In all, 42 patients were retrieved between 1910 and 2000. Six were excluded after morphological review for the following reasons: cystosarcoma phyllodes (n=4), fibromatosis (n=1), benign haemangioma (n=1). Totally, 11 cases were also excluded after showing a positive stain with wide spectrum screening keratin, and being considered metaplastic carcinoma.
Overall, 25 remaining patients constituted the study group and are summarised in
. There were 25 women age range 24–81 (mean 45 years). In total, 24 cases presented with lump, two of them associated with pain. In one case, it presented as an incidental mammographic finding. Contralateral breast sarcoma had been diagnosed elsewhere 3 years earlier in one case, renal cell carcinoma 5 years later in one case, colon cancer 4 years earlier in one case, skin melanoma and uterine cancer in one case 16 and 27 years earlier, respectively. No history of prior radiation was found in any case, therefore excluding postradiation sarcoma. The duration of symptoms for 16 patients ranged between 1 month to 40 years (mean 3.2 years).
Patients clinical and pathological characteristics in our series
Surgical treatment was excision in five cases, mastectomy in 19 cases (modified, four; simple, five; radical, five; not specified, five), and unknown in one case. Adjuvant therapy was administered in five cases (radiation, four; chemotherapy, one).
The right breast was affected in 10 cases, while the left was affected in 15 cases.
Gross description was available in 12 cases. Eight tumours were described as well-circumscribed, four as infiltrative of which two were angiosarcoma. Tumour size was available on 18 patients, and the mean tumour size was 5.7
cm (range 0.3–12.0). Angiosarcomas tended to be larger in size with a mean of 10
cm (range, 8–12
After present review, histopathological diagnoses were fibrosarcoma (n=6), angiosarcoma (n=6), pleomorphic sarcoma (n=6), leiomyosarcoma (n=2), myxofibrosarcoma (n=3), hemangiopericytoma (n=1) and osteosarcoma (n=1). Tumours were graded as low grade (grade 1, one; grade 2, nine), and high grade (grade 3, seven; grade 4, eight). Necrosis was observed in four cases (three high-grade tumours). In all, 11 (range, 0–43) mitoses were found on average in 10 HPF. Heterologous component was seen in one case of osteosarcoma. Seven had pushing margins while 16 had infiltrative ones.
An in situ ductal carcinoma component was observed in one case. In this case of pleomorphic sarcoma, keratin staining was negative in neoplastic cells with adequate internal control (the in situ component as well as benign entrapped ducts).
There was no metastasis in the 15 cases where axillary node dissection was performed.
Follow-up and survival analysis ()
Overall mean and median follow-up were, respectively, 10.5 and 6.4 years (range, 7 months–41 years). Local recurrence was observed in 11 patients and ranged from 2 to 36 months (mean 15 months), while distant metastasis was observed in 10 patients, in order of frequency affecting the lungs (n=7), bones (n=6), liver (n=5), spleen (two) and skin (two). In one case, other sites were also kidney, pancreas, adrenal, omentum, epicardium and mediastinum. Of the 25 patients, 12 have died of disease and six of other causes. At the last follow-up, seven patients were still alive with a mean and median follow-up of 10.2 and 10.9 years, respectively.
Five-year overall (OS) and cause-specific survival (CSS) were 66 and 70%, respectively. Five-year OS and CSS were both 91% for tumours
cm, and 50% for tumours >5
cm. Tumour size was significantly associated with OS (risk ratio=1.3 per 1
cm increase; 95% CI, 1.02–1.7; P
=0.036). There was no significant difference between low- and high-grade lesions (OS were 60 and 70%, P
=0.14, CSS were 70 and 70%, P
=0.5, respectively) or tumours showing infiltrative compared to pushing margins (OS were 65 and 71%, P
=0.47, CSS were 65 and 86%, P
=0.94, respectively) in terms of OS or CSS.
Although there was no statistically significant association between tumour size and metastasis or recurrence, mean tumour size of patients with recurrence or metastasis was 7.7
cm, compared to 4.9 and 4.3
cm, respectively, for patients without recurrence or metastasis. Four out of five patients treated with simple excision had recurrence or metastasis.
By the most common histopathologic types, all but one patient with angiosarcoma (4/5), one patient with fibrosarcoma, and two patients with pleomorphic sarcoma, died of disease.
Figure 1 Overall survival following surgery, according to tumour size (5 vs >5cm). The numbers in parentheses indicate the number of patients still at risk at selected time points.