Desmoplastic melanoma V. S. Devaraj,

:

a clinico-pathological

review

A. L. H. Moss and J. C. Briggs

Departmenrs qf’ Plastic Surgery and Histopathology, Frenchay Hospital, Bristol SUMMARY. Desmoplastic Melanoma (DM) represents a rare histological variant of melanoma. It has been described in isolated case reports as a cutaneous tumour with a high incidence of local recurrence and nodal metastases, requiring early aggressive surgery. However, overall clinical experience of the tumour is limited. 13 patients with DMM seen over a 20 year period were reviewed. The mean age at presentation was 67 years (range 34+7), and 2/3 of the lesions were in the head and neck. Tumour thickness averaged 5.78 mm (Breslow). 7 patients developed recurrence, 4 as regional nodes, and 3 as skin nodules. Four of these patients developed disseminated disease, of whom 3 died. The mean time to first recurrence was 26 months and mean follow-up time in the review was 40 months (range 3-141 months). were ulcerated at presentation. including three of the four patients who died with disseminated disease. The larger tumours measured 30-35 mm in the long axis, but most were smaller (20-25 mm). Ten patients had noticed the lesion appearing within the preceding few months, although 2 reported that it had been present for more than 12 months. The mean age at presentation was 67 years (range 34-87) (Table 1), and 9 lesions involved the head and neck, including one arising on the palate. The remaining 4 lesions were on the limbs, two involving the nail-bed of the thumb.

Around 1000 people die from melanoma in the UK annually and the incidence is rising. The clinicomorphological types of melanoma include superficial spreading melanoma (73.7 %), nodular melanoma (9.3 94). lentigo maligna melanoma (4.8 %) and acrallentiginous melanoma (3.2%) (Dubin et al., 1986). Other rare types constitute a further 5 %-lo %. Histologically desmoplastic melanoma (DM) has been recognised as a distinct variant of melanoma since the initial report by Conley et al. in 197 1. It is characterised by malignant spindle shaped tumour cells invading the dermis and a desmoplastic stroma (Fig. I). In the majority of patients examination of the skin adjacent to a DM will reveal lentigo maligna (Fig. 2). although a few cases of DM have been reported in association with superficial spreading melanoma. Desmoplasia has also been noted in acral and lentiginous melanoma (Egbert et al., 1988). Conley c’t ul. (1971) described 7 patients, the youngest aged 13 years, with spindle cell tumours and extreme desmoplasia arising from pigmented skin lesions mainly in the head and neck. 4 of the 7 patients died of metastatic melanoma. and the authors emphasised that DM may be deceptively bland yet have the capacity for local recurrence and metastasis. The first British case report was published in 1982 (Berry et cd.) and to date. more than 100 cases of DMM have been reported (Egbert et al., 1988). Patients The medical records of patients on the Melanoma Registry at Frenchay Hospital, Bristol, with a histological diagnosis of Desmoplastic Melanoma were reviewed. Between January 1966 and September 1988, 13 cases were reported, 8 female and 5 male (Table 1).

Fig. I

Results

Figure I-Photomicrograph of patient AT. Hacmatoxylin and Eosin stained slide showing the spindle fibrosarcoma-like cells with no junctional element to give a clue as tn the mclanomatous nature of the tumour.

All lesions were either patches or nodules, 7 of which were pigmented. Five patients had tumours which 595

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British Journal of Plastic Surgery

Fig. 3

Fig. 2 Figure 2-Patient

GR, DM with Lentigo

Figure >Patient

Maligna.

previous

WN. Skin recurrence in a scalp wide excision and split skin graft.

showing

the

Discussion 12 patients at presentation had clinical stage 1 disease, and only one was considered stage 2, (JW), with a palatal DM and nodes in the neck. Breslow tumour thickness averaged 5.78 mm (range 1.92-18.0 mm), Clarke’s level 4/5. Recurrence of local disease occurred in 7 patients, four in the regional nodes and three in the skin (Fig. 3). Of this group, 4 developed disseminated disease with pulmonary metastases. The mean time to first recurrence was 26 months, (range l&45 months) (Table I). The mean follow-up time from initial presentation was 40 months (range 3-141 months).

Table 1 Clinico-pathological

Clinical

Over 2/3 of lesions reported involve the head and neck area and although many present after the sixth decade, 1 in 10 tumours has been reported in patients under 40 years of age. DM appears to be slightly more common in men (male : female ratio 3 : 2), although not so in this series (Table 1). The spindle cell component has indistinct borders and may invade deeply. If not removed early and completely, local recurrence and metastases are common. The metastases may contain either a pure spindle cell tumour, a classical malignant

details

Name

Age/sex

Sire

KC AD DT GR JW* EB BT* JC ow* MD* WN cw* AT

34 f 81 f 70 f 83 ,f 55 f 19 m 53 f 59 m 66 m 64 f 82 m 66 m 71 f

Knee Cheek Nose Cheek Palate Face Thumb Face Thumb Foot Scalp Chin Temple

Breslow (mm) 1.9 2.1 2.9 3.0 3.4 3.1 5.0 5.3 6.0 8.0 9.5 Unassessable 18

* = ulcerated tumour t = dissemination m = male f = female recurrence: time to first recurrence (n = nodal, s = skin) follow-up : from first presentation All biopsies were excisional except for the patients with thumb nail-bed incisional biopsies.

Recurrence (monrhs)

Follow-up (months)

5

30 11 45 23 17 34

5

16s

well well died well tdied well tdied well tdied well well well talive

Clarke 415 5 :,5 5 5 5 5 :,5

s n n n n s

lesions, and two patients

52 25 54 21 30 29 48 3 36 141 4L 12 24

(CW, AT), who underwent

initial

Desmoplastic Melanoma : A Clinico-Pathological

Review

melanoma or a combination of cell types. Jain and Allen (1989) followed up 42 patients with DM, of whom 23 developed one or more local recurrence, 17 developed distant spread. and 14 had both local recurrence and distant spread. Lymph node metastases were not recorded in any patients. Similarly, Beenken et al. (1989) observed that despite much thicker primary tumours than other melanomas and a higher incidence of locally recurrent disease, true lymph node metastasis amongst 14 patients with DM was 3 times less frequent than those patients with non-DM. The margin of excision per se does not influence the local or regional recurrence rate. Tunzortr origin The origin of the desmoplasia from melanocytes (derivatives of the neurocristic cell population) is now generally accepted (Reed, 1983). Thus some desmoplastic melanomas may resemble histologically a pattern of nerve differentiation, or lie proximal to nerves which may be expanded with increased numbers of spindle cells in the perineural space (Reed and Leonard, 1979). This phenomenon, neurotropism, occurs to varying degrees and when marked constitutes a histological sub-type of DM-neurotropic melanoma. Histolog> There appears to be a morphological continuum from pure spindle cell melanomas to DM. Some patients with DM have focal areas typical of spindle cell melanoma, although collagen formation is not as extensive, nor are tumour cells arranged in fascicles as in DM (Jain and Allen, 1989). The histological differential diagnosis is large, and includes both benign and malignant mesenchymal proliferations (Egbert et al., 1988). Electron microscopy may show premelanosomes in the dermal neoplastic cells (From et al., 1983). Antisera to SIOO protein and Vimentin may prove useful as immunohistochemical markers, although NKl /C3 antimelanoma monoclonal antibody is invariably unhelpful (Walsh et al., 1988). A newer antibody, HMB-45, is frequently negative with DM although very useful with other types of melanoma (Cook, 1990). Prognostic

indices

The importance of pathological details including the number of mitoses/high power field, the presence of regression/flare or vascular/lymphatic invasion is as yet unclear. Treatnwnt An excision biopsy is essential. Resection margins in melanoma are determined by the extent of tumour and functional and cosmetic consequences of surgery. Although 2-3 cm may be adequate, DM tumours may extend to the deep resection margin histologically, blending with surrounding normal stroma and wide local re-excision with removal of tissue down to fascia,

Fig. 4 Figure Q-Patient AT. Skin recurrence involved supra-orbltal Pulmonary me&stases followed the recurrence.

nerve.

muscle, periosteum or bone has been advocated (Beenken et al., 1989). These authors also note that lesions overlying the parotid gland should be treated with a wide local excision and superficial parotidectomy. There is a high potential for spread along peripheral nerves. possibly related to the neurotropic tendency, and involved nerves may require resection in continuity. Spread along the inferior alveolar nerve resulted in a hemi-mandibulectomy (patient JW), (Table 1) and another patient (AT) developed a skin recurrence which involved the supra-orbital nerve (Fig. 4). Similarly, spread along the infra-orbital nerve requiring orbital exenteration has been reported (Reiman et ul., 1987). Two patients who developed local recurrence at 17 and 34 months from the initial presentation, underwent a nodal clearance or wide local excision. Both remained disease free at review (141 and 41 months respectively)., Elective neck dissection is not thought appropriate in DM as the incidence of locally recurrent disease is higher than true lymph node metastasis (Beenken P! ~1.. 1989). The possible role of chemotherapy, immunotherapy, radiotherapy and adjuvant therapy is not known (Reiman et ul.. 1987: Beenken et al., 1989). DM, although rare. occurs with significant frequency to justify recognition on clinical and histological grounds. The clinical presentation may be atypical local recurrence or metastatic tumour may present as a vague subcutaneous nodule. The high potential for perineural spread requires aggressive local resection but the overall prognosis is poor. The spindle cell desmoplasic component may be amelanotic, histologically bland and overlooked. The difficulty in defining the full extent of the lesion and adequacy ofclearance may require multiple sections or further specimens. A variable degree of neurotropism, often with lack of melanin pigment. may further encourage misdiagnosis.

Acknowledgement The authors Reconstructive

would like to thank Surgeons at Frenchay

the Consultant Plastic and Hospital. Bristol. Mr R. W.

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British Journal

of Plastic Surgery

Hiles, Mr R. W. Pigott, Mr P. L. G. Townsend and Mr C. D. Reid for allowing them to report on their cases.

Reed, R. J. and Leonard, D. D. (1979). Neurotropic Melanoma: a variant of Desmoplastic Melanoma. American Journal of Surgica/

References

Reiman, H. M., Goellner, J. R., Shields, J. A., Elder, D., Arbizo, V., Hedges, T. and Augsburger, J. (1987). Orbital involvement with Desmoplastic Melanoma. British Journal of Ophthalmology, 71,

Pathology, 3, 301.

Beenken, S., Byers, R., Smith, L., Goepfert, H. and Shallenberger, R. (1989). Desmoplastic Melanoma. Histological correlation with behavior and treatment. Archives of Otolaryngology, Head and Neck Surgery, 115, 374. Berry, R. B., Subbuswamy, S. G. and Hackett, M. E. (1982).

Desmoplastic

Malignant

Melanoma:

The first British report.

British Journal of Plastic Surgery, 35, 324. Conley, J., Lattes, R. and Orr, W. (1971). Desmoplastic Malignant Melanoma (A rare variant of spindle cell melanoma). Cancer, 28. 914. Cook, M. (1990). Personal communication. Dubin, N., Moseson, M. and Pasrernack, B. (1986). Epidemiology of

malignant melanoma: pigmentary traits, ultraviolet radiation, and the identification of high-risk populations. Recent Research in Cuncer Research. 102, 56. Egbert, B., Kempson, R. and Sagebiel,

R. (1988). Desmoplastic Malignant Melanoma. A clinicohistopathologic study of 25 cases.

Cancer, 62, 2033. From, L., Hanna, W., Kahn, H. J., Gross, J., Marks, A. and Baumal, R. (1983). Origin of desmoplasia in desmoplastic malignant melanoma. Human Pathology, 4, 1072. Jain. S. and Allen. P. W. C1989). Desmoulastic Malignant Melanoma and its variants. American journal ofSurgical Pathology, 13.358. Man, D., Weiner, L. J. and Reiman, H. M. Jr. (1981). Desmoplastic Malignant Melanoma. British Journal of Plastic Surgery, 34, 19. Reed, R. J. (1983). Neuromesenchyme. American Journal of Dermatology und Pathology. 5, 358.

279. Valensi, Q. J. (1977). Desmoplastic Malignant Melanoma. A report on two additional cases. Cancer, 39. 286. Walsh, N., Roberts, J., Orr, W. and Simon, G. (1988).Desmoplastic

Malignant Melanoma. A clinicopathologic

study of 14 cases.

Archives of Pathology and Laboratory Medicine. 112, 922.

The Authors V. S. Devaraj, FRCS, Senior Registrar, Department

of Plastic and Reconstructive Surgery, The Radcliffe Infirmary. Oxford ; formerly S.H.O., Department of Plastic Surgery, Frenchay Hospital, Bristol. A. L. H. Moss, FRACS, Consultant Plastic and Reconstructive Surgeon, St. George’s Hospital, London; formerly Senior Registrar, Department of Plastic Surgery, Frenchay Hospital. J. C. Briggs, FRCPatb, Consultant Histopathologist. Frenchay Hospital, Bristol BS16 1LE. Requests for reprints to Dr Briggs.

This paper is an update of one presented at the summer meeting of the British Association of Plastic Surgeons, in Bristol, July 4-5, 1989. Paper received 30 January 1991. Accepted 7 April 1992, after revision.

Desmoplastic melanoma: a clinico-pathological review.

Desmoplastic Melanoma (DM) represents a rare histological variant of melanoma. It has been described in isolated case reports as a cutaneous tumour wi...
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