236
Journal of the Royal Society of Medicine Volume 85 April 1992
2 Hallopeau H. Sur un cas de lichen de Wilson gingival avec neoplasie voisine dans la region maxillaire. Bull Soc Fr Dermatol Syphiligr 1910;17:33 3 Dechaume M, Payen J, Piriou J. Le lichen plan isole de la muquese buccale: considerations anatomocliniques d'apres 50 observations dont 30 avec examen histologique. Presse Med 1957;65:2133-5 4 Fulling H-J. Cancer development in oral lichen planus. Arch Dermatol 1973;108:667-9 5 Marks R, Black M, Wilson Jones E. Epidermal cell kinetics in lichen planus. Br J Dermatol 1973;88:37-45
6 Krutchkoff DJ, Eisenberg E. Lichenoid dysplasia: a distinct histopathologic entity. Oral Surg Oral Med Oral Pathol 1985; 60:308-15 7 Krolls SO, Hoffman S. Squamous cell carcinoma of the soft tissues: a statistical analysis of 14,253 cases by age, sex, and race of patients. J Am Dent Assoc 1976;92:571-4
(Accepted 24 September 1991)
Squamous cell carcinoma arising in rhinophyma
Case presented to Section of Plastic Surgery, 8 January 1991
D A Ross BSc FRCS M P Davies FRCS Regional Plastic Surgery Unit, Queen Mary's University Hospital, Roehampton, London SW15 Keywords: rhinophyma; malignant change
We present a case of squamous cell carcinoma arising in a long-standing rhinophyma. This association is rare and the literature is reviewed. The extensive invasion of the tumour is emphasized.
Case report A 54-year-old man presented with an ulcer arising in a rhinophyma of approximately 10 years standing. The ulcer had been present for 3 years, but had grown markedly in the 12 months preceding admission, and it had been this and not the large rhinophyma which had led him to seek the advice of his GP (Figure 1). The initial attempt to resect the ulcer and shave the rhinophyma was abandoned when it became apparent that the ulcer represented the tip of the iceberg of a tumour which extended deeply into the nasal septum and laterally under an obscuring layer of rhinophymatous tissue into the skin over the maxillae. Histology confirmed a poorly differentiated squamous cell carcinoma with dysplastic areas in the rhinophymatous skin at the lateral margins. A CT scan showed no evidence of bony invasion but at a second, wider excision, the entire nose and majority of the bony nasal septum were removed in order to gain tumour
-, :8.
...
, , ....
..
Figure 1. Pre-operative view, showing the patient's rhinophyma and ulcer
Figure 2. Postoperative view, showing the extent of resection
clearance (Figure 2). A split skin graft was applied to the defect and a prosthesis fitted. He was subsequently referred for adjuvant radiotherapy and showed no sign of recurrence 6 months later. Discussion
Rhinophyma is seen mainly in men in their 5th-6th decades. Wolfe reviewed 60 cases occurring over 20 years and found only two cases in women'. The aetiology remains obscure with putative factors including alcohol, diet and vitamin deficiency1. In one report Freeman found 33% of patients with rhinophyma to have a history of alcohol abuse. Two cases were noted to follow prolonged steroid therapy2. Macroscopically it appears as a form of acne rosacea, with proliferation and hyperplasia of sebaceous glands. Acanthosis, manifested by hyperplasia of all the layers of the epidermis, has also been noted to be a common feature3. Acute and chronic inflammation is superimposed upon this along with intermittent areas of fibrosis. Malignant change (squamous cell carcinoma in particular), is a well rognized complication of chronic inflammation as seen mi ulcers, osteomyelitis and even burn scars. It is also seen in association with the chronic inflammatory dermatoses such as lupus vulgaris, discoid lupus erythematosus and hypertrophic lichen planus. However, despite te mked level of cellular activity and inflammtion seen in rhinophyma the association between it and malignant change is rare; until 1966 only five cases were reported in the literature. Broadbent described two cases of squamous cell carcinoma arising in longstanding rhinophyma, however the tumours were well defined with no evidence of occult spread so strikingly seen in our case4. In 1967 Acker and Helwig reported five cases of basal cell carcinoma arising in 47 patients with long-standing rhinophyma3. The authors concluded that basal cell carcinoma is more likely to arise in rhinophymatous than 'normal' skin, this argument being based on comparison between the incidence of basal cell
0141^0768/92/ 040236-0402.00/0 © 1992 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 85 April 1992
carcinoma occurring in rhinophymatous skin and that ofthe population at large. Dysplastic areas were present at the lateral excision margins in our patient suggesting an unstable, premalignant phase. Brubaker reported a case where clear histological progression from hyperplasia to carcinoma was seen5. However, the case for recognizing rhinophyma as a premalignant condition remains a poor one and is generally unaccepted. Most skin carcinomata arise as a result of actinic damage which is usually limited to the most superficial layers. The hidden nature of the tumour seen in our case suggests that invasion occurred into the deeper, subcutaneous layers at a relatively early stage which may explain the extensive occult infiltration at the time of presentation. In a recent case report, a sebaceous carcinoma was discovered quite unexpectedly on routine histological analysis following paring of a chronic rhinophyma which had been noted to undergo a rapid increase in size; further emphasizing the case for care when assessing these supposedly innocuous lesions6. Our case, along with those
A case of malignant epithelioid schwannoma arising on the face
D A Ross BSc FRCS K Schafler FRCPath Regional Plastic Surgery Unit, Queen Mary's University Hospital, Roehampton, London SW15 Keywords: malignant schwannoma; malignant epithelioid schwannoma
Malignant schwannoma is a rare but well recognized tumour of peripheral nerve and neurofibromas accounting for approximately 10% of soft tissue sarcomas. Diagnostic difficulty may occur when the tumour does not appear to arise from a nerve or is poorly differentiated. In this situation the tumour shares certain histological features with malignant melanoma. One may also have to consider a number of even rarer variants such as malignant epithelioid schwannoma. Case report A 74-year-old Caucasian woman presented with a 3 year history of a nodular, non-pigmented lesion at the base ofthe right alar. The appearance suggested a basal cell carcinoma.
237
discussed above, would suggest long standing rhinophyma may be susceptible to malignant change and that close attention is warranted to any associated lesions. A small lesion may be the 'tip of the iceberg', References 1 Wolfe MM. Rhinophyma; with new aetiological and therapeutic considerations. Laryngoscope 1943;53:172-80 2 Freeman BS. Reconstructive rhinoplasty for rhinophyma. Plast Reconstr Surg 1970;46:265-70 3 Acker DW, Helwig EB. Rhinop with carcinoma. Arch Dermat 1967;95:250-4 4 Broadbent NRG, Cort DF. Squamous cell carcinoma in longstanding rhinophyma. Br J Plastic Surg 1977;31:308-9 5 Brubaker DB, Hellstom RH. Carcinoma arising in rhinophyma. Arch Dermatol 1977;113:847-8 6 Motley RJ, Douglas-Jones AF. Sebaceous carcinoma: an unusual cause of a rapidly enlarging rhinophyma. Br J Dermatol 1991;124:283-4 (Accepted 18 June 1991)
Histology revealed a tumour composed of pallisades of spindle cells but no evidence of junctional melanocyte activity. The tumour did not arise from a preexisting peripheral nerve lesion nor were there any stigmata of neurofibromatosis. Thirty-two months later she represented with an area of ulceration on the rim of the right alar. Biopsy confirmed recurrence and reexcision necessitated the removal of both soft tissue and the anterior wall of the right maxilla. Histology showed tumour to have invaded the right infraorbital nerve (Figure 1), which was resected at the floor of the orbit. A final procedure, through the floor ofthe orbit, succeeded in removing the residual tumour and she remains well with no signs of recurrence at 6 months.
Case presented to Section of Plastic Surgery, 8 January 1991
Discussion Malignant schwannoma is a rare tumour but well recognized in association with peripheral nerve tumours, especially von Recklinghausen's disease'. In the apparent absence of a preceding nerve lesion, diagnosis of these tumours may be exceedingly difficult and require ultrastructural and immunohistochemical studies. Differential diagnoses comprise malignant melanoma, poorly differentiated carcinoma, lymphoma and rare variants. These include the malignant 'Triton' tumour2, glandular malignant schwannoma3, and malignant epithelioid schwannoma. Malignant epithelioid schwannoma has been chronicled in several series in association with peripheral nerves and arising below the clavicles4, but descriptions of lesions developing on the head and neck are considerably more rare and confined to individual case reports. The mean age ofpresentation in these reports was 71 years. Three ofthe four reported cases, including our patient, were female. As in two other reports our patient presented with a nodular, unpigmented lesion on the face5'6. In the third case the tumour arose in the mandible of a 27-year-old
Ethiopian woman7.
Figure 1. Low power view ofthe tumour (H&E, x25) Note the spindle celled configuration on the left and a more solid epithelioid tumour island on the right
The histology of the initial specimen from our patient revealed several classical features of this tumour. In certain areas epithelioid cells are apparent, some multinucleate, and these were more prevalent in the recurrent tumour, a feature noted before'. Histology of the infraorbital nerve in the recurrent specimen clearly showed the tumour to be invading it as opposed to arising from it (Figure 2), and consequently it falls into the rare category of malignant epithelioid schwannoma developing in soft tissue'. In certain areas the tumour cells merged with the perineurium of small nerves
0141-0768/92/ 040237-02/$02.00/O © 1992 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 85 April 1992
2 Hallopeau H. Sur un cas de lichen de Wilson gingival avec neoplasie voisine dans la region maxillaire. Bull Soc Fr Dermatol Syphiligr 1910;17:33 3 Dechaume M, Payen J, Piriou J. Le lichen plan isole de la muquese buccale: considerations anatomocliniques d'apres 50 observations dont 30 avec examen histologique. Presse Med 1957;65:2133-5 4 Fulling H-J. Cancer development in oral lichen planus. Arch Dermatol 1973;108:667-9 5 Marks R, Black M, Wilson Jones E. Epidermal cell kinetics in lichen planus. Br J Dermatol 1973;88:37-45
6 Krutchkoff DJ, Eisenberg E. Lichenoid dysplasia: a distinct histopathologic entity. Oral Surg Oral Med Oral Pathol 1985; 60:308-15 7 Krolls SO, Hoffman S. Squamous cell carcinoma of the soft tissues: a statistical analysis of 14,253 cases by age, sex, and race of patients. J Am Dent Assoc 1976;92:571-4
(Accepted 24 September 1991)
Squamous cell carcinoma arising in rhinophyma
Case presented to Section of Plastic Surgery, 8 January 1991
D A Ross BSc FRCS M P Davies FRCS Regional Plastic Surgery Unit, Queen Mary's University Hospital, Roehampton, London SW15 Keywords: rhinophyma; malignant change
We present a case of squamous cell carcinoma arising in a long-standing rhinophyma. This association is rare and the literature is reviewed. The extensive invasion of the tumour is emphasized.
Case report A 54-year-old man presented with an ulcer arising in a rhinophyma of approximately 10 years standing. The ulcer had been present for 3 years, but had grown markedly in the 12 months preceding admission, and it had been this and not the large rhinophyma which had led him to seek the advice of his GP (Figure 1). The initial attempt to resect the ulcer and shave the rhinophyma was abandoned when it became apparent that the ulcer represented the tip of the iceberg of a tumour which extended deeply into the nasal septum and laterally under an obscuring layer of rhinophymatous tissue into the skin over the maxillae. Histology confirmed a poorly differentiated squamous cell carcinoma with dysplastic areas in the rhinophymatous skin at the lateral margins. A CT scan showed no evidence of bony invasion but at a second, wider excision, the entire nose and majority of the bony nasal septum were removed in order to gain tumour
-, :8.
...
, , ....
..
Figure 1. Pre-operative view, showing the patient's rhinophyma and ulcer
Figure 2. Postoperative view, showing the extent of resection
clearance (Figure 2). A split skin graft was applied to the defect and a prosthesis fitted. He was subsequently referred for adjuvant radiotherapy and showed no sign of recurrence 6 months later. Discussion
Rhinophyma is seen mainly in men in their 5th-6th decades. Wolfe reviewed 60 cases occurring over 20 years and found only two cases in women'. The aetiology remains obscure with putative factors including alcohol, diet and vitamin deficiency1. In one report Freeman found 33% of patients with rhinophyma to have a history of alcohol abuse. Two cases were noted to follow prolonged steroid therapy2. Macroscopically it appears as a form of acne rosacea, with proliferation and hyperplasia of sebaceous glands. Acanthosis, manifested by hyperplasia of all the layers of the epidermis, has also been noted to be a common feature3. Acute and chronic inflammation is superimposed upon this along with intermittent areas of fibrosis. Malignant change (squamous cell carcinoma in particular), is a well rognized complication of chronic inflammation as seen mi ulcers, osteomyelitis and even burn scars. It is also seen in association with the chronic inflammatory dermatoses such as lupus vulgaris, discoid lupus erythematosus and hypertrophic lichen planus. However, despite te mked level of cellular activity and inflammtion seen in rhinophyma the association between it and malignant change is rare; until 1966 only five cases were reported in the literature. Broadbent described two cases of squamous cell carcinoma arising in longstanding rhinophyma, however the tumours were well defined with no evidence of occult spread so strikingly seen in our case4. In 1967 Acker and Helwig reported five cases of basal cell carcinoma arising in 47 patients with long-standing rhinophyma3. The authors concluded that basal cell carcinoma is more likely to arise in rhinophymatous than 'normal' skin, this argument being based on comparison between the incidence of basal cell
0141^0768/92/ 040236-0402.00/0 © 1992 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 85 April 1992
carcinoma occurring in rhinophymatous skin and that ofthe population at large. Dysplastic areas were present at the lateral excision margins in our patient suggesting an unstable, premalignant phase. Brubaker reported a case where clear histological progression from hyperplasia to carcinoma was seen5. However, the case for recognizing rhinophyma as a premalignant condition remains a poor one and is generally unaccepted. Most skin carcinomata arise as a result of actinic damage which is usually limited to the most superficial layers. The hidden nature of the tumour seen in our case suggests that invasion occurred into the deeper, subcutaneous layers at a relatively early stage which may explain the extensive occult infiltration at the time of presentation. In a recent case report, a sebaceous carcinoma was discovered quite unexpectedly on routine histological analysis following paring of a chronic rhinophyma which had been noted to undergo a rapid increase in size; further emphasizing the case for care when assessing these supposedly innocuous lesions6. Our case, along with those
A case of malignant epithelioid schwannoma arising on the face
D A Ross BSc FRCS K Schafler FRCPath Regional Plastic Surgery Unit, Queen Mary's University Hospital, Roehampton, London SW15 Keywords: malignant schwannoma; malignant epithelioid schwannoma
Malignant schwannoma is a rare but well recognized tumour of peripheral nerve and neurofibromas accounting for approximately 10% of soft tissue sarcomas. Diagnostic difficulty may occur when the tumour does not appear to arise from a nerve or is poorly differentiated. In this situation the tumour shares certain histological features with malignant melanoma. One may also have to consider a number of even rarer variants such as malignant epithelioid schwannoma. Case report A 74-year-old Caucasian woman presented with a 3 year history of a nodular, non-pigmented lesion at the base ofthe right alar. The appearance suggested a basal cell carcinoma.
237
discussed above, would suggest long standing rhinophyma may be susceptible to malignant change and that close attention is warranted to any associated lesions. A small lesion may be the 'tip of the iceberg', References 1 Wolfe MM. Rhinophyma; with new aetiological and therapeutic considerations. Laryngoscope 1943;53:172-80 2 Freeman BS. Reconstructive rhinoplasty for rhinophyma. Plast Reconstr Surg 1970;46:265-70 3 Acker DW, Helwig EB. Rhinop with carcinoma. Arch Dermat 1967;95:250-4 4 Broadbent NRG, Cort DF. Squamous cell carcinoma in longstanding rhinophyma. Br J Plastic Surg 1977;31:308-9 5 Brubaker DB, Hellstom RH. Carcinoma arising in rhinophyma. Arch Dermatol 1977;113:847-8 6 Motley RJ, Douglas-Jones AF. Sebaceous carcinoma: an unusual cause of a rapidly enlarging rhinophyma. Br J Dermatol 1991;124:283-4 (Accepted 18 June 1991)
Histology revealed a tumour composed of pallisades of spindle cells but no evidence of junctional melanocyte activity. The tumour did not arise from a preexisting peripheral nerve lesion nor were there any stigmata of neurofibromatosis. Thirty-two months later she represented with an area of ulceration on the rim of the right alar. Biopsy confirmed recurrence and reexcision necessitated the removal of both soft tissue and the anterior wall of the right maxilla. Histology showed tumour to have invaded the right infraorbital nerve (Figure 1), which was resected at the floor of the orbit. A final procedure, through the floor ofthe orbit, succeeded in removing the residual tumour and she remains well with no signs of recurrence at 6 months.
Case presented to Section of Plastic Surgery, 8 January 1991
Discussion Malignant schwannoma is a rare tumour but well recognized in association with peripheral nerve tumours, especially von Recklinghausen's disease'. In the apparent absence of a preceding nerve lesion, diagnosis of these tumours may be exceedingly difficult and require ultrastructural and immunohistochemical studies. Differential diagnoses comprise malignant melanoma, poorly differentiated carcinoma, lymphoma and rare variants. These include the malignant 'Triton' tumour2, glandular malignant schwannoma3, and malignant epithelioid schwannoma. Malignant epithelioid schwannoma has been chronicled in several series in association with peripheral nerves and arising below the clavicles4, but descriptions of lesions developing on the head and neck are considerably more rare and confined to individual case reports. The mean age ofpresentation in these reports was 71 years. Three ofthe four reported cases, including our patient, were female. As in two other reports our patient presented with a nodular, unpigmented lesion on the face5'6. In the third case the tumour arose in the mandible of a 27-year-old
Ethiopian woman7.
Figure 1. Low power view ofthe tumour (H&E, x25) Note the spindle celled configuration on the left and a more solid epithelioid tumour island on the right
The histology of the initial specimen from our patient revealed several classical features of this tumour. In certain areas epithelioid cells are apparent, some multinucleate, and these were more prevalent in the recurrent tumour, a feature noted before'. Histology of the infraorbital nerve in the recurrent specimen clearly showed the tumour to be invading it as opposed to arising from it (Figure 2), and consequently it falls into the rare category of malignant epithelioid schwannoma developing in soft tissue'. In certain areas the tumour cells merged with the perineurium of small nerves
0141-0768/92/ 040237-02/$02.00/O © 1992 The Royal Society of Medicine
