Which type of surgical consult is recommended for a 20-50 year old adult with dermatofibrosarcoma protuberans (DFSP) and no underlying medical conditions, general surgery (Gen Surg) or orthopedic (Ortho) surgery?

Surgical Consultation for Dermatofibrosarcoma Protuberans

For a 20-50 year old adult with DFSP, neither general surgery nor orthopedic surgery is the appropriate primary consult—you should refer to a dermatologic surgeon trained in Mohs micrographic surgery or to a specialized sarcoma center with soft tissue sarcoma expertise. 1

Primary Surgical Management Approach

The optimal surgical approach for DFSP is Mohs micrographic surgery or wide local excision with complete circumferential peripheral and deep margin assessment (CCPDMA/PDEMA), not standard general surgical or orthopedic excision. 1, 2

Why Standard Surgical Services Are Inadequate

• DFSP requires specialized margin control techniques that general surgeons and orthopedic surgeons typically do not perform 1
• The tumor exhibits highly irregular finger-like extensions into subcutaneous tissue that are missed with standard excision techniques 2
• Historical recurrence rates with inadequate surgical technique range from 10-60%, compared to 0-6.6% with Mohs surgery 1, 3
• Multidisciplinary consultation at a center with specialized expertise should be strongly considered, especially for large or recurrent DFSP, as decisions about diagnosis and resection may be complex 1

Appropriate Referral Pathways

Refer to dermatologic surgery for:

• Mohs micrographic surgery (preferred for tissue-sparing complete removal with comprehensive margin control, particularly in cosmetically sensitive areas) 2
• This achieves the lowest recurrence rates (0-6.6%) compared to wide local excision (1.7-30.8%) 1

Refer to specialized sarcoma center for:

• Large tumors where extensive subcutaneous extension is suspected 1
• Recurrent DFSP 1
• Any case where fibrosarcomatous transformation (FS-DFSP) is identified on biopsy, as this variant has 10-23.5% metastatic risk and requires sarcoma-level management 1, 4
• Cases where deep anatomic structures (major vessels, nerves, bone) are involved 1

When General Surgery or Orthopedics Might Be Involved

General surgery or orthopedic surgery may participate as part of a multidisciplinary team only in specific circumstances:

• If the tumor involves deep structures requiring resection of fascia, muscle, or bone that exceeds dermatologic surgical expertise 1
• For complex reconstructive procedures after tumor excision with negative margins is confirmed 1, 2
• Never for the primary tumor excision itself, as standard surgical techniques lack the margin control necessary for DFSP 1

Critical Surgical Principles

• Wide undermining must be avoided prior to confirmation of clear margins, as this displaces potentially positive margins and hampers interpretation of re-excisions 1
• Any reconstruction involving extensive undermining or tissue movement must be delayed until negative histologic margins are verified 1, 2
• If Mohs/PDEMA is unavailable and margins are uncertain, split-thickness skin grafting should be used to monitor for recurrence rather than complex flap reconstruction 1

Common Pitfalls to Avoid

Do not allow inadvertent excision by general surgery or orthopedics without proper margin assessment:

• If DFSP has already been excised inadequately (common scenario), a conservative re-excision with primary closure achieving negative margins is acceptable, but this still requires specialized surgical expertise 1
• 52.4% of patients in one series had positive margins when operated on without proper preoperative planning 3

Ensure adequate preoperative tissue diagnosis:

• Punch or incisional biopsy including the deeper subcutaneous layer is mandatory 1, 2
• Superficial biopsies frequently miss the diagnosis 1
• CD34 immunostaining should be positive (virtually all DFSP cases) and Factor XIIIa negative to confirm diagnosis 1, 5

Bottom Line Algorithm

1. Confirm diagnosis with deep biopsy (punch/incisional including subcutaneous layer) with CD34/Factor XIIIa immunostaining 1, 2
2. Refer to dermatologic surgery for Mohs micrographic surgery as first-line treatment 1, 2
3. Refer to specialized sarcoma center if tumor is large, recurrent, involves deep structures, or shows fibrosarcomatous transformation 1, 4
4. Involve general surgery or orthopedics only as adjunct for deep structure resection or delayed reconstruction after margin clearance 1
5. Never proceed with standard surgical excision by general surgery or orthopedics as primary treatment 1