eMedicine Specialties > Oncology > Sarcomas of Soft Tissue and Bone

Angiosarcoma: Follow-up

Author: Belen Carsi, MD, PhD, FRCS, Consultant, Department of Orthopedics, University College Hospital London
Coauthor(s): Franklin Sim, MD, Chairman of Orthopedic Oncology, Professor, Department of Orthopedic Surgery, Mayo Medical School
Contributor Information and Disclosures

Updated: Jan 9, 2009

Follow-up

Further Inpatient Care

• Soft tissue angiosarcoma  
  • With larger higher-grade angiosarcomas, adjuvant radiotherapy is effective in reducing local recurrence.
  • Postoperative radiotherapy can be delivered intraoperatively, by brachytherapy, or by external beam. The brachytherapy technique results in rates of tumor control similar to those obtained with external beam, with a similar rate of wound complications. Moreover, it presents the advantage of requiring only 5 days, rather than the 5-6 weeks needed for external beam, and reduces radiation scatter.
  • Brachytherapy is often the technique of choice in angiosarcomas near joints or gonads.
• Bone angiosarcoma: Specialist use combinations of radiation therapy and chemotherapy as adjuvant methods of treatment, but significant data about their effectiveness are lacking.
• Cutaneous angiosarcoma  
  • Postoperative radiotherapy is warranted in cases with unsatisfactory margins, large tumor size, deep extension, and multicentricity.
  • Radical radiation therapy in the form of high-field electron beam therapy shows promise in prolonging survival of patients with localized lesions.

Further Outpatient Care

• Soft tissue angiosarcoma  
  • Recurrent neoplasms typically (80% of cases) develop within 2 years of the resection. Thus, the follow-up should be extremely stringent, ie, every 3 months in the first 2 years.
  • A chest radiograph every 6 months during this period is mandatory. If the chest radiograph reveals a suspicious nodule, obtain a CT scan of the chest for confirmation.
  • MRI is the most accurate technique for detecting locally recurrent or residual sarcoma. The baseline postoperative MRI examination serves a vital role in the evaluation.
  • After the first 2 years, schedule visits every 6 months for the next 3 years. After 5 years, see patients annually.
  • The following is a differential diagnosis for signal abnormality on a postoperative MRI:
    • The residual or recurrent neoplasm usually is a discrete, ovoid, or rounded soft tissue mass. Nonspecific signal characteristics are present, with intermediate-to-low signal intensity in T1-weighted images and high signal on T2-weighted images. It enhances with gadolinium. Comparison with prior postoperative examinations is essential.
    • Postsurgical/postradiation change usually appears as a regional distribution of signal abnormality, with a linear, trabeculated, or latticelike morphology. This has low-to-intermediate signal on T1-weighted images and high signal on T2-weighted images.
    • Scars show a linear morphology. Scars correspond with skin thickening and the loss of adjacent subcutaneous fat. A scar has low signal on both T1-weighted images and T2-weighted images, although they can present a linear high-signal intensity on T2-weighted images and a variable enhancement with gadolinium.
    • Fluid collection develops as an abscess, seroma, or hematoma. Seromas are the most common. They represent a signal intensity lower than muscle on T1-weighted images and high signal on T2-weighted images and are differentiable from recurrence with gadolinium. Hematomas usually appear in the subacute stage at the time of the first postoperative MRI. Hematomas show a characteristic high signal on T1-weighted images and T2-weighted images from the methemoglobin. Abscesses present as low signal on T1-weighted images and high signal on T2-weighted images and may show some low-signal intensity depending on the presence of a fibrous capsule. Following gadolinium administration, they appear as a nonenhancing fluid collection with a thick, nodular, peripherally enhancing rim.
• Bone angiosarcoma: The follow-up is similar to that outlined for the soft tissue angiosarcomas.
• Cutaneous angiosarcoma  
  • Patients need clinical examination every 3 months to detect possible recurrences. Palpation of the cervical lymph nodes remains a major tool.
  • Imaging studies include CT scan and MRI of the head and neck and plain chest radiograph and CT chest scans every 3 months for 1 year, every 6 months for 2 more years, and then annually. Distant metastasis can occur in a late fashion.

Deterrence/Prevention

• Prevention of angiosarcoma is included in cancer prevention guidelines. The reduction of cancer mortality is achieved via reduction in the incidence of cancer.
• Prevention strategies include avoiding carcinogens and adopting lifestyle or dietary factors that modify cancer-causing factors or genetic predispositions, alter of carcinogen metabolism, or alter end-organ effects of carcinogens. Prevention also includes the successful treatment of preneoplastic lesions.

Complications

• Neurovascular injury
• Range of motion and strength
• Complications from radiotherapy
  • Wound complications
  • Fractures
• Complications from chemotherapy
  • Fever
  • Dose-dependent myelosuppression
  • Nausea and vomiting unresponsive to antiemetics
  • Moderate-to-severe fatigue
  • Alopecia or hemialopecia in intraarterial chemotherapy
• Complications from surgery
  • Anesthetic complications
  • Blood loss
  • Infection, sepsis
  • Wound complications
  • Iatrogenic neurovascular injury
  • Deep vein thrombosis, pulmonary embolism
  • Limited limb function

Prognosis

• Soft tissue angiosarcoma  
  • Angiosarcoma of the soft tissue is a high-grade sarcoma with a high rate of death and short survival time.
  • A large number of patients, 50% in some series, also had metastasis, and a significant number (20%) had local recurrences.⁷
  • Older patient age, retroperitoneum location, and larger tumor size are unfavorable prognostic factors.
  • Approximately 66% of retroperitoneal angiosarcomas recur locally in the tumor bed and can recur diffusely throughout the peritoneal cavity (angiosarcomatosis).
• Bone angiosarcoma  
  • High-grade angiosarcomas exhibit extremely aggressive behavior with rapid local growth and early disseminated metastasis.
  • Prognosis depends on the histologic grade, with the disease-free survival rate reported as 95% in grade 1 tumors, 62% in grade 2, and 20% in grade 3. Multicentricity does not affect prognosis.
• Cutaneous angiosarcoma^1,9   
  • Despite aggressive treatment, prognosis is poor. The median time of survival ranges from 15-24 months, with a 5-year survival rate of 12-33%. Local failure and metastases to local cervical lymph nodes are common. The lung is the most common site of distant metastasis, followed by the liver and bone, although these tend to occur late.
  • Unlike other sarcomas, grade is not useful in predicting survival. No correlation exists between appearance (eg, ulcerated, nodular, diffuse) and survival or local recurrence.
  • Findings of significantly favorable prognostic importance appear to be tumor size (<5 cm), complete surgical resection, and a moderate or marked lymphoid infiltrate in and around the tumor.
  • Unresectable lesions and metastatic disease at diagnosis suggest a dismal prognosis. Death can occur either from local extension or metastasis.
  • Delayed diagnosis and treatment explain, in part, the poor prognosis of cutaneous angiosarcomas.

Patient Education

For excellent patient education resources, visit eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education articles Mastectomy and Breast Cancer.

Miscellaneous

Medicolegal Pitfalls

• Failure to obtain an adequate history and thorough enough physical examination to identify risk factors (eg, toxic exposure, radiation therapy)
• Failure to consider obtaining and documenting informed consent in individuals who require the use of materials previously related to the development of angiosarcoma (eg, Dacron grafts, bone wax, surgical sponges, metal foreign bodies)
• Failure to consider changes in signs or symptoms as a possible manifestation of local or distant relapse
• Failure to consider a diagnosis of angiosarcoma for any lump or mole arising in a radiation field
• Failure to provide adequate follow-up care to cancer survivors

Special Concerns

Future and controversies for angiosarcoma treatment

• Chemotherapy with doxorubicin may be promising for metastatic and/or unresectable tumors, although few studies compare intraarterial versus systemic chemotherapy and the role of preoperative versus postoperative radiotherapy.
• Liposomal encapsulation of doxorubicin may reduce cardiac effects and overcome multidrug resistance 1 (MDR-1). Positron emission tomography scanning measures metabolic activity of the tumor and may become an additional imaging technique to correlate with tumor grade.
• Paclitaxel as a single agent has substantial activity against angiosarcoma of the scalp or face, even in patients previously treated with chemotherapy or radiation therapy. Further investigation is warranted to define the optimal treatment dose and schedule.¹⁷
• Several recent approaches attack a proliferating endothelium. Close interactions between endothelial cells and the bloodstream appear to make the vasculature a practical target for tumor therapy. Most of these therapies use drugs that have not been approved for use by the US Food and Drug Administration or drugs approved for other uses. Caution should be used when these drugs are prescribed out of clinical trials.
  • Antiangiogenic drugs (eg, combrestatin, angiogenesis inhibitors [2-methoxyestradiol, CNTOT 95, NP-470]) cut off the blood supply to tumors. Interferon alfa-2a has shown antiangiogenesis activity through down-regulation of basic fibroblast growth factor, which is commonly overexpressed in angiosarcoma. Good responses to this treatment have been reported.^18,15
  • After demonstrating that rapidly proliferating tumor endothelial cells are susceptible to immunotoxins in experimental models, specific antibodies conjugated with efficient cytotoxins (eg, ricin), virus (eg, parvovirus) or radioactive isotopes may be used to target the tumor vessels.¹⁹
  • Radiotherapy following treatment with radiosensitizers (eg, halogenated pyrimidines, platinum salts, gadolinium porphyrins) has been especially helpful in treating tumors when surgery is considered too risky or is contraindicated.

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