Acral lentiginous melanoma

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Acral lentiginous melanoma
Melanoma (1).jpg
Specialty Oncology, dermatology   OOjs UI icon edit-ltr-progressive.svg
Symptoms Areas of dark pigmentation [1]
CausesMalignant melanocytes [2] [3]
Diagnostic method Biopsy [4]
TreatmentBiologic immunotherapy agents [5]
FrequencyMales = Females [6]

Acral lentiginous melanoma (ALM) is a type of skin cancer. [6] It typically begins as a uniform brownish mark before becoming darker and wider with a blurred, irregular border. ALM is most frequently seen on the foot of a person with darker skin but can also be found in non-sun exposed areas such as the palms, soles, and under finger and toenails. [6] It may become bumpy and ulcerate. [6] When under the nail it typically appears as dark longitudinal streaks. [7] As it grows, ALM may also spread to other areas of the body. [7]

Contents

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] ALM makes up less than 5% of all melanomas, but is considered the most common subtype in people with darker skin and is rare in people with lighter skin types. [9] It is not caused by exposure to sunlight or UV radiation, and wearing sunscreen does not protect against it. 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. [10]

Epidemiology

The absolute incidence of ALM is the same for people of all skin colors, and has not changed significantly for decades. [10] 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. [11] The average age at diagnosis is between sixty and seventy years. [12] Males and females are affected equally, but females tend to be diagnosed at earlier stages. [6] [7]

Signs and symptoms

Typical signs of acral lentiginous melanoma include the following [1]

Other uncommon presentations can include:

Warning signs are new areas of pigmentation, or existing pigmentation that shows change. If caught early, ALM has a similar cure rate as the other types of superficial spreading melanoma. [14] In contrast to cutaneous melanoma which utilizes the ABCDE rule (Asymmetry, Border, Color, Diameter, Evolving) to help identify lesions suspicious for skin cancer, an alternative pneumonic CUBED (Colored lesion, Uncertain diagnosis, Bleeding lesion, Enlargement of the lesion, Delay in healing) has been proposed for ALM based on the differences in signs and presentations between the two cancers. [15]

ALM can also cause other non-specific symptoms if it spreads to certain areas of the body: [16]

Causes

Acral lentiginous melanoma is a result of malignant melanocytes at the membrane of the skin (outer layers). [2] [3] The pathogenesis of ALM remains unclear, however injury or mechanical stress might play a role in its development. [17] [18] Unlike cutaneous melanoma, it is not caused by sunlight or UV radiation. [10]

Diagnosis

Graphic illustrating a punch biopsy Skin Punch Biopsy.png
Graphic illustrating a punch biopsy

Although the ideal method of diagnosis of melanoma is complete excisional biopsy, [19] alternative methods may be required based on 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 may provide enough information to suggest if a lesion is cancerous. 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, [20] and will help to guide further treatment if necessary. In order to establish the Breslow's depth of the lesion, the most raised section of the pigmented area should be sampled. [21] If the melanoma involves the nail fold or the nail bed, complete excision of the nail unit might be required for accurate sampling.

In the event that the melanoma spreads to other sites such as the lymph nodes, another biopsy called the Sentinel lymph node biopsy may provide more information in terms of outcomes. [21] More extensive melanomas may require wider excision (margins of 0.5 cm or more), digital amputation, lymphangiogram with lymph node dissection, or chemotherapy. [22]

Histology

Acral lentiginous melanoma (ALM) Skin Tumors-027.jpg
Acral lentiginous melanoma (ALM)

The main characteristic of acral lentiginous melanoma is continuous proliferation of atypical melanocytes at the dermoepidermal junction. [23] Other histological signs of acral lentiginous melanoma include dermal invasion and desmoplasia. [24] This invasion usually occurs many years after the initial lesion first appears. [7]

According to Scolyer et al., [25] ALM "is usually characterized in its earliest recognisable form as single atypical melanocytes scattered along the junctional epidermal layer".

Treatment

The mainstay of treatment of acral lentiginous melanoma is wide local excision. [7] If metastatic, biologic immunotherapy agents like ipilimumab, pembrolizumab, and nivolumab; BRAF inhibitors, such as vemurafenib and dabrafenib; or a MEK inhibitor trametinib may be used. [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, [26] therefore, to preserve function and aesthetics it is recommended that clinicians default to digit-sparing surgery. Secondary amputation may be considered if the surgery margins are not clear of cancerous cells, or if patients develop a recurrence of the melanoma.

Prognosis

The prognosis of acral lentiginous melanoma is based on multiple factors including gender, age, race, Breslow depth, staging, and sentinel lymph node positivity. [7] Out of these factors, it is believed that sentinel lymph node positivity provides the strongest prediction of cancer recurrence and death. [27] [28] When compared to cutaneous malignant melanoma (CMM), ALM has a poorer prognosis in terms of survival rates. [29] This poorer prognosis is thought to be related to the fact that ALM is usually diagnosed at a later stage than other skin cancers; which may be due to ALM occurring on areas of the body that are harder to notice, especially in the elderly population. [7]

Prevention

Since acral lentiginous melanoma is not linked to sun or UV exposure and the cause is not well-understood, there are no specific preventative measures. [7] However, Patient education can be geared towards populations in which ALM is more common to increase awareness of the warning signs of ALM and other melanomas to help with earlier detection. [30] Early detection is one of the most important factors in disease-specific survival. [7]

Differential Diagnoses

Image of a toenail fungal infection presenting similarly to ALM Paronychia green discolor.jpg
Image of a toenail fungal infection presenting similarly to ALM

Other benign skin lesions that may mimic acral lentiginous melanoma include: Lentigo (sun spots), Acral Nevi (moles), or Onychomycosis (fungal infection of the nail). These skin lesions can appear as dark brown spots, like ALM, but can be distinguished on close examination or dermatoscopy. [7] Other types of skin cancers like squamous cell carcinoma can also present similarly to ALM. [7]

Society and culture

Jamaican musician Bob Marley died of the condition in 1981, at the age of 36. [31]

See also

Related Research Articles

<span class="mw-page-title-main">Melanocytic nevus</span> Benign skin tumor of pigment-producing cells

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.

<span class="mw-page-title-main">Melanoma</span> Skin cancer originating in melanocytes

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.

<span class="mw-page-title-main">Nevus</span> Mole or birthmark; visible, circumscribed, chronic skin lesion

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.

<span class="mw-page-title-main">Superficial spreading melanoma</span> Medical condition

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.

<span class="mw-page-title-main">Dysplastic nevus</span> Mole in the skin with an abnormal appearance

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.

Lymph node biopsy is a test in which a lymph node or a piece of a lymph node is removed for examination under a microscope.

<span class="mw-page-title-main">Lentigo maligna</span> Medical condition

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.

<span class="mw-page-title-main">Lentigo</span> Small pigment spots on skin

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.

<span class="mw-page-title-main">Vulvar cancer</span> Cancer involving the vulva

Vulvar cancer is a cancer of the vulva, the outer portion of the female genitals. It most commonly affects the labia majora. Less often, the labia minora, clitoris, or Bartholin's glands are affected. Symptoms include a lump, itchiness, changes in the skin, or bleeding from the vulva.

<span class="mw-page-title-main">Blue nevus</span> Type of melanocytic tumor

A blue nevus is a type of coloured mole, typically a single well-defined blue-black bump.

<span class="mw-page-title-main">Skin biopsy</span> Removal of skin cells for medical examination

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.

<span class="mw-page-title-main">Amelanotic melanoma</span> Medical condition

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.

<span class="mw-page-title-main">Benign melanocytic nevus</span> Medical condition

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.

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.

<span class="mw-page-title-main">Acral nevus</span> Medical condition

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.

Skin cancer, or neoplasia, is the most common type of cancer diagnosed in horses, accounting for 45 to 80% of all cancers diagnosed. Sarcoids are the most common type of skin neoplasm and are the most common type of cancer overall in horses. Squamous-cell carcinoma is the second-most prevalent skin cancer, followed by melanoma. Squamous-cell carcinoma and melanoma usually occur in horses greater than 9-years-old, while sarcoids commonly affect horses 3 to 6 years old. Surgical biopsy is the method of choice for diagnosis of most equine skin cancers, but is contraindicated for cases of sarcoids. Prognosis and treatment effectiveness varies based on type of cancer, degree of local tissue destruction, evidence of spread to other organs (metastasis) and location of the tumor. Not all cancers metastasize and some can be cured or mitigated by surgical removal of the cancerous tissue or through use of chemotherapeutic drugs.

Vaginal melanoma is a rare malignancy that originates from melanocytes in the vaginal epithelium. It is also known as a melanocytic tumor or as a malignant melanoma. It is aggressive and infrequently cured. The median overall survival is 16 months. Vaginal melanoma accounts 5.5% of all vaginal cancers and only 1% of all melanomas diagnosed in women. Vaginal melanomas are frequently diagnosed in advanced stages of the disease. The prognosis is poor and the most important risk factor is the presence of lymph node metastases.

References

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