Malignant Transformation in Burn Scar Marjolin Ulcer, a case report in Sanglah General Hospital Denpasar.
Case Report
Malignant Transformation in Burn Scars, Marjolin’s Ulcer: A Case Report in Sanglah
General Hospital Denpasar
By:
Anne Saputra, MD
Dr. dr. Nyoman Putu Riasa, Sp.BP-RE (K)
General Surgery
School of Medicine Udayana University
Sanglah General Hospital Denpasar
2015
Malignant Transformation in Burn Scars, Marjolin’s Ulcer: A Case Report in Sanglah
General Hospital Denpasar
Anne Saputra1, Nyoman Putu Riasa2
1
General Surgery Resident School of Medicine Udayana University, Sanglah General Hospital
Denpasar, Bali, Indonesia
2
Head of Department of Plastic Surgery and Reconstruction School of Medicine Udayana
University, Sanglah General Hospital Denpasar, Bali, Indonesia
Abstract
Introduction: Marjolin’s Ulcer (MU) is a rare malignancy but highly aggressive ulcerating that
is most often presented in an area of chronic burn wounds. This potentially fatal complication
typically occurs after a certain latency period. The true incidence MU is largely unknown, 1.2 to
2 percent of skin cancers were restricted to carcinomas arising from burn scars. Commonest
region of the body involved are the lower limb, over flexion creases and junctions of mobile
areas. The disease is also rare in Sanglah General Hospital Denpasar with the aim to improving
outcome in MU is early recognition and adequate treatment.
Case: a 54-year-old man presented an area of ulceration in the left popliteal fossa which was
burned in childhood. While contracture were severe, there was a lag period of almost 40 years
before persistent breakdown and ulceration occurred. The entire area of ulceration was excised
widely with pathologic studies showed squamous cell carcinoma, and the surrounding contracted
regions were released, creating a defect which required regional flap and split thickness skin
graft. Six months follow up patients remains well.
Conclusion: Chronic trauma with repeated re-epithelization in a poorly vascularized area is
probably a causative factor for malignant transformation in old burn scars. Excision and
gradually reconstruction can improve patient’s quality of life and activity of daily living.
Key words: Burn scar, Marjolin’s Ulcer, squamous cell carcinoma, radical surgery.
Introduction
Malignant neoplasm arising in chronic, non-healing wounds has been known since ages
about one hundred years ago and its named Marjolin’s Ulcer (MU). This scar malignancy arises
in burned, constantly injuried or chronically inflamed skin1,2. MU is a rare malignancy but highly
aggressive ulcerating squamous cell carcinoma (SCC) that is most often presented in an area of
chronic burn wounds. This potentially fatal complication typically occurs after a certain latency
period. The suspicion of malignancy is raised with crusting, bleeding, increase in pain or size of
the ulcer3. The true incidence MU is largely unknown, 1.2 to 2 percent of skin cancers were
restricted to carcinomas arising from burn scars4.
Case Report
A 54 year old man was admitted to Sanglah Hospital on October 2014 with non healing
ulcer increasingly widespread on contracted left popliteal since a month ago. The ulcer was very
small to begin with but has increased over a period of time to attain the present size. History of
burned on the left leg at between 8-10 years of age when his clothes caught fire. While
contractures were severe, there was a lag period of almost 40 years before persistent breakdown
and ulceration occurred. There was no history of diabetes, hypertension, or tuberculosis. Local
examination revealed an oval, ulcerative growth measuring 5 x 3 cm with elevated, irregular
margin and necrotic floor. The inguinal lymph node impalpable.
His cardiovascular, respiratory, gastrointestinal and central nervous system were normal.
Laboratory finding TLC of 13.780 /cu mm with neutrophilic leucocytosis. Liver function, kidney
function tests were normal.
Gross examination: The specimen of involved areas with elevated, indurated margins and
necrotic floor of the popliteal region were excised widely with 3 cm of free margins to the
border/base of the ulcer was performed and also released the popliteal contracture. The denuded
areas were resurfaced immediately reconstruction with split-thickness skin grafts and regional
flap on the wound after excision. Left inguinal lymph nodes was carefully assessed by palpation,
without lymph node dissection in this case.
Histopatological resulted well differentiated squamous cell carcinoma. Contractures of
the medial aspect of the left leg were at a second operation. The patient remains well 8 months
later. The pathological result was negative.
A
C
B
D
E
Fig. (A) 54-year-old male MU patient burned. (B) Wide excision margin. (C) Regional flap
design. (D) Reconstruction with skin-thickness skin graft and flap. (E) Postoperatively follow up
Discussion
MU is defined as a tumor arising from a chronic wound, scar or chronic inflammation.
Jean Nicholas Marjolin originally described the malignant transformation of cutaneous scars in
1828. The mechanism of malignant change is supposed to be a sequence of repeated trauma in
scars. There is a consensus on the importance of chronic irritation. Repeated ulceration to the
scar and subsequent initiation of re-epithelialization provides a prolonged stimulus for cellular
proliferation and may increase the rate of spontaneous mutations.6
The patient was 54 years and developed cancer after a period of more than 40 years, due
to burn leading to chronic ulcer and presence of chronic inflammation of the wound. An
association between latency period and malignant transformation was first suggested by
Lawrence.7 After a certain period of existence of a chronic scar, early stages of MU usually
present with symptoms of burning and itching, followed by blisters and prurigo. During this
period, which we called the “pre-ulceration period”, the surface of the scars remains intact. The
duration of the pre-ulceration period, or “the age of the scar”, may be important for the
prognosis.7
A new ulcer forms whenever the integrity of skin is compromised by spontaneous
rupture, scratching, or lack of self-care. After ulceration, some patients will experience repeated
cycles of healing followed by rupturing of skin, which is called repeated ulceration period.7 At
this stage, ulcers protrude and deepen, accompanied with severe pain, purulent discharge, foul
odor, and bleeding. This patients presence as flat ulcer with indurated, elevated margins without
discharge and bleeding.
The unique difference between burn scars and scars of other wounds is that the scar is
spread out on the surface, whereas scars of incisions, punctures and lacerations extend into the
deeper tissues. In burns the amount of epithelial regeneration is much greater. Scar tissue
resulting from burns undergoes greater contraction and may continue for many weeks. As a
result the cicatrix pulls and puckers neighboring tissues while the scar itself grows thicker. The
amount of scar tissue contraction varies with the extent, depth and location of the burn. Emphasis
is placed on contractures and tension of scars because it is in such conditions that ulceration is
easily provoked.9
Malignant transformation in 75%-90% cases is SCC.3 The neoplasm such as basal cell
carcinoma, melanoma, osteosarcoma and fibrosarcoma have been reported.3 The microscopic
grade of the tumor varies from well differentiated to poorly differentiated. Since biopsy remains
the gold standard for the diagnosis, it should be applied for suspicious lesions that have not
healed in 3 months.
The macroscopic appearance in our case was flat ulcer with indurated, elevated margins
and microscopic examination revealed well differentiated SCC without involving joint space or
bone.
Amputation is considered where the lesion is large, deeply infiltrating, extending into
joint cavities and bone. Adjuvant radiation and chemotherapy may be given if the patient refuses
surgery or the lesion is unreachable.3
The patient was treated with wide local excision, with reconstruction with split-thickness
skin grafts and regional flap. Long term follow up is recommended in all cases of MU as there is
high risk of metastasis to the brain, liver, kidney and lungs, mainly with lesions of the lower
extremities. The patient is follow up regularly and there is no evidence of metastasis.
The survival rates are reported as 52% and 34% respectively at 5 to 10 years.5 This report
suggest that the diagnosis of MU must be considered in a chronic ulcer of burn etiology with
increase in size and persisting for over a long period. Moreover, since the majority of MUs occur
in long duration unstable scars of ungrafted full-thickness burns, the joint regions, especially
flexion creases, are more commonly involved due to predisposition to activity-related repeated
ulceration.8
Conclusion
MU usually occurs in old burn sites that were not skin grafted and were left to heal
secondarily. Although it is believed that there is a long latency period of years after burn injury
before the occurrence of malignancy. There is possibility of recurrence after radical surgery
because of the aggressive behavior of this type of cancer, appropriate radical treatment allows an
adequate control of the disease. Patient should be followed-up for the rest of their life, as MU
more aggressive than initial skin carcinomas. Early grafting of the burn site can prevent the
formation malignant neoplasm. The condition should be suspected in a non-healing chronic ulcer
on a burn scar.
References
1. Kadir AR: Burn scar neoplasm. Ann Burns Fire Disasters 2007, 20:185-188.
2. Copcu E, Aktas A, Sisman N, Oztan Y: Thirty-one cases of Marjolin’s ulcer. Clin Exp
Derm 2003, 28:138-141
3. Agale S, Kulkarni D, Valand A. Marjolin’s Ulcer – A Diagnostic Dilemma: A study of
clinicopathological features and trend in western India. JAPI 2009; 57
4. Treves N, Pack GT: The development of cancer in burn scars. Surg Gynecol Obstet 1930,
51:749-782.
5. Agullo FJ. Image the month – Diagnosis: Marjolin’s ulcer. 2006;141:1-3
6. Yu Nanze, Long X, Hernandez J et al. Marjolin’s ulcer: a preventable malignancy arising
from scars. World Journal of Surgical Oncology 2013;11:313
7. Lawrence EA. Carcinoma arising in the scars of thermal burns. Surg Gynecol Obstet
1952;95:579-588
8. Kerr-Valentic MA, Samimi K, Rohlen BH et al. Marjolin’s ulcer: modern analysis of an
ancient problem. Plast Reconst Surg 2009;123:184-191
9. Giblin T, Pickrell K, Pitts W et al. Malignant degeneration in burn scars: Marjolin’s
Ulcer. Annals of Surgery August 1965;291-297
Contact:
Anne Saputra, MD
General Surgery Resident School of Medicine Udayana University, Sanglah General Hospital
Denpasar, Bali, Indonesia
+62 81290100099
anne.saputra@gmail.com
48th Ratna St. Denpasar, Bali, Indonesia
Supervisor:
Dr. dr. Nyoman Putu Riasa, Sp.BP-RE (K)
Head Department of Plastic Surgery School of Medicine Udayana University, Sanglah General
Hospital Denpasar, Bali, Indonesia
Malignant Transformation in Burn Scars, Marjolin’s Ulcer: A Case Report in Sanglah
General Hospital Denpasar
By:
Anne Saputra, MD
Dr. dr. Nyoman Putu Riasa, Sp.BP-RE (K)
General Surgery
School of Medicine Udayana University
Sanglah General Hospital Denpasar
2015
Malignant Transformation in Burn Scars, Marjolin’s Ulcer: A Case Report in Sanglah
General Hospital Denpasar
Anne Saputra1, Nyoman Putu Riasa2
1
General Surgery Resident School of Medicine Udayana University, Sanglah General Hospital
Denpasar, Bali, Indonesia
2
Head of Department of Plastic Surgery and Reconstruction School of Medicine Udayana
University, Sanglah General Hospital Denpasar, Bali, Indonesia
Abstract
Introduction: Marjolin’s Ulcer (MU) is a rare malignancy but highly aggressive ulcerating that
is most often presented in an area of chronic burn wounds. This potentially fatal complication
typically occurs after a certain latency period. The true incidence MU is largely unknown, 1.2 to
2 percent of skin cancers were restricted to carcinomas arising from burn scars. Commonest
region of the body involved are the lower limb, over flexion creases and junctions of mobile
areas. The disease is also rare in Sanglah General Hospital Denpasar with the aim to improving
outcome in MU is early recognition and adequate treatment.
Case: a 54-year-old man presented an area of ulceration in the left popliteal fossa which was
burned in childhood. While contracture were severe, there was a lag period of almost 40 years
before persistent breakdown and ulceration occurred. The entire area of ulceration was excised
widely with pathologic studies showed squamous cell carcinoma, and the surrounding contracted
regions were released, creating a defect which required regional flap and split thickness skin
graft. Six months follow up patients remains well.
Conclusion: Chronic trauma with repeated re-epithelization in a poorly vascularized area is
probably a causative factor for malignant transformation in old burn scars. Excision and
gradually reconstruction can improve patient’s quality of life and activity of daily living.
Key words: Burn scar, Marjolin’s Ulcer, squamous cell carcinoma, radical surgery.
Introduction
Malignant neoplasm arising in chronic, non-healing wounds has been known since ages
about one hundred years ago and its named Marjolin’s Ulcer (MU). This scar malignancy arises
in burned, constantly injuried or chronically inflamed skin1,2. MU is a rare malignancy but highly
aggressive ulcerating squamous cell carcinoma (SCC) that is most often presented in an area of
chronic burn wounds. This potentially fatal complication typically occurs after a certain latency
period. The suspicion of malignancy is raised with crusting, bleeding, increase in pain or size of
the ulcer3. The true incidence MU is largely unknown, 1.2 to 2 percent of skin cancers were
restricted to carcinomas arising from burn scars4.
Case Report
A 54 year old man was admitted to Sanglah Hospital on October 2014 with non healing
ulcer increasingly widespread on contracted left popliteal since a month ago. The ulcer was very
small to begin with but has increased over a period of time to attain the present size. History of
burned on the left leg at between 8-10 years of age when his clothes caught fire. While
contractures were severe, there was a lag period of almost 40 years before persistent breakdown
and ulceration occurred. There was no history of diabetes, hypertension, or tuberculosis. Local
examination revealed an oval, ulcerative growth measuring 5 x 3 cm with elevated, irregular
margin and necrotic floor. The inguinal lymph node impalpable.
His cardiovascular, respiratory, gastrointestinal and central nervous system were normal.
Laboratory finding TLC of 13.780 /cu mm with neutrophilic leucocytosis. Liver function, kidney
function tests were normal.
Gross examination: The specimen of involved areas with elevated, indurated margins and
necrotic floor of the popliteal region were excised widely with 3 cm of free margins to the
border/base of the ulcer was performed and also released the popliteal contracture. The denuded
areas were resurfaced immediately reconstruction with split-thickness skin grafts and regional
flap on the wound after excision. Left inguinal lymph nodes was carefully assessed by palpation,
without lymph node dissection in this case.
Histopatological resulted well differentiated squamous cell carcinoma. Contractures of
the medial aspect of the left leg were at a second operation. The patient remains well 8 months
later. The pathological result was negative.
A
C
B
D
E
Fig. (A) 54-year-old male MU patient burned. (B) Wide excision margin. (C) Regional flap
design. (D) Reconstruction with skin-thickness skin graft and flap. (E) Postoperatively follow up
Discussion
MU is defined as a tumor arising from a chronic wound, scar or chronic inflammation.
Jean Nicholas Marjolin originally described the malignant transformation of cutaneous scars in
1828. The mechanism of malignant change is supposed to be a sequence of repeated trauma in
scars. There is a consensus on the importance of chronic irritation. Repeated ulceration to the
scar and subsequent initiation of re-epithelialization provides a prolonged stimulus for cellular
proliferation and may increase the rate of spontaneous mutations.6
The patient was 54 years and developed cancer after a period of more than 40 years, due
to burn leading to chronic ulcer and presence of chronic inflammation of the wound. An
association between latency period and malignant transformation was first suggested by
Lawrence.7 After a certain period of existence of a chronic scar, early stages of MU usually
present with symptoms of burning and itching, followed by blisters and prurigo. During this
period, which we called the “pre-ulceration period”, the surface of the scars remains intact. The
duration of the pre-ulceration period, or “the age of the scar”, may be important for the
prognosis.7
A new ulcer forms whenever the integrity of skin is compromised by spontaneous
rupture, scratching, or lack of self-care. After ulceration, some patients will experience repeated
cycles of healing followed by rupturing of skin, which is called repeated ulceration period.7 At
this stage, ulcers protrude and deepen, accompanied with severe pain, purulent discharge, foul
odor, and bleeding. This patients presence as flat ulcer with indurated, elevated margins without
discharge and bleeding.
The unique difference between burn scars and scars of other wounds is that the scar is
spread out on the surface, whereas scars of incisions, punctures and lacerations extend into the
deeper tissues. In burns the amount of epithelial regeneration is much greater. Scar tissue
resulting from burns undergoes greater contraction and may continue for many weeks. As a
result the cicatrix pulls and puckers neighboring tissues while the scar itself grows thicker. The
amount of scar tissue contraction varies with the extent, depth and location of the burn. Emphasis
is placed on contractures and tension of scars because it is in such conditions that ulceration is
easily provoked.9
Malignant transformation in 75%-90% cases is SCC.3 The neoplasm such as basal cell
carcinoma, melanoma, osteosarcoma and fibrosarcoma have been reported.3 The microscopic
grade of the tumor varies from well differentiated to poorly differentiated. Since biopsy remains
the gold standard for the diagnosis, it should be applied for suspicious lesions that have not
healed in 3 months.
The macroscopic appearance in our case was flat ulcer with indurated, elevated margins
and microscopic examination revealed well differentiated SCC without involving joint space or
bone.
Amputation is considered where the lesion is large, deeply infiltrating, extending into
joint cavities and bone. Adjuvant radiation and chemotherapy may be given if the patient refuses
surgery or the lesion is unreachable.3
The patient was treated with wide local excision, with reconstruction with split-thickness
skin grafts and regional flap. Long term follow up is recommended in all cases of MU as there is
high risk of metastasis to the brain, liver, kidney and lungs, mainly with lesions of the lower
extremities. The patient is follow up regularly and there is no evidence of metastasis.
The survival rates are reported as 52% and 34% respectively at 5 to 10 years.5 This report
suggest that the diagnosis of MU must be considered in a chronic ulcer of burn etiology with
increase in size and persisting for over a long period. Moreover, since the majority of MUs occur
in long duration unstable scars of ungrafted full-thickness burns, the joint regions, especially
flexion creases, are more commonly involved due to predisposition to activity-related repeated
ulceration.8
Conclusion
MU usually occurs in old burn sites that were not skin grafted and were left to heal
secondarily. Although it is believed that there is a long latency period of years after burn injury
before the occurrence of malignancy. There is possibility of recurrence after radical surgery
because of the aggressive behavior of this type of cancer, appropriate radical treatment allows an
adequate control of the disease. Patient should be followed-up for the rest of their life, as MU
more aggressive than initial skin carcinomas. Early grafting of the burn site can prevent the
formation malignant neoplasm. The condition should be suspected in a non-healing chronic ulcer
on a burn scar.
References
1. Kadir AR: Burn scar neoplasm. Ann Burns Fire Disasters 2007, 20:185-188.
2. Copcu E, Aktas A, Sisman N, Oztan Y: Thirty-one cases of Marjolin’s ulcer. Clin Exp
Derm 2003, 28:138-141
3. Agale S, Kulkarni D, Valand A. Marjolin’s Ulcer – A Diagnostic Dilemma: A study of
clinicopathological features and trend in western India. JAPI 2009; 57
4. Treves N, Pack GT: The development of cancer in burn scars. Surg Gynecol Obstet 1930,
51:749-782.
5. Agullo FJ. Image the month – Diagnosis: Marjolin’s ulcer. 2006;141:1-3
6. Yu Nanze, Long X, Hernandez J et al. Marjolin’s ulcer: a preventable malignancy arising
from scars. World Journal of Surgical Oncology 2013;11:313
7. Lawrence EA. Carcinoma arising in the scars of thermal burns. Surg Gynecol Obstet
1952;95:579-588
8. Kerr-Valentic MA, Samimi K, Rohlen BH et al. Marjolin’s ulcer: modern analysis of an
ancient problem. Plast Reconst Surg 2009;123:184-191
9. Giblin T, Pickrell K, Pitts W et al. Malignant degeneration in burn scars: Marjolin’s
Ulcer. Annals of Surgery August 1965;291-297
Contact:
Anne Saputra, MD
General Surgery Resident School of Medicine Udayana University, Sanglah General Hospital
Denpasar, Bali, Indonesia
+62 81290100099
anne.saputra@gmail.com
48th Ratna St. Denpasar, Bali, Indonesia
Supervisor:
Dr. dr. Nyoman Putu Riasa, Sp.BP-RE (K)
Head Department of Plastic Surgery School of Medicine Udayana University, Sanglah General
Hospital Denpasar, Bali, Indonesia