Marjolin’s ulcer
BMJ 2015; 351 doi: https://doi.org/10.1136/bmj.h3997 (Published 19 August 2015) Cite this as: BMJ 2015;351:h3997
- Robert Choa, plastic surgery specialty registrar1,
- Sukbhir Rayatt, consultant plastic and reconstructive surgeon1,
- Kamal Mahtani, general practitioner and National Institute for Health Research clinical lecturer2
- 1Department of Plastic Surgery Royal Stoke University Hospital, Stoke on Trent ST4 6QG, UK
- 2Nuffield Department of Primary Care Health Sciences, University of Oxford, Oxford, UK
- Correspondence to: R Choa rob_choa{at}hotmail.com
- Accepted 28 May 2015
The bottom line
Consider Marjolin’s ulcer in patients with a longstanding wound or scar that has undergone recent change and is refractory to basic wound care
If a Marjolin’s ulcer is suspected, refer for incisional biopsies of all suspicious areas, including the edge
Treatment is curative if the tumour is diagnosed early; these aggressive lesions can metastasise, resulting in high mortality
The tissue viability nurse refers a 72 year old woman with lower limb venous stasis and a six year history of a non-healing ulcer on her shin (figure⇓) to her general practitioner. The GP refers her to the plastic surgery team, where incisional biopsy of the ulcer confirms squamous cell carcinoma (SCC), consistent with a Marjolin’s ulcer. Further examination shows palpable lymph nodes in the right inguinal region.
Marjolin’s ulcer of the right anterolateral leg. The ulcer has an irregular border and base. It had bled intermittently and had increased in size despite compression bandaging
What is a Marjolin’s ulcer?
Marjolin’s ulcers are tumours that most commonly arise from areas of chronic inflammation or injury and develop over many years.1 They are most common in old burn scars, but they are also seen in traumatic wounds, venous stasis ulcers, pressure sores, osteomyelitic fistulas, and other chronic wounds.
The classic description is of an SCC,2 but other cancers, such as basal cell carcinoma and malignant melanoma, have also been reported as Marjolin’s ulcers.3
Marjolin’s ulcers are more aggressive than primary cutaneous cancers and have higher rates of local recurrence and metastasis.4
How common is it?
Marjolin’s ulcers are rare, so no accurate incidence figures are available. The relative risk for patients with a venous leg ulcer developing SCC, compared with the normal population developing a non-melanoma skin cancer, is quoted as 5.8.5 This is relevant given that the incidence of leg ulcers in the United Kingdom is estimated at 1% of the population.6 By contrast, a recent review of 140 chronic ulcers in burns found that 46 (32.85%) were malignant.7
Why is it missed?
Reasons for Marjolin’s ulcers being missed include complex distracting comorbidities,8 clinical inexperience,9 and non-representative histological sampling.10
Marjolin’s ulcers can have a long latency period from the time of the original injury until malignant transformation, with an average time of 29 years reported in a recent review.1
Chronic wounds such as pressure sores in patients with spinal injury may be insensate, so patients may not be aware of changes to the wound. Furthermore, Marjolin’s ulcers do not always exhibit the typical characteristics of a malignant ulcer, resulting in a delay in biopsy.1
Why does this matter?
Marjolin’s ulcers are generally considered to be high risk SCCs—five year survival rates of high risk SCCs with distant metastases are 25-40%.11 It is unclear whether this is due to late detection or aggressive tumour behaviour, but late presentation increases the chances of a patient being untreatable.1
How is it diagnosed?
Clinical features
An ulcerated cutaneous tumour and malignant transformation of a pre-existing ulcer, scar, or wound can present differently. Whereas SCCs often have the classic features of an everted edge, exophytic growth, and bleeding, Marjolin’s ulcers may have none of these features. Other features that may be present include an irregular base or margin, excess granulation tissue, and an increase in size despite appropriate treatment.
Ulcerated wounds heal from the edges, where there is rapid turnover of cells in an attempt to re-epithelialise, so malignant transformation may be more common at the wound edge. Malignant transformation may occur only at one edge, while the rest of the wound shows signs of healing, and this can lead to the diagnosis being missed.12
A high index of suspicion is needed when any chronic wound undergoes sudden or unexpected changes. These changes may be in the form of symptoms, such as a new onset of pain13; odour—for example, a foul smelling discharge; appearance—a new mass or nodule; or drainage, with increased volume, character, or appearance of exudate. However, a change in a wound is not a prerequisite for the diagnosis of Marjolin’s ulcer.
Given the potential for metastases, always examine the draining lymph node basins.
Investigations
The gold standard for diagnosis of these lesions is histological analysis. This is generally accurate, with false positive and false negative rates less than 2%.14 In general, consider an incisional or wedge biopsy of any wound that doesn’t heal within three months.15 Such biopsies—taken from any suspicious areas of the wound, including the edge and centre of the ulcer—are usually sufficient for diagnosis. Refer patients for biopsy whenever Marjolin’s ulcer is suspected because a negative result is preferable to an advanced tumour that has been overlooked.
Computed tomography and magnetic resonance imaging can help delineate the extent of bone destruction and periosteal reaction, but they are not essential for diagnosis.
How is it managed?
As with other high risk cutaneous cancers, treatment must be planned in the setting of a skin cancer multidisciplinary team. Wide local excision with skin grafting is the treatment of choice in most cases, but more complex reconstructions with free tissue transfer are occasionally needed if bone or vital structures are exposed. In advanced cases, particularly in developing countries, limb amputation is needed to control the disease.
Notes
Cite this as: BMJ 2015;351:h3997
Footnotes
This is one of a series of occasional articles highlighting conditions that may be more common than many doctors realise or may be missed at first presentation. The series advisers are Anthony Harnden, professor of primary care, Department of Primary Care Health Sciences, University of Oxford, and Richard Lehman, general practitioner, Banbury. To suggest a topic for this series, please email us at practice{at}bmj.com.
Contributors: All authors helped conceive, draft, and critically revise this article. They have all approved this version. RC is guarantor.
Competing interests: We have read and understood BMJ policy on declaration of interests and declare the following interests: none
Patient consent not required (patient anonymised, dead, or hypothetical).
No patients were involved in the making of this article.