Page 16 - Delaware Medical Journal - March 2016
P. 16
TABLE 2: Description of Presenting Physical Exam of Patients Presenting
with Skin Lesion
PATIENT ID
DESCRIPTION
1
Multiple blisters with 2.0 x 3.0 cm plaque in outer quadrant of left breast
2
Single site of bluish discoloration
3
Multiple bleeding nodules centrally located in breast
4
Multiple reddish streaks near nipple areolar complex
5
Red dots on medial aspect of the breast
sarcomas, and local recurrence. The latter of these patients also had distant metastatic disease to both bone and lung. This patient was treated with palliative radiation for a recurrence after resection. However she developed a second local recurrence and this was also treated with palliative radiation. The seventh patient is alive with
3.2 years of follow up. No grading of this tumor was available. This patient did have a local recurrence which was re-excised. None of the seven patients had lymphedema of the breast or upper extremities
of discoloration of the skin. (Table 2) Two patients presented with breast masses. All seven patients in this study had only local disease at diagnosis. There were a variety of closure methods for wound management. Three patients were closed primarily, two received pathologic grading of the tumor consisting of, four high grade, one intermediate, and one low grade.
DISCUSSION
Radiation associated angiosarcoma of the breast is a rare condition. A review of the literature by Depla et al. in 2014 produced
74 articles with only 222 patients reported.13 There have not been
any randomized controlled trials to evaluate the treatment of this condition. During the review of patients evaluated at our institution three of these patients had been diagnosed within the last three months of this report.
previously reported. The average age of presentation was 70 years. The time from radiation exposure to RAAS was 8.5 years, which
is slightly longer than previously documented with a mean of
7 – 7.5 years.7, 14 Five of the seven patients reviewed presented with skin lesions as the initial physical sign. RAAS typically presents with a rash while primary angiosarcomas are more likely to present as a mass.7
Multiple skin nodules on presentation have been shown to have a poor prognosis with a zero percent survival at two years.15 Two of our patients who are currently deceased presented with multiple skin nodules with an overall survival of two years.
One limitation of our review is that the follow up for our
patients is relatively short. Three of the patients included had been diagnosed within three months of data collection. Of the remaining four, one patient was lost to followup. Only one of the remaining patients is still alive with a follow up of 3.2 years. The
two deceased patients had overall survivals of 2.8 years
and 1.3 years, respectively. Both presented with local recurrence at six months. Both of these patients were treated with wide local excision.
The primary modality of treatment for our patient population was surgery alone. One patient did receive treatment with Paclitaxel. A second patient also received palliative radiation treatment for a local recurrence. No patient received adjuvant radiation therapy. It has been suggested that an axillary lymph node dissection (ALND) could be a risk factor for RAAS by Smith et al.16 Their data revealed 14 patients with RAAS who had ALND, and suggested that the vast majority
if not all patients with secondary angiosarcoma of the breast had undergone ALND. This presumption does not seem to be supported by the fact that only three of our seven patients underwent ALND. Torres et al., in one of the largest single institution reports on RAAS, showed 27 patients who developed RAAS with lumpectomy alone.12 An area of future study would be the evaluation of whether ALND or sentinel lymph node procedures are independent risk factors for the development of RAAS, with proposed mechanism of lymphatic stasis.
SURGERY
Early diagnosis of this condition is imperative for successful treatment. It is not uncommon for the diagnosis of RAAS to be protracted. In order to successfully diagnose this condition it is
80
Del Med J | March 2016 | Vol. 88 | No. 3
