Case report Dermat<!fibrosarcoma protuberans 
Recurrent dermatofibrosarcoma 
protuberans treated with Mohs 
micrographic surgery 
M. Koclric, V. Padovese, R. Stankovic, A. Adami, G. Grandi, R. Bussani, C. Vidali and G. Trevisan 
SUMMARY 
We present the case of a 68-year-old woman with a recurrent dermatofibrosarcoma protuberans on the 
neck and the upper back, surgically treated four times over a yearlong period. Neither radiotherapy nor 
chemotherapy have been effective. After the last recurrence the Mohs micrographic surgery has been 
applied. The tumor has been excised and cut in a considerable number of fragments, which have then 
been examined microscopically. A second procedure has been necessary to completely remove the 
tumoral mass. Four months later there have been no signs of local recurrence any more. The Mohs 
micrographic surgery is the treatment of choice for dermatofibrosarcoma protuberans, since classical 
surgical excision would have been, as our case reveals, difficult and vain. 
Introduction 
The dermatofibrosarcoma protuberans is a locally 
aggressive and highly recurrent malignant neoplasm of 
dermal origin. Histologically, it is composed of spindle 
cells resembling more or less mature fibroblasts (1, 2): 
The tumor occurs in middle-aged persons, most 
frequently on the trunk. Initially, it appears asa unique 
nodule that evolves later in a multinodular indurated 
plaque, usually flesh-colored, reddish or blue. The 
clinical course is characterized by a painless, progressive 
and irregular growth. In the advanced pluse it may 
become painful and ulcerated. Metastases have also 
been described, but they are rare and may occur after 
the repeated recurrences (1, 2). 
The differential diagnosis in the early phase includes 
a dermatofibroma and keloid. In the advanced phase 
the tumor's slow growth rate, a characteristic color and 
irregular margins suggest the diagnosis dermatofibro-
sarcoma (1). 
In general, wide surgical excision is considered 
necessa1y to avoid reappearance of the tumor. Recu-
rrence rate using undefined surgical margins is estimated 
from 49 to 53%. It drops to 10-20% when wide surgical 
margins (3 cm of clinically uninvolved tissue) reaching 
down to the superficial fascia, are used. Such procedure 
may lead however to a significant functional and 
aesthetical impairment(l). Chemotherapy is not indica-
Acta Dermatoven APA Vol 9, 2000, No 4 ------------------149 
Dermatofibrosarcoma protuberans 
ted being reserved for metastatic disease (1 , 2). 
Radiotherapy has a limited role; it may however be 
useful , combined with surgery when margins are 
positive or even alone in cases of non-operable tumors 
(3 - S). 
Case report 
A 68-year-old woman was affected by dermato-
fibrosarcoma protuberans extending from the posterior 
cervical region to the left upper part of the back (Fig. 
1). Initially it appeared as an asymptomatic , flesh-
colored, rapidly growing nodule, 2 cm in diameter. 
The tumor was completely excised and the histo-
pathology confirmed the clinical diagnosis. Three 
months later, the patient underwent a further surgical 
intervention because of a postoperative recurrence. 
Three months afterwards, as the tumor rapidly recurred, 
radiotherapy with photons of a linear accelerator ( 4 
MeV) was applied. The complete dose amounted to 
6000 cGy divided into 30 fractions, with single dose of 
200 cGy per fraction. This treatment was not successful; 
thus the patient underwent a new surgical excision, as 
there was again evidence of tumor's recurrence. Three 
months after the last operation chemotherapy with 
Adriamycin (60 mg/ m2, 110 mg once weekiy) and 
dacarbazine (220 mg/ m2, daily dose 400 mg, for five 
consecutive days, every fourth week) repeatedly for six 
cycles was introduced. There was no sign of tumor 
regression. 
For this reason, three months after the. chemo-
therapy, Mohs micrographic surgery has been perfor-
med. The tumor has been excised and divided into six 
large fragments. Due to their large size, each one has 
been cut into nine smaller specimens and examined 
microscopically, by multiple horizontal sections from 
the base to the epidermis in order to identify the per-
sisting foci of tumoral microinvasion. 
The tissue was fixed in 10% buffered formalin, 
conventionally processed and embedded in paraffin 
(wax). Sections 4 „m thick were stained with haemato-
xylin and eosin. Representative slides were studied by 
immunohistochemistry using an automated immuno-
stainer with the avidin-biotin-peroxidase method. Anti-
bodies used were CD34 and S-100 protein. 
Histologically, the neoplasm was composed of 
densely packed, monomorphous spindle cells arranged 
in a storiform pattern. Small fibrosarcomatous-like areas 
were seen at the periphery of the lesion, where the 
tumor was often characterized by a fascicular growth 
pattern of spindle cells or by a honeycomb-like pattern 
(Fig. 2). Only scattered mitoses were observed. Immu-
nostaining for CD34 resulted focally positive. The tumor 
was entirely negative for S-100 protein. 
1.50 
Figure 1. Recurrent dermatofibrosarcoma 
protuberans on the neck and the upper back: 
preoperative appearance. 
Figure 2. lrregular strands of spindle cells with 
monomorphous nuclear pattern. No atypical 
mitoses are evident. H. E. x 100. 
Case report 
Acta Dermatoven APA Vol 9, 2000, No 4 
Case report 
Two areas of deep tumoral involvement have been 
found (Fig. 3). 
The second and ultimate excision, necessary to 
remove selectively these two areas, has been done. The 
surgical wound has been closed with a rotation-advan-
cement flap. Four months later there is no evidence of 
local recurrence (Fig. 4). 
Discussion 
The Mohs micrographic surgery is an excisional 
technique with complete histological margins' control. 
This technique allows the complete removal of the 
neoplasm, examining all its margins, while conserving 
as much uninvolved tissue as possible (6, 7). 
It is the most effective surgical treatment for the epi-
thelial and mesenchimal neoplasms, characterized by 
contiguous growth, either primary or recurrent, having 
indistinct clinical margins and found in particular, high-
risk areas. Particularly aggressive and extended tumors 
may need wide and destroying excisions for they may 
infiltrate other organs and vita! tissues. Tumors, recurring 
after surgical excision or irradiation, may show unpre-
dictable subclinical growth within and around scar tissue 
from previous treatments (8). 
The advantages of this method are related to the 
high oncologic effectiveness and the maximal normal 
tissue saving. 
The tumor is excised and cut into severa! pieces. 
The proper orientation of fragments is preserved by 
putting references on the wound's edge and by color-
coding specimens' edges with different dyes (9, 10). 
Figure 4. Result at four months postoperatively. 
Dermatofibrosarcoma protuberans 
MOHSMAP 
TRUNK 
INFERIOR 
Figure 3. Mohs map: tumor divided into six 
large fragments and persistence of tumor in two 
areas. 
After those specimens are placed upside down, the 
edges are teased on the same plane as the basement 
and a microscopic examination of the horizontal 
sections is performed. If there is evidence of persisting 
tumoral islets, they can be localized on the surgical 
wound and selectively removed. Thus, only the residual 
neoplastic areas are excised while healthy surrounding 
tissue is maximally spared. The procedure continues 
step by step until the entire tumor is excised and a three-
dimensional view of the whole neoplasm with its 
microscopic extensions may be reconstructed (9, 10). 
Conclusion 
Although the follow-up period is quite short, it 
appears rather significant in view of the previous rapidly 
recurrent behavior of the tumor. 
Data from the literature suggest that Mohs micro-
graphic surgery is the treatment of choice for dermato-
fibrosarcoma protuberans. Therefore, it should always 
Acta Dermatoven APA Vol 9, 2000, No 4 ------------------ JJJ 
Case report Dermatofibrosarcoma protuberans 
be the treatment of choice in order to avoid recurrences 
and complications. 
Acknowledgement 
We wish to thank Mr. Luciana Bortoletto for his care 
in arranging the histological specimens. 
Our case confirms that Mohs micrographic surge1y 
can reduce the recurrence rate that is rather high after 
classical surgical excision. 
AUTHORS' 
ADDRESSES 
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treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996; 35: 82-7. 
3. Ballo MT, Zagars GK, Pisters P, Pollack A. The role of radiation therapy in the management of 
dermatofibrosarcoma protuberans. Int J Radia! On col Biol Phys 1998; 40: 823-7. 
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dermatofibrosarcoma protuberans. Soft Tissue Tumours Working Group. Eur J Cancer 1997; 33: 1055-
60. 
5. Suit H, Spiro I, Mankin HJ, EfirdJ. Radiation in the management of patient with dermatofibrosarcoma 
protuberans. J Ciin Oncol 1996; 14: 2365-9. 
6. Ratner D. Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. J Am 
Acad dermatol 1997; 37: 600-13. 
7. Swanson NA. Mohs surgery: technique, indications, applications and the future. Arch Dermatol 1983; 
119: 761-73. 
8. Lynn AD et al. Guidelines of care for Mohs micrographic surgery. J Am Acad Dermatol 1995; 33: 271-
8. 
9. Shriner DL, Me Coy DK, Goldberg DJ. Mohs micrographic surgery. J Am Acad Dermatol 1998; 39: 79-
97. 
10. Miller PK, Roenigk RK, Brodland DG. Cutaneous micrographic surgery: Mohs procedure. Mayo Ciin 
Proc 1992; 67: 971-80. 
Metka Kodric, MD, Institute oj Dermatology, University ojTrieste, Strada di 
Fiume 447, 34149 Trieste, Italy 
Valeska Padovese, MD, same address 
Relja Stankovic, MD, same adress 
AdelinoAdami, MD, Division oj Surgery ''Prima Chirurgica", 
Piazza del!' Ospitale 2, 34129 T'rieste, Italy 
Rossana Bussani, MD, same address 
Christiana Vidali, MD, Department oj Radiotherapy, Via Pieta 19, 
34129 T'rieste, Italy 
Giusto Trevisan, MD, projessor and chairman, Institute ojDermatology, 
University ojTrieste, Strada diFiume 447, 34149 Trieste, Italy 
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