News Update on Angiosarcoma : Dec 2020

Angiosarcoma of the breast

A case of angiosarcoma of the breast with a fatal outcome is reported, and the world literature on this rare type of breast tumor is reviewed. Familiarity with the clinical and pathologic features of this entity is essential in avoiding underdiagnosis and delay of definitive treatment. The three‐year survival rate of reported cases was 38%. However, the three‐year, disease‐free survival was only 14%. Five cases with five‐year, disease‐free survival have been reported to date. Eighteen patients (21%) showed contralateral breast involvement; in half of these, it occurred before other evidence of tumor dissemination. Simple mastectomy appears to be the optimal treatment of choice, since axillary node involvement is extremely rare. The role of irradiation and chemotherapy as adjuvant therapy remains to be evaluated. [1]

Angiosarcoma: A report of 67 patients and a review of the literature

BACKGROUND

Angiosarcomas (AS) are rare, aggressive tumors. Optimal treatment has not been well defined. The authors undertook a retrospective review of patients seen at their institution with the intent of identifying prognostic factors and optimal treatment strategies.

METHODS

Between 1955 and 1990, 67 patients with AS were seen at the University of California, at Los Angeles Medical Center. Follow‐up ranged from 1 to 173 months with a median of 30 months.

RESULTS

The overall prognosis was poor. The actuarial 2‐ and 5‐year disease free survivals (DFS) were 44% and 24%, respectively. Of 52 recurrences after primary treatment, 81% (42 of 52) had a component of local failure. Twenty‐eight patients had developed distant metastases at last follow‐up. Of patients who received surgery (S) and radiation therapy (RT), with or without chemotherapy (CT), 5‐year actuarial DFS was 43%, compared with 17% for patients who underwent S+/−CT as initial treatment (P = 0.03). Only 9% of patients (1 of 11) treated with RT+/−CT were rendered free of disease.

CONCLUSIONS

Patients with AS usually present with high grade histology, and with multifocal disease. There is a propensity for both local recurrence and distant metastases. Our results and a review of the literature, suggest that S plus RT offers the best chance for long term control of this aggressive tumor. The role of CT remains undefined. [2]

Angiosarcoma of the breast

BACKGROUND

Angiosarcoma of the breast is a rare entity. The objectives of this study were to evaluate prognostic factors and determine outcomes in a large contemporary series of patients.

METHODS

Clinical and pathologic factors were analyzed in all patients with angiosarcoma of the breast treated between 1990 and 2003. Overall survival (OS) and disease‐free survival (DFS) were estimated using the methods of Kaplan and Meier. Multivariate analyses were performed to evaluate prognostic factors.

RESULTS

Fifty‐five women with angiosarcoma of the breast were identified. The median age was 49.1 years, and the median follow‐up time was 3.7 years. The median OS and DFS were 2.96 years and 2.26 years, respectively. For the 32 patients with primary angiosarcoma of the breast 5‐year OS was 59%. Twenty‐three (42%) patients had received prior radiation therapy for the treatment of breast cancer. These patients with radiation therapy‐associated angiosarcoma were on average 30 years older and less likely to present with distant metastatic disease than patients presenting with radiation‐naive angiosarcoma of the breast. Although radiation‐naive patients appeared to have had better early DFS and OS, the Kaplan–Meier curves were not statistically different between patients with radiation therapy‐associated disease and radiation therapy‐naive patients. On multivariate analysis, tumor recurrence (P = 0.006) was the only significant adverse prognostic factor noted for OS.

CONCLUSIONS

In this series of 55 patients with angiosarcoma of the breast, radiation therapy‐naive angiosarcomas occurred in younger patients, but they behaved similarly to radiation therapy‐associated angiosarcomas. [3]

Angiosarcoma of Large Intestine: A Case Report

Angiosarcomas are collectively one of the rare forms of soft tissue neoplasms. That of the large intestine is much rarer. We report a case of large intestinal angiosarcoma. This is a case of a 30-year-old man who presented with intestinal obstruction, and after resection, it was histologically diagnosed as Angiosarcoma of large bowel. [4]

Cardiac Angiosarcoma of the Right Atrium with Cardiac Tamponade

Aims: We report a case of cardiac sarcoma, which showed cardiac tamponade as the first clinical symptom and sign. Accumulation of such cases could lead to better treatment for patients with cardiac angiosarcoma.

Presentation of Case: A 56-year-old man presented with loss of consciousness. An echocardiogram showed cardiac tamponade, requiring an emergent exploration of the pericardium. We confirmed that the tumor originated in the right atrium and advanced to the right ventricle beyond the right coronary artery. A thickened right atrium with a small hole was resected, followed by reconstruction of the right atrium using a large patch of autologous pericardium. Cardiac angiosarcoma was pathologically identified. The patient died of multiple lung metastases 6 months after the operation, despite subsequent radiation therapy.

Discussion and Conclusion: Cardiac angiosarcoma shows extremely aggressive growth, as observed in our case. More clinical reports with cardiac angiosarcoma could aid in improving diagnostic and treatment modalities. [5]

Reference

[1] Chen, K.T., Kirkegaard, D.D. and Bocian, J.J., 1980. Angiosarcoma of the breast. Cancer, 46(2), pp.368-371.

[2] Mark, R.J., Poen, J.C., Tran, L.M., Fu, Y.S. and Juillard, G.F., 1996. Angiosarcoma: a report of 67 patients and a review of the literature. Cancer: Interdisciplinary International Journal of the American Cancer Society, 77(11), pp.2400-2406.

[3] Vorburger, S.A., Xing, Y., Hunt, K.K., Lakin, G.E., Benjamin, R.S., Feig, B.W., Pisters, P.W., Ballo, M.T., Chen, L., Trent III, J. and Burgess, M., 2005. Angiosarcoma of the breast. Cancer: Interdisciplinary International Journal of the American Cancer Society, 104(12), pp.2682-2688.

[4] M. Sahabi, S., Abubakar, N., Abdullahi, K., Mohammed, U., T. Olayinka, L., O. Ekochin, K. and M. Bashir, B. (2017) “Angiosarcoma of Large Intestine: A Case Report”, Cardiology and Angiology: An International Journal, 6(1), pp. 1-4. doi: 10.9734/CA/2017/31373.

[5] Sato, H., Aizawa, K., Muraoka, A., Akutsu, H. and Misawa, Y. (2015) “Cardiac Angiosarcoma of the Right Atrium with Cardiac Tamponade”, Journal of Advances in Medicine and Medical Research, 12(5), pp. 1-5. doi: 10.9734/BJMMR/2016/22051.

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