Page 15 - Delaware Medical Journal - March 2016
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CASE REVIEW
TABLE 1: Summary of Selected Patient Characteristics of Patients with
Radiation Associated Angiosarcoma (RAAS)
VARIABLES
RAAS (n=7)
Age at Diagnosis (years)
70 (range 58-87)
Time from RT to RAAS (years)
8.5 (range 4.0-14.9)
Local Recurrence (patients)
3
Years to Recurrence
0.9 (range 0.5-1.8)
Presented with Skin Lesion (patients)
5
Presented with Mass (patients)
2
Lymphedema (Breast/UE) (patients)
0
Axillary Dissection (patients)
3
High Grade RAAS (patients)
4
Surgeons need to be familiar with RAAS, because commonly presenting characteristics can be subtle; and early diagnosis is imperative.
Angiosarcoma of the breast is divided into primary versus secondary. Primary angiosarcoma encompasses sarcoma of the breast occurring without any known risk factors.5 interest in this topic has increased in recent years.6 Scow et al. showed that these two entities have distinct clinically presentations, and that both carry a poor prognosis.7
Radiation associated angiosarcoma of the breast is a rare malignancy with an incidence reported of 0.9 per 1000
cases at 15 years.8 The tumor itself has an insidious onset typically occurring as a bluish/purplish skin discoloration diagnosis is a latency period of typically six to seven years.9 54 percent.10-12
Currently surgery is the mainstay of treatment. Surgical options include wide local excision, segmental mastectomy, and total mastectomy. Options for adjuvant therapy include chemotherapy and radiation. Interestingly, in 2008 a retrospective review of 73 manuscripts only produced 222 patients.13 The rarity of this disease has made evaluation of of this rare condition in a one month period.
MATERIALS AND METHODS
The Christiana Care Health System Institutional Review Board approval was requested and approved for this research. The Christiana Care Department of Pathology and the Helen F. Graham Cancer Center and Research Institute databases were queried from 2001 – 2011 and 2011-2015 respectively for soft tissue neoplasms of the breast. Cases from these data-bases were then cross checked to ensure accuracy of the diagnosis of RAAS of the breast. A total of 2,153 patients with diagnosis of malignant
RAAS: Radiation Associated Angiosarcoma, RT: Radiation Therapy, UE: Upper Extremity
Cases were individually reviewed for accuracy. Of the ten
remaining three were primary sarcomas of the breast. Radiation
the primary cancer. The following data were extracted: age of patient at diagnosis; latency from initial radiation to sarcoma; local recurrence; breast location; breast appearance; presence
of metastatic disease; surgical treatment; technique of breast
RESULTS
The mean age of the patient at presentation was 70 years (range 58-87). The mean time from initial presentation to the diagnosis of radiation associated angiosarcoma 8.5 years (range 4-15). (Table 1) Of the seven patients, three had a recent diagnosis of RAAS with limited follow up. The fourth patient was lost to follow up. Patients 2.8 and 1.3 years, respectively. Both of these patients had high grade
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