Acral lentiginous melanoma | |
---|---|
Specialty | Oncology, dermatology |
Symptoms | Areas of dark pigmentation [1] |
Causes | Malignant melanocytes [2] [3] |
Diagnostic method | Biopsy [4] |
Treatment | Biologic immunotherapy agents [5] |
Frequency | Males = Females [6] |
Acral lentiginous melanoma is a type of skin cancer. [6] It typically begins as a uniform brownish mark before becoming darker and wider with a blurred irregular edge, most frequently seen in the foot of a person with darker skin. [6] It may become bumpy and ulcerate. [6] Just under the nail it typically appears as dark longitudinal streaks, and it may spread. [7]
Melanoma is a group of serious skin cancers that arise from pigment cells (melanocytes); acral lentiginous melanoma is a kind of lentiginous [8] skin melanoma. [6] Acral lentiginous melanoma is the most common subtype in people with darker skins and is rare in people with lighter skin types. [7] It is not caused by exposure to sunlight or UV radiation, and wearing sunscreen does not protect against it. Acral lentiginous melanoma is commonly found on the palms, soles, under the nails, and in the oral mucosa. It occurs on non-hair-bearing surfaces of the body, which have not necessarily been exposed to sunlight. It is also found on mucous membranes. [9]
The absolute incidence of ALM is the same for people of all skin colors, and has not changed significantly for decades. [9] However, because rates of other melanomas are low in non-white populations, ALM is the most common form of melanoma diagnosed amongst Asian and sub-Saharan African ethnic groups. [10] The average age at diagnosis is between sixty and seventy years. [11]
Males and females are affected equally. [6]
Typical signs of acral lentiginous melanoma include the following [1]
Warning signs are new areas of pigmentation, or existing pigmentation that shows change. If caught early, acral lentiginous melanoma has a similar cure rate as the other types of superficial spreading melanoma. [12]
Acral lentiginous melanoma is a result of malignant melanocytes at the membrane of the skin (outer layers). [2] [3] The pathogenesis of acral lentiginous melanoma remains unknown at this time. [13] It is not caused by sunlight or UV radiation. [9]
Although the ideal method of diagnosis of melanoma is complete excisional biopsy, [14] alternatives may be required according to the location of the melanoma. Dermatoscopy of acral pigmented lesions is very difficult but can be accomplished with diligent focus. Initial confirmation of the suspicion can be done with a small wedge biopsy or small punch biopsy. [4] Thin deep wedge biopsies can heal very well on acral skin, and small punch biopsies can give enough clue to the malignant nature of the lesion. Once this confirmatory biopsy is done, a second complete excisional skin biopsy can be performed with a narrow surgical margin (1 mm). This second biopsy will determine the depth and invasiveness of the melanoma, [15] and will help to define what the final treatment will be. If the melanoma involves the nail fold and the nail bed, complete excision of the nail unit might be required. Final treatment might require wider excision (margins of 0.5 cm or more), digital amputation, lymphangiogram with lymph node dissection, or chemotherapy. [16]
The main characteristic of acral lentiginous melanoma is continuous proliferation of atypical melanocytes at the dermoepidermal junction. [17] Other histological signs of acral lentiginous melanoma include dermal invasion and desmoplasia. [18]
According to Scolyer et al., [19] ALM "is usually characterized in its earliest recognisable form as single atypical melanocytes scattered along the junctional epidermal layer".
Therapies for metastatic melanoma include the biologic immunotherapy agents ipilimumab, pembrolizumab, and nivolumab; BRAF inhibitors, such as vemurafenib and dabrafenib; and a MEK inhibitor trametinib. [5]
When arising in the nailbed of a digit, the evidence suggests that digit-sparing surgery (wide excision and grafting) has similar outcomes to amputation, [20] therefore, to preserve function it is recommended that clinicians default to digit-sparing surgery and if the margins are involved or patients develop recurrence, then secondary amputation can be considered.
It has been demonstrated that acral lentiginous melanoma has a poorer prognosis compared to that of cutaneous malignant melanoma (CMM). [21]
Jamaican musician Bob Marley died of the condition in 1981, at the age of 36. [22]
A melanocytic nevus is usually a noncancerous condition of pigment-producing skin cells. It is a type of melanocytic tumor that contains nevus cells. A mole can be either subdermal or a pigmented growth on the skin, formed mostly of a type of cell known as a melanocyte. The high concentration of the body's pigmenting agent, melanin, is responsible for their dark color. Moles are a member of the family of skin lesions known as nevi, occurring commonly in humans. Some sources equate the term "mole" with "melanocytic nevus", but there are also sources that equate the term "mole" with any nevus form.
Melanocytes are melanin-producing neural crest-derived cells located in the bottom layer of the skin's epidermis, the middle layer of the eye, the inner ear, vaginal epithelium, meninges, bones, and heart found in many mammals and birds. Melanin is a dark pigment primarily responsible for skin color. Once synthesized, melanin is contained in special organelles called melanosomes which can be transported to nearby keratinocytes to induce pigmentation. Thus darker skin tones have more melanosomes present than lighter skin tones. Functionally, melanin serves as protection against UV radiation. Melanocytes also have a role in the immune system.
Melanoma is the most dangerous type of skin cancer; it develops from the melanin-producing cells known as melanocytes. It typically occurs in the skin, but may rarely occur in the mouth, intestines, or eye. In women, melanomas most commonly occur on the legs; while in men, on the back. Melanoma is frequently referred to as malignant melanoma. However, the medical community stresses that there is no such thing as a 'benign melanoma' and recommends that the term 'malignant melanoma' should be avoided as redundant.
Nevus is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. The term originates from nævus, which is Latin for "birthmark"; however, a nevus can be either congenital or acquired. Common terms, including mole, birthmark, and beauty mark, are used to describe nevi, but these terms do not distinguish specific types of nevi from one another.
Superficial spreading melanoma (SSM) is a type of skin cancer that typically starts as an irregularly edged dark spot typically on sun-exposed part of the body. The colour may be variable with dark, light and reddish shades; occasionally no color at all. It typically grows in diameter before spreading to deeper tissue, forming a bump or becoming an ulcer. Itching, bleeding and crust formation may occur in some. The backs and shoulders of males and legs of women are particularly prone.
A dysplastic nevus or atypical mole is a nevus (mole) whose appearance is different from that of common moles. In 1992, the NIH recommended that the term "dysplastic nevus" be avoided in favor of the term "atypical mole". An atypical mole may also be referred to as an atypical melanocytic nevus, atypical nevus, B-K mole, Clark's nevus, dysplastic melanocytic nevus, or nevus with architectural disorder.
Ocular melanosis (OM) is a blue-gray and/or brown lesion of the conjunctiva that can be separated into benign conjunctival epithelial melanosis (BCEM) and primary acquired melanosis (PAM), of which the latter is considered a risk factor for uveal melanoma. The disease is caused by an increase of melanocytes in the iris, choroid, and surrounding structures. Overproduction of pigment by these cells can block the trabecular meshwork through which fluid drains from the eye. The increased fluid in the eye leads to increased pressure, which can lead to glaucoma. In humans, this is sometimes known as pigment dispersion syndrome.
Lentigo maligna is where melanocyte cells have become malignant and grow continuously along the stratum basale of the skin, but have not invaded below the epidermis. Lentigo maligna is not the same as lentigo maligna melanoma, as detailed below. It typically progresses very slowly and can remain in a non-invasive form for years.
A lentigo is a small pigmented spot on the skin with a clearly defined edge, surrounded by normal-appearing skin. It is a harmless (benign) hyperplasia of melanocytes which is linear in its spread. This means the hyperplasia of melanocytes is restricted to the cell layer directly above the basement membrane of the epidermis where melanocytes normally reside. This is in contrast to the "nests" of multi-layer melanocytes found in moles. Because of this characteristic feature, the adjective "lentiginous" is used to describe other skin lesions that similarly proliferate linearly within the basal cell layer.
A blue nevus is a type of coloured mole, typically a single well-defined blue-black bump.
Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 days. It is commonly performed by dermatologists. Skin biopsies are also done by family physicians, internists, surgeons, and other specialties. However, performed incorrectly, and without appropriate clinical information, a pathologist's interpretation of a skin biopsy can be severely limited, and therefore doctors and patients may forgo traditional biopsy techniques and instead choose Mohs surgery.
Amelanotic melanoma is a type of skin cancer in which the cells do not make any melanin. They can be pink, red, purple or of normal skin color, and are therefore difficult to diagnose correctly. They can occur anywhere on the body, just as a typical melanoma can.
Melanonychia is a black or brown pigmentation of a nail, and may be present as a normal finding on many digits in Afro-Caribbeans, as a result of trauma, systemic disease, or medications, or as a postinflammatory event from such localized events as lichen planus or fixed drug eruption.
Nevus spilus, also known as speckled lentiginous nevus, is a light brown or tan birth mark, speckled with small, dark spots or small bumps. If it occurs in a segmental pattern then it is sometimes referred to as a Zosteriform speckled lentiginous nevus.
A benign melanocytic nevus is a cutaneous condition characterised by well-circumscribed, pigmented, round or ovoid lesions, generally measuring from 2 to 6 mm in diameter. A benign melanocytic nevus may feature hair or pigmentation as well.
Pseudomelanoma is a cutaneous condition in which melanotic skin lesions clinically resemble a superficial spreading melanoma at the site of a recent shave removal of a melanocytic nevus.
Balloon cell nevus is a benign nevus. It appears like a melanocytic nevus.
Oral pigmentation is asymptomatic and does not usually cause any alteration to the texture or thickness of the affected area. The colour can be uniform or speckled and can appear solitary or as multiple lesions. Depending on the site, depth, and quantity of pigment, the appearance can vary considerably.
An acral nevus is a cutaneous condition of the palms, soles, fingers, or toes, characterized by a skin lesion that is usually macular or only slightly elevated, and may display a uniform brown or dark brown color, often with linear striations.
Animal-type melanoma is a rare subtype of melanoma that is characterized by heavily pigmented dermal epithelioid and spindled melanocytes. Animal-type melanoma is also known to be called equine-type melanoma, pigment synthesizing melanoma, and pigmented epithelioid melanocytoma (PEM). While melanoma is known as the most aggressive skin cancer, the mortality for PEM is lower than in other melanoma types. Animal-type melanoma earned its name due to the resemblance of melanocytic tumors in grey horses.