- Email: [email protected]
Marjolin’s ulcer on the nose
Burns 28 (2002) 701–704
Marjolin’s ulcer on the nose Eray Copcu a,∗ , Nil Çulhaci b a
Plastic and Reconstructive Surgery Department, Medical Faculty, Adnan Menderes University, 09100 Aydin, Turkey b Pathology Department, Medical Faculty, Adnan Menderes University, 09100 Aydin, Turkey Accepted 9 May 2002
Abstract Malignancies in scars are generally known as Marjolin’s ulcer. The majority of these arise from burn injuries. Although Marjolin’s ulcer consists of all kinds of skin cancer, squamous cell carcinoma is the main cancer type reported in the literature. The pathogenesis of this tumor is due to chronic irritation of the effected area and mostly involves the extremities and scalp area. This report presents an unusually located and rare type of Marjolin’s ulcer: on the nose and baso-squamotic in type. A 54-year-old man, 33 years after burn, presented with an unhealed ulcer on his nose which had been present for 2 years. The case was managed by tumor excision and a naso-labial transposition flap. To our knowledge, this is the first report of Marjolin’s ulcer developing on post-burned skin of the nose. © 2002 Elsevier Science Ltd and ISBI. All rights reserved. Keywords: Marjolin’s ulcer; Nose; Skin; Tumor; Burn
1. Introduction Marjolin’s ulcer is a well-recognized malignancy that arises from previously traumatized, chronically inflamed or scarred skin. As cited by Fleming et al. [1], this tumor was first described by Marjolin in 1823 and was defined as “ulcer canchroides.”A full description was made by Dupuytren 2 years later [2]. “Marjolin’s ulcer” has now become a term that is used generally to describe any squamous cell carcinoma arising in a chronically inflamed skin. The majority of burn scar carcinomas occur after a lag period in burns that were not been grafted following injury [3,4]. The cancer can be seen on all parts of the body; the extremities, trunk and scalp are the major locations. There has not to date been any report in the literature of Marjolin’s ulcer on the nose.
2. Case report A 54-year-old man presented, suffering from an ulcerated and non-healed wound on his nose (Fig. 1). He had a burn scar on his face that extended from the midface to the chest. Interview with the patient revealed that he had sustained a flame burn to his face and chest 33 years ago in a traffic accident. He reported no active treatment following injury. ∗
Corresponding author. Fax: +90-256-212-01-46. E-mail address: [email protected] (E. Copcu).
The burned face and chest healed spontaneously, leaving a large scar. He had no operations in the subsequent 33 years. Two years ago, he noticed a small ulcer on his nose and scratched it unwittingly. The lesion remained small until approximately 6 months before his presentation to hospital. He had never experienced any pain nor sought medical help for the lesion before presentation. Examination revealed an ulcerative lesion measuring 23 mm × 15 mm on his nose, over the lateral crus of right lower lateral cartilage. Macroscopically, the lesion had a reddish halo with a central necrotic crater and irregular edges. Physical examination and ultrasonographic investigation showed no signs of lymphatic involvement. Additionally, no metastatic involvement was found on the investigation of abdomen, chest and brain. Routine laboratory test results were normal. The presumptive diagnosis was Marjolin’s ulcer. Incisional biopsy clarified the diagnosis and the pathology result was given as a baso-squamous carcinoma. The whole lesion was excised together with the affected left lower cartilage. The inner nasal lining was tumor-free. The defect was covered with a right naso-labial transposition flap (Fig. 2). On histo pathological examination tumor diameters were 13 mm × 10 mm the histology showed islands in the dermis. Nuclear pallisading was present at the edge of the clusters. Adjacent clusters exhibited transitions to squamous cells and contained keratin pearls. There were also focal areas of necrosis. The small basoloid cells had moderately pleomorphic, densely hyperchromatic nuclei with scant cytoplasm. Larger cells with
0305-4179/02/$22.00 © 2002 Elsevier Science Ltd and ISBI. All rights reserved. PII: S 0 3 0 5 - 4 1 7 9 ( 0 2 ) 0 0 0 9 3 - 1
702
E. Copcu, N. Çulhaci / Burns 28 (2002) 701–704
Fig. 1. Marjolin’s ulcer on the nose. Pre-operative view.
more cytoplasm, vesicular nuclei and small nucleoli were seen as well. All these findings indicated that the lesion was baso-squamous carcinoma (Figs. 3 and 4). The surgical margins of the specimen were found to be tumor-cell free.
Fig. 2. Post-operative view. Defect was closed with naso-labial transposition flap.
3. Discussion Marjolin’s ulcer is a rare tumor in which various etiological factors have been encountered. Some of these are irritation, poor lymphatic regeneration, antibodies, mutations and
Fig. 3. Microscopically view. Hematoxylen eosin 40× magnification.
E. Copcu, N. Çulhaci / Burns 28 (2002) 701–704
703
Fig. 4. Microscopically view. Hematoxylen eosin 100× magnification.
local toxins [12]. Discoid lupus erythematosus and acne conglobata may also undergo malignant change in time. Castillo and Goldsmith [11] showed that immune system deficiency may have a role on development of Marjolin’s ulcer. All of these circumstances result in an inflammatory state that is claimed to end up with a Marjolin’s ulcer. The most commonly met factor, however, is irritation of inflamed skin. This inflammation is often due to burn injuries but at the same time vaccination [5], snake bite [6], osteomyelitis [7], pilonoidal abscess [8], pressure sores [9], venous stasis [10] may be other sources of inflammation for this tumor. Although many different cell types can be seen in these lesions, the major histological type is squamous cell carcinoma. Basal cell carcinoma (BCC) [13], malignant melanoma [1], and mesenchymal malignancies [12] are the less predominant types of the Marjolin’s ulcer. As in our case, baso-squamous carcinoma of the skin is another rare type of Marjolin’s’ ulcer. Multiple factors act together in malignant transformation on burn scar tissue. Sun exposure is a major etiologic factor in the etiopathogenesis of the skin tumours [14]. Nose skin tumors are very common, most being BCC [15] because the nose is one of the parts of the body most often exposed to the sun. This anatomical structure is an unusual area for the Marjolin’s ulcer and our report is the first in the literature. It is usually located on the extremities [16–18], scalp [19,20], or neck [21]. There is always a lag period to appearance of the tumor. This period takes approximately 30 years as in our case [12,17,21], whereas short periods are relatively rare [12] with the shortest reported wound of 6 weeks [22]. Marjolin’s ulcers are generally accepted as very aggressive tumors [17] having higher regional metastasis and fatality rate [23,24]. Although it is a well defined skin tumor, the treatment of it is still controversial. Radical excisional
surgery is the most appropriate treatment, but there is no consensus on the indications for lymph node dissection (LND). If a clinically palpable lymph node is present, dissection it should be carried out [12]. Most of the studies did not support the performance of prophylactic node dissection [17,25], whereas one study claims that the presence of a clinically palpable lymph node is an indication for LND. In another study, Novick et al. [24] suggest prophylactic LND in cases of lower extremity tumors. Bad prognosis is the characteristic of Marjolin’s ulcer and death from Marjolin’s ulcer is not uncommon [26,27]. Finally, this tumor is an uncommon type of skin cancer in the developing countries as strongly emphasized in Paredes’ study [28]. In a patient with chronically irritated skin, good health care will help to prevent a bad outcome such as Marjolin’s ulcer.
References [1] Fleming MD, Hunt JL, Purdue GF, Sandstad J. Marjolin’s ulcer: a review and reevaluation of a difficult problem. J Burn Care Rehabil 1990;11:460–9. [2] Applebaum J, Burrows WM, Greenway HJ. Acute Marjolin’s ulcer. J Assoc Mil Dermatol 1985;11:57–61. [3] Horton CE. The malignant potential of burn scar. Plast Reconstr Surg 1958;22:348–51. [4] Mosborg DA, Crane RT, Tami T, Parjer GS. Scar carsinoma of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:1038– 40. [5] Steffen C. Marjolin’s ulcer. Report of two cases and evidence that Marjolin did not describe cancer arising in scars of burns. Am J Dermatopathol 1984;6:187–93. [6] Smith J, Mello LF, Nogueira Neto NC, Meohas W, Pinto LW, Campos VA, et al. Malignancy in chronic ulcers and scars of the leg (Marjolin’s ulcer): a study of 21 patients. Skeletal Radiol 2001;30:331–7.
704
E. Copcu, N. Çulhaci / Burns 28 (2002) 701–704
[7] Hensel KS, Ono CM, Doukas WC. Squamous cell carcinoma in chronic ulcerative lesions: a case report and literature review. Am J Orthop 1999;28:253–6. [8] Lerner HJ, Dietrick G. Squamous cell carcinoma of the pilonidal sinus: report of a case and review of the literature. J Surg Oncol 1979;11:117–83. [9] Berkwits L, Yarkony GM, Lewis V. Marjolin’s ulcer complicating a pressure ulcer: case report and literature review. Arch Phys Med Rehabil 1986;67:831–3. [10] Nkere UU, Banjar A. Malignant change in a chronic skin lesion induced by an intravenous cannula. Postgrad Med J 1997;73:45–7. [11] Castillo JL, Goldsmith HS. Burn scar malignancy in a possible depressed immune setting. Surg Forum 1968;19:511–3. [12] Celikoz B, Demiriz M, Selmanpakoglu N. A shorter lag period of mesenchymal malignancy on Marjolin’s ulcer. Burns 1997;23:72–4. [13] Ozyazgan I, Kontacs O. Basal cell carcinoma arising from surgical scars: a case and review of the literature. Dermatol Surg 1999;25: 965–8. [14] Casson PR, Robins P. Malignant tumors of the skin. In: McCarthy JG, editor. Plastic Surgery, vol. 4. Philadelphia: Saunders, 1990, 3614. [15] McCormack CJ, Kelly JW, Dorevitch AP. Differences in age and body site distribution of the histological subtypes of basal cell carcinoma. Arch Dermatol 1997;133:593–6. [16] Ames FC, Hickey RC. Squamous cell carcinoma of the skin of the extremities. Int Adv Surg Oncol 1980;3:179–99. [17] Barr LH, Menard JW. Marjolin’s ulcer: the LSU experience. Cancer 1983;52:173–5.
[18] Bean DJ, Rees RS, O’Leary JP, Lynch JB. Carcinoma of the hand: a 20-year experience. South Med J 1984;7(7):998–1000. [19] Clements B, Lewis H, Lewis H, McKinstrey S, Gray J, Byrnes D. A late, fatal complication of a high energy thermal injury to the scalp. Ann Plast Surg 1995;35:650–3. [20] Curry SS, Gaither DH, King Jr LE. Squamous cell carcinoma arising in dissecting perifolliculitis of the scalp: a case report and review of secondary squamous cell carcinomas. J Am Acad Dermatol 1981;4:673–8. [21] Simmons MA, Edwards JM, Nigam A. Marjolin’s ulcer presenting in the neck. J Laryngol Otol 2000;114:980–2. [22] Hammond JS, Thomsen S, Ward CG. Scar carcinoma arising in a skin graft donor site. J Trauma 1987;27:681–3. [23] Moller R, Reymann F, Hou-Jensen K. Metastases in dermatological patients with squamous cell carcinoma. Arch Dermatol 1979;115:703–5. [24] Novick M, Gard DA, Hardy SB, Spira M. Burn scar carcinoma: a review and analysis of 46 cases. J Trauma 1977;17(10):809–17. [25] Hill BB, Sloan DA, Lee EY, McGrath PC, Kenady DE. Marjolin’s ulcer of the foot caused by non-burn trauma. South Med J 1996;89:707–10. [26] Alcolado JC, Ray K, Baxter M, Edwards CW, Dodson PM. Malignant change in dermatitis artefacta. Postgrad Med J 1993;69:648– 50. [27] Konigova R, Rychterova V. Marjolin’s ulcer. Acta Chir Plast 2000;42:91–4. [28] Paredes F. Marjolin ulcer. Acta Med Port 1998;11:185–7.
Marjolin’s ulcer on the nose Eray Copcu a,∗ , Nil Çulhaci b a
Plastic and Reconstructive Surgery Department, Medical Faculty, Adnan Menderes University, 09100 Aydin, Turkey b Pathology Department, Medical Faculty, Adnan Menderes University, 09100 Aydin, Turkey Accepted 9 May 2002
Abstract Malignancies in scars are generally known as Marjolin’s ulcer. The majority of these arise from burn injuries. Although Marjolin’s ulcer consists of all kinds of skin cancer, squamous cell carcinoma is the main cancer type reported in the literature. The pathogenesis of this tumor is due to chronic irritation of the effected area and mostly involves the extremities and scalp area. This report presents an unusually located and rare type of Marjolin’s ulcer: on the nose and baso-squamotic in type. A 54-year-old man, 33 years after burn, presented with an unhealed ulcer on his nose which had been present for 2 years. The case was managed by tumor excision and a naso-labial transposition flap. To our knowledge, this is the first report of Marjolin’s ulcer developing on post-burned skin of the nose. © 2002 Elsevier Science Ltd and ISBI. All rights reserved. Keywords: Marjolin’s ulcer; Nose; Skin; Tumor; Burn
1. Introduction Marjolin’s ulcer is a well-recognized malignancy that arises from previously traumatized, chronically inflamed or scarred skin. As cited by Fleming et al. [1], this tumor was first described by Marjolin in 1823 and was defined as “ulcer canchroides.”A full description was made by Dupuytren 2 years later [2]. “Marjolin’s ulcer” has now become a term that is used generally to describe any squamous cell carcinoma arising in a chronically inflamed skin. The majority of burn scar carcinomas occur after a lag period in burns that were not been grafted following injury [3,4]. The cancer can be seen on all parts of the body; the extremities, trunk and scalp are the major locations. There has not to date been any report in the literature of Marjolin’s ulcer on the nose.
2. Case report A 54-year-old man presented, suffering from an ulcerated and non-healed wound on his nose (Fig. 1). He had a burn scar on his face that extended from the midface to the chest. Interview with the patient revealed that he had sustained a flame burn to his face and chest 33 years ago in a traffic accident. He reported no active treatment following injury. ∗
Corresponding author. Fax: +90-256-212-01-46. E-mail address: [email protected] (E. Copcu).
The burned face and chest healed spontaneously, leaving a large scar. He had no operations in the subsequent 33 years. Two years ago, he noticed a small ulcer on his nose and scratched it unwittingly. The lesion remained small until approximately 6 months before his presentation to hospital. He had never experienced any pain nor sought medical help for the lesion before presentation. Examination revealed an ulcerative lesion measuring 23 mm × 15 mm on his nose, over the lateral crus of right lower lateral cartilage. Macroscopically, the lesion had a reddish halo with a central necrotic crater and irregular edges. Physical examination and ultrasonographic investigation showed no signs of lymphatic involvement. Additionally, no metastatic involvement was found on the investigation of abdomen, chest and brain. Routine laboratory test results were normal. The presumptive diagnosis was Marjolin’s ulcer. Incisional biopsy clarified the diagnosis and the pathology result was given as a baso-squamous carcinoma. The whole lesion was excised together with the affected left lower cartilage. The inner nasal lining was tumor-free. The defect was covered with a right naso-labial transposition flap (Fig. 2). On histo pathological examination tumor diameters were 13 mm × 10 mm the histology showed islands in the dermis. Nuclear pallisading was present at the edge of the clusters. Adjacent clusters exhibited transitions to squamous cells and contained keratin pearls. There were also focal areas of necrosis. The small basoloid cells had moderately pleomorphic, densely hyperchromatic nuclei with scant cytoplasm. Larger cells with
0305-4179/02/$22.00 © 2002 Elsevier Science Ltd and ISBI. All rights reserved. PII: S 0 3 0 5 - 4 1 7 9 ( 0 2 ) 0 0 0 9 3 - 1
702
E. Copcu, N. Çulhaci / Burns 28 (2002) 701–704
Fig. 1. Marjolin’s ulcer on the nose. Pre-operative view.
more cytoplasm, vesicular nuclei and small nucleoli were seen as well. All these findings indicated that the lesion was baso-squamous carcinoma (Figs. 3 and 4). The surgical margins of the specimen were found to be tumor-cell free.
Fig. 2. Post-operative view. Defect was closed with naso-labial transposition flap.
3. Discussion Marjolin’s ulcer is a rare tumor in which various etiological factors have been encountered. Some of these are irritation, poor lymphatic regeneration, antibodies, mutations and
Fig. 3. Microscopically view. Hematoxylen eosin 40× magnification.
E. Copcu, N. Çulhaci / Burns 28 (2002) 701–704
703
Fig. 4. Microscopically view. Hematoxylen eosin 100× magnification.
local toxins [12]. Discoid lupus erythematosus and acne conglobata may also undergo malignant change in time. Castillo and Goldsmith [11] showed that immune system deficiency may have a role on development of Marjolin’s ulcer. All of these circumstances result in an inflammatory state that is claimed to end up with a Marjolin’s ulcer. The most commonly met factor, however, is irritation of inflamed skin. This inflammation is often due to burn injuries but at the same time vaccination [5], snake bite [6], osteomyelitis [7], pilonoidal abscess [8], pressure sores [9], venous stasis [10] may be other sources of inflammation for this tumor. Although many different cell types can be seen in these lesions, the major histological type is squamous cell carcinoma. Basal cell carcinoma (BCC) [13], malignant melanoma [1], and mesenchymal malignancies [12] are the less predominant types of the Marjolin’s ulcer. As in our case, baso-squamous carcinoma of the skin is another rare type of Marjolin’s’ ulcer. Multiple factors act together in malignant transformation on burn scar tissue. Sun exposure is a major etiologic factor in the etiopathogenesis of the skin tumours [14]. Nose skin tumors are very common, most being BCC [15] because the nose is one of the parts of the body most often exposed to the sun. This anatomical structure is an unusual area for the Marjolin’s ulcer and our report is the first in the literature. It is usually located on the extremities [16–18], scalp [19,20], or neck [21]. There is always a lag period to appearance of the tumor. This period takes approximately 30 years as in our case [12,17,21], whereas short periods are relatively rare [12] with the shortest reported wound of 6 weeks [22]. Marjolin’s ulcers are generally accepted as very aggressive tumors [17] having higher regional metastasis and fatality rate [23,24]. Although it is a well defined skin tumor, the treatment of it is still controversial. Radical excisional
surgery is the most appropriate treatment, but there is no consensus on the indications for lymph node dissection (LND). If a clinically palpable lymph node is present, dissection it should be carried out [12]. Most of the studies did not support the performance of prophylactic node dissection [17,25], whereas one study claims that the presence of a clinically palpable lymph node is an indication for LND. In another study, Novick et al. [24] suggest prophylactic LND in cases of lower extremity tumors. Bad prognosis is the characteristic of Marjolin’s ulcer and death from Marjolin’s ulcer is not uncommon [26,27]. Finally, this tumor is an uncommon type of skin cancer in the developing countries as strongly emphasized in Paredes’ study [28]. In a patient with chronically irritated skin, good health care will help to prevent a bad outcome such as Marjolin’s ulcer.
References [1] Fleming MD, Hunt JL, Purdue GF, Sandstad J. Marjolin’s ulcer: a review and reevaluation of a difficult problem. J Burn Care Rehabil 1990;11:460–9. [2] Applebaum J, Burrows WM, Greenway HJ. Acute Marjolin’s ulcer. J Assoc Mil Dermatol 1985;11:57–61. [3] Horton CE. The malignant potential of burn scar. Plast Reconstr Surg 1958;22:348–51. [4] Mosborg DA, Crane RT, Tami T, Parjer GS. Scar carsinoma of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:1038– 40. [5] Steffen C. Marjolin’s ulcer. Report of two cases and evidence that Marjolin did not describe cancer arising in scars of burns. Am J Dermatopathol 1984;6:187–93. [6] Smith J, Mello LF, Nogueira Neto NC, Meohas W, Pinto LW, Campos VA, et al. Malignancy in chronic ulcers and scars of the leg (Marjolin’s ulcer): a study of 21 patients. Skeletal Radiol 2001;30:331–7.
704
E. Copcu, N. Çulhaci / Burns 28 (2002) 701–704
[7] Hensel KS, Ono CM, Doukas WC. Squamous cell carcinoma in chronic ulcerative lesions: a case report and literature review. Am J Orthop 1999;28:253–6. [8] Lerner HJ, Dietrick G. Squamous cell carcinoma of the pilonidal sinus: report of a case and review of the literature. J Surg Oncol 1979;11:117–83. [9] Berkwits L, Yarkony GM, Lewis V. Marjolin’s ulcer complicating a pressure ulcer: case report and literature review. Arch Phys Med Rehabil 1986;67:831–3. [10] Nkere UU, Banjar A. Malignant change in a chronic skin lesion induced by an intravenous cannula. Postgrad Med J 1997;73:45–7. [11] Castillo JL, Goldsmith HS. Burn scar malignancy in a possible depressed immune setting. Surg Forum 1968;19:511–3. [12] Celikoz B, Demiriz M, Selmanpakoglu N. A shorter lag period of mesenchymal malignancy on Marjolin’s ulcer. Burns 1997;23:72–4. [13] Ozyazgan I, Kontacs O. Basal cell carcinoma arising from surgical scars: a case and review of the literature. Dermatol Surg 1999;25: 965–8. [14] Casson PR, Robins P. Malignant tumors of the skin. In: McCarthy JG, editor. Plastic Surgery, vol. 4. Philadelphia: Saunders, 1990, 3614. [15] McCormack CJ, Kelly JW, Dorevitch AP. Differences in age and body site distribution of the histological subtypes of basal cell carcinoma. Arch Dermatol 1997;133:593–6. [16] Ames FC, Hickey RC. Squamous cell carcinoma of the skin of the extremities. Int Adv Surg Oncol 1980;3:179–99. [17] Barr LH, Menard JW. Marjolin’s ulcer: the LSU experience. Cancer 1983;52:173–5.
[18] Bean DJ, Rees RS, O’Leary JP, Lynch JB. Carcinoma of the hand: a 20-year experience. South Med J 1984;7(7):998–1000. [19] Clements B, Lewis H, Lewis H, McKinstrey S, Gray J, Byrnes D. A late, fatal complication of a high energy thermal injury to the scalp. Ann Plast Surg 1995;35:650–3. [20] Curry SS, Gaither DH, King Jr LE. Squamous cell carcinoma arising in dissecting perifolliculitis of the scalp: a case report and review of secondary squamous cell carcinomas. J Am Acad Dermatol 1981;4:673–8. [21] Simmons MA, Edwards JM, Nigam A. Marjolin’s ulcer presenting in the neck. J Laryngol Otol 2000;114:980–2. [22] Hammond JS, Thomsen S, Ward CG. Scar carcinoma arising in a skin graft donor site. J Trauma 1987;27:681–3. [23] Moller R, Reymann F, Hou-Jensen K. Metastases in dermatological patients with squamous cell carcinoma. Arch Dermatol 1979;115:703–5. [24] Novick M, Gard DA, Hardy SB, Spira M. Burn scar carcinoma: a review and analysis of 46 cases. J Trauma 1977;17(10):809–17. [25] Hill BB, Sloan DA, Lee EY, McGrath PC, Kenady DE. Marjolin’s ulcer of the foot caused by non-burn trauma. South Med J 1996;89:707–10. [26] Alcolado JC, Ray K, Baxter M, Edwards CW, Dodson PM. Malignant change in dermatitis artefacta. Postgrad Med J 1993;69:648– 50. [27] Konigova R, Rychterova V. Marjolin’s ulcer. Acta Chir Plast 2000;42:91–4. [28] Paredes F. Marjolin ulcer. Acta Med Port 1998;11:185–7.