Melanoma of nail unit

Introduction

Melanoma of the nail unit is usually a variant of acral lentiginous melanoma (melanoma arising on the palms of the hands and soles of the feet). Other types of melanoma rarely arising under the nails are nodular melanoma and desmoplastic melanoma.

Melanoma of the nail unit usually affects either a thumbnail or great toenail, but any finger or toenail may be involved. The term includes:

• Subungual melanoma (melanoma originating from the nail matrix)
• Ungual melanoma (melanoma originating from under the nail plate)
• Periungual melanoma (melanoma originating from the skin beside the nail plate)

Who gets melanoma of nail unit?

Melanoma of the nail unit is rare, accounting for only about 1% melanoma in white-skinned individuals. It arises in people of all races, whatever their skin colour. Although no more common in dark skin than fair skin, it is the most common type of melanoma diagnosed in deeply pigmented individuals. It is most diagnosed between the age of 40 and 70 [1].

It is not thought to be due to sun exposure. Trauma may be a factor, accounting for the greater incidence in the great toe and thumb.

Management of melanoma is evolving. For up to date recommendations, refer to the Australian Cancer Council Clinical practice guidelines for the diagnosis and management of melanoma

What does melanoma of the nail unit look like?

Subungual melanoma often starts as a pigmented band visible the length of the nail plate (melanonychia). Over weeks to months, the pigment band:

• Becomes wider, especially at its proximal end (cuticle)
• Becomes more irregular in pigmentation including light brown, dark brown
• Extends to involve the skin of the adjacent proximal or lateral nail fold (Hutchinson sign)
• May develop a nodule, ulcerate or bleed
• May cause thinning, cracking or distortion of the nail plate (nail dystrophy).

However, in up to half of all cases, subungual melanoma is amelanotic (not pigmented). Ungual melanoma can form a nodule under the nail plate, lifting it (onycholysis). It may sometimes look like a wart (verrucous). It is usually painless, but an advanced tumour invading underlying bone may cause severe pain.

A melanocytic naevus of the nail matrix results in a pigmented band (melanonychia). This tends to be narrower than 3 mm (but can be wider) and a uniform brown or dark brown colour. Benign pigmentation observed in the cuticle, or proximal nail fold is referred to as pseudo-Hutchinson sign.

Melanoma of nail unit

Melanoma of nail unit

Melanoma of nail unit

See more images of melanoma of the nail unit.

How is the diagnosis of nail unit melanoma made?

Subungual melanoma may be suspected clinically because of a wide (> 3 mm) new or changing pigment band in a single nail. The dermatoscopic examination may reveal more details showing pigmented lines of varying colour, width and spacing. These lines tend to lose their usual tendency to run parallel to each other along the length of the nail. Ungual melanoma forms a non-pigmented lump under the nail plate, eventually resulting in its destruction.

The diagnosis of melanoma is confirmed by biopsy of the nail matrix and nail bed. It can be a difficult diagnosis requiring examination by expert dermatopathologists. The pathologist should report whether the melanoma is in-situ or invasive. The description of invasive melanoma should include its thickness in millimetres and what level of tissue has been invaded.

If the clinical diagnosis is a subungual haematoma (a purple mark under the nail due to bleeding or bruising), the nail may be observed for a few weeks. Normal-appearing nail should then be seen growing behind the mark. Dermoscopy is helpful, as haematoma does not conform to the band-like pattern of subungual melanoma. However, it must be remembered that melanoma may bleed.

Other diagnoses that are often considered include onychomycosis (fungal infection), paronychia, pyogenic granuloma and squamous cell carcinoma. See also subungual melanoma pathology.

Algorithms for diagnosis of melanoma of the nail unit

ABCD criteria for subungual melanoma in situ

Lee et al have suggested ABCD nail criteria to diagnose subungual melanoma in situ when a patient presents with longitudinal melanonychia. The diagnosis was confirmed in their 26 patients with subungual melanoma in situ and 28 patients with nail matrix naevi [2].

• A — adult age (age >18 years)
• B — brown bands in a brown background
• C — colour in periungual skin
• D — one digit.

ABCDEF guidelines for pigmented nail lesions

Levit et al described ABCDEF guidelines to assess pigmented nail lesions [3].

• A: Age 40–70 years of age; African, Japanese, Chinese, and Native American heritage
• B: Brown-black band ≥ 3mm with variegated borders
• C: Change or lack of change in the nail band or nail morphology
• D: Digit most commonly involved (thumb, big toe, or index finger)
• E: Extension of discolouration into the skin surrounding the nail (Hutchinson sign)
• F: Family or personal history of melanoma

Dermoscopic clues to differentiate pigmented nail bands

In the International Dermoscopy Society survey evaluating dermoscopic clues in pigmented nail bands, melanoma cases were significantly associated with [4]:

• The width of the pigmented band occupying more than two-thirds of the nail plate
• Grey and black colours
• Irregularly pigmented lines
• Hutchinson sign and micro-Hutchinson sign (skin pigmentation noted on dermoscopy but not on clinical examination)
• Nail dystrophy.

Granular pigmentation, a newly defined dermoscopic criterion, was found in 40% of melanomas and only in 3.51% of benign lesions. The dermoscopic feature of any pigmentation in the hyponychia (the distal pulp of the finger under the nail plate) is parallel pigmented lines on the ridges (in contrast to naevi in which the pigmentation affects furrows or may be diffuse).

What is the treatment of melanoma of the nail unit?

The melanoma must be removed surgically. This requires removal of the entire nail apparatus. Sometimes the end of the finger or toe is amputated.

Some patients may be offered sentinel node biopsy to determine whether the melanoma has spread to local lymph nodes.

What is the outlook for patients with melanoma affecting the nail unit?

The main factor associated with the risk of spread of melanoma (metastasis) and death is the thickness of the melanoma at the time of complete excision of the primary tumour. Delay in diagnosis is common with subungual melanoma, particularly when it affects the toe, and some of these tumours have already spread at the time of diagnosis.

The 5-year survival rate ranges widely from 16% to 87%, depending on the series, with two larger series in the 51% to 55% range [5].