Dermatofibrosarcoma protuberans: Our experience of 59 cases

  • Authors:
    • Alessio Stivala
    • Giuseppe A.G. Lombardo
    • Gianluca Pompili
    • Maria Stella Tarico
    • Filippo Fraggetta
    • Rosario Emanuele Perrotta
  • View Affiliations

  • Published online on: August 30, 2012
  • Pages: 1047-1055
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Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor with intermediate malignancy. It is initially located on the skin from where it is able to infiltrate the deep structures and has a tendency to recur locally following inadequate excision. A t(17;22)(q22;q13) chromosome translocation is the main cytogenetic alteration responsible for the onset of DFSP. Treatment options include complete surgical excision by performing conventional surgery with wide margins (>3 cm) or Mohs micrographic surgery. A retrospective study was conducted in our Department of Plastic and Reconstructive Surgery and all data were collected from medical records of 59 DFSP patients within this department from 1999 to 2011. A total of 13 of 59 (22%) cases were treated with conventional excision; 3 (5%) cases resulted in tumor-free margins, 8 (14%) cases required surgical revision and 2 (3%) cases lead to recurrence. A total of 46 of 59 (78%) cases were treated with wide excision; 43 (73%) cases resulted in tumor-free margins, 3 (5%) cases required surgical revision and 0 (0%) cases lead to recurrence. In conclusion, the data collected reveal the controversy surrounding the adoption of general guidelines regarding safe margins. Further studies are required to investigate the possibility of obtaining genotypically altered margins from margins that may appear phenotypically healthy.


In 1924, Darier and Ferrand (1) first reported a case of progressive and recurrent dermatofibroma. One year later, Hoffman (2) described the tendency of the dermatofibroma tumor to develop into protruding nodules and termed the condition dermatofibrosarcoma protuberans (DFSP). In 1962, Taylor and Helwig (3) analyzed the histological characteristics of DFSP and in 1992 it was discovered that immunopositivity for CD34 correlated with negative immunostaining for factor XIIIa (4,5). Then, in 1997, Simon et al (6) identified that a translocation between chromosome 17 and 22 was the distinguishing cytogenetic alteration in neoplastic tissues responsible for the development of DFSP.

DFSP is classified as a rare tumor, however, it may be considered as the most common stromal tumor of cutaneous origin (7). Numerous epidemiological studies in the USA have reported a mean annual incidence rate between 0.8 and 4.5 cases per million individuals (810). Rutgers et al (11) reviewed 902 DFSP cases and identified a 3:2 incidence ratio of males to females. However, Criscione and Weinstock (12) studied 9 population-based cancer registries with a total of 2,885 DFSP cases and reported a higher incidence in females. It has also been found that DFSP has a higher incidence rate among individuals aged between 20 and 50 years (13); however, several studies have reported more than 160 pediatric cases of acquired DFSP and more than 35 congenital cases (7,1419). In addition, it has been demonstrated that giant cell fibroblastoma is the juvenile form of dermatofibroblastoma arising in childhood (20).

Materials and methods

Patient data

A retrospective study was conducted in our Department of Plastic and Reconstructive Surgery and all data were collected from medical records of 59 DFSP patients within this department from 1999 to 2011 (Table I). The histopathological diagnosis and immunohistochemical studies were conducted by the Department of Anatomical Pathology within the same hospital. Each medical record included the age, gender, tumor location and presentation, clinical features, treatment modality, histopathological report, closure type and prognosis of each patient. Informed consent was obtained from each patient.

Table I

Summary of 59 cases of dermatofibrosarcoma protuberans between 1999 and 2011.

Table I

Summary of 59 cases of dermatofibrosarcoma protuberans between 1999 and 2011.

Patient no.Date of diagnosisAge (years)GenderLocationPresentationClinical featuresSurgical modality Immunohistochemistry and cytogenetic resultsClosure typeRecurrence or surgical revision
121/04/199920MBackOn prior scarsMorphea-likeWECD34+ Vimentin+1 closureNo
219/05/200030FLeft armOn apparently normal cutisMorphea-likeWECD34+ Actin 1A4-Vimentin+Local flapSurgical revision
323/09/200030MAbdomenOn apparently normal cutisProtrudingWECD34+ Vimentin+1 closureNo
403/10/20009FLeft ankleOn prior surgical scarProtruding DFSPWECD34+1 closureNo
503/10/200068FLeft shoulderOn apparently normal cutisProtruding DFSPWECD34+Local flapNo
603/11/200054FForehead and root of the noseOn prior fronto-temporalMorphea-like, protruding DFSP fibrosarcomaCECD34+ Vimentin+S100-Local flapRecurrence
706/11/200060FRight inguinal regionOn prior surgical scarProtruding DFSPWECD34+ Vimentin+CD68+1 closureNo
827/01/200157FRight supraclavicular regionOn apparently normal cutisProtruding DFSPCECD34+1 closureSurgical revision
926/06/200136FRight flankOn apparently normal cutisMorphea-likeCECD34+ CD68-1 closureRecurrence
1004/07/200134MLeft thighOn apparently normal cutisProtruding DFSPWECD34-GraftNo
1129/10/200114FLeft legOn apparently normal cutisMorphea-likeWECD34+1 closureNo
1209/05/200227FLeft shoulderOn apparently normal cutis Atrophoderma-likeCECD34+1 closureSurgical revision
1330/09/200232FBackOn prior surgical scarProtruding DFSPWECD34+ S-100+1 closureNo
1410/12/200266MScalpOn prior actinic keratosis Atrophoderma-likeWECD34+ Actin 1A4+1 closureNo
1528/12/200234Right supraclavicular regionOn apparently normal cutisProtruding DFSPWECD34+ CD68-Local flapNo
1610/01/200324MRight cheekOn apparently normal cutisProtruding DFSPCECD34+ Vimentin+Local flapNo
1703/02/200357MNapeOn apparently normal cutisProtruding DFSPWECD34+ Vimentin+Actin 1A4-EMA-CD31+ Factor VIII+CD99+ Bcl-2+1 closureNo
1821/03/200364FRight legOn prior blue nevus and lentigo simplexPigmented DFSPWEHemosiderin+ CD34+S-1001 closureNo
1907/04/200367MRight footOn apparently normal cutisMorphea-likeWECD34+1 closureNo
2028/04/200328FLeft breastOn apparently normal cutisPigmented DFSPWECD34+ Actin 1A4+S-100-Local flapNo
2106/05/200354FLeft breastOn apparently normal cutisProtruding DFSPWECD34+ S-100Local flapNo
2216/06/200314FLeft shoulderOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
2323/06/200326FLeft armOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
2403/07/200321FRight thighOn apparently normal cutisPigmented DFSPWECD34+GraftNo
2521/07/200326FLeft shoulderOn apparently normal cutisProtruding DFSPWECD34+ Vimentin+Local flapNo
2611/08/200318MRight forearmOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
2717/09/200324MRight armOn apparently normal cutisProtruding DFSPWECD34+ Actin 1A4+Desmin, CK-1 closureNo
2827/10/200339FAbdomenOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
2924/03/200419FRight armOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
3005/08/200428MChestOn apparently normal cutisMorphea-likeWECD34+1 closureSurgical revision
3118/09/200425MLeft legOn apparently normal cutisUlcerated protruding DFSPWECD34+Actin 1A4+ Bcl-2+1 closureNo
3221/10/200419FScalpOn prior surgical scarUlcerated protruding DFSPWECD34+ Actin 1A4-S-100-1 closureNo
3312/05/200419FLeft shoulderOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
3405/01/200523FChestOn apparently normal cutisProtruding DFSPWECD34+1 closureSurgical revision
3508/04/200552FLeft laterocervical regionOn apparently normal cutisProtruding DFSPCECD34+1 closureSurgical revision
3612/04/200543FLeft inguinal regionOn prior surgical scarProtruding DFSPWECD34+Local flapNo
3710/05/200537MLeft shoulderOn apparently normal cutisMorphea-likeCECD34+1 closureSurgical revision
3814/10/200531MRight inguinal regionOn apparently normal cutisMorphea-likeCECD34+1 closureSurgical revision
3911/02/200656FAbdomenOn apparently normal cutisMorphea-likeWECD34+1 closureNo
4001/04/200632MRight armOn apparently normal cutisProtruding DFSPWECD34+ Vimentin+CD163-Actin 1A4-1 closureNo
4120/12/200629MAbdomenOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
4206/02/200728FLeft armOn apparently normal cutisPigmented DFSPWECD34+1 closureNo
4312/03/200752FLeft scapular regionOn apparently normal cutisMorphea-likeCECD34+ S-1001 closureSurgical revision
4426/06/200763MLeft armOn apparently normal cutisProtruding DFSPWECD34+1 closureNo
4516/11/200737MAbdomenOn apparently normal cutisMorphea-likeWECD34+1 closureNo
4609/01/200837FLeft armOn apparently normal cutis Atrophoderma-likeWECD34+1 closureNo
4729/02/200837FRight armOn pigmented cutisPigmented DFSPWECD34+GraftNo
4819/03/200869FRight shoulderOn apparently normal cutisProtruding DFSPWECD34+ S-100Actin 1A41 closureNo
4930/03/200953MRight thighOn apparently normal cutisProtruding DFSPWECD34+GraftNo
5028/07/200957MScalpOn apparently normal cutisProtruding DFSPWECD34+ CD31-Desmin-1 closureNo
5104/12/200929MLeft cheekOn apparently normal cutisProtruding DFSPCECD34+ CD117+CD31-CD68-CD163-Local flapSurgical revision
5223/12/200935FVulvaOn apparently normal cutisProtruding DFSPCECD34+1 closureNo
5329/04/201033FPalpebral commissureOn apparently normal cutisProtruding DFSPCECD34+Local flapNo
5420/07/201043FLeft thighOn apparently normal cutisProtruding DFSPWECD34+ CD68+GraftNo
5524/12/201042MLeft armOn apparently normal cutisPigmented DFSPWECD34+1 closureNo
5615/03/201132FGluteusOn apparently normal cutisProtruding DFSPWECD34+Local flapNo
5720/05/20111FLeft flankOn apparently normal cutisProtruding DFSPWECD34+1 closureSurgical revision
5804/08/201147MTemporal regionOn apparently normal cutisProtruding DFSPWECD34+Local flapNo
5904/10/201164MLeft shoulderOn apparently normal cutisProtruding DFSPWECD34+1 closureNo

[i] DFSP, dermatofibrosarcoma protuberans; CE, conventional excision; WE, wide excision.


Surgical treatment was achieved by performing conventional and wide excision. Conventional surgery was adopted in areas where wide excision would have been difficult to perform, including the root of the nose, vulva, cheek and palpebral commissure. The mean margin used in this type of treatment was 1.07 cm. The majority of cases treated were subjected to wide excision with a mean margin of 3.4 cm. Both surgical options were performed by removing the skin, subcutaneous tissue and superficial fascia.

All specimens excised were subjected to formalin fixation and sectioning to confirm the tumor-free margins and histopathological positivity. The immunohistochemical stainings adopted in this study included: CD34 antigen (clone, QBEnd/10, NCL; Novacastra), Vimentin (clone, V9; Thermo Scientific), ACTML, Van Gieson, hematoxylin and eosin, Protein S100 (clone, 4C4.9; Thermo), CD68 (clone, KP1; NCL) and Perl's iron staining.

Follow up

Patient follow-up for recurrences ranged from 3 to 120 months with a mean follow-up time of 62 months. A variety of surgical techniques were adopted for the most suitable wound closure, including primary intention, local flaps or grafting.

No patient within this study had been treated with chemotherapy or radiation prior to treatment at our institution, and no additional adjuvant treatments were performed.


Clinical and surgical data are presented in Tables IIII. As demonstrated in Table II, the mean patient age at time of diagnosis was 37 years (71% of cases were <50-years-old and 29% of cases were >50-years-old), and the majority of cases presented were female (61%). The tumor was located on the trunk, upper extremities, lower extremities and head and neck region in 49, 20, 15 and 16% of cases, respectively. The protruding form of DFSP was the most frequent clinical variety presented, occurring in 66% of all cases. Notably, 17% of patients reported a prior alteration of the cutis, including previous wounds, surgical scars, actinic keratosis, blue nevus and lentigo simplex. Additionally, no cases presented were recurrent at the time of their initial diagnosis.

Table III

Correlation between excision modality and prognosis.

Table III

Correlation between excision modality and prognosis.

No. of cases treated
PrognosisConventional excision (%)Wide excision (%)Total (%)
Tumor free3 (5)43 (73)46 (78)
Surgical revision8 (14)3 (5)11 (19)
Tumor recurrence2 (3)0 (0)2 (3)
Total13 (22)46 (78)59 (100)

Table II

Clinical features of 59 DFSP patients.

Table II

Clinical features of 59 DFSP patients.

Clinical featuresNo. of casesTotal cases (%)
Age at diagnosis
  Mean age (years)37-
Clinical presentation
  Protruding DFSP3966
  Pigmented DFSP610
  Upper extremities1120
  Lower extremities915
  Head and neck region1016
  On apparently normal cutis4983
  On pigmented/prior altered cutis1017

[i] DFSP, dermatofibrosarcoma protuberans.

A total of 13 of 59 (22%) cases were treated with conventional excision due to the difficult location of the tumor (Table III). Following surgery, 3 (5%) cases had tumor-free margins, 8 (14%) cases required surgical revision and 2 (3%)cases lead to recurrence. Although conventional excision may lead to a higher recurrence rate (as demonstrated by this study), in certain cases prognosis may be positively affected by this treatment option. The remaining 46 (78%) cases were treated with wide excision. No patient developed recurrences and only 3 (5%) cases required surgical revision. Additionally, no patient referred to in Tables IIII presented metastatic disease.


The main etiological factor in the development of DFSP is the presentation of several prior traumas, including surgical scars (21), trauma scars (22), burns (23), radiodermatitis (24), vaccination sites (25), sites of central venous lines (26) and insect bites (27).

The most common anatomical site affected by DFSP is the trunk (42–72%), with the majority of cases found on the chest and trunk. A total of 16 to 30% of DFSP cases are located on the proximal extremities (particularly on the legs) and up to 16% of cases affect the head and neck areas (7,13).

Clinically, DFSP behaves as a slow-growing asymptomatic plaque and consequently, the majority of patients consult their doctors at a late stage (13). Initially, the neoplasm presents as a violaceous reddish-brown or pink indolent plaque with a hard consistency; at this stage the lesion may be misinterpreted as a hypertrophic scar. Over time, the tumor diffusely infiltrates the deep layers of the skin and the dermis, which leads to the development of several multiple nodules, which are indurated to palpation and adherent to the surrounding tissues, including the subcutaneous fat, fascia, muscle, periosteum and bone.

Martin et al (28) distinguished three clinical forms of non-protruding DFSP: morphea-like, atrophoderma-like and angioma-like (Table IV). However, the most frequent presentation described in adults is a large plaque presenting multiple nodules on its surface.

Table IV

Clinical variants of the preprotuberant stage of DFSP as described by Martin et al (25).

Table IV

Clinical variants of the preprotuberant stage of DFSP as described by Martin et al (25).

VariantClinical featuresOnset
Morphea-likeWhite or brown indurated plaque appearing as a scar, morphea, morpheaform basal cell carcinoma or dermatofibroma plaqueChildhood
Atrophoderma-likeSoft depressed white or brown plaque similar to atrophoderma or anetodermaCongenital
Angioma-likeIndurated red or violaceous plaques with clinical appearance similar to vascular malformations or angiomasUncommon

[i] DFSP, dermatofibrosarcoma protuberans.

DFSP is characterized by a low rate of metastasis and an eccentric growth rate, which may determine a high level of local invasion. It was found that conventional surgery leads to local recurrence in up to 30% of cases (13).

Kim (29) described seven histological DFSP subtypes of which 90% of cases are represented by ‘classic’ DFSP (Table V). Histologically, the ‘classic’ subtype of DFSP appears as a well-differentiated fibrosarcoma initially located on the dermis. The neoplasm is composed of a poor stroma with a dense growth of monomorphous fusiform cells and a large elongated nucleus characterized by little pleomorphism and a low mitotic index. In addition, spindle cells are irregularly organized in linked fascicles with a storiform arrangement. Taylor and Helwig (3) reported a typical diagnostic pattern of DFSP, which is represented by a cartwheel arrangement where cells are arranged radially around a central acellular collagenous area.

Table V

Histological subtypes of DFSP as described by Kim (29).

Table V

Histological subtypes of DFSP as described by Kim (29).

SubtypeHistological characteristicsClinical features
‘Classic’ DFSPMonomorphous fusiform cells (spindle cells) with large elongated nucleus and poor cytoplasm, low mitotic indexMost common subtype (90%)
Rare metastasis (≤0.5%)
Giant cell fibroblastomaGiant multinucleated cells, sinusoidal vessels, myxoid stromaFrequently observed in childhood
Bednar tumorMelanocytes and deposits of melaninObserved in African and American patients
Sclerotic DFSPAbundant stroma with several layers of collagen and areas of denser cellularityRare subtype
Myxoid DFSPSpindle cells grouped in nodules with eosinophilic cytoplasmRare subtype
Atrophic DFSPAtrophic mid-dermis with subcutaneous tissue close to the epidermisFrequently observed in childhood
Fibrosarcomatous DFSPHigh mitotic index with high cellularity and marked nuclear pleomorphismSubtype with the highest recurrence rate and metastatic potential, most aggressive variant

[i] DFSP, dermatofibrosarcoma protuberans.

DFSP has the tendency to expand from the central focus and invade the surrounding tissues. The tentacle-like projections invade the septa and fat lobules and adopt a honeycomb (30%) or multilayered (70%) subcutaneous pattern (30,31). In both patterns, tentacle-like projections can be inadvertently omitted during wide excision, determining a possible cause of tumor recurrence followed by fascia, muscle and bone invasion.

The first immunohistochemical marker identified for DFSP was the CD34 antigen. It is expressed in up to 90% of cases, differentiating DFSP from other fibrohistiocytic tumors (32,33) (Table VI). Previous studies have revealed that CD34 is also expressed by other sarcomas and benign fibrohistiocytic lesions, including solitary fibrous tumor (34), sclerotic fibroma (35), superficial acral fibromyxoma (36), cellular digital fibromas (37), dermatofibromas (38) and nuchal-type fibroma (39). Consequently, CD34 may be considered as a non-specific marker for DFSP. Other immunohistochemical markers, including factor XIIIa, stromelysin III, apolipoprotein D and CD163, have been found to be positive in dermatofibromas and negative in DFSP (13,3941). Bandarchi et al (42) also reported that D2-40 may be used as a marker for the differential diagnosis between DFSP and dermatofibroma (Table VI).

Table VI

Immunohistochemical markers used for differential diagnosis of DFSP.

Table VI

Immunohistochemical markers used for differential diagnosis of DFSP.

MarkerExpressedNot expressed
CD34DFSP, inflammatory fibrosarcoma, myofibrosarcoma, angiosarcoma, epithelioid sarcomaDermatofibroma, malignant fibrous histiocytoma, pediatric myofibromatosis, fibrosarcoma, hypertrophic scars or keloids
Factor XIIIaDermatofibromaDFSP
Stromelysin IIIDermatofibromaDFSP
Apolipoprotein DDermatofibromaDFSP

[i] DFSP, dermatofibrosarcoma protuberans.

Molecular biology techniques, including reverse-transcriptase polymerase chain reaction and fluorescent in situ hybridization, have revealed that DFSP is characterized by supernumerary ring chromosomes or a reciprocal translocation between chromosome 17 and chromosome 22 t(17;22) (q22;q13) (6,43). This translocation involves the collagen type 1 α 1 (COL1α1) gene located on chromosome 17 and the PDGFβ gene located on chromosome 22. In DFSP, COL1α1 is highly expressed and acts as an inducer of gene transcription (44). COL1α1-PDGFβ fusion leads to the transcription of a fully active PDGFβ protein, which triggers mitosis through the activation of the PDGFβ receptor (PDGFβR) via auto-crine and paracrine stimulation of its functional ligand (45). The PDGFβR is composed of three structural domains: an extracellular binding, a transmembrane and a cytoplasmic domain with tyrosine kinase activity. The tyrosine kinase activates an intracellular signaling cascade that affects physiological cell processes, including chemotaxis, proliferation and apoptosis (13).

Primary treatment of DFSP consisted of complete surgical excision of the lesion. It has been reported (46) that standard surgical resection leads to a local recurrence rate of up to 60%, which is due to the occult spreading of the tentacle-like projections beneath the clinically normal-appearing skin margins.

The main challenge in DFSP surgery is to achieve satisfactory local control. To obtain the lowest recurrence rate, two surgical treatments may be performed: wide local excision and Mohs micrographic surgery (MMS). In addition to surgical methods (recurrent and metastatic lesions), molecular targeted therapy with imatinib mesylate may be considered as a suitable alternative or additional treatment option for DFSP.

Several studies have demonstrated a significant correlation between wide excisions and low recurrence rates (4648) (Table VII). According to previous studies, it is recommended that surgical excisions be performed at least 2–3 cm away from the gross margin. Furthermore, it is important to perform a three dimensional en bloc removal of the tumor, including skin, subcutaneous tissue and fascia. If the underlying bone structures are affected it is necessary to perform a wide resection of the periosteum and bone (7).

Table VII

Correlation between surgical margins and local recurrence rate in DFSP wide local excision.

Table VII

Correlation between surgical margins and local recurrence rate in DFSP wide local excision.

Author (Refs.)Surgical margin (cm)Recurrence rate (%)
Chang et al (46)5<5
Fiore et al (47)320
Gloster et al (48)<240

[i] DFSP, dermatofibrosarcoma protuberans.

MMS can be used to produce a local control that is more effective. MMS is a surgical procedure characterized by precise histological resection margin control. According to Dim-Jamora and Perone (49), MMS should be the first choice for DFSP treatment. The most important technical aspect in MMS is continual sequential horizontal sectioning (5–7 μm) with immediate microscopic examination of the frozen sections of the resected tissue until a clear margin is obtained. Guillen and Cockerell (50) revealed through MMS that tentacle-like formations can extend beyond 3 cm in the horizontal direction. Loss and Zeitouni (51) consider MMS as the treatment of choice in anatomically challenging areas, including the head or neck. Although the efficacy of MMS is highly recognized, this technique is also considered to be elaborate, time-consuming and labor-intensive (7).

According to the cytogenetic role of PDGFβR in the pathogenesis of DFSP, several studies have focused on the most suitable strategy to inhibit the mitogen process. Imatinib competes with adenosine triphosphate and prevents tyrosine kinase receptor autophosphorylation. This leads to inhibition of the aberrant signal transduction pathway and a partial restoration of intracellular signaling.

Data in the present study demonstrate the controversy surrounding the adoption of general guidelines regarding safe margins. However, we are confident to use the guidelines proposed by Chang et al (46), Fiore et al (47) and Gloster et al (48). Future treatments for DFSP may adopt other parameters, including the cytogenetical study of surgical margins, since margins that are phenotypically recognized to be tumor-free, may hide the genetical translocation t(17;22) (q22;q13). Further studies should investigate the possibility of obtaining genotypically altered margins from margins that may appear phenotypically healthy. This may improve the accuracy of tumor excision and the predictability of further possible recurrences.



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November 2012
Volume 4 Issue 5

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Online ISSN:1792-1082

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Stivala A, Lombardo GA, Pompili G, Tarico MS, Fraggetta F and Perrotta RE: Dermatofibrosarcoma protuberans: Our experience of 59 cases. Oncol Lett 4: 1047-1055, 2012
Stivala, A., Lombardo, G.A., Pompili, G., Tarico, M.S., Fraggetta, F., & Perrotta, R.E. (2012). Dermatofibrosarcoma protuberans: Our experience of 59 cases. Oncology Letters, 4, 1047-1055.
Stivala, A., Lombardo, G. A., Pompili, G., Tarico, M. S., Fraggetta, F., Perrotta, R. E."Dermatofibrosarcoma protuberans: Our experience of 59 cases". Oncology Letters 4.5 (2012): 1047-1055.
Stivala, A., Lombardo, G. A., Pompili, G., Tarico, M. S., Fraggetta, F., Perrotta, R. E."Dermatofibrosarcoma protuberans: Our experience of 59 cases". Oncology Letters 4, no. 5 (2012): 1047-1055.