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Often, this disease evolves from a precursor lesion, usually a dysplastic nevus. Otherwise it arises in previously normal skin. A prolonged radial growth phase, where the lesion remains thin, may eventually be followed by a vertical growth phase where the lesion becomes thick and nodular. As the risk of spread varies with the thickness, early SSM is more frequently cured than late nodular melanoma.
The microscopic hallmarks are:
- Large melanocytic cells with nest formation along the dermo-epidermal junction.
- Invasion of the upper epidermis in a pagetoid fashion (discohesive single cell growth).
- The pattern of rete ridges is often effaced.
- Invasion of the dermis by atypical, pleomorphic melanocytes
- Absence of the 'maturation' typical of naevus cells
- Mitoses
A Spitz nevus (also known as an epithelioid and spindle-cell nevus, benign juvenile melanoma, and "Spitz's juvenile melanoma") is a benign melanocytic nevus, a type of skin lesion, affecting the epidermis and dermis.
The name "juvenile melanoma" is generally no longer used as it is misleading: it is not a melanoma, it is a benign lesion; and it can also occur in adults, not only in children.
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.
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.
Characteristics include a blue/black stain of skin initially. Skin is thin, about 4-5 cell layers thick, which is often related to aging. Histological features include epidermal atrophy and increased number of melanocytes.
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.
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.
Blue nevi may be divided into the following types:
- A "patch blue nevus" (also known as an "acquired dermal melanocytosis", and "dermal melanocyte hamartoma") is a cutaneous condition characterized by a diffusely gray-blue area that may have superimposed darker macules.
- A "blue nevus of Jadassohn–Tièche" (also known as a "common blue nevus", and "nevus ceruleus") is a cutaneous condition characterized by a steel-blue papule or nodule.
- A "cellular blue nevus" is a cutaneous condition characterized by large, firm, blue or blue-black nodules.
- An "epithelioid blue nevus" is a cutaneous condition most commonly seen in patients with the Carney complex.
- A "deep penetrating nevus" is a type of benign melanocytic skin tumor characterized, as its name suggests, by penetration into the deep dermis and/or subcutis. Smudged chromatic is a typical finding. In some cases mitotic figures or atypical melanocytic cytology are seen, potentially mimicking a malignant melanoma. Evaluation by an expert skin pathologist is advisable in some cases to help differentiate from invasive melanoma.
- An "amelanotic blue nevus" (also known as a "hypomelanotic blue nevus") is a cutaneous condition characterized by mild atypia and pleomorphism.
- A "malignant blue nevus" is a cutaneous condition characterized by a sheet-like growth pattern, mitoses, necrosis, and cellular atypia.
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.
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 congenital melanocytic nevus appears as a circumscribed, light brown to black patch or plaque, potentially very heterogeneous in consistency, covering any size surface area and any part of the body.
As compared with a melanocytic nevus, congenital melanocytic nevi are usually larger in diameter and may have excess terminal hair, a condition called hypertrichosis. If over 40 cm projected adult diameter with hypertrichosis, it is sometimes called giant hairy nevus; more usually these largest forms are known as large or giant congenital melanocytic nevus. The estimated prevalence for the largest forms is 0.002% of births.
Melanocytic Nevi often grow proportionally to the body size as the child matures. As they mature, they often develop thickness, and become elevated, although these features can also be present from birth. Prominent terminal hairs often form, especially after puberty. With maturity, the nevus can have variation in color, and the surface might be textured with proliferative growths.
Neurocutaneous melanosis is associated with the presence of either giant congenital melanocytic nevi or non-giant nevi of the skin. It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. Neurocutaneous melanosis is characterized by the presence of congenital melanocytic nevi on the skin and melanocytic tumors in the leptomeninges of the central nervous system.
Hypermelanotic nevi must be differentiated from other types of pigmented skin lesions, including:
- Lentigo simplex
- Solar lentigo
- Café au lait macule
- Ink-spot lentigo
- Mucosal melanotic macule
- Mongolian spot (dermal melanocytosis)
Phakomatosis pigmentovascularis is subdivided into five types:
- Type 1 PWS + epidermal nevus
- Type 2 (most common): PWS + dermal melanocytosis +/- nevus anemicus
- Type 3: PWS + nevus spilus +/- nevus anemicus
- Type 4: PWS + nevus spilus + dermal melanocytosis +/- nevus anemicus
- Type 5: CMTC (Cutis marmorata telangiectatica congenita) + dermal melanocytosis
They all can contain capillary malformation. Type 2 is the most common and can be associated with granular cell tumor. Some further subdivide each type into categories A & B; with A representing oculocutaneous involvement and subtype B representing extra oculocutaneous involvement. Others have proposed fewer subtypes but currently this rare entity is mostly taught as having five subtypes currently.
Some sources equate the term mole with "melanocytic nevus". Other sources reserve the term "mole" for other purposes such as the animal of the same name.
Melanocytic nevi represent a family of lesions. The most common variants are:
- Location:
- Junctional nevus: the nevus cells are located along the junction of the epidermis and the underlying dermis. A junctional nevus is flat and brown to black.
- Compound nevus: a mixture of junctional and intradermal proliferation. Compound nevi are slightly raised and brown to black. Beauty marks are usually compound nevi of either the acquired variety or congenital variety.
- Intradermal nevus: the nevus cells are located in the dermis only. Intradermal nevi are raised; most are flesh-colored (not pigmented).
- Dysplastic nevus (nevus of Clark): usually a compound nevus with cellular and architectural dysplasia. Like typical moles, dysplastic nevi can be flat or raised. While they vary in size, dysplastic nevi are typically larger than normal moles and tend to have irregular borders and irregular coloration. Hence, they resemble melanoma, appear worrisome, and are often removed to clarify the diagnosis. Dysplastic nevi are markers of risk when they are numerous (atypical mole syndrome). According to the National Cancer Institute (NIH), doctors believe that, when part of a series or syndrome of multiple moles, dysplastic nevi are more likely than ordinary moles to develop into the most virulent type of skin cancer called melanoma.
- Blue nevus: It is blue in color as its melanocytes are very deep in the skin. The nevus cells are spindle shaped and scattered in deep layers of the dermis. The covering epidermis is normal.
- Spitz nevus: a distinct variant of intradermal nevus, usually in a child. They are raised and reddish (non-pigmented). A pigmented variant, called the 'nevus of Reed', typically appears on the leg of young women.
- Acquired nevus: Any melanocytic nevus that is not a congenital nevus or not present at birth or near birth. This includes junctional, compound and intradermal nevus.
- Congenital nevus: Small to large nevus present at or near time of birth. Small ones have low potential for forming melanomas, however the risk increases with size, as in the giant pigmented nevus.
- Giant pigmented nevus: these large, pigmented, often hairy congenital nevi. They are important because melanoma may occasionally (10 to 15%) appear in them.
- Intramucosal nevus: junctional nevus of the mucosa of the mouth or genital areas. In the mouth, they are found most frequently on the hard palate.
- Nevus of Ito and nevus of Ota: congenital, flat brownish lesions on the face or shoulder.
- Mongolian spot: congenital large, deep, bluish discoloration which generally disappears by puberty. It is named for its association with East Asian ethnic groups but is not limited to them.
- Recurrent nevus: Any incompletely removed nevus with residual melanocytes left in the surgical wound. It creates a dilemma for the patient and physician, as these scars cannot be distinguished from a melanoma.
Spindle cell carcinoma is a type of cancer that begins in the skin or in tissues that line or cover internal organs and that contains long spindle-shaped cells. It is also called sarcomatoid carcinoma.
Congenital melanocytic nevi may be divided into the following types:
- "Small-sized congenital melanocytic nevus" is defined as having a diameter less than 2 cm.
- "Medium-sized congenital melanocytic nevus" is defined as having a diameter more than 2 cm but less than 20 cm.
- "Giant congenital melanocytic nevus" (also known as "Bathing trunk nevus," "Garment nevus," "Giant hairy nevus", and "Nevus pigmentosus et pilosus") is defined by one or more large, darkly pigmented and sometimes hairy patches.
According to the American Academy of Dermatology, the most common types of moles are skin tags, raised moles and flat moles. Benign moles are usually brown, tan, pink or black (especially on dark-colored skin). They are circular or oval and are usually small (commonly between 1–3 mm), though some can be larger than the size of a typical pencil eraser (>5 mm). Some moles produce dark, coarse hair. Common mole hair removal procedures include plucking, cosmetic waxing, electrolysis, threading and cauterization.
Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.
Individuals with a basal-cell carcinoma typically present with a shiny, pearly skin nodule. However, superficial basal-cell cancer can present as a red patch similar to eczema. Infiltrative or morpheaform basal-cell cancers can present as a skin thickening or scar tissue – making diagnosis difficult without using tactile sensation and a skin biopsy. It is often difficult to visually distinguish basal-cell cancer from acne scar, actinic elastosis, and recent cryodestruction inflammation.
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.
These nevi represent excess growth of blood vessels, including capillaries.
- Nevus simplex (also known as a stork bite, salmon patch, or Nevus flammeus neonatorum)
A seborrheic keratosis, also known as seborrheic verruca, basal cell papilloma, or a senile wart, is a non-cancerous (benign) skin tumour that originates from cells in the outer layer of the skin (keratinocytes). Like liver spots, seborrheic keratoses are seen more often as people age.
The tumours (also called lesions) appear in various colours, from light tan to black. They are round or oval, feel flat or slightly elevated, like the scab from a healing wound, and range in size from very small to more than across. They can often come in association with other skin conditions, including basal cell carcinoma. Sometimes seborrheic keratosis and basal cell carcinoma occur at the same location, and sometimes seborrheic keratosis progresses to basal cell carcinoma. At clinical examination the differential diagnosis include warts and melanoma. Because only the top layers of the epidermis are involved, seborrheic keratoses are often described as having a "pasted on" appearance. Some dermatologists refer to seborrheic keratoses as "seborrheic warts", because they resemble warts, but strictly speaking the term "warts" refers to lesions that are caused by human papillomavirus.
Nevus sebaceus or sebaceous nevus (the first term is its Latin name, the second term is its name in English; also known as an "organoid nevus" and "nevus sebaceus of Jadassohn") is a congenital, hairless plaque that typically occurs on the face or scalp. Such nevi are present at birth, or early childhood, affecting males and females of all races equally. The condition is named for an overgrowth of sebaceous glands in the area of the nevus.
Skin growths such as benign tumors and basal cell carcinoma can arise in sebaceous nevi, usually in adulthood. Rarely, sebaceous nevi can give rise to sebaceous carcinoma. However, the rate of such malignancies is now known to be less than had been estimated. For this reason, excision is no longer automatically recommended.
Giant-cell fibroma is a type of fibroma not associated with trauma or irritation. It can occur at any age and on a mucous membrane surface. The most common oral locations are on the gingiva of the mandible, tongue, and palate. It is a localized reactive proliferation of fibrous connective tissue.
Giant-cell fibroma (GCF) is a benign non-neoplastic lesion first described by Weathers and Callihan (1974). It occurs in the first three decades of life and predominates in females (Houston, 1982; Bakos, 1992). Clinically, the GCF presents as an asymptomatic, papillary and pedunculated lesion. The most predominant location is the mandibular gingiva (Houston, 1982; Bakos, 1992). Histologically, the GCF is distinctive, consisting of fibrous connective tissue without inflammation and covered with stratified squamous hyperplastic epithelium. The most characteristic histological feature is the presence of large spindle-shaped and stellate-shaped mononuclear cells and multinucleated cells. These cells occur in a variety of lesions, such as the fibrous papule of the nose, ungual fibroma, acral fibrokeratoma, acral angiofibroma and desmoplastic fibroblastoma (Swan, 1988; Pitt et al., 1993; Karabela-Bouropoulou et al., 1999; Jang et al., 1999).
Despite many studies, the nature of the stellated multinucleate and mononuclear cell is not clear (Weathers and Campbell, 1974; Regezi et al., 1987; Odell et al., 1994; Magnusson and Rasmusson, 1995).
The histopathologic classification includes:
- "Nodular basal cell carcinoma" (also known as "classic basal-cell carcinoma") most commonly occurs on the sun-exposed areas of the head and neck.
- "Cystic basal cell carcinoma" is morphologically characterized by dome-shaped, blue-gray cystic nodules.
- "Cicatricial basal cell carcinoma" (also known as "morpheaform basal cell carcinoma," and "morphoeic basal cell carcinoma") is an aggressive variant with a distinct clinical and histologic appearance.
- "Infiltrative basal cell carcinoma" is an aggressive type characterized by deep infiltration.
- "Micronodular basal cell carcinoma" is characterized by a micronodular growth pattern.
- "Superficial basal cell carcinoma" (also known as "superficial multicentric basal-cell carcinoma") occurs most commonly on the trunk and appears as an erythematous patch.
- "Pigmented basal cell carcinoma" exhibits increased melanization. About 80% of all basal-cell carcinoma in Chinese are pigmented while this subtype is uncommon in white people.
- "Rodent ulcer" (also known as a "Jacob's ulcer") is a large skin lesion of nodular basal-cell carcinoma with central necrosis. Almost all cancers can metastasize except glioma (maligancy of the central nervous system) and the rodent ulcer.
- "Fibroepithelioma of Pinkus" most commonly occurs on the lower back.
- "Polypoid basal cell carcinoma" is characterized by exophytic nodules (polyp-like structures) on the head and neck.
- "Pore-like basal cell carcinoma" resembles an enlarged pore or stellate pit.
- "Aberrant basal cell carcinoma" is characterized by the formation of basal-cell carcinoma in the absence of any apparent carcinogenic factor, occurring in odd sites such as the scrotum, vulva, perineum, nipple, and axilla.
See also:
- Nevoid basal-cell carcinoma syndrome