Primary pleural epithelioid angiosarcoma manifesting as a loculated hemothorax: A case report and literature review focusing on CT findings

Primary pleural angiosarcoma (PPA) is an extremely rare and clinically aggressive tumor. We report the case of a 66-year-old man having PPA with chest computed tomography (CT) scan showing a large oval-shaped, nonenhancing high attenuation cystic mass in the left hemithorax. Morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma. Pleural angiosarcoma should be considered in the differential diagnosis of spontaneous hemothorax manifesting as high attenuation loculated pleural fluid on CT.     

Primary malignant cardiac tumors

Approximately 10% of surgically resected heart tumors are malignant. Of these, over 90% are sarcomas, and the remainder lymphomas. Sarcomas of the heart may be of a variety of histologic types. Angiosarcomas are usually right-sided, typically in the atrium. Most other heart sarcomas arise in the left atrium and may be clinically mistaken for myxoma. Left atrial sarcomas are typically pleomorphic, and may have areas of osteosarcoma or chondrosarcoma.     

Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience

Primary cardiac sarcomas are exceptionally rare. We present a 10-year, single-institution experience with 24 primary adult cardiac sarcomas. These cases were retrieved from the Department of Pathology data file of the Methodist Hospital at Houston, TX. Clinical presentation and pathologic features were analyzed. Histologic classification was followed according to the criteria set by the World Health Organization, and grading according to the system proposed by the Federation Nationale des Centres de Lutte Contrele Cancer. There were 14 men and 10 women (male/female, 1.4:1) with a mean age of 42.2 years (range 20-68 years). The tumors involved the right atrium in 14 cases, left atrium in 6 cases, right ventricle in 2 cases, and left ventricle in 2 cases. The tumor size ranged from 2.0 to 17.0 cm (mean 7.2 cm), and, histologically, there were 10 angiosarcomas, 9 unclassified sarcomas, 3 synovial sarcomas, and 2 leiomyosarcomas. All 10 angiosarcomas originated from the right atrium, whereas 5 of the unclassified sarcomas were from the left atrium. Although cases were limited, no predilection site was found for the other histologic types. All tumors were graded as 2 (5 cases) or 3 (19 cases) in differentiation. The prognosis was poor with a median survival time of 25 months after diagnosis. The grade was not statistically significant on survival (P = .14). In conclusion, angiosarcoma and unclassified sarcomas are the most common sarcomas of the heart accounting for 76%, but rare tumors such as synovial sarcoma and leiomyosarcoma may also occur in this organ. The survival of cardiac sarcomas is poor.     

Radiation-associated angiosarcoma of the breast: An international multicenter analysis

IntroductionRadiation-associated angiosarcoma (RAAS) is a rare and serious complication of breast irradiation. Due to the rarity of the condition, clinical experience is limited and publications on this topic include only retrospective studies or case reports.Materials and methodsAll patients diagnosed with RAAS between January 2000 and December 2017 in twelve centers across the Czech Republic and Slovakia were evaluated.ResultsData of 53 patients were analyzed. The median age at diagnosis was 72 (range 44–89) years. The median latency period between irradiation and diagnosis of RAAS was 78 (range 36–172) months. The median radiation dose was 57.6 (range 34–66) Gy. The whole breast radiation therapy with radiation boost to the tumor bed was the most common radiotherapy regimen. Total mastectomy due to RAAS was performed in 43 patients (81%), radical excision in 8 (15%); 2 patients were not surgically treated due to unresectable disease. Adjuvant chemotherapy followed surgical therapy of RAAS in 18 patients, 3 patients underwent adjuvant radiotherapy. The local recurrence rate of RAAS was 43% and the median time from surgery to the onset of recurrence was 7.5 months (range 3–66 months). The 3-year survival rate was 56%, the 5-year survival rate was only 33%. 46% of patients died during the follow-up period.ConclusionThe present data demonstrate that RAAS is a rare condition with high local recurrence rate (43%) and mortality (the 5-year survival rate was 33%.). Early diagnosis of RAAS based on biopsy is crucial for treatment with radical intent. Surgery with negative margins constitutes the most important part of the therapy; the role of adjuvant chemotherapy and radiotherapy is still unclear.     

Radiation-induced angiosarcoma of the parotid gland after postoperative radiotherapy for hypopharyngeal carcinoma

Secondary carcinogenesis within the irradiation range is one of the most severe problems in cancer survivors. A 60-year-old woman developed hypopharyngeal carcinoma, and she received radical surgery and postoperative radiotherapy. Eight years later, brown pigmentation and induration were observed in the left subaural region. Fine-needle aspiration biopsy revealed malignancy and the parotid tumor was diagnosed as recurrence of hypopharyngeal carcinoma. Neoadjuvant chemotherapy followed by radical parotidectomy was performed. The pathological diagnosis was angiosarcoma, which was most likely induced by past irradiation. About two months after surgery, lung metastases were detected. Docetaxel did not affect to lung metastases, but paclitaxel therapy was partially effective. The lung tumors increased in size, and brain metastases developed, resulting in death. Both neoadjuvant chemotherapy and radical surgery played important roles in the local disease control. Administration of newer agents as adjuvant chemotherapeutic agent should also be considered for improving the prognosis.     

Primary central nervous system angiosarcoma with recurrent acute subdural hematoma

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma.This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.     

Management of Localized Breast Angiosarcoma by North American Radiation and Medical Oncologists

Introduction

Primary breast angiosarcoma is a rare malignancy with no clinical trials to guide management. The current use of surgery, chemotherapy, and radiotherapy among North American oncologists is unknown.

Intravascular (”intimal”) epithelioid angiosarcoma: clinicopathological and immunohistochemical analysis of three cases

Angiosarcomas are rare malignant mesenchymal tumours, characterized morphologically by anastomosing vascular channels lined by atypical and proliferative active endothelial cells. An epithelioid cytomorphology of tumour cells is often seen focally in angiosarcoma, whereas purely epithelioid angiosarcomas are rare. Although angiosarcomas show a vascular differentiation they are almost never confined to pre-existing blood vessels. We describe three cases of intravascular epithelioid angiosarcoma arising in the carotid artery of a 60-year-old man, in the infrarenal part of the abdominal aorta and both renal arteries of a 69-year-old woman, and in the abdominal aorta of a 68-year-old man. In all cases malignant tumour tissue was found incidentally after disobliteration of thrombosed vessels. Histologically, purely epithelioid angiosarcoma composed of solid sheets of epithelioid tumour cells was seen; immunohistochemistry confirmed the endothelial differentiation of neoplastic cells. The reported cases show that angiosarcoma can occasionally arise within a pre-existing vessel. Received: 18 May 1999 / Accepted: 8 June 1999     

Enormous hemangiosarcoma of the heart

This report describes a 26-year-old patient with hemangiosarcoma of the heart and summarizes the clinicopathological features in previous reports of patients with cardiac angiosarcoma. The patient was admitted to our hospital because of a syncope and one episode of nocturnal dyspnea and hemoptysis. In his history he complained of progressive weakness and loss of weight over the past 2 months. Echocardiography and computed tomography of the chest showed inhomogeneous masses in the pericardial cavity completely surrounding the heart and involving the ascending aorta and the superior vena cava. Histological examination of the tissue obtained from the mass by fine needle technique revealed a poorly differentiated malignant tumor of mesenchymal origin. Exploratory thoracotomy followed by tumor biopsies showed an inoperable cardiac hemangiosarcoma of enormous size with multiple metastases in both lungs. Palliative tumor resection was not performed. During the postoperative course the patient still required controlled ventilation. After 3 days of cytostatic chemotherapy no regression of tumor mass was seen by chest radiography. Cardiorespiratory insufficiency was progressive, and the patient died within 3 weeks after admission.