European Journal of Cardiovascular Medicine

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft-tissue sarcoma that arises from dermal fibroblasts and accounts for less than 0.1% of all malignancies. It commonly affects young to middle-aged adults and typically presents as an indurated plaque or a firm nodular mass that grows gradually over months or years, often without pain or systemic symptoms. Because of its subtle early appearance and resemblance to benign lesions such as dermatofibroma, morphea, keloid, or hypertrophic scar, DFSP is frequently misdiagnosed, leading to significant delays in detection and treatment (1). Although metastasis is rare, DFSP is locally aggressive, infiltrating subcutaneous tissue, fascia, and sometimes muscle, resulting in a high recurrence rate when excised with inadequate margins. Clinical examination alone is often insufficient to determine the extent of spread, making imaging an important component of pre-operative assessment.

Various imaging techniques are used for DFSP evaluation. MRI is widely considered the best modality because it provides excellent contrast resolution, allowing assessment of tumour depth, involvement of adjacent structures, and surgical margin planning. DFSP typically appears as an iso- to hypointense lesion on T1-weighted images and hyperintense on T2-weighted sequences, often with infiltrative margins and septations (2). Ultrasound can be useful for superficial lesions and may show a hypoechoic mass with lobulated margins or internal vascularity. CT, although less sensitive for soft tissue contrast, helps detect rare cases with bone erosion or in situations where MRI is not available (3). In recent years, dermoscopy has gained importance as a non-invasive tool, revealing features such as arborising or polymorphic vessels, shiny white streaks, and pink-brown structureless areas. However, dermoscopic findings vary, and overlapping patterns with other spindle-cell tumours make interpretation challenging (4).

Despite the availability of these tools, the existing literature shows important gaps. Most studies focus on a single imaging technique, especially MRI, and describe radiological patterns in small numbers of patients. Very few studies attempt to correlate imaging findings with clinical parameters such as lesion duration, growth rate, presence of protuberant nodules, recurrence status, or previous surgical interventions. Studies by Li et al. (5) and Teixeira et al. (2) concentrated mainly on MRI characteristics without linking them to clinical behaviour or surface changes. Similarly, dermoscopic studies are mostly descriptive and do not evaluate deeper infiltration seen on ultrasound or MRI. There is also a lack of work integrating multimodal imaging dermoscopy, ultrasound, and MRI to explore whether combined features improve diagnostic confidence or provide clues regarding tumour aggressiveness. The absence of clinicoradiological correlation makes it harder for clinicians to interpret imaging findings meaningfully and plan wide local excision or Mohs micrographic surgery appropriately. A structured correlation between clinical findings and imaging characteristics is therefore needed to strengthen early diagnosis, reduce recurrence, and guide surgeons more accurately in planning tumour clearance.

The present study aims to comprehensively describe the imaging features of dermatofibrosarcoma protuberans using available modalities and analyse how these radiological findings correlate with clinical presentation. By establishing a clearer clinicoradiological relationship, the study seeks to enhance diagnostic accuracy, improve understanding of tumour behaviour, and support better pre-operative planning.

Study Design and Setting

This was a prospective observational study conducted jointly in the Departments of Dermatology, Venereology and Leprosy (DVL) and Radiology at Mamata Medical College, Khammam. The study was carried out over a period of two years, from May 2022 to June 2024. All patients attending the dermatology outpatient unit with clinically suspected or biopsy-confirmed dermatofibrosarcoma protuberans (DFSP) during the study period were screened for eligibility.

Sample Size and Selection Criteria

A total of 30 patients were included in the study. Patients of all ages and both sexes with clinically diagnosed DFSP and willing to undergo imaging evaluation were enrolled after obtaining informed consent. Patients with recurrent tumours previously treated elsewhere, incomplete records, poor-quality imaging, or those who declined participation were excluded. The diagnosis of DFSP was confirmed by histopathological examination in all cases, which served as the reference standard.

Clinical Evaluation

For each patient, detailed clinical information was recorded, including age, sex, duration of lesion, site of involvement, history of trauma or previous treatment, presence of protuberant nodules, and symptoms such as pain or rapid enlargement. Clinical measurements including size, colour, consistency, and surface changes were noted systematically.

Imaging Protocol

All enrolled patients underwent imaging according to departmental protocol. Ultrasound examination was performed for all cases to assess lesion echotexture, depth, margins, vascularity, and involvement of underlying structures. MRI was carried out whenever feasible, especially for lesions larger than 2 cm, lesions located over complex anatomical areas, or when deeper invasion was suspected. Standard MRI sequences included T1-weighted, T2-weighted, STIR, and post-contrast images. CT imaging was used selectively in cases requiring evaluation of possible bone involvement. Imaging findings such as signal characteristics, pattern of enhancement, infiltration into subcutaneous tissue or muscle, and margin definition were documented. Dermoscopy was performed in the dermatology department for accessible lesions, and features including vascular structures, pigment network, shiny white lines, and background colour were recorded.

Clinicoradiological Correlation

Imaging findings were correlated with clinical features to assess patterns of concordance, including lesion depth versus duration, infiltrative margins versus clinical induration, and dermoscopic vascularity versus MRI enhancement. Histopathology findings were used as the gold standard for comparison. All data were compiled and analysed descriptively to understand the relationship between clinical presentation and imaging characteristics.

Ethical Considerations

Institutional ethical committee approval was obtained before the start of the study. Written informed consent was taken from all participants, and confidentiality was maintained throughout the study.

Statistical Analysis

Data were analysed using standard statistical software (SPSS version 24.0). Descriptive statistics were expressed as mean ± standard deviation for continuous variables and proportions for categorical variables. p-values < 0.05 were considered statistically significant

Table 1: Baseline Clinical Characteristics of Patients with DFSP (n = 30)

In this cohort of 30 patients, the mean age was 38.6 ± 12.4 years with a slight male predominance. Most lesions were long-standing, with an average duration of 28.3 ± 14.9 months, and primarily involved the trunk (46.7%) or extremities (33.3%). Protuberant nodules were present in 70% of cases, while rapid recent enlargement and pain were reported in 40% and 26.7% respectively. A history of trauma (10%) or prior treatment (16.7%) was noted in a minority of patients (Table 1).

Table 2: Clinical Characteristics of Lesions in Patients with DFSP (n = 30)

The average lesion size in the study group was 4.8 ± 1.9 cm. Most lesions were skin-coloured or reddish-brown, while a smaller proportion appeared hyperpigmented or pale. On palpation, the majority were firm (73.3%), with fewer showing induration or soft consistency. Protuberant nodules were the most frequent surface change (70%), followed by smooth plaques (36.7%), while atrophic changes, telangiectasia, and ulceration were less common (Table 2).

Table 3: Ultrasound Characteristics of DFSP Lesions (n = 30)

Ultrasound evaluation showed a mean lesion thickness of 12.6 ± 4.3 mm. Most lesions were hypoechoic, with 60% appearing homogeneous and 40% showing heterogeneous echotexture. Ill-defined or infiltrative margins were seen in 73.3% of cases, while 46.7% demonstrated a lobulated contour and 36.7% showed tentacle-like projections. Colour Doppler revealed mild to moderate vascularity in most cases, with marked vascularity in 20%. Subcutaneous fat involvement was common (70%), whereas deeper spread to fascia and muscle was observed in 30% and 10% of patients, respectively (Table 3).

Figure 1: CT Findings in DFSP Patients Undergoing CT Evaluation (n = 6)

In the subset of six patients who underwent CT, soft-tissue extension beyond the fascia and absence of bone involvement were the most common findings (50% each). Cortical irregularity was noted in 33.3%, while definite cortical breach or erosion was seen in only one patient (16.7%). Adjacent periosteal reaction was also present in 16.7% of cases. No lesions demonstrated calcification (Figure 1).

Figure 2: Dermoscopic Features in Patients with DFSP (n = 30)

Dermoscopy revealed predominantly pink to skin-coloured backgrounds, with pink being the most common (40%), followed by skin-coloured lesions (33.3%). Vascular patterns were frequently observed, most notably linear irregular (40%) and arborising vessels (30%), with dotted and mixed patterns seen less often. Pink–brown structureless areas were the most common pigment-related feature (60%), followed by shiny white lines in 36.7% and a delicate pigment network in 23.3% of cases. These findings together reflect the vascular and fibrous stromal components characteristic of DFSP (Figure 2).

Table 4: MRI Characteristics of Lesions in DFSP Patients (n = 30)

MRI evaluation showed that most lesions exhibited hypointense signals on T1-weighted images (70%) and hyperintense signals on T2-weighted and STIR sequences (80% and 86.7%, respectively), with a subset demonstrating heterogeneous patterns. Contrast enhancement was predominantly moderate to intense, reflecting vascular tumour stroma. The mean maximum diameter was 5.2 ± 1.8 cm, with a mean invasion depth of 14.1 ± 5.2 mm. Ill-defined or infiltrative margins were observed in 36.7–40% of lesions, indicating deeper spread along tissue planes. Subcutaneous fat involvement was common (80%), while fascial and muscle invasion occurred in 43.3% and 16.7% of cases, respectively, suggestive of advanced disease in a subset of patients (Table 4).

Table 5: Correlation Between Clinical and Ultrasound Parameters (n = 30)

A significant positive correlation was noted between clinical lesion size and ultrasound-measured lesion thickness (r = 0.72, p < 0.001), indicating that larger lesions tended to demonstrate greater depth of invasion. Longer duration of lesions was significantly associated with infiltrative projections on ultrasound (p = 0.013), suggesting progressive extension into deeper planes over time. Protuberant nodular lesions showed higher vascularity grades on Doppler evaluation (p = 0.027), reflecting increased stromal activity. Pain at presentation was also significantly correlated with deeper invasion involving subcutaneous tissue or muscle (p = 0.020), indicating that deeper infiltration may be clinically symptomatic (Table 5).

Table 6: Correlation Between Clinical Features and MRI Findings (n = 30)

A strong correlation was observed between clinical lesion size and MRI-measured depth of invasion (r = 0.69, p < 0.001), indicating that larger lesions tended to infiltrate deeper tissues. Longer duration of lesions showed a significant association with fascial involvement (p = 0.022), suggesting progressive spread along deeper planes over time. Protuberant nodules demonstrated a significant relationship with stronger enhancement patterns on MRI (p = 0.008), reflecting higher vascularity and stromal activity. Additionally, pain at presentation correlated with muscle invasion (p = 0.026), implying that deeper involvement is more likely to produce symptoms (Table 6).

Table 7: Correlation Between Ultrasound and MRI Parameters (n = 30)

There was a very strong correlation between ultrasound-measured thickness and MRI depth of invasion (r = 0.81, p < 0.001), indicating high concordance between both modalities in assessing tumour extent. Infiltrative margins identified on ultrasound were significantly associated with corresponding infiltrative margins on MRI (p = 0.001), reflecting consistency in detecting invasive spread across techniques. Doppler vascularity showed a moderate but significant correlation with contrast enhancement on MRI (r = 0.63, p = 0.001), suggesting that increased tumour vascularity on ultrasound corresponds to stronger enhancement patterns on MRI (Table 7).

Table 8: Correlation Between Dermoscopic and Imaging Features (n = 30)

Dermoscopy findings showed meaningful correlations with deeper imaging features. Arborising and irregular vascular patterns were significantly associated with stronger contrast enhancement on MRI (p = 0.009), suggesting that surface vascular morphology reflects deeper stromal vascularity. The presence of shiny white lines correlated with infiltrative margins on MRI (p = 0.015), indicating that collagen-rich stromal changes visible dermoscopically may correspond to deeper invasive extensions. Pink–brown structureless areas were significantly associated with heterogeneous echotexture on ultrasound (p = 0.031), reflecting internal structural variability consistent with tumour infiltration (Table 8).

This prospective study evaluated the clinical features and multimodal imaging characteristics of dermatofibrosarcoma protuberans (DFSP) and analysed their correlations in 30 patients over two years. DFSP is a rare, slow-growing, locally aggressive cutaneous sarcoma with high recurrence despite low metastatic potential, consistent with existing literature [6]. The mean age in our study was in the late thirties with slight male predominance, and trunk and extremities were the most common sites, comparable to previous reports that DFSP typically affects young to middle-aged adults and favours the trunk and limbs [7]. The mean lesion duration exceeded two years, reflecting delayed diagnosis due to early resemblance to benign conditions such as keloid or dermatofibroma [6]. Protuberant nodules were observed in most cases and pain in a subset, consistent with the transition from plaque-like lesions to nodular growth described in established studies [8].

Ultrasound in our study showed predominantly hypoechoic lesions with lobulated or infiltrative margins and high rates of subcutaneous fat involvement, aligning with prior reports describing hypoechoic dermal masses with tentacle-like extensions and altered hypodermis [9]. Increased Doppler vascularity, especially in nodular lesions, matches findings from recent multimodal ultrasound studies demonstrating enhanced vascular patterns in DFSP [10]. The correlation between pain and deeper invasion in our cohort indicates that ultrasound may serve as a useful early tool to suspect advanced local spread.

MRI findings were characteristic, with most lesions showing T1 hypointensity, T2/STIR hyperintensity, and moderate to intense enhancement, consistent with classical MRI descriptions of DFSP as a superficial but infiltrative enhancing mass [11]. Infiltrative or tail-like margins seen in a subset of cases also reflect known microscopic radial extensions responsible for incomplete excision and recurrence [12]. The significant correlation between imaging depth on MRI and ultrasound, and between lesion size and deeper invasion, supports literature suggesting that lesion progression parallels deeper tissue involvement [13].

CT performed selectively demonstrated cortical irregularity or bone involvement in few cases, similar to reports where bone erosion occurs mainly in long-standing or anatomically constrained tumours, while calcification remains uncommon [14].

Dermoscopy revealed pink to skin-coloured backgrounds with linear irregular or arborising vessels and shiny white streaks, matching established dermoscopic patterns described by Bernard et al. and subsequent studies [15]. Correlation of vascular dermoscopic features with MRI enhancement and surface structural changes with infiltrative margins suggests that dermoscopy may reflect deeper tumour biology, consistent with emerging literature emphasising multimodal assessment [16, 17].

Overall, our findings reinforce that combined clinical, dermoscopic, ultrasound, and MRI evaluation improves early suspicion, depth assessment, and pre-operative planning. Ultrasound serves as an initial, easily accessible modality, while MRI remains essential for mapping deeper invasion and surgical margin planning. A multimodal approach helps identify high-risk lesions and may reduce recurrence through better delineation of tumour boundaries. Limitations include small sample size, single-centre design, selective use of MRI/CT, and limited follow-up for recurrence outcomes. Larger multicentre studies with advanced imaging techniques and long-term follow-up are needed to further validate these correlations

DFSP in this cohort presented as long-standing plaques progressing to nodules, most commonly on the trunk and extremities. Ultrasound revealed hypoechoic infiltrative lesions with high rates of subcutaneous involvement, while MRI confirmed T1 hypointensity, T2/STIR hyperintensity, and infiltrative margins, with deeper invasion in advanced cases. Dermoscopy showed characteristic vascular and stromal patterns that correlated with radiological features. Significant associations between lesion size, duration, vascularity, and depth of infiltration support multimodal evaluation for diagnosis and surgical planning. Integrating dermoscopy, ultrasound, and MRI may enhance early detection, guide appropriate surgical margins, and reduce recurrence risk.

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