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Derm CasesDermatology / Soft-tissue sarcoma

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.

8 minosce2 min readVerification in progress

Target exams

NEET-PGINICETUSMLEPLABMRCP
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Target exams

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

  1. Recognise classic DFSP morphology and demographics.
  2. Plan biopsy and key IHC (CD34 vs Factor XIIIa).
  3. Explain COL1A1–PDGFB fusion / PDGFR pathway.
  4. Prefer Mohs or wide excision with generous margins; discuss recurrence rates conceptually.
  5. Identify fibrosarcomatous transformation as metastasis risk escalator.
  6. Indicate imatinib for unresectable/recurrent/metastatic disease.
[7]

Examiner checklist (mark each domain / 10)

DomainKey actions expected
RecognitionSlow-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 / IHCStoriform 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]
SurgeryPreferred 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 conceptClassic DFSP is locally aggressive with low metastatic risk; incomplete excision drives local recurrence; head/neck and large/deep tumours higher risk
Fibrosarcomatous DFSPHerringbone 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 therapyImatinib (PDGFR-targeted TKI) for unresectable, recurrent, metastatic, or neoadjuvant downstaging selected cases; multidisciplinary sarcoma team
CommunicationLong-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.
[1] [3] [6]
References7ShowHide
  1. [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. [2]Alchorne MMA, Conceição KDC, Barraza LL, et al. Dermatology in black skin. Anais Brasileiros de Dermatologia, 2024.PMID 38310012
  3. [3]Allen A, Ahn C, Sangüeza OP. Dermatofibrosarcoma Protuberans. Dermatologic Clinics, 2019.PMID 31466588
  4. [4]Acosta AE, Vélez CS. Dermatofibrosarcoma Protuberans. Current treatment options in oncology, 2017.PMID 28795284
  5. [5]Tillman BN, Liu JC. Cutaneous Sarcomas. Otolaryngologic clinics of North America, 2021.PMID 33602520
  6. [6]Jozwik M, Bednarczuk K, Osierda Z Dermatofibrosarcoma Protuberans: An Updated Review of the Literature. Cancers (Basel), 2024.PMID 39335097
  7. [7]Das S, et al. Beyond COL1A1::PDGFB: Rare fusions and their clinical implications in dermatofibrosarcoma protuberans World J Clin Cases, 2025.PMID 41356086
PreviousOSCE — hypopigmented truncal scale: pityriasis versicolor diagnosis and antifungal therapyDermatology / Mycology / General PracticeNextOSCE — infantile haemangioma: natural history, propranolol, and PHACES screeningDermatology / Paediatrics / Vascular tumours