Derm Cases · Dermatology / Soft-tissue sarcoma
OSCE — indurated trunk plaque: DFSP diagnosis, Mohs preference and imatinib pathway
An 8-minute OSCE station on dermatofibrosarcoma protuberans clinical recognition, CD34/COL1A1-PDGFB biology, Mohs vs wide excision, fibrosarcomatous transformation risk, and imatinib for unresectable disease.
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NEET-PGINICETUSMLEPLABMRCP
Prompt
An 8-minute OSCE station on dermatofibrosarcoma protuberans clinical recognition, CD34/COL1A1-PDGFB biology, Mohs vs wide excision, fibrosarcomatous transformation risk, and imatinib for unresectable disease.
Brief (to candidate)
A 42-year-old man has a slowly enlarging, firm blue-red-brown indurated plaque on the shoulder/trunk for 3 years, now becoming multinodular and “protuberant.” A prior clinic called it a scar/keloid. You have 8 minutes to suspect DFSP, plan biopsy/IHC, choose surgery (Mohs vs wide margins), and outline imatinib use and fibrosarcomatous risk.
Candidate instructions
- Recognise classic DFSP morphology and demographics.
- Plan biopsy and key IHC (CD34 vs Factor XIIIa).
- Explain COL1A1–PDGFB fusion / PDGFR pathway.
- Prefer Mohs or wide excision with generous margins; discuss recurrence rates conceptually.
- Identify fibrosarcomatous transformation as metastasis risk escalator.
- Indicate imatinib for unresectable/recurrent/metastatic disease.
Examiner checklist (mark each domain / 10)
| Domain | Key actions expected |
|---|---|
| Recognition | Slow-growing indurated blue-red-brown plaque/nodule on trunk of young–middle-aged adult; may progress to multinodular protuberant mass; often misdiagnosed as scar/keloid/DF for years[3][6] |
| Histology / IHC | Storiform spindle cells infiltrating fat in honeycomb pattern; diffuse CD34+, Factor XIIIa− (vs DF opposite pattern); molecular COL1A1–PDGFB from t(17;22)/ring chromosome supports diagnosis and imatinib rationale[1][3][4] |
| Surgery | Preferred margin-controlled surgery: Mohs micrographic surgery (lowest local recurrence in many series) or wide local excision with ~2–3 cm clinical margins when Mohs unavailable; incomplete margins → re-excise[1][4][6] |
| Recurrence concept | Classic DFSP is locally aggressive with low metastatic risk; incomplete excision drives local recurrence; head/neck and large/deep tumours higher risk |
| Fibrosarcomatous DFSP | Herringbone high-grade areas, increased mitoses, CD34 loss in transformed foci → metastatic risk rises substantially (order of ~15–30% cited in reviews) — needs aggressive local control ± RT and staging/MDT sarcoma care[1][6] |
| Systemic therapy | Imatinib (PDGFR-targeted TKI) for unresectable, recurrent, metastatic, or neoadjuvant downstaging selected cases; multidisciplinary sarcoma team |
| Communication | Long-term surveillance for local recurrence; do not dismiss “recurrent scar” on trunk |
Model key actions
- Suspect DFSP in chronic indurated trunk plaque mislabelled keloid/scar; biopsy with CD34.[3][6]
- Plan Mohs (or wide margins) as definitive local therapy.[1]
- Use imatinib when unresectable/metastatic; escalate if fibrosarcomatous change.[4][1]
Common errors
- Treating DFSP as keloid with steroids alone.
- Narrow excision like a dermatofibroma.
- Missing CD34/Factor XIIIa distinction from DF.
- Ignoring fibrosarcomatous histology as a game-changer for metastasis risk.
- Failing to re-excise positive margins.
References7ShowHide
- [1]Saiag P, Lebbe C, Brochez L, et al. Diagnosis and treatment of dermatofibrosarcoma protuberans. European interdisciplinary guideline - update 2024. European journal of cancer (Oxford, England : 1990), 2025.PMID 39904126
- [2]Alchorne MMA, Conceição KDC, Barraza LL, et al. Dermatology in black skin. Anais Brasileiros de Dermatologia, 2024.PMID 38310012
- [3]Allen A, Ahn C, Sangüeza OP. Dermatofibrosarcoma Protuberans. Dermatologic Clinics, 2019.PMID 31466588
- [4]Acosta AE, Vélez CS. Dermatofibrosarcoma Protuberans. Current treatment options in oncology, 2017.PMID 28795284
- [5]Tillman BN, Liu JC. Cutaneous Sarcomas. Otolaryngologic clinics of North America, 2021.PMID 33602520
- [6]Jozwik M, Bednarczuk K, Osierda Z Dermatofibrosarcoma Protuberans: An Updated Review of the Literature. Cancers (Basel), 2024.PMID 39335097
- [7]Das S, et al. Beyond COL1A1::PDGFB: Rare fusions and their clinical implications in dermatofibrosarcoma protuberans World J Clin Cases, 2025.PMID 41356086