Angiosarcoma Treatment & Management

Updated: Feb 03, 2017
  • Author: Belen Carsi, MD, PhD, FRCS; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Treatment of soft-tissue angiosarcoma

For stage I angiosarcomas, the National Comprehensive Cancer Network (NCCN) recommends surgery to obtain adequate oncologic margins. For stage II-III disease that can be resected with acceptable functional outcomes, the NCCN also recommends preoperative radiation therapy (category 1) or preoperative chemoradiation or chemotherapy (category 2B). [22] The NCCN lists the following agents as having activity against angiosarcoma:

• Paclitaxel

• Docetaxel

• Vinorelbine

• Sorafenib [23]

• Sunitinib

• Bevacizumab

Ravi et al report exceptional response to treatment with pazopanib in a patient with angiosarcoma that harbored amplification of vascular endothelial growth factor receptor (VEGFR) and that had not responded to sorafenib. These authors suggest that a subset of patients with angiosarcoma with genomic alterations in vascular signaling genes may respond well to pazopanib. [24]

Multiple randomized studies using doxorubicin-based chemotherapy fail to show a survival benefit from neoadjuvant chemotherapy. However, a meta-analysis suggests improved local control and disease-free survival with chemotherapy, but no survival advantage. [12, 25]

Response to preoperative chemotherapy is only 40-50%, with the most active regimens, and toxicity is significant. Consequently, specialists reserve preoperative chemotherapy for patients with high-grade lesions. The regimen is continued in those patients who respond with tumor shrinkage after two to three courses of multiagent chemotherapy after tumor resection.

Pasquier et al reported effective treatment in seven patients with advanced angiosarcoma using the combination of twice-daily propranolol (40 mg) and weekly metronomic vinblastine (6 mg/m2) and methotrexate (35 mg/m2). All patients responded; one patient showed a complete response and three showed very good partial responses. Median progression-free and overall survival was 11 months (range 5–24) and 16 months (range 10–30), respectively. [26] In January 2017, propranolol was granted orphan drug status in Europe for the treatment of soft-tissue sarcoma. [27]

Offer patients with unresponsive tumors different treatment regimens. Response to neoadjuvant chemotherapy can be observed, but it does not always correlate with radiographic response.

Radiotherapy

The use of irradiation in conjunction with surgery continues to evolve and results in 80% of local control and excellent functional and cosmetic outcome. [10] However, consider that 50% of angiosarcomas have distant metastases, and irradiation does not improve survival. Better definition of the extent of the disease with the use of MRI helps to further delineate the radiotherapy fields and decrease long-term morbidity. Intraoperative radiation, brachytherapy, or more external beam therapy can complement preoperative external beam radiotherapy.

The disadvantage of preoperative radiation is that a higher wound complication rate may delay surgery (1 wk of healing per 10 Gy of radiation delivered). The advantages of preoperative radiation are as follows:

  • Optimization for surgery

  • Smaller volume of external beam fields

  • Less hypoxic tissue

  • Potential to reduce the chance of intraoperative implantation

  • Potential improvement in local control in advanced tumors

Bone angiosarcoma

Evidence of multicentricity must be sought before making any decision regarding therapy. Patients have presented with lesions affecting as many as 45 different bones. In such cases, consider neoadjuvant chemotherapy.

A chemotherapeutic regimen common for sarcomatous tumors can be administered (ifosfamide and doxorubicin used together or sequentially). If clinical or radiographic improvement is not observed, consider a second regimen with cyclophosphamide, etoposide, and cisplatin. Gemcitabine may be effective as second line or third-line therapy.

Cutaneous angiosarcoma

The best outcomes are reported with surgery followed by radiotherapy. The role of chemotherapy in cutaneous angiosarcoma has not yet been established, although for patients with metastasis or tumors deemed unresectable, doxorubicin (intraarterial or systemic) is indicated. [25]

Paclitaxel as a single agent has shown substantial activity against angiosarcoma of the scalp or face, even in patients previously treated with chemotherapy or radiation therapy. [28] Further investigation is warranted to define the optimal treatment dose and schedule.

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Surgical Care

Surgical treatment of angiosarcoma of the soft tissue, retroperitoneum, and abdomen is as follows [6] :

  • Target obtaining wide surgical margins, with at least 2 cm of unaffected tissue surrounding the tumor. The resection should include skin when applicable and the soft tissue around the angiosarcoma. Include biopsy sites, including the biopsy tract, en bloc with the specimen.

  • Resection of large lesions can be extremely difficult and sometimes requires amputation for local control; however, local control does not prevent distant relapse

  • Free surgical margins sometimes have anatomic constraints, especially in retroperitoneal tumors

Surgical treatment of angiosarcoma of bone is as follows:

  • Surgical resection and radiation therapy are the standard treatment for localized disease

  • Low-grade lesions lead to similar benefits with either technique

  • Treat high-grade lesions as malignant bone neoplasms, with a combination of radical en bloc excision followed by radiotherapy and/or chemotherapy

  • The number of lesions in a limb may render limb salvage impossible, and amputation may be indicated

Surgical treatment of cutaneous angiosarcoma is as follows [14] :

  • Surgical treatment is contraindicated in tumors extending into vital structures, in those of massive size, or in those with multicentricity

  • The lesion may be solitary or multicentric and frequently extends laterally throughout the dermis, making gross assessment of surgical margins difficult and necessitating multiple biopsies of the surrounding tissues

  • In the primary treatment of angiosarcomas of the scalp, recognizing the horizontal and vertical extensions of the tumor is essential, which can only be discerned by microscopic examination of all the margins of the resected specimen; the primary excision of the scalp should be full-thickness, including the pericranium and, if indicated, the outer table of the cranial vault; the margins should be wide (at least 5 cm) on all sides

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