AN AUTOPSY CASE OF PRIMARY ANGIOSARCOMA OF THE PERICARDIUM MIMICKING MALIGNANT MESOTHELIOMA

The pathology of a rare case of primary diffuse angiosarcoma of the pericardium is reported. Grossly, the heart was entirely encased by the pericardial tumor, and the myocardium was only superficially invaded by the tumor. The tumor tissue extended directly to the mediastinum, where the great vessels were embedded in the tumor. A few minute distant metastases were found only in the bilateral lungs and pulmonary hilar lymph nodes. Microscopically, the tumor tissue was composed of malignant cells forming vascular channels admixed with solid areas. Histo- and immunohistochemically, no mesothelial characteristics were evident. Factor VHI-related antigen and Ulex'europaeus I lectin were positive, implying that the tumor was of vascular origin. Grossly, and in part microscopically, this case resembled malignant diffuse mesothelioma, indicating that pericardial angiosarcoma may sometimes mimick malignant mesothelioma. ACTA PATHOL JPN 38: 1345-1351, 1988.     

Basement membrane in Kaposi's sarcoma: an immunohistochemical and ultrastructural study

Basement membranes were investigated in early angiomatous and late sarcomatoid stages of Kaposi's sarcoma (KS). Seven frozen skin biopsies of KS from five elderly Mediterranean people and one renal allograft recipient were labelled, using an immunoperoxidase technique, for basement membrane-specific macromolecules, laminin and type IV-collagen. Twenty-seven other frozen cutaneous lesions including haemangio and lymphangiosarcomas, benign vascular tumours, and various epithelial, melanocytic, fibrohistiocytic, fibrosarcomatous and muscular tumours were processed in the same way. In addition an ultrastructural study was performed in two cases of KS, one haemangiosarcoma and one lymphangiosarcoma. Intense labelling was observed for both type IV-collagen and laminin, which appeared closely co-distributed, in all areas of KS. Staining pattern was often regular and continuous around neoplastic vessels in early lesions of KS, as in benign vascular lesions, whereas in late nodular lesions large amounts of basement membrane components were present in intercellular spaces between densely aggregated spindle cells. In contrast, ultrastructural examination disclosed early disruption of basement membranes around neoplastic vessels, and occasional fragments of external lamina were seen at the interface between KS spindle cells and collagen. Similar results were obtained in angiosarcomas both by immunohistological and ultrastructural study. In the comparative group, laminin and type IV-collagen were present, in significantly fewer quantities and in various distinctive patterns, in epithelial, melanocytic, fibrohistiocytic and muscular tumors. This study deals with basement membrane modifications in early and late lesions of KS and provides further evidence in favour of the endothelial nature of the spindle cells of KS.     

Angiosarcoma of the breast: Report of a case and autopsy findings

We report a case of angiosarcoma of the breast and the autopsy findings. The patient was a 35-year-old premenopausal woman who complained of a tumor in her left breast. We found a tumor measuring 55 mm in diameter in the lower external quadrant. The tumor was elastic and soft, smooth surfaced, well-defined and mobile. Dimpling sign or change of skin color were not observed. Clinically it was diagnosed as phyllodes tumor, but tumorectomy revealed primary angiosarcoma of the breast. Further extended surgery was recommended, but the patient refused additional therapy. Histological findings revealed a free surgical margin and neither lymph node metastasis nor distant metastasis were clinically observed. Seven months later, local recurrence in the same breast was recognized and finally radical mastectomy was carried out. Histological findings showed recurrence of angiosarcoma in the left breast but lymph node metastasis was not detected. Two months after mastectomy, metastases to the cervical and thoracic vertebrae were observed and radiation therapy was performed. Sixteen months from onset, she died due to multi-organ failure as general metastases of angiosarcoma. At autopsy, metastases to many organs including the digestive system were observed. The incidence of primary angiosarcoma of the breast is low but its prognosis is poor. This case emphasized the difficulties in clinical diagnosis and treatment for the angiosarcoma of the breast.     

Epithelioid angiosarcoma of the thyroid

Epithelioid angiosarcomas of the thyroid usually develop in people living in Alpine regions, and only rare cases arising in subjects living in nonmountainous areas have been reported. We describe the clinicopathological features of a series of seven cases collected from non-Alpine areas. All patients were adults. The tumours appeared as haemorrhagic, unencapsulated, sometimes cystic nodules. In two cases multinodularity was present. They were composed of large, epithelioid cells, which lined vascular-like spaces or were arranged in solid sheets. Intracytoplasmic lumina containing red blood cells were identified. Neoplastic cells were diffusely positive for factor VIII-related antigen, Ulex europaeus agglutinin, CD31 and keratin peptides. Ultrastructural studies were performed in four cases and showed features of endothelial differentiation. An average follow-up of 3.8 years disclosed that four patients died of disease after a median survival time of 5 months, whereas 3 patients are still alive with no evidence or residual disease 27, 32 and 66 months after thyroidectomy. The good prognosis in these patients appears to be related mainly to the absence of extraglandular tumour spread at the time of surgery.     

Malignant vascular lesions of bone: radiologic and pathologic features

The malignant vascular tumors of bone represent an uncommon diverse group of tumors with widely variable clinical and radiographic presentations. Although the radiographic imaging features of the lytic osseous lesions typically seen with this group of tumors are relatively nonspecific, the propensity to develop multifocal disease in an anatomic region is a feature that can be helpful in suggesting the diagnosis of a vascular tumor. The differential diagnosis varies according to the age of the patient and presence of solitary or multifocal disease. The histologic features are variable and range from tumors with vasoformative features to those that mimic mesenchymal neoplasm or metastatic carcinoma. Familiarity with the radiographic and pathologic spectrum of disease is essential for making an accurate diagnosis in this diverse group of neoplasms. This paper will provide a review of the nomenclature for the malignant vascular tumors of bone and discuss the radiographic and pathologic differential diagnosis. Received: 17 December 1999 Revision requested: 8 February 2000 Revision received: 19 May 2000 Accepted: 9 June 2000     

Non-lymphoid lesions that may mimic cutaneous hematopoietic neoplasms histologically

This review considers neoplastic lesions that originate in the skin, and which have the potential to imitate hematopoietic proliferations at a histological level. They include lymphoepithelioma-like carcinoma, Merkel cell carcinoma, benign lymphadenoma, pseudolymphomatous angiosarcoma, lymphadenoid dermatofibroma, lymphomatoid atypical fibroxanthoma, histiocytoid (epithelioid) hemangioma, and inflamed melanocytic lesions. The clinical and pathological features of those tumors are considered.     

Pyogenic granuloma of the large intestine: Case report and review of reported cases in the adult

Pyogenic granuloma (PG) is a polypoid lobular capillary hemangioma rarely described in the large bowel. We describe the case of a 72-year-old man with recent weight loss, anemia, and change in bowel habit. A 3 cm × 2 cm polypoid lesion in the right flexure of the colon was observed and treated with extended right hemicolectomy. A review of the English-language literature on PG of the large bowel in adults yielded eight previously reported cases. The age of the patients ranged from 26 to 80 years with a mean of 54.8 years. The lesions were all solitary, except one case of multiple lesions in the colon. Another case was associated with satellite lesions. Seven cases were located in the colon and two in the rectum. The mean maximum diameter of the lesions was 1.6 cm (range 0.5–3 cm). Colorectal bleeding was the most common clinical symptom. Due to its unusual appearance, PG can be misdiagnosed and mistreated as colon carcinoma with extensive surgery. Polypectomy or endoscopy mucosal resection is the optimal treatment. In large or deep lesions, a surgical excision may be necessary. Pathologists should be familiar with this condition so as to avoid overdiagnosis as a malignant vascular tumor.     

头面部血管肉瘤1例

患者男,80岁。头面部出现簇集血疱样皮疹40d。皮损组织病理:可见不规则的血管腔,管腔由内皮细胞排列而成,内皮细胞异型性。免疫组化检查:CD31,CD34,Vimetin(+)。诊断:头面部血管肉瘤。