Epithelioid angiosarcoma of the ilium: a case report

Bone epithelioid angiosarcoma (EA) is rare and characterized by large, mildly to moderately pleomorphic epithelioid cells, with abundant eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. The tumors may arise in various locations in bone and the patients may present with unifocal or multifocal osseous disease. We present a unifocal lesion case of EA of the ilium in a 62-year-old woman. A needle biopsy of the ilium was performed and first diagnosed poorly differentiated adenocarcinoma based on CKpan and CK18 immunopositivity. The tumor was treated initially with curettage followed by chemotherapy. The final diagnosis on the surgical specimen was epithelioid angiosarcoma.     

Bone marrow metastasis of angiosarcoma

We report a rare finding of bone marrow metastasis from an angiosarcoma. The patient was a 36-year-old man who initially presented with a high-grade angiosarcoma of the spleen and was treated with splenectomy and chemotherapy. He developed leukoerythroblastic anemia three years after splenectomy. Bone marrow biopsy revealed extensive infiltrate by angiosarcoma with typical features of spindle tumor cells and anastomosing vascular channels. The immunohistochemistry showed tumor cells positive for the endothelial markers of CD31, CD34, and von Willebrand factor. Angiosarcomas are rare and aggressive tumors. Although metastases occur commonly, bone marrow findings have been rarely documented. We have found in the literature two other cases of bone marrow metastasis of angiosarcoma, and all these patients had a primary tumor of the spleen. It would seem that splenic angiosarcomas have a virtually unique propensity for infiltration in the bone marrow.     

Angiosarcoma of the pericardium: a case report

Background: Primary malignant tumors of the pericardium are rare, and most primary malignant pericardium tumors are mesotheliomas. Primary pericardial angiosarcoma is extremely rare, and it is associated with a poor prognosis. Case presentation: We report of a 47-year-old woman who complained of activity-related chest tightness and shortness of breath. Computed tomography, magnetic resonance imaging, and transesophageal echocardiography revealed an enlarged pericardium with hematic and solid components. An exploratory pericardiotomy was performed, and the results of the histological examination were suggestive of spindle cell hemangioendothelioma. She survived for 9 months after surgery without chemotherapy and radiotherapy, and she had a relatively good quality of life. Conclusion: Primary pericardial angiosarcoma is difficult to diagnose, and it has a poor prognosis. Pericardiotomy, radiation therapy, and chemotherapy were associated with a prolongation of survival.     

Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases

Primary cardiac angiosarcoma is a rare but the most common malignant neoplasm of the heart in adults. The objective of this study is to analyze the clinicopathologic characteristics of primary cardiac angiosarcoma. Ten cases of primary cardiac angiosarcoma treated in a single institution were analyzed for their clinical, pathologic, and immunohistochemical features. There were 6 men and 4 women, with a mean age of 40 years (range, 20-61 years). The patients commonly presented with dyspnea and distant metastasis. All tumors were located in the right atrium, with a mean tumor size of 6.8 cm. Tumors were hemorrhagic, with variegated tan-brown solid areas. Histologically, they exhibited high-grade morphology with mixed solid growth and anatomizing channels. Frequent mitoses and tumor necrosis were common. The tumors were strongly positive for CD31, CD34, FLI-1, and WT-1 but negative for AE1/3, D2-40, human herpesvirus 8, and epidermal growth factor receptor. The tumor cells were focally reactive to p53, with a high rate of Ki-67 expression. A complete tumor resection was not possible in any of the patients because of the size or extensive local invasion of the tumor. Overall survival ranged from 1 to 81 months (mean, 26.6 months) after initial histologic diagnosis. Primary cardiac angiosarcomas are rare tumors that commonly arise in the right atrium. The mean age is much younger than that of soft tissue angiosarcoma. Regional tumor extension and distant metastasis are extremely common at the time of diagnosis. Surgical resection with adjuvant chemotherapy is currently the preferred treatment, and survival time appears to be inversely correlated with the tumor size and degree of regional tumor extension at the time of surgery.     

Cardiac angiosarcoma.

Primary tumors of the heart are extremely rare, with a prevalence rate of around 0.01% in collective autopsy studies. Majority of the primary cardiac tumors are benign. Myxoma is the most common primary cardiac tumor, while angiosarcoma is the commonest primary malignant tumor. We present a case report of a 20-year-old patient with history of breathlessness and hemoptysis. A malignant mass was seen arising in the right atrium, with pericardial effusion and multiple metastases in the lung. A complete autopsy was performed and a diagnosis of cardiac angiosarcoma was confirmed on histology and immunohistochemistry.     

Angiosarcoma associated with germ cell tumors

In two patients with malignant germ cell tumors angiosarcoma developed through two apparently different mechanisms. In one case the angiosarcoma probably developed as a complication of therapeutic radiation, since radiation changes were demonstrated in tissue adjacent to the neoplasm and since the angiosarcoma was not associated with elements of germ cell tumor. The absence of associated germ cell elements does not support the development of the angiosarcoma from a teratoma. In the second case, however, it is likely that the angiosarcoma developed as a result of malignant change within teratomatous foci, since angiosarcomatous elements were intermingled with teratomatous elements and the patient's primary germ cell tumor contained malignant and atypical teratomatous elements as well as prominent vascular proliferation. Malignant change within teratomatous components of germ cell tumors is a phenomenon of increasing importance in this era of effective chemotherapy for germ cell tumors. The development of angiosarcoma as a potential complication of testicular carcinoma has not been reported previously.     

骨原发性上皮样血管肉瘤2例报道并文献复习

目的提高对骨原发性上皮样血管肉瘤(epithelioid angiosarcoma,EA)的认识,避免误诊。方法对2例原发于骨的EA进行临床病理、组织学及免疫组化分析,并进行文献复习。结果 2例均为男性,为骨组织内的多灶性、溶骨性病变,组织学上肿瘤主要由实性片状排列的上皮样细胞组成,瘤细胞核大,空泡状,含有明显核仁,可见细胞内空泡和血管腔样结构形成。免疫组化标记显示瘤细胞表达CD31、FⅧRAg、CK、vimentin。分别进行单纯化疗和手术治疗。1例失访,1例2个月后死亡。结论骨原发性EA是一种具有上皮样特征的高度恶性血管源性肿瘤,必须与转移癌等鉴别,CD31、CK等血管标记物对鉴别诊断具有重要的意义。     

Histologic diagnosis of hepatic tumors

Many types of tumors, benign and malignant, primary and metastatic, can occur in the liver. Diagnostic criteria for two relatively common and two uncommon primary malignant liver tumors are presented. Hepatocellular carcinoma, the most common primary malignant hepatic tumor, is recognized when the tumor cells show features of liver cell differentiation, such as a trabecular growth pattern, intercellular bile canaliculi, eosinophilic granular cytoplasm, bile production or other hepatic synthetic products. Intrahepatic cholangiocarcinoma shows glandular differentiation, similar to other adenocarcinomas. It often cannot be distinguished from metastatic adenocarcinoma, and the diagnosis requires the exclusion of an extrahepatic primary site. Undifferentiated (embryonal) sarcoma is a rare primary liver tumor of children and young adults. It shows undifferentiated stellate and spindle cells in a myxoid matrix. Hepatic angiosarcoma is a malignant endothelial cell proliferation, beginning in the hepatic sinusoids, eventually filling the vascular structures, and often progressing to tumorous vascular masses. The histologic diagnosis of all types of liver tumors requires the application of strict morphologic criteria to light microscopic observations. Diagnoses that do not conform to such criteria are often inaccurate.     

Extracutaneous angiosarcomas metastatic to the lungs: clinical and pathologic features of twenty-one cases.

Angiosarcomas are rare malignant vascular tumors with a high rate of metastasis involving lungs (most commonly), liver, regional lymph nodes, bone, and other sites. In this study, we have reviewed the clinical presentation and histopathology of 21 cases of extracutaneous angiosarcoma metastatic to the lungs. Tumors with exclusively pleural involvement were excluded. Patients presented with dyspnea, chest pain, and/or hemoptysis lasting a few weeks to months. Radiologically, the most common finding comprised multiple peripheral lung nodules (57%), often accompanied by infiltrates. For 11 cases (52%), the primary tumor was not identified at the time of presentation. Vasoformative areas were identified in 15 cases (71%). Nine cases comprised spindle cells (43%), two contained epithelioid cells (9.5%), and 10 consisted of both spindle and epithelioid cells (48%). Nuclear pleomorphism was at least moderate in all cases. However, five tumors contained regions of minimal nuclear atypia. Hemorrhage, siderophages, and fibrosis were commonly present. Immunohistochemical staining (IHS) was performed on 14 cases. Thirteen tumors showed reactivity for vascular markers. Tumor cells reacted for Von Willebrand factor in 13 of 14 cases, and CD31 and CD34 were each positive in 2/2 cases. Two cases (of nine examined) also expressed cytokeratins. Because the tumor often first presented in the lungs before the primary sarcoma was identified, the clinical impression included both benign and malignant entities. For patients with primary cardiac tumors, symptoms referable to the primary tumor complicated the clinical presentation, and radiologic evaluation supported a clinical diagnostic impression of non-neoplastic pericarditis. Thus, angiosarcoma in the lung may elude diagnosis until histopathologic evaluation of the lung biopsy.     

Oral and salivary gland angiosarcoma: a clinicopathologic study of 29 cases.

Angiosarcomas of the oral and salivary gland area are extremely rare, mostly presented as case reports. We wanted to study the clinicopathologic features of a series of oral and salivary gland angiosarcomas. Cases coded as "angiosarcoma" were retrieved from the Oral and Maxillofacial Pathology Department of the Armed Forces Institute of Pathology. Patient folders and pathology were reviewed and recorded; immunohistochemistry and follow-up were obtained. Inclusion required oral or salivary gland location, vasoformative growth, cytologic atypia, mitoses, and vascular markers. Skin, bone, and subcutaneous angiosarcomas were excluded. Primary and secondary (metastatic) oral angiosarcomas were included. The 22 primary angiosarcomas involved tongue (n = 9), parotid (n = 4), lip (n = 4), submandibular gland (n = 3), and 1 each of soft and hard palate. The 7 secondary angiosarcomas involved the gingiva (n = 4) and parotid gland (n = 3). Overall, patient ages ranged from 6-90 years (mean, 55 years). There were 15 males and 14 females. Symptoms included a mass with recent enlargement and bleeding. Tumor sizes ranged from 0.8-7.0 cm (mean, 2.6 cm). Histologically, all tumors were vasoformative; 86% had solid and 17% had distinctive papillary areas. Eight (28%) were classified as the epithelioid subtype. Immunohistochemical stains showed that the tumor cells were positive for Factor VIIIrag in 19/21, CD31 in 16/19, CD34 in 7/12, and Ulex in 1/1. Primary tumors were classified as low grade (n = 7, in all locations except salivary gland), intermediate (n = 7), and high grade (n = 8); all secondary tumors were high grade. Follow-up was available on 14/22 primary and 7/7 secondary angiosarcomas. Of primary tumors, two tongue angiosarcoma patients died at 1 and 9 years, but 4 were alive without disease over a mean of 7.3 years (range, 1-13 years). Four primary salivary gland angiosarcoma patients were alive without disease over a mean of 5.8 years (range, 1-14 years), and 1 had only a late (15 years) metastasis and death (at 20 years). Three primary lip angiosarcoma patients were without disease over a mean of 14.3 years (range, 13-16 years). Of secondary tumors, three salivary gland angiosarcoma patients died within 1 year, and all four secondary gingival angiosarcoma patients died of disease within 3 years. Assessing follow-up of primary oral and salivary gland angiosarcoma patients by grade, 5 patients with high-grade tumors had no evidence of disease over a mean of 7.6 years (range, 1-16 years), 3 patients with intermediate-grade tumors had no evidence of disease over a mean of 12.7 years (range, 11-14 years), 2 patients with intermediate-grade tumors died of disease at 9 and 20 years, 3 patients with low-grade tumors had no evidence of disease over a mean of 6.3 years (range, 1-14 years), and 1 patient with low-grade tumor died of disease at 1 year. Primary oral and salivary gland angiosarcomas, albeit rare, mostly involve the tongue, parotid gland, and lip of adults, often with relatively good outcome. Although the most common angiosarcoma morphology in this area is spindled vasoformative and solid, almost one third of oral and salivary gland angiosarcomas are the rare epithelioid angiosarcoma variant. Most gingival and few parotid angiosarcomas appear to be metastases from other locations, with many patients succumbing to death within 3 years. Despite predominantly high- or intermediate-grade morphology, patients with primary angiosarcoma of the tongue, salivary gland, and lip have a better prognosis than do patients with primary cutaneous or deep soft tissue angiosarcoma, including those patients with secondary oral and salivary gland involvement.     

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal tumor of intermediate malignant potential with characteristic clinicopathologic and genetic features. Although bone involvement accompanies nearly one-fourth of reported cases of soft tissue PHEs, primary intraosseous PHE is rare. Herein, we report five cases of primary intraosseous PHEs. Male to female ratio was 4:1, with an average age of 28 years (age range, 5–44 years). Radiologically, tumors presented as lytic lesions in the proximal femur (two), diaphysis of the tibia (one), distal radius (one) and vertebrae (one). Multifocal lesions were observed in four cases. Histopathologic examination revealed plump spindle cells and prominent nucleoli. New bone formation was noted in three cases. Immunohistochemically, all tumors were positive for CD31 and negative for CD34. Pan Cytokeratin (CK) (AE1/3) was positively expressed in all, except a single tumor, in which CK7 and Cam5.2 were expressed. INI1/SMARCB1 was completely retained in all tumors. A single patient underwent surgical resection. During follow-up, two cases showed no evidence of disease within two and five years, respectively. Differential diagnosis of a PHE of bone includes osteoblastoma, epithelioid angiosarcoma, metastatic carcinoma, metastatic rhabdomyosarcoma, and epithelioid sarcoma. Caution must be exercised as pan CK (AE1/3) might not be expressed; therefore, the use of other cytokeratins, such as Cam5.2 is recommended. Awareness of such an entity in bone is the key to the diagnosis.     

Primary ovarian angiosarcoma presenting as malignant cells in ascites: case report and review of the literature

Primary angiosarcoma of the ovary is a rare tumor, with less than 25 cases reported in the literature. These tumors are thought to arise from carcinosarcomas, teratomas or the ovarian vasculature, and occur at any age. The prognosis is dismal in the majority of cases. We present the case of a 19-year old female who presented with abdominal distention and abdominal pain. Tapping of peritoneal effusion showed groups of pleomorphic cells that were negative for epithelial and germ cell markers. Immunohistochemistry performed on the surgical specimen of the ovary, and subsequently on the effusion specimen showed staining for endothelial markers. The patient was treated post-operatively with chemotherapy, but died one year following diagnosis. This is the first reported case of an ovarian angiosarcoma that metastasized to the peritoneal cavity, with a resulting malignant effusion. Despite the rarity of metastasis from gynecological sarcomas in effusions, this possibility needs to be included in the differential diagnosis of malignant effusions that are negative for epithelial and germ cell markers.     

Primary angiosarcoma of the cuboidal bone: a case report

Primary angiosarcoma of bone is a rare neoplasm. The unique cytomorphologic and ultrastructural aspects of a primary angiosarcoma of the cuboid bone diagnosed by fine-needle aspiration are presented. Smears and cell block preparations revealed single and clustered large, pleomorphic, and spindle-shaped cells with a markedly hemorrhagic background. Electron microscopy revealed characteristic features of endothelial cells including rare Weibel-Palade bodies. The clinical, cytologic, histologic, and ultrastructural aspects of the case are discussed.     

Gastrointestinal stromal tumor colliding with angiosarcoma

The collision of gastric tumors is rare, and those involving gastrointestinal stromal tumor and angiosarcoma have not been previously reported. This article reports a gastric tumor involving collision of gastrointestinal stromal tumor and angiosarcoma. The patient was an 81-year-old man who presented with dyspepsia and large gastric mass. The mass showed a proliferation of spindle cells expanding the muscularis propria. Most of the tumor consisted of spindle cells with low-grade cytology that were positive for CD117 (c-kit), indicating gastrointestinal stromal tumor. Occasional prominent foci showed dissecting and anastomosing channels of ectatic vascular spaces lined by cytologically malignant cells that were positive focally for CD117 and diffusely positive for multiple vascular markers, indicating angiosarcoma.     

Primary angiosarcoma of the male breast

We report an exceptional case of primary breast angiosarcoma in a 58-year-old man. This is a very rare breast tumor (0.04% of breast tumors) which may be difficult to diagnose. Treatment is now standardized: radical mastectomy associated with adjuvant chemotherapy for grade III or poorly differentiated tumors. Prognosis is variable, depending on tumor size and histological grade. Diagnosis should be established as early as possible because the 10-year overall survival rate is 80% for low grade tumors and only 20% for high grade tumors.     

Multimodality management of a case of primary osteogenic sarcoma of the zygoma

Craniofacial osteogenic sarcomas are rare primary malignant bone tumors and very few cases involving zygomatic bone were reported in literature. We present our experience of multimodality management of a case of primary osteogenic sarcoma of zygoma. Wide radical excision of the tumor including the parotid gland was done followed by three cycles of adjuvant chemotherapy and fifty Gy of external radiotherapy. The patient is disease-free at two years follow-up. Till 1970s, craniofacial osteogenic sarcomas were managed mainly by radical surgery with a high local failure rate. With the advances made in the field of radiotherapy and chemotherapy, multimodality therapy is playing a major role in the treatment of these aggressive tumors with better overall and disease-free survival.     

Fine-needle aspiration cytology and immunocytochemistry of soft-tissue tumors and osteo/chondrosarcomas of the head and neck

The purpose of the study was to present the clinical and cytological findings of 28 cases of malignant or borderline mesenchymal tumors of the head and neck, of which 22 originated from soft tissue and 6 were found in bone or cartilage. The basic procedures employed involved a cytologic review and subclassification of fine-needle aspiration (FNA) smears from the tumors, which were diagnosed as: pleomorphic sarcomas (5 poorly differentiated, 1 angiosarcoma, and 1 epithelioid sarcoma), spindle-cell sarcomas (2 leiomyosarcomas, 2 malignant mesenchymal tumors, and 1 malignant schwannoma), myxoid sarcomas (2 liposarcomas and 1 high-grade tumor), round-cell tumors (1 malignant histiocytic tumor and 1 chloroma), osteosarcomas (3), chondrosarcomas (3), and borderline tumors (2 pleomorphic lipoma, 1 myxolipoma, 1 cranial fasciitis, and 1 fibromatosis). Histological correlation and problems in subtyping on both cytologic and histological material are discussed. It is concluded that FNA cytology can be used with high accuracy to diagnose musculoskeletal tumors in rare sites such as the head and neck.     

Angiosarcoma of the breast: a clinicopathologic analysis of cases from the last 10 years

Breast angiosarcoma may occur de novo, or as a complication of radiation therapy, or chronic lymphedema secondary to axillary lymph node dissection for mammary carcinoma. In our effort to characterize the clinicopathologic features of breast angiosarcoma, we reviewed all breast angiosarcoma cases in the University of Kansas Medical Center and Ohio State University Medical Center archives from 1997 to 2007. Clinical histories and follow-up data for identified patients were reviewed. The tumors were graded histologically according to Rosen's method. Only 11 angiosarcomas were identified among more than 5000 malignant breast neoplasms (0.1%-0.2% incidence) for the last 10 years. Eight cases (6 high grade, 1 intermediate grade, 1 low grade) were identified as postradiation angiosarcoma (postradiation time interval, 4-12 years), and 3 cases were identified as primary angiosarcomas (1 high grade, 2 low grade). Follow-up (median, 36 months) revealed that 3 cases of postradiation angiosarcoma recurred as skin and/or chest wall lesions and 1 case of primary angiosarcoma developed liver metastases (all high-grade). In conclusion, breast angiosarcoma remains a rare disease. Rosen's method for grading breast angiosarcoma is easy to implement and correlates well with clinical outcome. There are no distinct clinical or histologic differences between primary and postradiation breast angiosarcomas.     

Dedifferentiated chondrosarcoma of the rib with a malignant mesenchymomatous component: An autopsy case report

A rare variant of dedifferentiated chondrosarcoma wlth malignant mesenchymomatous component in a 57-year-old male is reported. The patient presented with a posterior mediastinal mass arising from the left eighth and ninth ribs showing well differentiated, low-grade chondrosarcoma. Five years later, local recurrence occurred and an excised specimen also showed the same histological features as the primary tumor. Another 6 years later, the tumor recurred and metastasized to the multiple organs, the patient dying 4 months later. Autopsy revealed that the recurrent and metastatic tumors showed malignant mesenchymomatous 'dedifferentiation' of chondrosarcoma composed of rhab domyosarcoma, angiosarcoma, chondrosarcoma, osteosarcoma, and leiomyosarcoma, in addition to fibrosarcomatous areas. Although the less differentiated component of dedifferentiated chondrosarcoma usually shows the histological features of malignant fibrous histiocytoma and fibrosarcoma, multilineage differentiation can occur in that component. The phenomenon of 'dedifferentiation' in chondrosarcoma and the relationship to and distinction from malignant mesenchymoma of soft tissue and bone are discussed.