Marjolin's ulcer
Burn-scar carcinoma; ulcer carcinoma; scar carcinoma
Marjolin's ulcer is a malignancy — most commonly cutaneous squamous cell carcinoma — arising in long-standing scars, chronic ulcers, sinus tracts of osteomyelitis, hidradenitis suppurativa, lupus vulgaris or pressure sores. The classical latency is 20–40 years between initial injury and malignant transformation. It carries a worse prognosis than conventional cSCC, with regional lymph-node metastatic rates of 20–35% and elevated mortality, partly due to delayed diagnosis. The cornerstone of management is wide local excision (or amputation when limb-threatening) plus regional lymph-node assessment.
Pathogenesis
- Chronic inflammation, repeated cycles of ulceration and re-epithelialisation, impaired lymphatic drainage and altered immunity drive carcinogenesis.
- Burn scars (particularly contractures across joints) are the most frequent substrate — accounts for the term.
- Other substrates: venous ulcers, pressure sores, hidradenitis suppurativa, sinus tracts of chronic osteomyelitis, lupus vulgaris, lichen sclerosus, pilonidal sinus, vaccination scars.
- Acute Marjolin (transformation <1 year after injury) — has been described, behaves more like ordinary cSCC.
- Chronic Marjolin (typical, 20–40 years' latency) — more aggressive.
Risk factors
- Burn scars not covered by skin grafts (left to heal by secondary intention) carry the highest risk.
- Lower limb predominance (~40%) followed by trunk, head/neck, upper limb.
- Male:female ~3:1 (reflects burn epidemiology).
- Latency typically 20–40 years; range <1 year (acute) to >60 years.
Clinical features
- New non-healing ulcer or nodule arising within a previously stable scar or chronic wound.
- Pain, malodour, friable bleeding, exophytic growth.
- Sentinel symptom: any change in a chronic wound — increased size, induration, ulceration depth, or odour — should prompt biopsy.
- Regional lymphadenopathy on examination is common at presentation.
Histology
- Most often well- or moderately-differentiated squamous cell carcinoma.
- Less common: BCC, melanoma, sarcoma, sebaceous carcinoma — full histology mandatory.
- Multiple deep biopsies through ulcer base and edges are essential — superficial biopsy may show only chronic inflammation or pseudoepitheliomatous hyperplasia.
Management
- Wide local excision with at least 1–2 cm margins and full-thickness reconstruction (skin graft, local or free flap).
- Limb-threatening or extensive disease may require amputation.
- Imaging staging — CT chest/abdomen/pelvis ± MRI of involved region; PET-CT in selected cases.
- Sentinel lymph node biopsy or selective neck/groin dissection — increasingly recommended given the high rate of occult nodal disease.
- Adjuvant radiotherapy — for incomplete margins, perineural invasion, multiple positive nodes or ENE.
- Cemiplimab (TA802) for advanced disease unsuitable for surgery / RT.
- Long-term surveillance — high recurrence and second-primary risk.
Prognosis
5-year overall survival 30–60% — substantially worse than conventional cSCC. Adverse prognostic factors: lower-limb tumours, >5 cm size, perineural invasion, nodal metastasis, post-burn substrate, late presentation. Prevention by early skin grafting of burns to avoid unstable scars, plus surveillance of long-standing wounds, is the most effective intervention.
References
- Pekarek B et al. A comprehensive review on Marjolin's ulcers. Eplasty; 2011.
- Copcu E. Marjolin's ulcer: a preventable complication of burns? Plast Reconstr Surg; 2009.
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