0307693891000218
Clinical and Experimental Dermatology 1991; 16: 63-65.
Basal-cell carcinoma complicating a port-wine stain P.DUHRA AND I.S.FOULDS The Skm Hospital, George Road, Edgbaston, Birmingham B15 lPR, UK Accepted for publication M) July 1990 Summary A rare radio-resistant basal-cell carcinoma is described which presented as a recently enlarged non-ulcerated nodule on a port-wine stain. The literature on basal-cell carcinoma occurring on a port-wine stain is reviewed, and the aetiologieal .significance of thorium-X, sun-exposure and vascular changes in the development of malignancy on this type of haemangioma is discussed. The natural history of a port-wine stain (PWS) is to darken gradually in colour and become thickened and raised, with advancing age. The development of nodules is not an infrequent occurrence in later life, and these may occasionally evolve rapidly.' However, they are usually benign and result from an increased number of vascular channels, together with an enlargement of diameter.' We describe a case of a recently enlarged nodule on a PWS which resulted from the development of a basal-cell carcinoma (BCC). I
Case report A 63-year-()ld man, who was otherwise healthy, had a PWS since birth on the left side of the face in the region innervated by the second division of the trigeminal nerve. This haemangioma had gradually darkened in colour and become raised and thickened. However, since March 1987 a rapidly enlarging lump was noticed in the PWS situated below the outer canthus of the left eye (Fig. 1). There was mild intermittent pruritus but no pain or bleeding from it. A photograph taken 15 years earlier showed a matchhead-si/.ed lump at the exact site of the current nodule. However, no treatment had been given in the past for the PWS. Examination revealed a hard nonulcerated nodule measuring 2-5x1-5 cm, which was mobile over the underlying bone, and there was no regional lymphadenopathy. Histological analysis of a biopsy specimen taken from the base of the nodule showed ectatic blood vessels and solar elastosis in the upper dermis. In addition, islands of BCC were found Correspondence: Dr P.Duhra, Skin Department, St Mark's Annexe, Coventry and Warwickshire Hospital, Stoney Stanton Road, C>)ventry CVl 4FH, UK.
Figure 1. A nodular BCC arising in upper part of PWS.
arising from a hair follicle and extending into the subcutaneous layer as well as encircling dilated vascular channels (Fig. 2). Initial treatment with a course of radical radiotherapy using 250-kV X-rays and delivering 50 Gy fractionated over 3 weeks yielded a poor response. The radiation produced a conjunctivitis and oedema of the eyelids but the malignant nodule showed little change apart from lightening of colour. The patient was subsequently referred for plastic surgery and the entire haemangioma 63
64
P.DUHRA AND I.S.FOULDS
Figure 2. A BCC arising from a hair folliele and surrounding ectatic vaseular channels (H&E x 325).
including the nodule was excised and the wound closed by a cervico-facial flap (Fig. 3). The final result was cosmetically satisfying for the patient. Discussion The development ofa BCC on a PWS is a rare occurrence with only six cases previously documented in the English literature (Table 1).' ^ These patients, together with ours, were all males, and 50% of cases had been irradiated previously. In a case report by Rogers,"* a patient received 35 local applications of thorium X from the age of 3 years and a BCC developed at 10 years of age. Courtemanehe^ reported a patient in whom thorium X was applied over several months at the age of 7 years and a BCC arose 9 years later. Both authors incriminated thorium X in carcinogenesis. However, Sarkany and Caron** reviewed the literature on the carcinogenic potential of thorium X but were unable to find any supportive evidence. They
Figure 3. Appearance 4 weeks after surgery,
concluded that a BCC eould evolve spontaneously on a PWS as exemplified by their own case and that the development of malignancy in the reports of Rogers and Courtemanche was probably fortuitous and unrelated to the previous exposure to thorium X. However, the early onset of BCC in the cases reported by Rogers and Courtemanche is strongly suggestive of a carcinogenic potential of thorium X. In a recent report by MaganaGarcia et al.^, a previously untreated PWS was complicated by the onset of multiple BCCs, suggesting that the vascular changes in this type of haemangioma might predispose to the development of BCC. This view was supported by a report ofa BCC developing in a non-sunexposed PWS,' but further reports of similar cases are needed before an association between PWS and BCC can be accepted as genuine. The predominence of right-sided lesions in previously reported cases of BCC and PWS probably reflects the more common occurrence of haemangioma on the right side, although the reason for this is
BASAL-CELL CARCINOMA AND A PORT-WINE STAIN
65
Table I, Previously reported eases of basal-cell carcinoma and port-v^ine stain Reference Martin-Scott' Rogers'' Courtemanche' Sarkany and Caron" Sagi et at.' Magana-tiarcia and Magiina-I.ozano^
Sex Age (years) M M M M \\ \\
Previous irradiation
Anatomical site
44
None Thorium \ at the age of } Thorium X at the age of 7 None Grenz rays at the age of ft
Right Right Right Right Right
49
None
Left cheek and ehin
38 10 16 40
unclear.^ The greater incidence of BCC and PWS in men may be related to increased occupational sun exposure and to women camouflaging their vascular naevj, thereby further reducing UV exposure. In conclusion, nodule formation on a PWS is not always benign, as illustrated by our case, and exclusion of malignancy is recommended in any recently enlarged firm nodule on this type of haemangioma.
hypochondrium side of faee side of face temple side of face
2. Barsky SH, Rosen S, Geer DE, Noe JM, The nature and evolution iifport-vvine stains: a computer-assisted study._7owrna/o//ni!«/i^iilive Dermatology 1980; 74: 154 157. 3. Scott IM, Basal cell epithelioma on the siie of an angioma. British Jotirnuhif Dermatology 194H; 60: 69-711. 4. Rogers J. Rodent ulcer follov\'ing thorium X therapy, British Jiiiirnal oj Dermatology 1954; 66: 31, 5. (Courtemanche ,AD. Thorium X-induced basal-cell carcinoma: a case report, British Journal of Plastic Surgery 1961; 14: 254 257. 6. Sarkany I, C.aron GA. Ba.sal-eell t-pithelioma on a port-wine stain. British Journat of Dermatology 1965; 77: 16 19. 7. Sagi E, Aram H, Peled IJ, Basal cell carcinoma developing in a References nevusflammeu.s.Cutis 1984; 33: 311-318. 1. Williams HE. Hemangioma. In: Demis DJ, Crounse RG, Dobson 8. Magana-Garcia M, Magana-Lozano M. Multiple basal cell carjRI*, McGuire JS, eds. Clinical Dermatology. Philadelphia: Harper cinomas arising in port-wine haemangiomas, British Journat of t 1986: 2; Unit 7-63: 1- 6. 1988; 119: 393-396.
Clinical and Experimental Dermatology 1991; 16: 63-65.
Basal-cell carcinoma complicating a port-wine stain P.DUHRA AND I.S.FOULDS The Skm Hospital, George Road, Edgbaston, Birmingham B15 lPR, UK Accepted for publication M) July 1990 Summary A rare radio-resistant basal-cell carcinoma is described which presented as a recently enlarged non-ulcerated nodule on a port-wine stain. The literature on basal-cell carcinoma occurring on a port-wine stain is reviewed, and the aetiologieal .significance of thorium-X, sun-exposure and vascular changes in the development of malignancy on this type of haemangioma is discussed. The natural history of a port-wine stain (PWS) is to darken gradually in colour and become thickened and raised, with advancing age. The development of nodules is not an infrequent occurrence in later life, and these may occasionally evolve rapidly.' However, they are usually benign and result from an increased number of vascular channels, together with an enlargement of diameter.' We describe a case of a recently enlarged nodule on a PWS which resulted from the development of a basal-cell carcinoma (BCC). I
Case report A 63-year-()ld man, who was otherwise healthy, had a PWS since birth on the left side of the face in the region innervated by the second division of the trigeminal nerve. This haemangioma had gradually darkened in colour and become raised and thickened. However, since March 1987 a rapidly enlarging lump was noticed in the PWS situated below the outer canthus of the left eye (Fig. 1). There was mild intermittent pruritus but no pain or bleeding from it. A photograph taken 15 years earlier showed a matchhead-si/.ed lump at the exact site of the current nodule. However, no treatment had been given in the past for the PWS. Examination revealed a hard nonulcerated nodule measuring 2-5x1-5 cm, which was mobile over the underlying bone, and there was no regional lymphadenopathy. Histological analysis of a biopsy specimen taken from the base of the nodule showed ectatic blood vessels and solar elastosis in the upper dermis. In addition, islands of BCC were found Correspondence: Dr P.Duhra, Skin Department, St Mark's Annexe, Coventry and Warwickshire Hospital, Stoney Stanton Road, C>)ventry CVl 4FH, UK.
Figure 1. A nodular BCC arising in upper part of PWS.
arising from a hair follicle and extending into the subcutaneous layer as well as encircling dilated vascular channels (Fig. 2). Initial treatment with a course of radical radiotherapy using 250-kV X-rays and delivering 50 Gy fractionated over 3 weeks yielded a poor response. The radiation produced a conjunctivitis and oedema of the eyelids but the malignant nodule showed little change apart from lightening of colour. The patient was subsequently referred for plastic surgery and the entire haemangioma 63
64
P.DUHRA AND I.S.FOULDS
Figure 2. A BCC arising from a hair folliele and surrounding ectatic vaseular channels (H&E x 325).
including the nodule was excised and the wound closed by a cervico-facial flap (Fig. 3). The final result was cosmetically satisfying for the patient. Discussion The development ofa BCC on a PWS is a rare occurrence with only six cases previously documented in the English literature (Table 1).' ^ These patients, together with ours, were all males, and 50% of cases had been irradiated previously. In a case report by Rogers,"* a patient received 35 local applications of thorium X from the age of 3 years and a BCC developed at 10 years of age. Courtemanehe^ reported a patient in whom thorium X was applied over several months at the age of 7 years and a BCC arose 9 years later. Both authors incriminated thorium X in carcinogenesis. However, Sarkany and Caron** reviewed the literature on the carcinogenic potential of thorium X but were unable to find any supportive evidence. They
Figure 3. Appearance 4 weeks after surgery,
concluded that a BCC eould evolve spontaneously on a PWS as exemplified by their own case and that the development of malignancy in the reports of Rogers and Courtemanche was probably fortuitous and unrelated to the previous exposure to thorium X. However, the early onset of BCC in the cases reported by Rogers and Courtemanche is strongly suggestive of a carcinogenic potential of thorium X. In a recent report by MaganaGarcia et al.^, a previously untreated PWS was complicated by the onset of multiple BCCs, suggesting that the vascular changes in this type of haemangioma might predispose to the development of BCC. This view was supported by a report ofa BCC developing in a non-sunexposed PWS,' but further reports of similar cases are needed before an association between PWS and BCC can be accepted as genuine. The predominence of right-sided lesions in previously reported cases of BCC and PWS probably reflects the more common occurrence of haemangioma on the right side, although the reason for this is
BASAL-CELL CARCINOMA AND A PORT-WINE STAIN
65
Table I, Previously reported eases of basal-cell carcinoma and port-v^ine stain Reference Martin-Scott' Rogers'' Courtemanche' Sarkany and Caron" Sagi et at.' Magana-tiarcia and Magiina-I.ozano^
Sex Age (years) M M M M \\ \\
Previous irradiation
Anatomical site
44
None Thorium \ at the age of } Thorium X at the age of 7 None Grenz rays at the age of ft
Right Right Right Right Right
49
None
Left cheek and ehin
38 10 16 40
unclear.^ The greater incidence of BCC and PWS in men may be related to increased occupational sun exposure and to women camouflaging their vascular naevj, thereby further reducing UV exposure. In conclusion, nodule formation on a PWS is not always benign, as illustrated by our case, and exclusion of malignancy is recommended in any recently enlarged firm nodule on this type of haemangioma.
hypochondrium side of faee side of face temple side of face
2. Barsky SH, Rosen S, Geer DE, Noe JM, The nature and evolution iifport-vvine stains: a computer-assisted study._7owrna/o//ni!«/i^iilive Dermatology 1980; 74: 154 157. 3. Scott IM, Basal cell epithelioma on the siie of an angioma. British Jotirnuhif Dermatology 194H; 60: 69-711. 4. Rogers J. Rodent ulcer follov\'ing thorium X therapy, British Jiiiirnal oj Dermatology 1954; 66: 31, 5. (Courtemanche ,AD. Thorium X-induced basal-cell carcinoma: a case report, British Journal of Plastic Surgery 1961; 14: 254 257. 6. Sarkany I, C.aron GA. Ba.sal-eell t-pithelioma on a port-wine stain. British Journat of Dermatology 1965; 77: 16 19. 7. Sagi E, Aram H, Peled IJ, Basal cell carcinoma developing in a References nevusflammeu.s.Cutis 1984; 33: 311-318. 1. Williams HE. Hemangioma. In: Demis DJ, Crounse RG, Dobson 8. Magana-Garcia M, Magana-Lozano M. Multiple basal cell carjRI*, McGuire JS, eds. Clinical Dermatology. Philadelphia: Harper cinomas arising in port-wine haemangiomas, British Journat of t 1986: 2; Unit 7-63: 1- 6. 1988; 119: 393-396.
