hemangiosarcoma specifically than the double two-thirds rule indicates. These findings may be useful in an emergency setting to guide owners on potential diagnoses for dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass. However, there remains a portion of these dogs with benign conditions and nonhemangiosarcoma malignancies that may have a good long-term prognosis compared to dogs with hemangiosarcoma. Studies with higher levels of evidence, lower risks of bias, and large case numbers are needed in the literature.

[Clinical case of angiosarcoma of ethmoidal labyrinth and frontal sinus, issues of morphological verification of diagnosis]. / Klinicheskii sluchai angiosarkomy reshetchatogo labirinta i lobnoi pazukhi, voprosy morfologicheskoi verifikatsii diagnoza.

A clinical case of incorrect verification of the angiosarcoma of the ethmoidal labyrinth and frontal sinus in a 36-year-old man is presented. A feature of this case is the disregard of additional research methods without taking into account the unilateralism of the pathological process, which led to the wrong tactics of surgical treatment and the development of a relapse of the disease complicated by nasal liquorrhea. A thorough examination of the patient and an analysis of a series of computer tomograms of the paranasal sinuses suggested a diagnosis of malignancy. The patient underwent neoplasm removal with fistula plastic, and histological confirmation of the presumptive diagnosis was performed.

[Intimal pulmonary sarcoma: A case report of a rare differential diagnosis of chronic thromboembolic pulmonary hypertension]. / Das intimale Sarkom der Pulmonalarterie ­ Fallbericht einer seltenen Differenzialdiagnose der chronisch thromboembolischen pulmonalen Hypertonie.

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.