Rapidly Evolving Cervical Dermatofibrosarcoma Protuberans With Deep Muscle Infiltration: A Rare and Aggressive Presentation

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade sarcoma that typically presents as a slow-growing cutaneous nodule or plaque. We report a highly unusual case of DFSP involving the neck, which demonstrated rapid enlargement, necrosis, and deep muscle infiltration. A 45-year-old man presented with a large, bleeding cervical tumor that had evolved over two years. Imaging revealed extensive involvement of the neck and shoulder musculature without distant metastasis. Histopathologic evaluation confirmed the diagnosis of DFSP. Given the tumor's inoperability, treatment with imatinib was initiated, resulting in marked tumor regression. This case highlights an atypical presentation of DFSP in terms of both location and clinical behavior. It underscores the importance of repeated histologic assessment in the face of rapid progression, the role of imaging in surgical planning, and the value of targeted therapy when surgical options are limited.

Keywords: darier–ferrand dermatofibrosarcoma; dermatofibrosarcoma protuberans; imatinib mesylate; imatinib therapy; oncodermatology.

Figure 1. Clinical presentation of a well-defined subcutaneous tumor (~10 cm) in the basicervical region, with erythematous-violaceous overlying skin (yellow arrows). A second, superimposed angiomatous lesion, ulcerated and highly hemorrhagic, measuring approximately 6 cm is also visible (yellow asterisk). (A) Lateral view. (B) Frontal view

Figure 2. DFSP. (A) Tumor proliferation composed of spindle-shaped cells showing mild to moderate nuclear atypia (H&E, ×400). (B) Immunohistochemistry showing intense and diffuse positivity for CD34, consistent with DFSP

H&E: hematoxylin and eosin, DFSP: dermatofibrosarcoma protuberans

Figure 3. Helical CT scan in axial (A) and coronal (B) planes after iodinated contrast injection, showing an exophytic lesion (yellow asterisk) arising from the right posterior cervical soft tissues, with ill-defined, irregular margins. The lesion invades the right longus colli muscle (red arrow) and the ipsilateral trapezius muscle (yellow arrow)

CT: computed tomography

Figure 4. MRI showing a well-defined, lobulated mass (yellow asterisk) arising from the right basi-cervical soft tissues. The lesion appears isointense on T1-weighted images (A, B), hyperintense on T2/STIR sequences (C, D), and shows heterogeneous enhancement after contrast (E, F). It invades the ipsilateral longus colli (orange arrow) and trapezius muscles (red arrow)

MRI: magnetic resonance imaging

Figure 5. Marked increase in the size of the angiomatous lesion one month after the initial consultation. The lesion became largely necrotic and measured approximately 15 cm in diameter. (A) Lateral view. (B) Posterior view

Figure 6. Helical CT acquisition in axial (A) and sagittal (B) planes showing significant reduction in the size of the mass (yellow asterisk) arising from the right basi-cervical soft tissues, still in close contact with the ipsilateral trapezius muscle

CT: computed tomography

Figure 7. Marked reduction of the subcutaneous tumor four months after initiation of imatinib therapy (arrows), with complete regression of the angiomatous component (asterisk). (A) Posterior view. (B) Lateral view