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Amelanotic lentigo malignant melanoma: a case report and review of the literature
British Journal of Plastic Surgery (1991), 44.312-314 0 1991 The Trustees of British Association of Plastic Surgeons
Case Report Amelanotic lentigo malignant melanoma: a case report and review of the literature M. S. Irwin, D. M. Mercer and N. P. J. Walker The Department of Plastic Surgery, St Thomas’ Hospital, London SUMMARY.
A patient with an amelanotic lentigo malignant melanoma is presented which manifested itself initially as an erythematous flare with the subsequent development of nodules. The diagnosis was only made histologically. A computer search of the literature revealed 6 previous cases, and a review of these rare tumours is presented.
Case report A Bl-year-old nursing sister presented with a macular erythematous lesion over the left mandible which contained two pink nodules. The lesions had been present for 7 months, starting initially as reddened areas of flaky skin which appeared initially to resolve, but which then became nodular and enlarged. Biopsy of one of the nodules and surrounding erythema revealed that these were both invasive primary malignant melanoma (Fig. 1). The remaining tumour was excised and the defect grafted. An incidental finding was the presence of a pleural effusion and hilar mass secondary to a breast tumour treated 4 years earlier. The excised specimen measured 110 mm x 80 mm and included the pale area A (10 mm diameter), a nodule B (2 mm diameter) and the brown plaque C (15 mm diameter) (Fig. 1). Histology of this nodule showed an ulcerated invasive malignant melanoma extending deep into the reticular dermis (Clark’s level IV), with a maximum depth of invasion of 1.58 mm. The cells were epitheloid in appearance with brisk mitotic activity (Fig. 2). The adjacent epidermis showed extensive in situ changes representing macular amelanotic lentigo maligna (Fig. 3) with occasional foci of invasion to Clark’s level II. The brown plaque represented a distant discrete melanotic lentigo maligna. Extensive solar elastosis was present in the dermis.
Discussion Amelanotic lentigo malignant melanoma is a rare entity. A computer search of the world literature revealed that only six previous cases have been reported (Table 1). As with our patient, all initially presented as inconspicuous erythematous macules or plaques. In two publications (Burkett, 1979 and Borkovic and Schwartz, 1983) the clinical features were almost identical to those of our patient, namely
pale nodules with surrounding ill-defined macular erythema. Three of the patients had a past history of lentigo maligna and one of lentigo maligna melanoma. Review of this small series of patients suggests that amelanotic lentigo malignant melanoma is more common in elderly women as they represent six of the seven cases. The mean age of the affected patients is 66.3 years, with a range of 58 to 72 years. The lesions were present for a period ranging from 7 months to 10 years from onset to excision. Five of the six cases at histology were found to have areas of invasion ranging from Clark’s level II to IV. The sixth case (Pichler and Fritsch, 1988) showed pre-invasive in situ change only. Unfortunately, follow-up of the six cases is not discussed and we have no long-term follow-up of our own case. As a result we are unable to comment on the natural history of these lesions, or compare them with melanotic lentigo malignant melanoma. The outstanding feature in all the reported cases, including our own, is that the diagnosis was made histologically and not clinically. The long history which some patients gave suggests that it is a slow growing lesion like other lentigo malignant melanoma. Nevertheless, all the reported lesions became malignant. In view of the diagnostic difficulties, amelanotic lentigo malignant melanoma should be considered as part of the differential diagnosis of large long standing erythematous macules of the face, especially if they become nodular or irregular.
Acknowledgments We are indebted to Dr D. M. MacDonald MA, FRCP for referring this patient, and would like to thank Dr Philip McKee for his help in preparing the histological photographs.
312
Case Report
313
Fig. 1 Figure l--Side
view of the patient showing the left cheek following biopsy. Note the erythematous flare within which are (A) pale area, (B) amelanotic nodule, (C) brown plaque and (D) biopsy scars.
Fig. 3 Figure 2-Histological section from nodule B showing invasive amelanotic malignant melanoma. Note the nuclear pleomorphism, conspicuous nucleoli and mitotic acitivity. (x 102 magnification). Figme 3-Section through area A showing amelanotic lentigo maligna with basally located atypical melanocytes. Note the nuclear irregularity, hyperchromatism and extensive solar elastosis. ( x 66 magnification).
314
British Journal of Plastic Surgery Table
A summary of previously
reported
macular amelanotic
melanomas
Patient details Authors
Age
Sex
Site
Cramer (1967)
70
F
R. Calf
Appearance
History/duration
Histopathology
“Bowenoid” Round Well defined Slightly Elevated Two nodules . Surrounding Erythematous Macule Hypopigmented Macule Irregular Erythema In Previous Scar
3 years
L.M.M. Clarke 2. Amelanotic
10 years P.H. Pigmented L.M. (13 years prior)
L.M.M. Clarke 4. 1.65 mm deep Amelanotic L.M.M. Clarke 2. Amelanotic
l
l l l
66
F
L. Neck
l
Su and Bradley (1980) 68
F
L. Forearm
l
Burkett (1979)
l
7 years P.H. Pigmented L.M. (7 years prior)
Central Nodule . Ill Defined Surrounding Erythema
5 years P.H. L.M.M. (10 years prior)
L.M.M. Clarke 3. 0.5 mm deep
Erythematous Slightly Elevated
10 years
L.M.M. Clarke 3. 0.64 mm deep
Red Macule Round Well Defined
4 years
Macular Melanoma In situ Amelanotic
l
Borkovic and Schwartz (1983)
72
M
R. Shoulder
F
L. Calf
l
l
Hoffman et al. (1986) 69
l
l
Pichler and Fritsch (1988)
58
F
L. Cheek
l l l
P.H.
L.M. (10 years prior)
L.M.M. = Lentigo maligna melanoma. P.H. = Past History. L.M. =Lentigo Maligna.
References
The Authors
S. P. and Schwartz, R. A. (1983). Amelanotic lentigo maligna melanoma manifesting as a dermatitis like plaque.
Jhkovic,
Archives ofDermatology,
119,423.
Burke& J. M. (1979). Amelanotic lentigo maligna. Archives of Dermatology, 115,496.
Cramer, H. J. (1967). Unpigmentierte melanosis praeblastomatosa. Gleichzeiting ein Beitrag zur Differentialdiagnose des Morbus Bowen. Der Hautartz. 18,203. Hoffman, H., Lllke-PIewig, H. and PIewIg, G. (1986). Amelanotisches lentigo-maligna-melanom. Der Hautartz. 37,28 1. Plchler, E. and FrItsch, P. (1988). Macular amelanotic melanoma in situ. Dermatologica. 177,313. SU, W. P. D. and Bradley, R. R. (1980). Amelanotic lentigo maligna. Archives of Dermatology, 116,82.
M. S. Irwin, MB, BS, Senior House Officer in Plastic Surgery, St Thomas’ Hospital, London D. M. Mercer, FRCS, Senior Registrar in Plastic Surgery, Hand Unit, Derby Royal Infirmary, Derby N. P. J. Walker, MB, MRCP(UK), Senior Lecturer, Institute of Dermatology, St Thomas’ Hospital, London
Requests for reprints to: Dr M. S. Irwin, Department of Plastic Surgery, St Thomas’ Hospital, London SE1 7EH. Paper received 12 July 1990. Accepted 15 December 1990.
Case Report Amelanotic lentigo malignant melanoma: a case report and review of the literature M. S. Irwin, D. M. Mercer and N. P. J. Walker The Department of Plastic Surgery, St Thomas’ Hospital, London SUMMARY.
A patient with an amelanotic lentigo malignant melanoma is presented which manifested itself initially as an erythematous flare with the subsequent development of nodules. The diagnosis was only made histologically. A computer search of the literature revealed 6 previous cases, and a review of these rare tumours is presented.
Case report A Bl-year-old nursing sister presented with a macular erythematous lesion over the left mandible which contained two pink nodules. The lesions had been present for 7 months, starting initially as reddened areas of flaky skin which appeared initially to resolve, but which then became nodular and enlarged. Biopsy of one of the nodules and surrounding erythema revealed that these were both invasive primary malignant melanoma (Fig. 1). The remaining tumour was excised and the defect grafted. An incidental finding was the presence of a pleural effusion and hilar mass secondary to a breast tumour treated 4 years earlier. The excised specimen measured 110 mm x 80 mm and included the pale area A (10 mm diameter), a nodule B (2 mm diameter) and the brown plaque C (15 mm diameter) (Fig. 1). Histology of this nodule showed an ulcerated invasive malignant melanoma extending deep into the reticular dermis (Clark’s level IV), with a maximum depth of invasion of 1.58 mm. The cells were epitheloid in appearance with brisk mitotic activity (Fig. 2). The adjacent epidermis showed extensive in situ changes representing macular amelanotic lentigo maligna (Fig. 3) with occasional foci of invasion to Clark’s level II. The brown plaque represented a distant discrete melanotic lentigo maligna. Extensive solar elastosis was present in the dermis.
Discussion Amelanotic lentigo malignant melanoma is a rare entity. A computer search of the world literature revealed that only six previous cases have been reported (Table 1). As with our patient, all initially presented as inconspicuous erythematous macules or plaques. In two publications (Burkett, 1979 and Borkovic and Schwartz, 1983) the clinical features were almost identical to those of our patient, namely
pale nodules with surrounding ill-defined macular erythema. Three of the patients had a past history of lentigo maligna and one of lentigo maligna melanoma. Review of this small series of patients suggests that amelanotic lentigo malignant melanoma is more common in elderly women as they represent six of the seven cases. The mean age of the affected patients is 66.3 years, with a range of 58 to 72 years. The lesions were present for a period ranging from 7 months to 10 years from onset to excision. Five of the six cases at histology were found to have areas of invasion ranging from Clark’s level II to IV. The sixth case (Pichler and Fritsch, 1988) showed pre-invasive in situ change only. Unfortunately, follow-up of the six cases is not discussed and we have no long-term follow-up of our own case. As a result we are unable to comment on the natural history of these lesions, or compare them with melanotic lentigo malignant melanoma. The outstanding feature in all the reported cases, including our own, is that the diagnosis was made histologically and not clinically. The long history which some patients gave suggests that it is a slow growing lesion like other lentigo malignant melanoma. Nevertheless, all the reported lesions became malignant. In view of the diagnostic difficulties, amelanotic lentigo malignant melanoma should be considered as part of the differential diagnosis of large long standing erythematous macules of the face, especially if they become nodular or irregular.
Acknowledgments We are indebted to Dr D. M. MacDonald MA, FRCP for referring this patient, and would like to thank Dr Philip McKee for his help in preparing the histological photographs.
312
Case Report
313
Fig. 1 Figure l--Side
view of the patient showing the left cheek following biopsy. Note the erythematous flare within which are (A) pale area, (B) amelanotic nodule, (C) brown plaque and (D) biopsy scars.
Fig. 3 Figure 2-Histological section from nodule B showing invasive amelanotic malignant melanoma. Note the nuclear pleomorphism, conspicuous nucleoli and mitotic acitivity. (x 102 magnification). Figme 3-Section through area A showing amelanotic lentigo maligna with basally located atypical melanocytes. Note the nuclear irregularity, hyperchromatism and extensive solar elastosis. ( x 66 magnification).
314
British Journal of Plastic Surgery Table
A summary of previously
reported
macular amelanotic
melanomas
Patient details Authors
Age
Sex
Site
Cramer (1967)
70
F
R. Calf
Appearance
History/duration
Histopathology
“Bowenoid” Round Well defined Slightly Elevated Two nodules . Surrounding Erythematous Macule Hypopigmented Macule Irregular Erythema In Previous Scar
3 years
L.M.M. Clarke 2. Amelanotic
10 years P.H. Pigmented L.M. (13 years prior)
L.M.M. Clarke 4. 1.65 mm deep Amelanotic L.M.M. Clarke 2. Amelanotic
l
l l l
66
F
L. Neck
l
Su and Bradley (1980) 68
F
L. Forearm
l
Burkett (1979)
l
7 years P.H. Pigmented L.M. (7 years prior)
Central Nodule . Ill Defined Surrounding Erythema
5 years P.H. L.M.M. (10 years prior)
L.M.M. Clarke 3. 0.5 mm deep
Erythematous Slightly Elevated
10 years
L.M.M. Clarke 3. 0.64 mm deep
Red Macule Round Well Defined
4 years
Macular Melanoma In situ Amelanotic
l
Borkovic and Schwartz (1983)
72
M
R. Shoulder
F
L. Calf
l
l
Hoffman et al. (1986) 69
l
l
Pichler and Fritsch (1988)
58
F
L. Cheek
l l l
P.H.
L.M. (10 years prior)
L.M.M. = Lentigo maligna melanoma. P.H. = Past History. L.M. =Lentigo Maligna.
References
The Authors
S. P. and Schwartz, R. A. (1983). Amelanotic lentigo maligna melanoma manifesting as a dermatitis like plaque.
Jhkovic,
Archives ofDermatology,
119,423.
Burke& J. M. (1979). Amelanotic lentigo maligna. Archives of Dermatology, 115,496.
Cramer, H. J. (1967). Unpigmentierte melanosis praeblastomatosa. Gleichzeiting ein Beitrag zur Differentialdiagnose des Morbus Bowen. Der Hautartz. 18,203. Hoffman, H., Lllke-PIewig, H. and PIewIg, G. (1986). Amelanotisches lentigo-maligna-melanom. Der Hautartz. 37,28 1. Plchler, E. and FrItsch, P. (1988). Macular amelanotic melanoma in situ. Dermatologica. 177,313. SU, W. P. D. and Bradley, R. R. (1980). Amelanotic lentigo maligna. Archives of Dermatology, 116,82.
M. S. Irwin, MB, BS, Senior House Officer in Plastic Surgery, St Thomas’ Hospital, London D. M. Mercer, FRCS, Senior Registrar in Plastic Surgery, Hand Unit, Derby Royal Infirmary, Derby N. P. J. Walker, MB, MRCP(UK), Senior Lecturer, Institute of Dermatology, St Thomas’ Hospital, London
Requests for reprints to: Dr M. S. Irwin, Department of Plastic Surgery, St Thomas’ Hospital, London SE1 7EH. Paper received 12 July 1990. Accepted 15 December 1990.