Operative management of dermatofibrosarcoma protuberans of the breast Melissa H. Kinney, MD, and Sally M. Knox, MD
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a tendency towards local recurrence. A 26-year-old woman presented with a 3 × 2 cm raised, purple-hued lesion on her left breast. Excisional biopsy identified the lesion as a DFSP. She underwent two additional operations to achieve widely clear margins. Operative excision is the primary treatment of dermatofibrosarcoma protuberans of the breast.
D
ermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous tumor. It is characterized by its slow, infiltrative growth and marked tendency towards local recurrence after surgical resection (1). The incidence rate is 4.2 to 4.5 cases per million persons per year in the United States (2). The most common location of DFSP is the trunk, with 25% of cases affecting the chest and shoulder areas (1). At least 40 case reports of DFSP in the breast have been reported, but less than half address the surgical management (3–17), which we describe here. CASE DESCRIPTION A 26-year-old Hispanic woman presented to the surgery clinic at Baylor University Medical Center at Dallas complaining of a left breast lesion. The lesion had been present since an incision and drainage procedure in the area approximately 8 years earlier. She noted that it had slowly grown over this period. The lesion was approximately 2 × 3 cm in size. It was located 5 cm from the nipple at the 7 o’clock position on the left breast. The lesion had a keloid-like appearance in that it was purple-hued, protuberant, and irregular. She had no ulceration of the lesion or nipple discharge. She was not taking any hormone therapy. There was no family history of breast, ovarian, skin, or other cancers. An ultrasound revealed a 3 cm lesion of the dermis corresponding with the area of concern. An excisional biopsy of the lesion with gross margins using a radially oriented elliptical incision disclosed it to be DFSP (Figure 1). The lesion had a low mitotic index, but there was invasion into the underlying adipose tissue. All margins were positive for residual disease. She subsequently had wide local excision with a 2 cm margin of tissue taken circumferentially around the previous incision. Initial frozen section evaluation revealed that the deep margin was positive, so a new margin was Proc (Bayl Univ Med Cent) 2016;29(3):315–317
Figure 1. Histologic appearance of the lesion consistent with dermatofibrosarcoma protuberans.
taken. Frozen section analysis of the new margin was negative at the time of surgery, but on final analysis DFSP was seen at the lateral edge and another focus 2 mm away from the new deep margin. The patient underwent a third operation for wide local excision, again with 2 cm circumferential margins. All margins were clear by at least 1 cm on histological evaluation. The patient has done well postoperatively with a satisfactory cosmetic result (Figure 2).
Figure 2. Postsurgical appearance following wide local excision and primary closure. From the Department of Surgery, Baylor University Medical Center of Dallas. Corresponding author: Melissa Kinney, MD, Department of Surgery, Baylor University Medical Center at Dallas, 3500 Gaston Avenue, Dallas, TX 75246 (e-mail: [email protected]). 315
Table 1. Cases of dermatofibrosarcoma protuberans of the breast addressing surgical management Age (yr)
Size of lesion (cm)
Yeniay (3)
48
10
Recurrence
Mastectomy
Unknown
Yes
Cavus¸og˘lu (4)
26
2
Primary
Wide local excision with breast reduction pattern
5
Yes
Swan (5)
39
5
Recurrence
Wide local excision with latissimus dorsi flap
5
Yes
Dagregorio (6)
66
21
Primary
Wide local excision with reverse abdominal plasty
5
Yes
Castle (7)
30
Unknown
Primary
Wide local excision with pectoralis flap
Unknown
Unknown
McGregor (8)
33
Unknown
Dragoumis (9)
48
2
Park (10)
28
3.8
First author (ref)
Recurrence or primary
Margin size (cm)
Surgical management
Deep fascia excision
Primary
Wide local excision with skin graft
“Wide”
Yes
Recurrence
Wide local excision with primary closure
3
No
Primary
Wide local excision with primary closure
3
No
Kamiya (11)
75
1.2
Primary
Wide local excision with primary closure
3
Yes
Cottier (12)
75
1.8
Primary
Wide local excision with primary closure
5
Yes
Mikhail (13)
19
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a tendency towards local recurrence. A 26-year-old woman presented with a 3 × 2 cm raised, purple-hued lesion on her left breast. Excisional biopsy identified the lesion as a DFSP. She underwent two additional operations to achieve widely clear margins. Operative excision is the primary treatment of dermatofibrosarcoma protuberans of the breast.
D
ermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous tumor. It is characterized by its slow, infiltrative growth and marked tendency towards local recurrence after surgical resection (1). The incidence rate is 4.2 to 4.5 cases per million persons per year in the United States (2). The most common location of DFSP is the trunk, with 25% of cases affecting the chest and shoulder areas (1). At least 40 case reports of DFSP in the breast have been reported, but less than half address the surgical management (3–17), which we describe here. CASE DESCRIPTION A 26-year-old Hispanic woman presented to the surgery clinic at Baylor University Medical Center at Dallas complaining of a left breast lesion. The lesion had been present since an incision and drainage procedure in the area approximately 8 years earlier. She noted that it had slowly grown over this period. The lesion was approximately 2 × 3 cm in size. It was located 5 cm from the nipple at the 7 o’clock position on the left breast. The lesion had a keloid-like appearance in that it was purple-hued, protuberant, and irregular. She had no ulceration of the lesion or nipple discharge. She was not taking any hormone therapy. There was no family history of breast, ovarian, skin, or other cancers. An ultrasound revealed a 3 cm lesion of the dermis corresponding with the area of concern. An excisional biopsy of the lesion with gross margins using a radially oriented elliptical incision disclosed it to be DFSP (Figure 1). The lesion had a low mitotic index, but there was invasion into the underlying adipose tissue. All margins were positive for residual disease. She subsequently had wide local excision with a 2 cm margin of tissue taken circumferentially around the previous incision. Initial frozen section evaluation revealed that the deep margin was positive, so a new margin was Proc (Bayl Univ Med Cent) 2016;29(3):315–317
Figure 1. Histologic appearance of the lesion consistent with dermatofibrosarcoma protuberans.
taken. Frozen section analysis of the new margin was negative at the time of surgery, but on final analysis DFSP was seen at the lateral edge and another focus 2 mm away from the new deep margin. The patient underwent a third operation for wide local excision, again with 2 cm circumferential margins. All margins were clear by at least 1 cm on histological evaluation. The patient has done well postoperatively with a satisfactory cosmetic result (Figure 2).
Figure 2. Postsurgical appearance following wide local excision and primary closure. From the Department of Surgery, Baylor University Medical Center of Dallas. Corresponding author: Melissa Kinney, MD, Department of Surgery, Baylor University Medical Center at Dallas, 3500 Gaston Avenue, Dallas, TX 75246 (e-mail: [email protected]). 315
Table 1. Cases of dermatofibrosarcoma protuberans of the breast addressing surgical management Age (yr)
Size of lesion (cm)
Yeniay (3)
48
10
Recurrence
Mastectomy
Unknown
Yes
Cavus¸og˘lu (4)
26
2
Primary
Wide local excision with breast reduction pattern
5
Yes
Swan (5)
39
5
Recurrence
Wide local excision with latissimus dorsi flap
5
Yes
Dagregorio (6)
66
21
Primary
Wide local excision with reverse abdominal plasty
5
Yes
Castle (7)
30
Unknown
Primary
Wide local excision with pectoralis flap
Unknown
Unknown
McGregor (8)
33
Unknown
Dragoumis (9)
48
2
Park (10)
28
3.8
First author (ref)
Recurrence or primary
Margin size (cm)
Surgical management
Deep fascia excision
Primary
Wide local excision with skin graft
“Wide”
Yes
Recurrence
Wide local excision with primary closure
3
No
Primary
Wide local excision with primary closure
3
No
Kamiya (11)
75
1.2
Primary
Wide local excision with primary closure
3
Yes
Cottier (12)
75
1.8
Primary
Wide local excision with primary closure
5
Yes
Mikhail (13)
19
