Marjolin's Ulcer
Timour F. El-Husseini, MD
Marjolin's ulcer, which was first described more than 1.5 centuries ago, involves a
rare malignant transformation of chronic scar tissue or ulcer.1 Marjolin's ulcers
are rare. The delay between the occurrence of the scar and the malignant
transformation can be as long as 70 years.2
Case Study
A 59-year-old woman with a 36-year history of leprosy was referred to the clinic
by the community physician because of a spreading ulcer on the right foot that
failed to heal despite medical treatment (Slide 1, Slide 2, Slide 3, Slide 4). The
results of three successive skin smears for Mycobacterium leprae were negative.
Slide 1
Slide 2
Slide 3
Slide 4
Amputation was recommended because of the advanced neuropathic status.
However, the patient did not undergo amputation. She presented 5 months later
with progressive bleeding from the foot, as well as general malaise. Her
hemoglobin level was 6.9 g/dL. X-rays (Slide 5) showed further bone destruction
affecting most of the right forefoot.
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Slide 5
She consented to amputation (Slide 6), prior to which a skin biopsy showed welldifferentiated squamous cell carcinoma grade I (Slide 7), with infiltration to
subcutaneous tissue and bone. Results of a metastasis workup including computed
tomography of the lungs, ultrasound of the abdomen, radionuclide scan were
negative, and inguinal lymph nodes were not palpable.
Slide 6
Slide 7
Syme’s amputation was performed allowing a 5-cm safety margin. The
histological examination of the amputation stump showed that the resection
margin was free of local infiltration (Slide 8, Slide 9).
Slide 8
Slide 9
The amputation scar healed eventually but, at 8 months’ follow-up, the patient
had a small recurring ulcer; multiple small biopsies did not show malignant cells.
The ulcer was not healed but neither bled nor increased in size 11 months after the
procedure (Slide 10). The patient died 2 years later due to an unrelated cause.
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Slide 10
Discussion
In 1828, Jean Nicholas Marjolin described the formation of ulcers in chronic burn
scars. Although he did not specifically describe cancer or squamous cell
carcinoma,1 the term Marjolin’s ulcer is used to describe a carcinoma appearing in
established chronic scar tissue.3 The most common clinical examples are
decubitus ulcers and burn scars, but the definition covers conditions such as
chronic fistulae, sinuses from osteomyelitis, and chronic ulcerating scar tissue of
non-united fracture.4-7
A difference may exist between a true Marjolin’s ulcer and malignant
transformation arising in a sinus of chronic osteomyelitis (Table).5
Table. Differences Between Carcinomas Derived From Ulcers and Sinuses
Category
Marjolin's Ulcers
Location
Size
Nodes
Prognosis
Axial
Large
Yes
Poor
Osteomyelitis
Sinus
Appendicular
Small
No
Good
Adapted from: Esther RJ, Lamps L, Schwartz HS. Marjolin
ulcers: secondary carcinomas in chronic wounds. J South
Orthop Assoc. 1999; 8.3:181-187.
Squamous cell carcinoma developing on chronic skin lesions has a higher
incidence of metastasis (9% to 36%) as compared to carcinoma arising in
previously normal skin (1% to 10%). Squamous cell carcinoma that develops on
top of sinuses and ulcers seems to be more aggressive than the Marjolin’s ulcer of
scars or burns.8,9
3
Lifeso and Bull7 used a three-grade histopathological classification: grade I (well
differentiated), grade II (moderately differentiated), and grade III (poorly
differentiated). They found that the grade is the most important prognostic factor.
Patients with leprosy are at high risk for developing squamous cell carcinoma.
The incidence of squamous cell carcinoma in this group is 0.79:1000 per year,
and the risk for fatal metastatic spread is 5%.13 Higher number of cases of
squamous cell carcinoma secondary to leprosy ulcers are found in Asia and
Africa.10-12
Wide local excision with a margin of at least 1 cm of healthy tissue should be
performed in cases of Marjolin’s ulcer. Amputation is indicated when wide local
excision is not possible due to deep invasion, bone or joint involvement, infection,
or hemorrhage, or when excision would cause major functional disability. From
an oncologic standpoint, amputation is not superior to wide local excision.14
Regional lymph node dissection is indicated when nodes are palpable. Lymph
node dissection in the absence of palpable nodes, however, is controversial.15
Long-term follow-up is recommended in all cases of Marjolin’s ulcer. Ames and
Hickey16 reported that 98% of all recurrences were seen within 3 years of
excision. Most series indicate that the incidence of recurrence ranges from 20% to
50%. Most recurrences are regional, but metastases to the brain, liver, lung,
kidney, and distant lymph nodes have been reported.17 Novick reported a 54%
incidence of metastases from lower extremity lesions. This rate was more than
twice the metastatic rate from any other primary site. In the same series, the
overall 3-year survival rate was 66%.18 Barr and Menard14 reported a 5-year
survival rate of 60% for wide excision and 69% for amputation. If regional lymph
nodes are involved, the 3-year survival rate decreases to 35%.16
Conclusion
The presence of chronic ulcers and sinuses in orthopedic surgery may present the
orthopedic surgeon with a rare case of Marjolin's ulcer. Awareness about this
condition and a high index of suspicion are essential when an ulcer drains
excessively, bleeds, increases in size, or becomes more painful. Prompt
management of this slow-progressing, but ultimately fatal, malignancy is
imperative.
References
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