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Lentigo maligna melanoma is a melanoma that has evolved from a lentigo maligna. They are usually found on chronically sun damaged skin such as the face and the forearms of the elderly. The nomenclature is very confusing to both patients and physicians alike.
Lentigo maligna is the non-invasive skin growth that some pathologists consider to be a melanoma-in-situ. A few pathologists do not consider lentigo maligna to be a melanoma at all, but a precursor to melanomas. Once a lentigo maligna becomes a lentigo maligna melanoma, it is treated as if it were an invasive melanoma.
Lentigo maligna (also known as "lentiginous melanoma on sun-damaged skin") is a melanoma "in situ" that consists of malignant cells but does not show invasive growth. Lentigo maligna is not the same as lentigo maligna melanoma, and should be discussed separately. It typically progresses very slowly and can remain in a non-invasive form for years. The transition to true melanoma is marked by the appearance of a bumpy surface (itself a marker of vertical growth and invasion), at which point it is called lentigo maligna melanoma. It is normally found in the elderly (peak incidence in the 9th decade), on skin areas with high levels of sun exposure like the face and forearms. Some authors do not consider lentigo maligna to be a melanoma. It is commonly thought of as a melanoma precursor. Incidence of evolution to lentigo maligna melanoma is very low, about 2.2% to 5% in elderly patients.
It is also known as "Hutchinson's melanotic freckle". This is named for Jonathan Hutchinson.
Spitz nevi are uncommon. Their annual incidence was estimated in a coastal population of sub-tropical Queensland to be 1.4 cases per 100,000 people. For comparison, the annual incidence of melanoma in the same population, which is high by world standards is 25.4 cases per 100,000 people.
Although they are most commonly found on people in their first two decades of life, the age range for people with Spitz nevi is from 6 months to 71 years, with a mean age of 22 years and a median age of 19 years.
Ultraviolet light from the sun causes premature aging of the skin and skin damage that can lead to melanoma. Some scientists hypothesize that overexposure to UV, including excessive sunlight, may play a role in the formation of acquired moles. However, more research is needed to determine the complex interaction between genetic makeup and overall exposure to ultraviolet light. Some strong indications that this is so (but falling short of proof), are:
- The relative lack of moles on the buttocks of people with dysplastic nevi.
- Freckles (spots of melanin on the skin, and distinct from moles) are known to be influenced by sunlight.
Studies have found that sunburns and too much time in the sun can increase the risk factors for melanoma. This is "in addition to" those who have dysplastic nevi being at higher risk of this cancer (the uncertainty is in regard to acquiring "benign" moles). To prevent and reduce the risk of melanoma caused by UV radiation, the American Academy of Dermatology and the National Cancer Institute recommends staying out of the sun between 10 a.m. and 4 p.m. standard time (or whenever one's shadow is shorter than one's height). The National Cancer Institute also recommends wearing long sleeves and trousers, hats with a wide brim, sunscreens, and sunglasses that have UV-deflecting lenses.
An invasive tumor arising from a classical lentigo maligna. Usually a darkly pigmented raised papule or nodule, arising from a patch of irregularly pigmented flat brown to dark brown lesion of sun exposed skin of the face or arms in an elderly patient.
The best treatment of lentigo maligna is not clear as it has not been well studied.
Standard excision is still being done by most surgeons. Unfortunately, the recurrence rate is high (up to 50%). This is due to the ill defined visible surgical margin, and the facial location of the lesions (often forcing the surgeon to use a narrow surgical margin). The use of dermatoscopy can significantly improve the surgeon's ability to identify the surgical margin. The narrow surgical margin used (smaller than the standard of care of 5 mm), combined with the limitation of the standard bread loafing technique of fixed tissue histology - result in a high "false negative" error rate, and frequent recurrences. Margin controlled (peripheral margins) is necessary to eliminate the false negative errors. If breadloafing is utilized, distances from sections should approach 0.1 mm to assure that the method approaches complete margin control.
Where the lesion is on the face and either large or 5mm margins are possible, a skin flap or skin graft may be indicated/required. Grafts have their own risks of failure and poor cosmetic outcomes. Flaps can require extensive incision resulting in long scars and may be better done by plastic surgeons (and possibly better again by those with extensive LM or "suspicious of early malignant melanoma" experience.
Mohs surgery has been done with cure rate reported to be 77%. The "double scalpel" peripheral margin controlled excision method approximates the Mohs method in margin control, but requires a pathologist intimately familiar with the complexity of managing the vertical margin on the thin peripheral sections and staining methods.
Some melanocytic nevi, and melanoma-in-situ (lentigo maligna) have resolved with an experimental treatment, imiquimod (Aldara) topical cream, an immune enhancing agent. In view of the very poor cure rate with standard excision, some surgeons combine the two methods: surgical excision of the lesion, then three months treatment of the area with imiquimod cream.
Studies seem to conflict about the level of certainty associated with using imiquimod.
Another treatment to be considered where standard margins cannot be achieved or cosmetics are a major consideration is ultra-soft x-ray/grenz-ray radiation.
In the very elderly or those with otherwise limited life expectancy, the impact of major day surgery for excision with 5mm margins and large skin flap could be worse than doing nothing or the possibility of failed treatments with imiquimod or Grenz ray.
Superficial spreading melanoma (also known as "superficially spreading melanoma") (SSM) is usually characterized as the most common form of cutaneous melanoma in Caucasians. The average age at diagnosis is in the fifth decade, and it tends to occur on sun-exposed skin, especially on the backs of males and lower limbs of females.
Experts, such as the American Academy of Dermatology, say that vast majority of moles are benign. Nonetheless, the U.S. National Cancer Institute estimated that 62,480 new cases of melanoma and 8,420 related deaths would appear in the United States in the year 2008.
Data on the chances of transformation from melanocytic nevi to melanoma is controversial, but it appears that about 10% of malignant melanomas have a precursor lesion, of which about 10% are melanocytic nevi. Therefore, it appears that malignant melanoma quite seldomly (1% of cases) has a melanocytic nevi as a precursor.
Nevi and melanomas are a group of neoplasia.
Although a nevus and a melanoma are often treated as independent entities, there is evidence that a nevus can be a precursor for a melanoma.
Common mutations have been identified in nevi and melanomas.
Treatment is by excisional biopsy, wide local excision and possibly sentinel node biopsy. Spread of disease to local lymph nodes or distant sites (typically brain, bone, skin and lung) marks a decidedly poor prognosis.
The cause of Spitz nevi is not yet known. There is an association with sunburn, but causation is not established. Genetic studies of Spitz nevi have shown that most cells have the normal number of chromosomes, however a minority (25%) of cells have been shown to contain extra copies of parts of some chromosomes, such as the short arm of chromosome 11 (11p).
Spitz nevi characteristically have vertically arranged nests of nevus cells that have both a spindled and an morphology. Apoptotic cells may be seen at the dermoepidermal junction. The main histologic differential diagnoses are pigmented spindle cell nevus and malignant melanoma.
The decision to observe or treat a nevus may depend on a number of factors, including cosmetic concerns, irritative symptoms (e.g., pruritus), ulceration, infection, and concern for potential malignancy.
A pigmented spindle cell nevus (also known as a "Pigmented spindle cell tumor of Reed," and "Pigmented variant of Spitz nevus") is a cutaneous condition characterized by a dark brown to black macule or papule, usually less than 6 mm.
It was characterized in 1975.
Halo nevi are estimated to be present in approximately 1% of the general population, and are found to be more prevalent in people with vitiligo, malignant melanoma, or Turner syndrome. All races and sexes are equally susceptible to this disease, although a familial tendency has been reported. The average age of onset is in a person's teenage years.
Screening for melanoma in FAMMM kindreds should begin at age 10 with a baseline total body skin examination including scalp, eyes, oral mucosa, genital area, and nail, as family members may develop melanoma in their early teens.
At Mayo Clinic, FAMMM patients with a confirmed mutation and family history of pancreatic cancer are offered screening with either high-resolution pancreatic protocol CT, MRI, or endoscopic ultrasound starting at age 50 or 10 years younger than the earliest family member with pancreas cancer. They are counseled on the lack of evidence-based data to support screening, and on the limitations of our current technology to detect a lesion at a stage amenable to therapy.
Large and especially giant congenital nevi are at higher risk for malignancy degeneration into melanoma. Because of the premalignant potential, it is an acceptable clinical practice to remove congenital nevi electively in all patients and relieve the nevocytic overload.
Melanoma with features of a Spitz nevus (also known as a "Spitzoid melanoma") is a cutaneous condition characterized histologically with tissue similar to a spitz nevus and with overall symmetry and a dermal nodule of epithelioid melanocytes that do not mature with progressively deeper dermal extension.
Also known as "scoop", "scallop", or "shave" excisional biopsy, or "shave excision". A trend has occurred in dermatology over the last 10 years with the advocacy of a deep shave excision of a pigmented lesion An author published the result of this method and advocated it as better than standard excision and less time consuming. The added economic benefit is that many surgeons bill the procedure as an excision, rather than a shave biopsy. This save the added time for hemostasis, instruments, and suture cost. The great disadvantage, seen years later is the numerous scallop scars, and a very difficult to deal with lesions called a "recurrent melanocytic nevus". What has happened is that many "shave" excisions does not adequately penetrate the dermis or subcutanous fat enough to include the entire melanocytic lesion. Residual melanocytes regrow into the scar. The combination of scarring, inflammation, blood vessels, and atypical pigmented streaks seen in these recurrent nevus gives the perfect dermatoscopic picture of a melanoma. When a second physicians re-examine the patient, he or she has no choice but to recommend the reexcision of the scar. If one does not have access to the original pathology report, it is impossible to tell a recurring nevus from a severely dysplastic nevus or a melanoma. As the procedure is widely practiced, it is not unusual to see a patient with dozens of scallop scars, with as many as 20% of the scars showing residual pigmentation. The second issue with the shave excision is fat herniation, iatrogenic anetoderma, and hypertrophic scarring. As the deep shave excision either completely remove the full thickness of the dermis or greatly diminishing the dermal thickness, subcutanous fat can herniate outward or pucker the skin out in an unattractive way. In areas prone to friction, this can result in pain, itching, or hypertrophic scarring.
These nevi represent excess growth of blood vessels, including capillaries.
- Nevus simplex (also known as a stork bite, salmon patch, or Nevus flammeus neonatorum)
Pseudomelanoma (also known as a "recurrent melanocytic nevus", and "recurrent nevus") is a cutaneous condition in which melantic skin lesions clinically resemble a superficial spreading melanoma at the site of a recent shave removal of a melanocytic nevus.
Blue nevus (also known as "blue neuronevus", "dermal melanocytoma", and "nevus bleu") is a type of melanocytic nevus. The blue colour is caused by the pigment being deeper in the skin than in ordinary nevi. In principle they are harmless but they can sometimes be mimicked by malignant lesions, i.e. some melanomas can look like a blue nevus.
Studies have recorded an incidence of about 3–5 cases per 1,000 newborn babies.
Nevus spilus (also known as speckled lentiginous nevus and zosteriform lentiginous nevus) is a skin lesion that presents as a light brown or tan macule, speckled with smaller, darker macules or papules.
The histopathologic characteristics of melanoma in FAMMM kindreds are not different from those seen in sporadic cases of melanoma and, thus, are not useful in diagnosing the syndrome. Superficial spreading melanoma (SSM) and nodular melanoma are the most frequently encountered histological melanoma subtypes in patients with CDKN2A mutations, which is consistent with the relative early age of onset.
Nevoid melanoma is a cutaneous condition that may resemble a Spitz nevus or an acquired or congenital melanocytic nevus.