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A case report of extensive bilateral Marjolin's ulcers
A CASE R E P O R T
OF EXTENSIVE
BILATERAL
MARJOLIN'S ULCERS
By J. GHOSH, M.B., B.S.
Senior House Officer, Liverpool Regional Burns and Plastic Unit Whiston Hospital, Prescot, Lancs. MOULY (196o) noted that o f the cases of malignant ulcerations arising in scars which were described b y Marjolin in I828, none in fact was the result of a burn. Sevitt (I957) has pointed out that it was Hawkins who in 1825 described the first two cases of carcinoma in old burns scars. Despite these now well-recognised facts, this condition has retained its traditional name. Case R e p o r t . - - M r P. C., aged 60 years, sustained severe molten copper burns of the backs of both legs and popliteal fossm in 1937. He had always refused surgical treatment and carried on with dressings only, living alone and taking an extremely poor diet. Consequently the burns never healed, although he continued working as a furnace man until 1954, despite marked contracture of both knee joints. In November 1963 bleeding from his ulcers eventually forced him to accept admission to hospital. Examination Findings.--On admission he was malnourished and anaemic with a haemoglobin of 37 per cent. (5"5 g. per cent.). On the posterior aspect of both legs there were massive discharging ulcerations extending from above the knees to the ankles (Fig. I). In part the surfaces were granulating: in other areas there were irregular shallow ulcer craters with raised, rolled edges. The ulcers were indurated and adherent to the deeper structures. The area involved on the left leg was larger than on the right and had a large cutaneous horn on its infero-medial aspect, approximately IO by 4 by 3 cm. in size. There was a rather flatter and smaller horn on the right leg in a similar position. Both knees had fixed flexion deformities (left equals 15o degrees ; right equals 145 degrees). There were two fleshy but not tender lymph nodes palpable in the right groin, each I "5 cm. in diameter. No lymph node was felt in the left groin. The liver and spleen were not enlarged. Biopsy.--Biopsies were taken from several areas of both legs (Fig. 2). In addition there was a rectal biopsy to eliminate amyloidosis. Miscroscopic findings were reported as follows : I. Scars of Leg--Early Squamous-cell Carcinoma (Figs. 3, 4) . - T h e appearances are essentially similar in all the specimens. The squamous epithelium is thickened and keratinised and frequently forms warty papillm, which project on its surface. The rete pegs show budding at their tips. There is no deep invasion, but the amount of mitotic activity and the frequent formation of epithelial pearls in the down growths suggest true carcinoma rather than pseudoepitheliomatous hyperplasia. Wide excision appears warranted. 2. Rectal Biopsy.--There is no evidence of amyloidosis in appropriately stained sections. Treatment.--Anaemia and malnutrition were corrected first and the patient agreed to surgery. The right leg was treated first, and the left two weeks later. The ulcers were excised widely and deeply through normal tissue. The deep fascia was very thickened, contracted and hard. On the right side the medial head of the gastrocnemius, the tendon of semitendinosus and the sural nerves were sacrificed, and the medial and lateral popliteal nerves left exposed. Full extension of the knee joint was restored following this excision. On the left side no division of muscles was necessary, but in all other respects the operation was similar. All the excised areas were covered with sheets of medium thickness skin grafts, obtained from the corresponding thighs and the legs were immobilised in full extension in plaster of Paris cylinders. i (5
97
98
BRITISH JOURNAL OF PLASTIC SURGERq
FIG. I Pre-operative photograph (for clarification see line drawing, Figure 2).
0
BIOPSY SITES FIG. 2
Line drawing of pre-operative condition. The biopsy sites are indicated by circles.
FIG. 3 FIG. 4 Fig. 3.--Photomicrograph of biopsy. Low-power view--frequent mitoses and epithelial pearls seen. Fi~. 4.--Photomicrograph of biopsy. High-power view. Mitoses more closely seen,
EXTENSIVE BILATERAL MARJOLIN'S ULCERS
99
Histological Reports.--Both specimens show early squamous-ceU carcinoma, as in the original biopsies. There is no evidence of deep invasion and excision appears complete. Subsequent P r o g r e s s . - - N i n e months post-operatively his general condition had improved greatly and he was walking very well, though using one stick as he felt safer that way. The grafts on both legs were stable and there was no area of breakdown (Fig. 5). Right inguinal lymph nodes were still palpable and freely mobile but slightly firmer than previously. There was now a similar enlargement of a node in the left groin. Biopsy was refused.
FIG. 5 Post-operative photograph, showing well-healed surface. DISCUSSION
The case described here shows many typical features of Marjolin's ulcer, namely a long period of chronic ulceration prior to malignant change, the situation of the ulcer over a joint flexure, and the relatively superficial nature of the lesions which permits of a radical excision. The precise incidence of malignant change in burns scars is not known and reports are variable. Mason (1929) reported that 7"5 per cent. of 255 cases of carcinoma of the upper extremity developed in burns scars. He found that 9"4 per cent. of ninety-eight cases of carcinoma of the hand following irritation and injury (excluding post-radiation cases), originated in burned skin. On the other hand in large-scale investigations Levi (i956) found 1.3 per cent, to have arisen in burn sears and Treves and Pack (i93o)
IO0
BRITISH JOURNAL OF PLASTIC SURGERY
in a similar investigation reported an incidence of 2 per cent. Indeed Cruickshank et al. (I963) regard malignant change as unusual in any form of scar. This case also displays a number of unusual features. In the first place there were very extensive malignant changes proved by biopsy. Secondly the condition was bilateral and thirdly there were cutaneous horns on both legs. The association of cutaneous horns with a squamous-cell carcinoma in a burn scar was reported by Cohney ( I 9 6 2 ) and regarded by him as rare. Menkin (I96O) claims to have demonstrated a growth-promoting factor in inflammatory exudates which he suggests is capable of acting as a co-carcinogen, in a genetically susceptible organism. He postulates that such neoplastic transformation may be referable to a disequilibration of the cells brought about by a variety of means including chronic inflammation and irritation. Such an explanation might well apply to this present case in which the tissues had been irritated by trauma and chronic infection for a considerable period of time. Moreover, the presence of malignant change, not only in both lesions but in many areas in both legs, suggests a wide field of epithelial instability and accords well with Menkin's concept of zetiology. The prognosis of the condition varies in the series of cases reported. Treves and Pack (I93 o) found a high mortality, but Mouly (I96O) suggested a much better outlook. Certainly neoplastic change tends to be superficial and lymphatic spread is late in its occurrence. The early diagnosis and in particular an appreciation that excision must be very wide to ensure removal of the whole area of unstable epithelium, should afford reasonable prospects of cure. Above all it must be emphasised that Marjolin's ulcer is a preventable condition. If modern techniques are used to obtain early full skin cover, and if there is a careful follow-up to ensure that the affected areas remain healed, then Marjolin's ulcer should become merely an historical curiosity. This case was admitted under the care of Mr A. H. M. Littlewood, to whom I am grateful for permission to publish and for his interest and stimulus in the preparation of this paper. I am especially grateful to Dr A. S. Woodcock for the pathological reports, the photomicrographs and his very considerable help. I would also like to express my thanks to Mr M. Bavliss for the other photographs. REFERENCES COHNEY, B. C. (x962). Brit. J. plast. Surg., i5, 216. CRUICKSHANK, A. H., McCoNNELL, E. M., and MILLER, D. G. (1963). J. clin. Path., x6, 573LEVI, J. M. (1956). Quoted by SEVITT, S. (1957). MASON, M. L. (1929). Arch. Surg., I8, 2oi7. MENI(IN, V. (196o). Brit. reed. J., i, I585. MOULY, R. (196o). Trans. int. Soc. plast. Surg., 2nd Congress, 1959, p. i3 O. Edinburgh & London : E. & S. Livingstone. SEVlTT, S. (1957). " Burns: Pathology and Therapeutic Applications." London: Butterworth. TREVES, N., and PACK, G. T. (193o). Surg. Gynec. Obstet., 5I, 749-
Submitted for publication~ October i964.
OF EXTENSIVE
BILATERAL
MARJOLIN'S ULCERS
By J. GHOSH, M.B., B.S.
Senior House Officer, Liverpool Regional Burns and Plastic Unit Whiston Hospital, Prescot, Lancs. MOULY (196o) noted that o f the cases of malignant ulcerations arising in scars which were described b y Marjolin in I828, none in fact was the result of a burn. Sevitt (I957) has pointed out that it was Hawkins who in 1825 described the first two cases of carcinoma in old burns scars. Despite these now well-recognised facts, this condition has retained its traditional name. Case R e p o r t . - - M r P. C., aged 60 years, sustained severe molten copper burns of the backs of both legs and popliteal fossm in 1937. He had always refused surgical treatment and carried on with dressings only, living alone and taking an extremely poor diet. Consequently the burns never healed, although he continued working as a furnace man until 1954, despite marked contracture of both knee joints. In November 1963 bleeding from his ulcers eventually forced him to accept admission to hospital. Examination Findings.--On admission he was malnourished and anaemic with a haemoglobin of 37 per cent. (5"5 g. per cent.). On the posterior aspect of both legs there were massive discharging ulcerations extending from above the knees to the ankles (Fig. I). In part the surfaces were granulating: in other areas there were irregular shallow ulcer craters with raised, rolled edges. The ulcers were indurated and adherent to the deeper structures. The area involved on the left leg was larger than on the right and had a large cutaneous horn on its infero-medial aspect, approximately IO by 4 by 3 cm. in size. There was a rather flatter and smaller horn on the right leg in a similar position. Both knees had fixed flexion deformities (left equals 15o degrees ; right equals 145 degrees). There were two fleshy but not tender lymph nodes palpable in the right groin, each I "5 cm. in diameter. No lymph node was felt in the left groin. The liver and spleen were not enlarged. Biopsy.--Biopsies were taken from several areas of both legs (Fig. 2). In addition there was a rectal biopsy to eliminate amyloidosis. Miscroscopic findings were reported as follows : I. Scars of Leg--Early Squamous-cell Carcinoma (Figs. 3, 4) . - T h e appearances are essentially similar in all the specimens. The squamous epithelium is thickened and keratinised and frequently forms warty papillm, which project on its surface. The rete pegs show budding at their tips. There is no deep invasion, but the amount of mitotic activity and the frequent formation of epithelial pearls in the down growths suggest true carcinoma rather than pseudoepitheliomatous hyperplasia. Wide excision appears warranted. 2. Rectal Biopsy.--There is no evidence of amyloidosis in appropriately stained sections. Treatment.--Anaemia and malnutrition were corrected first and the patient agreed to surgery. The right leg was treated first, and the left two weeks later. The ulcers were excised widely and deeply through normal tissue. The deep fascia was very thickened, contracted and hard. On the right side the medial head of the gastrocnemius, the tendon of semitendinosus and the sural nerves were sacrificed, and the medial and lateral popliteal nerves left exposed. Full extension of the knee joint was restored following this excision. On the left side no division of muscles was necessary, but in all other respects the operation was similar. All the excised areas were covered with sheets of medium thickness skin grafts, obtained from the corresponding thighs and the legs were immobilised in full extension in plaster of Paris cylinders. i (5
97
98
BRITISH JOURNAL OF PLASTIC SURGERq
FIG. I Pre-operative photograph (for clarification see line drawing, Figure 2).
0
BIOPSY SITES FIG. 2
Line drawing of pre-operative condition. The biopsy sites are indicated by circles.
FIG. 3 FIG. 4 Fig. 3.--Photomicrograph of biopsy. Low-power view--frequent mitoses and epithelial pearls seen. Fi~. 4.--Photomicrograph of biopsy. High-power view. Mitoses more closely seen,
EXTENSIVE BILATERAL MARJOLIN'S ULCERS
99
Histological Reports.--Both specimens show early squamous-ceU carcinoma, as in the original biopsies. There is no evidence of deep invasion and excision appears complete. Subsequent P r o g r e s s . - - N i n e months post-operatively his general condition had improved greatly and he was walking very well, though using one stick as he felt safer that way. The grafts on both legs were stable and there was no area of breakdown (Fig. 5). Right inguinal lymph nodes were still palpable and freely mobile but slightly firmer than previously. There was now a similar enlargement of a node in the left groin. Biopsy was refused.
FIG. 5 Post-operative photograph, showing well-healed surface. DISCUSSION
The case described here shows many typical features of Marjolin's ulcer, namely a long period of chronic ulceration prior to malignant change, the situation of the ulcer over a joint flexure, and the relatively superficial nature of the lesions which permits of a radical excision. The precise incidence of malignant change in burns scars is not known and reports are variable. Mason (1929) reported that 7"5 per cent. of 255 cases of carcinoma of the upper extremity developed in burns scars. He found that 9"4 per cent. of ninety-eight cases of carcinoma of the hand following irritation and injury (excluding post-radiation cases), originated in burned skin. On the other hand in large-scale investigations Levi (i956) found 1.3 per cent, to have arisen in burn sears and Treves and Pack (i93o)
IO0
BRITISH JOURNAL OF PLASTIC SURGERY
in a similar investigation reported an incidence of 2 per cent. Indeed Cruickshank et al. (I963) regard malignant change as unusual in any form of scar. This case also displays a number of unusual features. In the first place there were very extensive malignant changes proved by biopsy. Secondly the condition was bilateral and thirdly there were cutaneous horns on both legs. The association of cutaneous horns with a squamous-cell carcinoma in a burn scar was reported by Cohney ( I 9 6 2 ) and regarded by him as rare. Menkin (I96O) claims to have demonstrated a growth-promoting factor in inflammatory exudates which he suggests is capable of acting as a co-carcinogen, in a genetically susceptible organism. He postulates that such neoplastic transformation may be referable to a disequilibration of the cells brought about by a variety of means including chronic inflammation and irritation. Such an explanation might well apply to this present case in which the tissues had been irritated by trauma and chronic infection for a considerable period of time. Moreover, the presence of malignant change, not only in both lesions but in many areas in both legs, suggests a wide field of epithelial instability and accords well with Menkin's concept of zetiology. The prognosis of the condition varies in the series of cases reported. Treves and Pack (I93 o) found a high mortality, but Mouly (I96O) suggested a much better outlook. Certainly neoplastic change tends to be superficial and lymphatic spread is late in its occurrence. The early diagnosis and in particular an appreciation that excision must be very wide to ensure removal of the whole area of unstable epithelium, should afford reasonable prospects of cure. Above all it must be emphasised that Marjolin's ulcer is a preventable condition. If modern techniques are used to obtain early full skin cover, and if there is a careful follow-up to ensure that the affected areas remain healed, then Marjolin's ulcer should become merely an historical curiosity. This case was admitted under the care of Mr A. H. M. Littlewood, to whom I am grateful for permission to publish and for his interest and stimulus in the preparation of this paper. I am especially grateful to Dr A. S. Woodcock for the pathological reports, the photomicrographs and his very considerable help. I would also like to express my thanks to Mr M. Bavliss for the other photographs. REFERENCES COHNEY, B. C. (x962). Brit. J. plast. Surg., i5, 216. CRUICKSHANK, A. H., McCoNNELL, E. M., and MILLER, D. G. (1963). J. clin. Path., x6, 573LEVI, J. M. (1956). Quoted by SEVITT, S. (1957). MASON, M. L. (1929). Arch. Surg., I8, 2oi7. MENI(IN, V. (196o). Brit. reed. J., i, I585. MOULY, R. (196o). Trans. int. Soc. plast. Surg., 2nd Congress, 1959, p. i3 O. Edinburgh & London : E. & S. Livingstone. SEVlTT, S. (1957). " Burns: Pathology and Therapeutic Applications." London: Butterworth. TREVES, N., and PACK, G. T. (193o). Surg. Gynec. Obstet., 5I, 749-
Submitted for publication~ October i964.