Subungual melanoma of the hand

SUBUNGUAL

MELANOMA

OF THE

HAND

D. A. HUDSON, J. E. J. KRIGE, R. M. STROVER and HELEN S. KING

From the University of Cape Town and the Melanoma Clinic, Groote Schuur Hospital, Cape Town, South Africa

Thirteen patients with subungual melanoma on the fingers had a mean delay before diagnosis of 1.2 years. Four patients presented with local recurrence after inadequate initial treatment and two presented with systemic metastases. Mean primary subungual melanoma thickness was 6.1 mm. and nine patients had Clark level IV or V disease. All patients underwent digital amputation. Two of seven patients who had localised disease initially are alive at 29 and 44 months. One of four patients who had locally recurrent melanoma is alive at 36 months. Both patients with systemic disease at presentation died. Advanced disease and delayed presentation contributed to the poor prognosis of subungual melanoma in our patients. Journal of Hand Surgery (British Volume, 1990) 15B: 288-290 Subungual melanoma is uncommon and represents less than 2% of all cutaneous melanoma diagnosed in western countries (Shaw and Koea, 1988). A higher proportion of subungual melanoma, however, occurs in dark-skinned and oriental patients (Gutman et al., 1985). Subungual melanoma is frequently mistaken for benign, traumatic or inflammatory lesions of the nail-bed and amelanotic subungual melanoma is rarely diagnosed clinically at an early stage (Leppard et al., 1974). The prognosis of subungual melanoma is poor compared with the survival rate of melanoma at other locations. One of the main reasons is that the clinical characteristics of early lesions of subungual melanoma have not been fully elucidated (Saida and Ohshima, 1989). Subungual melanoma is often grouped with other melanomas of the extremities (Krementz et al., 1982). There is evidence, however, that melanoma of the nail bed is a distinct entity with special clinical and pathological characteristics (Klausner et al., 1987). The present study evaluates subungual melanoma as a site-specific entity and analyses the results of treatment in 13 patients over 17 years at a major referral centre. Material and methods The records of all patients registered at the Melanoma Clinic, Groote Schuur Hospital, Cape Town between 1970 and 1987 were reviewed. During this 18-year period, 1460 patients with primary cutaneous melanoma were treated, of whom 22 (1.5%) had melanoma involving the nail-bed. 13 of these 22 patients had subungual melanomas of the fingers and form the basis of this study. Complete follow-up was available on all patients. Clinical data included patient characteristics, duration of tumour before diagnosis, stage and surgical treatment of the primary lesion (initial therapy, extent of definitive excision and timing of regional lymph node dissection), subsequent clinical course including site and management of recurrent disease and survival. Histological review of the primary lesions included subtype of melanoma, Breslow tumour thickness and Clark level of penetration. 288

Results The average age at presentation of the 13 patients was 47.5 years (range : 22-79 years). Seven patients were men and six were women. Seven subungual melanomas involved the thumb (right thumb: four; left thumb: three) and six occurred on the fingers (index: three; middle : two; little finger: one). The sites of disease were similar in men and women. The ethnic distribution was five white, five coloured, and three black. Eight subungual lesions were pigmented and five patients had ulcerated lesions with minimal or no pigmentation. The mean delay between onset of symptoms and diagnosis was 1.2 years (range: O-3 years). Melanoma was not recognised initially in six patients and several were treated for long periods for chronic paronychia, osteitis, bacterial or fungal nailbed infections or subungual haematoma. Mean Breslow depth for all subungual lesions was 6.1 mm. (range: 0.76-17 mm.). Nine patients (69.2%) had either Clark level IV or V lesions. The histogenetic subtypes were acral lentiginous melanoma in eight patients (61.5’/,> and nodular melanoma in three (23%). In two patients (15.4x), the lesions were unclassifiable histologically because of extensive necrosis and ulceration or previous biopsy of the nail bed. Seven patients had stage I disease localised to the primary site. Four patients presented with local recurrent disease (stage II) because of inadequate initial treatment and two patients had stage IV disease with both nodal and lung metastases. Five of the seven patients with stage I disease had an amputation at the metacarpo-phalangeal joint and two patients, both with Clark level V lesions involving periosteum, had ray amputations. Four patients with stage I disease developed positive axillary nodes at a meanof 5.3 months (range 1-12 months) after amputation of the digit and underwent a therapeutic axillary block dissection. Five of the seven patients with stage I disease died of systemic disease between two and 36 months (mean 19.2 months) after presentation. One of the four patients is alive 44 months after axillary dissection. Two of three patients died of systemic disease without THE

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developing nodal metastases and one is alive after 29 months. Two of four patients who presented with stage II disease had excision of the distal phalanx only performed elsewhere and both developed local recurrent disease at 7 and 12 months. Two patients had inadequate initial biopsies of the nail which were reported histologically as either inflammation or benign naevus. Both developed recurrent melanoma at the biopsy site 6 and 18 months later. All four patients with local recurrence had subsequent amputation of the digit. One patient developed nodal axillary disease at 32 months and three subsequently died of systemic disease between 24 and 41 months. One patient is disease-free at 36 months. Both patients who presented with stage IV disease had an amputation of the digit and therapeutic axillary block dissection to prevent fungation and died at 9 and 14 months.

MELANOMA

Fig. 1

Advanced infection.

subungual melanoma

with ulceration and secondary

Discussion Subungual melanoma is uncommon in Caucasians in contrast to black and oriental populations (Saida and Ohshima, 1989). The ethnic differences are pronounced both in the incidence and predilection of sites of melanoma. In a Japanese series, 19% of all diagnosed melanomas involved the nail-bed (Takematsu et al., 1985). While cutaneous melanoma is more common in women than men, the incidence of subungual melanoma in men and women is similar (Papachristou and Fortner, 1982). Subungual melanoma occurs with equal frequency on the fingers and toes in western populations, the thumbs and big toes being the most commonly involved digits (Patterson and Helwig, 1980). In the Japanese literature, however, 46 (73%) of 63 reported subungual melanomas occurred on the hands and 33 of these involved the thumb-nails (Takematsu et al., 1985). Subungual melanoma usually begins as a pigmented linear streak in the nail-bed, and, in the early stages, has a benign appearance and is symptomless (Saida and Ohshima, 1989). Frequently the patient’s attention is only drawn to the lesion after an injury (Briggs, 1985). Three (23%) of our patients gave a definite history of recent local trauma. This is less than in other series where the incidence of preceding trauma ranged from 29% to 44% (Patterson and Helwig, 1980; Takematsu, 1985). Involvement of the eponychium and paronychium is common with advancing disease. When left untreated, the nail becomes thickened, deformed and split and tumour may extrude through the nail-plate (Klausner et al., 1987). Ulceration, secondary infection and bleeding are common at this late stage, as shown in Figure 1, (Pack and Orpeza, 1967). Over a third of the subungual melanomas in our series were either amelanotic or had minimal pigmentation, making diagnosis particularly difficult. The clinical VOL. 15B No. 3 AUGUST

1990

differential diagnosis covers a wide spectrum including subungual haematoma, pyogenic granuloma, chronic paronychia, bacterial and fungal infections, junctional naevi and rare vascular tumours (Patterson and Helwig, 1980; Papachristou and Former, 1982; Hughes et al., 1985 ; Klausner et al., 1987). Two features suspicious of subungual melanoma are the associated pigmentation of the adjacent nail-fold (Hutchinson% sign) and progressive elevation of the nail from the nail-bed (Saida and Ohshima, 1989; Hughes et al., 1985). Distal migration of pigment away from the nail-bed may help to distinguish a melanoma from subungual haematoma (Papachristou and Former, 1982). Subungual melanotic bands unrelated to malignant melanoma are common in black and oriental patients, but rare in Caucasians (Leyden et al., 1972). Because of difficulty in diagnosis, clinical awareness and early biopsy are essential in suspicious or persisting lesions of the nail-bed or the nail itself, whether the abnormality is pigmented or not (Papachristou and Former, 1982). A generous biopsy is mandatory to obtain satisfactory diagnostic material and should include a portion of the proximal terminus of the nail matrix, the nail-bed and the nail-plate (Briggs, 1985). If the initial biopsy is inconclusive, more tissue should be taken (Patterson and Helwig, 1980). All pigmented subungual lesions should be considered melanoma until proved otherwise by histological examination of adequate biopsy material. The radial growth phase of malignant melanoma in the subungual region is easily confused histologically with a junctional naevus (Briggs, 1985) and the histological impression may appear so bland that biopsies are reported as benign (Patterson and Helwig, 1980). In Caucasians, however, an acquired subungual melanocy289

D. A. HUDSON,

J. E. J. KRIGE,

tic lesion is more likely to be a malignant melanoma than a benign naevus. The dermis of the nail-bed is thick, hard and adherent to the periosteum and may present difficulties to the pathologist in the interpretation, staging and classification of melanomas (Gutman et al., 1985). Often the histological material is inadequate for evaluation because of ulceration and necrosis (Daly et al., 1987). In the subungual region the interface between dermal layers may be poorly-defined, compromising interpretation of Clark’s levels of invasion, while the markedly thickened dermis may invalidate prognostic values when applying Breslow micrometric measurements (Krementz et al., 1982; Arrington et al., 1977). The majority of subungual melanomas in our patients, like other series (Feibleman et al., 1980), were acral lentiginous melanomas, a histogenetic type of melanoma now dominating reports of palmar and subungual melanomas (Krementz et al., 1982; Gutman et al., 1985). Surgery offers the only effective means of treatment. In the unusual circumstance where small superficial Clark I or II subungual lesions occur, distal phalangeal amputation is adequate (Gutman et al., 1985). In most lesions however, formal digital amputation is necessary. Lesser procedures inevitably led to local recurrence in our series. When the melanoma invades the periosteum or involves the proximal nail-fold, amputation of the metatarsal or metacarpal head should be included (Hughes et al., 1985). Patients with clinically palpable regional nodes require therapeutic lymph node dissection (Feibleman et al., 1980). The role of elective or prophylactic regional lymph node dissection in patients with extremity melanoma remains controversial. Some investigators have indicated that prophylactic lymphadenectomy in subungual melanoma offers a prognostic and therapeutic benefit (Das Gupta and Brasfield, 1965). In view of the substantial number of patients with subungual melanoma who develop positive regional lymph nodes during follow-up, current recommendations advocate prophylactic lymph node dissection in the high risk group of patients (Clark level 4 and 5 or deeper than 1.5 mm.) with subungual melanoma (Briggs, 1985). The results of chemotherapy and immunotherapy are disappointing and the only promising treatment to date is isolated regional hyperthermic limb perfusion with high dose melphalan (Baas et al., 1989). Subungual melanoma has a poor prognosis due to delay in diagnosis and aggressive biological behaviour (Klausner et al., 1987). Deep penetration ulceration and

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early metastatic dissemination are common (Shaw and Koea, 1988). The overall five-year survivals in other series were 16% (Patterson and Helwig, 1980), 35% (Pack and Orpeza, 1967) and 40% (Baas et al., 1989). Early recognition and adequate initial treatment could improve these poor survival figures. References ARRINGTON, J. H., REED, R. J., ICHINOSE, H. and KREMENTZ, E. T. (1977). Plantar lentiginous melanoma: A distinctive variant of human cutaneous malignant melanoma. American Journal of Surgical Pathology, 1: 2: 131-143. BAAS, P. C., HOEKSTRA, H. J., SCHRAFFORDT KOOPS, H., OOSTERHUIS, J. W. and OLDHOFF, J. (1989). Isolated Regional Perfusion in the Treatment of Subungual Melanoma. Archives of Surgery, 124: 373-376. BRIGGS, J. C. (1985). Subungual malignant melanoma: a review article. British Journalof Plastic Surgery, 38: 174-176. DALY, J. M., BERLIN, R. andURMACHER,C. (1987). SubungualMelanoma: A 25-Year Review of Cases. Journal of Surgical Oncology, 35: 107-l 12. DAS GUPTA, T. and BRASFIELD, R. (1965). Subungual Melanoma: 25-Year Review of Cases. Annals of Surgery, 161: 545-552. FEIBLEMAN, C. E., STOLL, H. and MAIZE, J. C. (1980). Melanomas of the Palm, Sole and Nailbed. A Clinicopathologic Study. Cancer, 46: 2492-2504. GUTMAN, M., KLAUSNER, J. M., INBAR, M., SKORNICK,Y., BARATZ, M. and ROZIN, R. R. (1985). Acral (volar-subungual) melanoma. British Journal of Surgery, 72: 61@-613. HUGHES, L. E., HORGAN, K., TAYLOR, B. A. and LAIDLER, P. (1985). Malignant melanoma of the hand and foot: diagnosis and management. British Journal of Surgery, 72: 811-815. KLAUSNER, J. M., INBAR, M., GUTMAN, M., WEISS, G., SKORNICK, Y., CHAICHIK, S. and ROZIN, R. R. (1987). Nail-Bed Melanoma. Journal of Surgical Oncology, 34: 208-210. KREMENTZ, E. T., REED, R. J., COLEMAN, W. P., SUTHERLAND, C. M., CARTER, R. D. and CAMPBELL, M. (1982). Acral Lentiginous Melanoma, A Clinico-pathologic Entity. Annals of Surgery, 195 : 632645. LEPPARD, B., SANDERSON, K. V. and BEHAN, F. (1974). Subungual Malignant Melanoma: Difficulty in Diagnosis. British Medical Journal, 1: 310-312. LEYDEN, J. J., SPOTT, D. A. and GOLDSCHMIDT, H. (1972). Diffuse and Banded Melanin Pigmentation in Nails. Archives of Dermatology, 105 : 548550. PACK, G. T. and OROPEZA, R. (1967). Subungual melanoma. Surgery Gynecology and Obstetrics, 124: 571-582. PAPACHRISTOU, D. N. and FORTNER, J. G. (1982). Melamona Arising Under the Nail. Journal of Surgical Oncology, 21: 219-222. PATTERSON, R. H. and HELWIG, E. B. (1980). Subungual Malignant Melanoma. Cancer, 46: 20742087. SAIDA, T. and OHSHIMA, Y. (1989). Clinical and Histopathologic Characteristics of Early Lesions of Subungual Malignant Melanoma. Cancer, 63 : 556 560. SHAW, J. H. F. and KOEA, J. B. (1988). Acral (volar-subungual) melanoma in Auckland, New Zealand. British Journal of Surgery, 75: 69-72. TAKEMATSU, H., OBATA, M., TOMITA, Y., KATO, T., TAKAHASHI, M. and ABE, R. (1985). Subungual Melanoma. A Clinicopathologic Study of 16 Japanese Cases. Cancer, 55: 2725-2731.

Accepted: 31 October 1989 Dr Don Hudson, Department of Plastic Surgery, Groote Schuur Hospital, South Africa.

Observatory

7925,

6 1990 The British Society for Surgery of the Hand 026&7681/90/00154288/$10.00

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