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Desmoplastic melanoma of the hand: Case reports and review of the literature
Desmoplastic Melanoma of the Hand: Case Reports and Review of the Literature Kevin C. Chung, MD, Edward R. Calkins, MD, Rebecca Crawford, MD, Riley S. Rees, MD, Ann Arbor, MI
Desmoplastic melanoma (DM), described by Conley et al. in 1971, is a variant of melanoma characterized by superficial melanotic dysplasia, underlying deep fibroblastic tumor, and neurotropism. 1Although histologically similar to lentigo maligna melanoma, DM presents with deeper dermal extension that produces a higher incidence of local recurrence and regional metastasis. The biological behavior of DM in extremities differs from that of acral lentiginous melanoma. The major difference is the site of occurrence. Acral lentiginous melanomas are confined to the atrichous surfaces of the hands or feet and the subungual areas of the fingers and toes. On the other hand, of the 13 cases of DM of the upper extremities reported in the world literature (Table 1), DM occurred only on the dorsal thumbs or the arms. Histologically, DM is distinguished from acral lentiginous melanoma by its predilection for perineural involvement. Ample immunohistochemical and ultrastructural evidence now exists to separate DM from the other types of melanoma? To our knowledge fewer than 200 cases of DM have been reported in the world literature since 1971. Of the 83 cases of DM collected in the world literature by Egbert et al. in 1988, 3 62 cases occurred on the head and neck region and only 7 cases occurred
From the Department of Surgery, Section of Plastic and Reconstructive Surgery, University of Michigan Hospital, Ann Arbor, MI. Received for publication Feb. 4, 1994; accepted in revised form March 9, 1995. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Kevin C. Chung, MD, Section of Plastic and Reconstructive Surgery, University of Michigan Hospital, 2130 Taubman Center, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-0340.
on the upper extremity. Our review of the world literature revealed only 13 cases of DM located on the upper extremities (Table 1). In this report, two patients with desmoplastic melanoma of the upper extremity are discussed along with their pathologic features and our proposed treatment options.
Case 1 The patient was an 85-year-old woman who presented with a 1-cm irregular brown lesion over the dorsum of her right thumb (Fig. 1). The lesion had been present for 6 months and was increasing in size. The fight-hand-dominant patient had no prior history of melanoma. Her chest x-ray films and liver function tests were normal. There was no palpable epitrochlear or axillary lymph node. The lesion appeared flat with an irregular border. Incisional biopsy was performed. Histologically, the tumor consisted of atypical junctional melanocytic hyperplasia with slender spindle cells between the collagen fibers in the dermis (Fig. 2). The spindle cells stained strongly for S-100 protein antigen. These findings were consistent with a level IV and tumor thickness 0.96-mm DM. 4'5 Thumb amputation was offered to the patient for definitive tumor control; however, she refused the thumb amputation because of the functional disability that would result. She underwent reexcision of the melanoma with a 1-cm surgical margin and the wound was dressed with homograft. The depth of the excision was above the extensor mechanism of the thumb, as anatomic constraint in the fingers prevented a 1-cm deep excision. After review of the permanent sections, which revealed complete removal of the melanoma, a full-thickness autograft was placed on the defect 2 days later. The Journal of Hand Surgery 873
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Table 1. Reported Cases o f Desmoplastic Melanoma of the Upper Extremity Author
Location
Reed and Leonard (1979) 9 From et al. (1983) j~ Walsh et al. (1988) 8
Egbert et al. (1988) 3 Devaraj et al. (1992) 2
Forearm Arm Arm Upper arm Thumb Arm Arm Arm Right forearm Forearm Right arm Thumb Thumb
Outcome
Recurrence x 2 Recurrence x 2 N/A N/A No recurrence No recurrence Recurrence N/A Recurrence N/A N/A Recurrence Recurrence
N/A, follow-up data not available.
At 18-month follow-up evaluation, the thumb had full range of motion and an excellent aesthetic result (Fig. 3). Physical examination, chest x-ray films, and liver function tests showed no tumor recurrence.
Case 2 A 32-year-old woman presented with a 2.5-cm brown, nodular lesion over her left upper forearm.
F i g u r e 1. Dorsal view of the right thumb showing a 1-cm brown-pigmented flat lesion at the distal thumb adjacent to the nail (large arrow). The other pigmented areas on the hand (small arrows) are actinic keratosis lesions.
F i g u r e 2. (A) The atypical junctional melanocytic proliferation (arrow A) and the abundance of spindle cells in the stroma (arrow B) (hemotoxylin & eosin, 10• can be seen. (B) The desmoplasia in the dennis; the arrow points to the spindle cell in the dermis (hemotoxylin & eosin, 20x).
Figure 3.
Outline of the healed skin graft on the thumb at 18 months (arrows).
The Journal of Hand Surgery / Vol. 20A No. 5 September 1995
The lesion had been present for 1 year and was increasing in size. She had no axillary adenopathy. Her chest x-ray film and liver function tests were normal. Incisional biopsy revealed a level IV, tumor thickness 1.58-mm DM. Histologically, the tumor consisted of intense fibroblastic reaction and atypical melanocytic proliferation at the dermal-epidermal junction. The tumor stained strongly for S-100 protein antigen. The biopsy site was reexcised with a 3-cm margin. The excision was extended to the depth of the extensor musculature beneath the fascia. The wound was covered with homograft. Permanent sections confirmed complete excision of the tumor. Her wound was covered with autograft 2 days after surgery. Follow-up physical examination, chest x-ray film, and liver function test at 18 months revealed no tumor recurrence. The skin graft had healed uneventfully.
Discussion Desmoplastic melanoma is a variant of melanoma that is difficult to diagnose since 71% of patients have amelanotic skin lesions? Histopathologic identification is confusing because of the intense fibroblastic reaction in the dermis and minimal atypical melanocytic proliferation at the dermal and epidermal junction? Desmoplastic melanoma frequently shows histologic features of lentigo maligna melanoma with dysplastic melanocytes in the epidermis. This similarity led Egbert et al. to suggest that DM may be a variant of lentigo maligna melanoma, since they can occur simultaneously in the same lesion? Biological behavior differentiates DM from lentigo maligna melanoma because of the intense fibroblastic dermal activity and significant invasive potential of DM. The spindle fibroblastic cells present in DM have the propensity to undergo metaplastic transformation along the nerve sheaths and preferentially exhibit perineural invasion. This apparent neurotropic behavior results in a marked enlargement of the cutaneous nerves. The origin of the fibroblastic reaction is unclear, but the neural crest origin of these melanocytes may contribute to their transformation into fibroblastic and neuritic cells. 6 Since DM is a rare tumor, the biological behavior of this lesion is not well described. Microstaging of DM may not be useful. Although the median thickness of DM at presentation is 4.3 mm, whereas classical melanoma is 1.2 mm, the 5-year survival in clinical stage I DM is higher (63%, compared to 48% for classical melanoma of the same Breslow depth, >4.0 mm).6 Multivariate analysis of metastasis of DM indicates that a thickness of more than 4 nun is not a significant
875
prognostic factor. 6Therefore, microstaging appears not to be a useful clinical tool. The factors that predict an increased risk of recurrence or disseminated disease in DM include failure to excise at least a 1-cm margin and head and neck primary or deeply invasive tumors. Reported series suggest a high rate of local recurrence? In the series by Smithers et al., 17 of 58 patients had local recurrences. Lymph node metastasis occurred in eight of these patients with local recurrences. 6 With regard to treatment, the Sydney Melanoma Unif recommends a 3-cm margin of excision and elective lymph node dissection for all types of melanoma of the hand with thickness greater than 4 mm. We agree with these reports if anatomic boundaries allow wide excision. When DM occurs on the arm, a 3-cm margin of excision may provide adequate local control. Covering the surgical defect with skin graft on the arm will allow early detection of recurrent disease. Because of the high local recurrence rate for DM of the finger, amputation of the involved finger is recommended in an effort to gain effective tumor control. However, as shown in our case 1, some patients may refuse thumb amputation because of the functional disability that results. In these situations, a 1 cm minimum margin of excision needs to be done, as the Queensland Melanoma Project indicated a significant rate of local recurrence for excision margins of less than 1 cm in DM. 6 However, since DM of the upper extremity is rare, the surgical margin has not been defined. Similarly, no data were available in the literature in regard to the value of lymph node dissection in this disease. Lymph node dissection may be reserved for patients with positive axillary nodes for tumor control. In summary, DM of the extremities is a rare disease. Recognition of its pathologic features will provide an accurate diagnosis and a responsible treatment plan. Because DM frequently recurs at the wound margin, the excision must be generous. Amputation is recommended in an effort to provide effective treatment for DM of the fingers.
References 1. Conley J, Lattes R, Orr W. Desmoplastic malignant melanoma (a rare variant of spindle cell melanoma). Cancer 1971;28:914-7. 2. Devaraj VS, Moss ALH, Briggs JC. Desmoplastic melanoma: a clinicopathologicalreview. Br J Plast Surg 1992;45:595-8. 3. Egbert B, Kempson R, Sagebiel R. Desmoplastic malignant melanoma:a clinicohistopathologicstudyof 25 cases. Cancer 1988;62:2033-2.
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Chung et al. / Desmoplastic Melanoma of the Hand
4. Clark WH, From L. The histogenesis and biological behavior of primary human malignant melanoma of the skin. Cancer Res 1969;29:705-10. 5. Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous melanoma. Ann Surg 1970;172:902-10. 6. Smithers BM, McLeod GR, Little JH. Desmoplastic melanoma: patterns of recurrence. World J Surg 1992;16: 186-90. 7. Quinn MJ, Wikramanayake R, Thompson JF, McCarthy WH. Non-subungual melanomas of the hand. J Hand Surg 1992;17B:433--6.
8. Walsh NMG, Roberts JT, Orr W, Simon GT. Desmoplastic malignant melanoma: a clinicopathologic study of 14 cases. Arch Pathol Lab Med 1988;112:922-7. 9. Reed RJ, Leonard DD. Neurotropic melanoma (a variant of desmoplastic melanoma). Am J Surg Pathol 1979;3:301-11. 10. From L, Hanna W, Kahn HJ, Gross J, Marks A, Baumal R. Origin of desmoplasia in desmoplastic malignant melanoma. Hum Pathol 1983;4:1072-80.
Desmoplastic melanoma (DM), described by Conley et al. in 1971, is a variant of melanoma characterized by superficial melanotic dysplasia, underlying deep fibroblastic tumor, and neurotropism. 1Although histologically similar to lentigo maligna melanoma, DM presents with deeper dermal extension that produces a higher incidence of local recurrence and regional metastasis. The biological behavior of DM in extremities differs from that of acral lentiginous melanoma. The major difference is the site of occurrence. Acral lentiginous melanomas are confined to the atrichous surfaces of the hands or feet and the subungual areas of the fingers and toes. On the other hand, of the 13 cases of DM of the upper extremities reported in the world literature (Table 1), DM occurred only on the dorsal thumbs or the arms. Histologically, DM is distinguished from acral lentiginous melanoma by its predilection for perineural involvement. Ample immunohistochemical and ultrastructural evidence now exists to separate DM from the other types of melanoma? To our knowledge fewer than 200 cases of DM have been reported in the world literature since 1971. Of the 83 cases of DM collected in the world literature by Egbert et al. in 1988, 3 62 cases occurred on the head and neck region and only 7 cases occurred
From the Department of Surgery, Section of Plastic and Reconstructive Surgery, University of Michigan Hospital, Ann Arbor, MI. Received for publication Feb. 4, 1994; accepted in revised form March 9, 1995. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Kevin C. Chung, MD, Section of Plastic and Reconstructive Surgery, University of Michigan Hospital, 2130 Taubman Center, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-0340.
on the upper extremity. Our review of the world literature revealed only 13 cases of DM located on the upper extremities (Table 1). In this report, two patients with desmoplastic melanoma of the upper extremity are discussed along with their pathologic features and our proposed treatment options.
Case 1 The patient was an 85-year-old woman who presented with a 1-cm irregular brown lesion over the dorsum of her right thumb (Fig. 1). The lesion had been present for 6 months and was increasing in size. The fight-hand-dominant patient had no prior history of melanoma. Her chest x-ray films and liver function tests were normal. There was no palpable epitrochlear or axillary lymph node. The lesion appeared flat with an irregular border. Incisional biopsy was performed. Histologically, the tumor consisted of atypical junctional melanocytic hyperplasia with slender spindle cells between the collagen fibers in the dermis (Fig. 2). The spindle cells stained strongly for S-100 protein antigen. These findings were consistent with a level IV and tumor thickness 0.96-mm DM. 4'5 Thumb amputation was offered to the patient for definitive tumor control; however, she refused the thumb amputation because of the functional disability that would result. She underwent reexcision of the melanoma with a 1-cm surgical margin and the wound was dressed with homograft. The depth of the excision was above the extensor mechanism of the thumb, as anatomic constraint in the fingers prevented a 1-cm deep excision. After review of the permanent sections, which revealed complete removal of the melanoma, a full-thickness autograft was placed on the defect 2 days later. The Journal of Hand Surgery 873
874
Chung et al. / Desmoplastic Melanoma of the Hand
Table 1. Reported Cases o f Desmoplastic Melanoma of the Upper Extremity Author
Location
Reed and Leonard (1979) 9 From et al. (1983) j~ Walsh et al. (1988) 8
Egbert et al. (1988) 3 Devaraj et al. (1992) 2
Forearm Arm Arm Upper arm Thumb Arm Arm Arm Right forearm Forearm Right arm Thumb Thumb
Outcome
Recurrence x 2 Recurrence x 2 N/A N/A No recurrence No recurrence Recurrence N/A Recurrence N/A N/A Recurrence Recurrence
N/A, follow-up data not available.
At 18-month follow-up evaluation, the thumb had full range of motion and an excellent aesthetic result (Fig. 3). Physical examination, chest x-ray films, and liver function tests showed no tumor recurrence.
Case 2 A 32-year-old woman presented with a 2.5-cm brown, nodular lesion over her left upper forearm.
F i g u r e 1. Dorsal view of the right thumb showing a 1-cm brown-pigmented flat lesion at the distal thumb adjacent to the nail (large arrow). The other pigmented areas on the hand (small arrows) are actinic keratosis lesions.
F i g u r e 2. (A) The atypical junctional melanocytic proliferation (arrow A) and the abundance of spindle cells in the stroma (arrow B) (hemotoxylin & eosin, 10• can be seen. (B) The desmoplasia in the dennis; the arrow points to the spindle cell in the dermis (hemotoxylin & eosin, 20x).
Figure 3.
Outline of the healed skin graft on the thumb at 18 months (arrows).
The Journal of Hand Surgery / Vol. 20A No. 5 September 1995
The lesion had been present for 1 year and was increasing in size. She had no axillary adenopathy. Her chest x-ray film and liver function tests were normal. Incisional biopsy revealed a level IV, tumor thickness 1.58-mm DM. Histologically, the tumor consisted of intense fibroblastic reaction and atypical melanocytic proliferation at the dermal-epidermal junction. The tumor stained strongly for S-100 protein antigen. The biopsy site was reexcised with a 3-cm margin. The excision was extended to the depth of the extensor musculature beneath the fascia. The wound was covered with homograft. Permanent sections confirmed complete excision of the tumor. Her wound was covered with autograft 2 days after surgery. Follow-up physical examination, chest x-ray film, and liver function test at 18 months revealed no tumor recurrence. The skin graft had healed uneventfully.
Discussion Desmoplastic melanoma is a variant of melanoma that is difficult to diagnose since 71% of patients have amelanotic skin lesions? Histopathologic identification is confusing because of the intense fibroblastic reaction in the dermis and minimal atypical melanocytic proliferation at the dermal and epidermal junction? Desmoplastic melanoma frequently shows histologic features of lentigo maligna melanoma with dysplastic melanocytes in the epidermis. This similarity led Egbert et al. to suggest that DM may be a variant of lentigo maligna melanoma, since they can occur simultaneously in the same lesion? Biological behavior differentiates DM from lentigo maligna melanoma because of the intense fibroblastic dermal activity and significant invasive potential of DM. The spindle fibroblastic cells present in DM have the propensity to undergo metaplastic transformation along the nerve sheaths and preferentially exhibit perineural invasion. This apparent neurotropic behavior results in a marked enlargement of the cutaneous nerves. The origin of the fibroblastic reaction is unclear, but the neural crest origin of these melanocytes may contribute to their transformation into fibroblastic and neuritic cells. 6 Since DM is a rare tumor, the biological behavior of this lesion is not well described. Microstaging of DM may not be useful. Although the median thickness of DM at presentation is 4.3 mm, whereas classical melanoma is 1.2 mm, the 5-year survival in clinical stage I DM is higher (63%, compared to 48% for classical melanoma of the same Breslow depth, >4.0 mm).6 Multivariate analysis of metastasis of DM indicates that a thickness of more than 4 nun is not a significant
875
prognostic factor. 6Therefore, microstaging appears not to be a useful clinical tool. The factors that predict an increased risk of recurrence or disseminated disease in DM include failure to excise at least a 1-cm margin and head and neck primary or deeply invasive tumors. Reported series suggest a high rate of local recurrence? In the series by Smithers et al., 17 of 58 patients had local recurrences. Lymph node metastasis occurred in eight of these patients with local recurrences. 6 With regard to treatment, the Sydney Melanoma Unif recommends a 3-cm margin of excision and elective lymph node dissection for all types of melanoma of the hand with thickness greater than 4 mm. We agree with these reports if anatomic boundaries allow wide excision. When DM occurs on the arm, a 3-cm margin of excision may provide adequate local control. Covering the surgical defect with skin graft on the arm will allow early detection of recurrent disease. Because of the high local recurrence rate for DM of the finger, amputation of the involved finger is recommended in an effort to gain effective tumor control. However, as shown in our case 1, some patients may refuse thumb amputation because of the functional disability that results. In these situations, a 1 cm minimum margin of excision needs to be done, as the Queensland Melanoma Project indicated a significant rate of local recurrence for excision margins of less than 1 cm in DM. 6 However, since DM of the upper extremity is rare, the surgical margin has not been defined. Similarly, no data were available in the literature in regard to the value of lymph node dissection in this disease. Lymph node dissection may be reserved for patients with positive axillary nodes for tumor control. In summary, DM of the extremities is a rare disease. Recognition of its pathologic features will provide an accurate diagnosis and a responsible treatment plan. Because DM frequently recurs at the wound margin, the excision must be generous. Amputation is recommended in an effort to provide effective treatment for DM of the fingers.
References 1. Conley J, Lattes R, Orr W. Desmoplastic malignant melanoma (a rare variant of spindle cell melanoma). Cancer 1971;28:914-7. 2. Devaraj VS, Moss ALH, Briggs JC. Desmoplastic melanoma: a clinicopathologicalreview. Br J Plast Surg 1992;45:595-8. 3. Egbert B, Kempson R, Sagebiel R. Desmoplastic malignant melanoma:a clinicohistopathologicstudyof 25 cases. Cancer 1988;62:2033-2.
876
Chung et al. / Desmoplastic Melanoma of the Hand
4. Clark WH, From L. The histogenesis and biological behavior of primary human malignant melanoma of the skin. Cancer Res 1969;29:705-10. 5. Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous melanoma. Ann Surg 1970;172:902-10. 6. Smithers BM, McLeod GR, Little JH. Desmoplastic melanoma: patterns of recurrence. World J Surg 1992;16: 186-90. 7. Quinn MJ, Wikramanayake R, Thompson JF, McCarthy WH. Non-subungual melanomas of the hand. J Hand Surg 1992;17B:433--6.
8. Walsh NMG, Roberts JT, Orr W, Simon GT. Desmoplastic malignant melanoma: a clinicopathologic study of 14 cases. Arch Pathol Lab Med 1988;112:922-7. 9. Reed RJ, Leonard DD. Neurotropic melanoma (a variant of desmoplastic melanoma). Am J Surg Pathol 1979;3:301-11. 10. From L, Hanna W, Kahn HJ, Gross J, Marks A, Baumal R. Origin of desmoplasia in desmoplastic malignant melanoma. Hum Pathol 1983;4:1072-80.