Angiosarcoma is a rare vascular malignant soft tissue tumor, with highly malignant, invasive, and multifocal characteristics of biology, which is prone to local recurrence and distant metastasis, so the prognosis is extremely poor. It rarely involves the diaphragm. We present the case of an adult patient who had a primary giant angiosarcoma of the left-sided diaphragm.
Angiosarcoma is a rare vascular malignant tumor originat-ing from the endothelial cells in small blood vessels, which represents 1-2% of soft tissue tumors [
A 58-year-old woman had a left-sided chest distress and short breath for more than one month duration. No other symptoms such as fever, ague, cough, emptysis, hemoptysis, night sweat, and fatigue were detected. High-density shadow was found in lower left chest after chest film examination, and the further CT revealed a mass opacities with inhomogeneous density and slight enhancement in lower left thoracic cavity, and no mediastinal lymph nodes, as well as a small amount of effusion (see Figure
CT findings of angiosarcoma of the diaphragm (tumors were located under the chest, a huge volume, heterogeneous enhancement, No mediastinal lymph nodes).
Upright fluorescence microscope was used for watch. Biopsy of the lesion showed irregular channels and ecstatic vascular spaces lined by plump hyperchromatic endothelial cells. The histology results were most compatible with moderately differentiated angiosarcoma, as shown in Figure
Histopathological microscopic structure of diaphragm angiosarcoma (tumor cell atypia, abnormal vessel-like cavities structure).
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Epithelioid cells strongly positive with CD34 (
After surgical treatment, the patient recovery was smoothly. Due to the multifocal and invasive characteristic of angiosarcoma, and combined with limited resection of the left-sided diaphragm, radiotherapy postoperatively 3 weeks later was used. The patient was treated by 3-dimensional conformal radiotherapy (3DCRT), the clinical target including the left side of diaphragm and the part of the lung tissues which adhered to the angiosarcoma. A dose of 60 Gy in 2 Gy fractions was given to 90% isodose volume in 3DCRT. In view of the effect is not clear, postoperative chemotherapy was not used. She was good by following up in outpatient department, and CAl25 was significantly decreased to 63.0 U/mL one month after radiotherapy.
Angiosarcoma with low morbidity and high invasiveness is evolved from endothelial cells or their derivatives of mesenchymal cells. Its incidence may be related to these factors, such as long-term chronic lymphedema, ionizing radiation, chemical exposure, trauma, and chronic infection [
Clinical manifestations of angiosarcoma are diverse, and its clinical features and imaging findings are different from different parts [
The diagnosis of angiosarcoma mainly depends on the histomorphological checks, and the immunohistochemistry has great significance for the diagnosis and differential diagnosis of angiosarcoma [
There is still no standard therapeutic method of angiosarcoma to follow. Surgical treatment, radiotherapy, chemotherapy, immune therapy, and other comprehensive therapy treatment are often adopted. Surgical excision is the main treatments of angiosarcoma. Radiotherapy or chemotherapy before or after the operation may reduce the local recurrence and metastasis [
The prognosis of angiosarcoma is bad [