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NEUROFIBROMA General Information Probably the most common benign peripheral nerve tumor, neurofibroma is a tumor of the nerve fiber. On occasion a solitary neurofibroma occurs, but more frequently they occur as part of neurofibromatosis. Neurofibromatosis is a genetic disorder that may be associated with multiple neurofibromas. The disease is transmitted by an autosomal dominant mutant gene. There are two types of neurofibromatosis. o Type 1 (von Recklinghausen's disease) affects 1 in 3000 people; however, the majority of cases are mild with a limited number of tumors. o Type 2, which is more severe, affects 1 in 50,000 people. Symptoms The spectrum of lesions in von Recklinghausen's disease varies widely. For example, the neurofibromas may involve small skin nerves and appear as small bumps, or they may be confined to large nerves and produce elephantiasis, or enlargement of affected body parts. Diagnosis Neurofibromatosis 1:The diagnosis requires two or more of the following: o Six or more brownish discolorations on the skin, more than 5mm in greatest diameter in pre-adolescents and greater than 15 mm in adolescents and adults. o Two or more neurofibromas. o Freckling in the arm pits or groin regions. o Optic glioma .

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NEUROFIBROMA

General Information

Probably the most common benign peripheral nerve tumor, neurofibroma is a tumor of the nerve fiber.

On occasion a solitary neurofibroma occurs, but more frequently they occur as part of neurofibromatosis. Neurofibromatosis is a genetic disorder that may be associated with multiple neurofibromas. The disease is transmitted by an autosomal dominant mutant gene.

There are two types of neurofibromatosis.

o Type 1 (von Recklinghausen's disease) affects 1 in 3000 people; however, the majority of cases are mild with a limited number of tumors.

o Type 2, which is more severe, affects 1 in 50,000 people.

Symptoms

The spectrum of lesions in von Recklinghausen's disease varies widely. For example, the neurofibromas may involve small skin nerves and appear as small bumps, or they may be confined to large nerves and produce elephantiasis, or enlargement of affected body parts.

Diagnosis

Neurofibromatosis 1:The diagnosis requires two or more of the following: o Six or more brownish discolorations on the skin, more than 5mm in greatest

diameter in pre-adolescents and greater than 15 mm in adolescents and adults.

o Two or more neurofibromas.

o Freckling in the arm pits or groin regions.

o Optic glioma.

o Two or more Lisch nodules, or small masses on the iris of the eyes.

o Bone lesions, called sphenoid dysplasia.

o A first degree relative with neurofibromatosis type 1.

Neurofibromatosis 2: The diagnosis requires either of the following:

o Eighth cranial nerve masses on both sides (bilateral).

o An immediate relative with neurofibromatosis 2, and either an eighth nerve mass on one side or two of the following: neurofibroma, meningioma, glioma or schwannoma.

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Treatment

The management of neurofibromas depends upon the symptoms. They do not typically need to be surgically removed.

Surgical exploration and excision may be required under the following circumstances: type of diagnosis, pain, cosmetic considerations, progressive neurological complications, compression of adjacent tissues and suspicion of a malignant tumor.

Painful neurofibromas occur just under the skin. These globular lesions may occur in the hand or other surfaces that are subject to pressure. Typically these lesions involve small bundles of superficial nerves and can be removed without creating a meaningful neurological deficit.

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EPIDERMOID CYST

Definition

Epidermoid cysts are small bumps that develop beneath the skin on your face, neck, trunk and sometimes your genital area. Slow-growing and often painless, they rarely cause problems or need treatment.

Although many people refer to epidermoid cysts as sebaceous cysts, they’re different. True sebaceous cysts are less common, and they arise from the glands that secrete oily matter that lubricates hair and skin (sebaceous glands). Epidermoid glands arise from the cells that make up the outer layers of skin (epidermal).

If the appearance of an epidermoid cyst bothers you, or if the cyst ruptures or becomes infected, it can be surgically removed. Epidermoid cysts are almost always noncancerous, but in rare cases, they can lead to skin cancers.

Symptoms

Epidermoid cysts:

Are round cysts or small bumps that are easy to move with your fingers Are usually white or yellow, though people with darker skin may have pigmented cysts

Range in size from less than 1/4 inch to nearly 2 inches (a few millimeters to 5 centimeters) in diameter

Occur on nearly any part of your body, including your fingernails, but are found most often on your face, trunk and neck

Sometimes an epidermoid cyst has a central opening — the remnant of a hair follicle from which the cyst originally formed — that's plugged by a tiny blackhead. You may be able to squeeze out a thick, cheesy material through this opening, but because of the risk of infection and scarring, it's best to leave this to your dermatologist.

Milia — tiny, deep-seated whiteheads that never seem to come to the surface of your skin — are miniature epidermoid cysts. They're especially common in older women and in men with significant sun damage on their cheeks and temples. They can also be caused or aggravated by long-term use of oil-based creams or cosmetics.

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Signs and symptoms of infection, which can occasionally occur, include:

A thick, yellow material draining from the cyst that may have a foul odor Redness, swelling and tenderness around the cyst

A similar looking conditionSometimes you may develop a small bump on your scalp that looks like an epidermoid cyst. These are almost always pilar or trichilemmal cysts, which usually have thicker walls than epidermoid cysts do and almost always move freely under your skin. The lining of this type of cyst differs slightly from that of an epidermoid cyst.

When to see a doctorMost epidermoid cysts aren't harmful, but you may want to have them removed for cosmetic reasons. See your doctor if you have a cyst that:

Grows rapidly Ruptures

Becomes painful

Occurs in a spot that's constantly irritated

Causes

The surface of your skin (epidermis) is made up of an extremely thin, protective layer of cells that your body continuously sheds. Most epidermoid cysts form when these surface cells, instead of exfoliating normally, move deeper into your skin and multiply. Most often, this occurs in areas where there are small hair follicles and larger oil glands (sebaceous glands), such as your face, neck, upper back and groin.

The epidermal cells form the walls of the cyst, and then secrete the protein keratin into the interior. The keratin is the thick yellow substance that sometimes drains from the cyst.

Several factors can lead to this abnormal proliferation of cells, including:

Damage to a hair follicle. Each hair grows from a follicle, a small pocket of modified skin in the dermis, the layer of skin just below the epidermis. Follicles damaged by injuries, such as abrasions or surgical wounds, can become blocked by surface cells.

A ruptured sebaceous gland. Located just above the hair follicles, sebaceous glands produce sebum, the oil that lubricates your skin and coats each hair shaft. These glands are easily ruptured by inflammatory skin conditions, especially acne, making them a likely site for epidermoid cysts.

Developmental defect. Epidermoid cysts can begin in a developing fetus when stem cells intended to form skin, hair or nails become trapped in cells forming other tissues.

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Heredity. Epidermoid cysts can develop in people with Gardner's syndrome, a rare genetic disorder that causes growths in the colon, or basal cell nevus syndrome, an inherited disease that leads to several serious defects.

Risk Factor

Nearly anyone can develop one or more epidermoid cysts, but these factors make you more susceptible:

Being past puberty. Though they can occur at any age, epidermoid cysts rarely appear before puberty.

Being male. Men are more likely to have epidermoid cysts.

Having a history of acne. Epidermoid cysts are especially common in people who've had acne.

Having significant sun exposure. Milia, seen mainly on the face, often occur in men and women with a long history of sun exposure.

Experiencing skin injuries. Any traumatic or crushing injury to your skin — slamming your hand in a car door, for instance — increases your risk of an epidermoid cyst.

Complication

In rare cases, epidermoid cysts can give rise to basal and squamous cell skin cancers. Because this occurs so seldom, epidermoid cysts usually aren't biopsied unless they're solid, immobile, infected or have other unusual characteristics that suggest a more serious problem. Besides cancer, other complications include:

Inflammation. Epidermoid cysts can become tender and swollen, even if they're not infected. Inflamed cysts are difficult to remove, and your doctor is likely to postpone treating them until the inflammation subsides.

Rupture. A ruptured cyst often leads to a boil-like abscess that requires prompt treatment.

Infection. An epidermoid cyst can become infected spontaneously or after a rupture.

Genital discomfort. Genital epidermoid cysts can lead to painful intercourse and urination.

Tests and Diagnosis

In most cases, your doctor can diagnose an epidermoid cyst based on its appearance, though he or she may refer you to a dermatologist for treatment.

Treatment

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Cysts that don't cause cosmetic or functional problems are usually left alone. A cyst that's inflamed, ruptured or infected may be treated with:

Injections. Your doctor may inject an inflamed, but uninfected, epidermoid cyst with a corticosteroid or triamcinolone acetonide (Kenalog) to help reduce inflammation.

Incision and drainage. In this procedure, your doctor makes a small cut in the cyst and expresses the contents. Although incision and drainage is relatively quick and easy, cysts often recur after this treatment.

Total excision. This surgical technique removes the entire cyst and so prevents recurrence. Excision is most effective when the cyst isn't inflamed. Your doctor may recommend first treating the inflammation with antibiotics, steroids, or incision and drainage and then waiting to perform excision for four to six weeks after inflammation resolves. Total excision requires sutures. Your doctor will remove sutures in your face within a week or so of total cyst excision, and will remove sutures elsewhere in your body within one to two weeks.

Minimal excision. Some doctors prefer this technique because it removes the whole cyst wall but causes minimal, if any, scarring. During the procedure, your doctor makes a tiny incision in the cyst, expresses the contents, and then removes the cyst wall through the incision. The small wound is usually left to heal naturally.

Lasers. To minimize scarring, your doctor may use a carbon dioxide laser to vaporize an epidermoid cyst on your face or other sensitive area.

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LIPOMA

A lipoma is a benign tumor composed of adipose tissue. It is the most common form of soft tissue tumor.[1] Lipomas are soft to the touch, usually movable, and are generally painless. Many lipomas are small (under one centimeter diameter) but can enlarge to sizes greater than six centimeters. Lipomas are commonly found in adults from 40 to 60 years of age, but can also be found in younger adults and children. Some sources claim that malignant transformation can occur,[2] while others say that this has yet to be convincingly documented.[3]

Types

There are several subtypes of lipomas:[4]:624–5

Adenolipomas are lipomas associated with eccrine sweat glands.[5]:627

Angiolipoleiomyomas are acquired, solitary, asymptomatic acral nodules, characterized histologically by well-circumscribed subcutaneous tumors composed of smooth muscle cells, blood vessels, connective tissue, and fat.[4]:627

Angiolipomas painful subcutaneous nodules having all other features of a typical lipoma.[4]:624[6]

Chondroid lipomas are deep-seated, firm, yellow tumors that characteristically occur on the legs of women.[4]:625

Corpus callosum lipoma is a rare congenital brain condition which may or may not present with symptoms.[7] This occurs in the corpus callosum, also known as the colossal commissure, which is a wide, flat bundle of neural fibers beneath the cortex in the human brain.

Hibernomas are lipoma of brown fat.

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Intradermal spindle cell lipomas are distinct in that it most commonly affects women, and has a wide distribution, occurring with relatively equal frequency on the head and neck, trunk, and upper and lower extremities.[4]:625[6]

Neural fibrolipomas are overgrowths of fibro-fatty tissue along a nerve trunk, which often leads to nerve compression.[4]:625

Pleomorphic lipomas , like spindle-cell lipomas, occur for the most part on the backs and necks of elderly men, and are characterized by floret giant cells with overlapping nuclei.[4]:625

Spindle-cell lipomas are asymptomatic, slow-growing subcutaneous tumors that have a predilection for the posterior back, neck, and shoulders of older men.[4]:625

Superficial subcutaneous lipomas, the most common type of lipoma, lie just below the surface of the skin.[3] Most occur on the trunk, thighs and the forearms, although they may be found anywhere in the body where fat is located.

Prevalences

Approximately one percent of the general population has a lipoma.[3] These tumors can occur at any age, but are most common in middle age, often appearing in people from 40 to 60 years old.[8] Cutaneous lipomas are rare in children, but these tumors can occur as part of the inherited disease Bannayan-Zonana syndrome.[9][10]

Lipomas are usually relatively small with diameters of about 1–3 cm,[11] but in rare cases they can grow over several years into "giant lipomas" that are 10–20 cm across and weigh up to 4–5 kg.[12][13]

Causes

The tendency to develop a lipoma is not necessarily hereditary although hereditary conditions, such as familial multiple lipomatosis, may include lipoma development.[14][15] Genetic studies in mice have shown a correlation between the HMG I-C gene (previously identified as a gene related to obesity) and lipoma development. These studies support prior epidemiologic data in humans showing a correlation between HMG I-C and mesenchymal tumors.[16]

Cases have been reported where minor injuries are alleged to have triggered the growth of a lipoma, called "post-traumatic lipomas."[17] However, the link between trauma and the development of lipomas is controversial.

Treatment

Usually, treatment of a lipoma is not necessary, unless the tumor becomes painful or restricts movement. They are usually removed for cosmetic reasons, if they grow very large, or for histopathology to check that they are not a more dangerous type of tumor such as a liposarcoma.

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[3] This last point can be important as the actual identity of a "bump" is not known until after it is removed and professionally examined.

Lipomas are normally removed by simple excision.[8] The removal can often be done under local anaesthetic, and take less than 30 minutes.[19] This cures the majority of cases, with about 1–2% of lipomas recurring after excision.[20] Liposuction is another option if the lipoma is soft and has a small connective tissue component. Liposuction typically results in less scarring; however, with large lipomas it may fail to remove the entire tumor, which can lead to regrowth.[21]

There are new methods being developed that are supposed to remove the lipomas without scarring. One of them is removal by the use of injection of compounds that trigger lipolysis, such as steroids or phosphatidylcholine.[8][22]

DERMOID CYST

Dermoid Cyst Overview

A dermoid cyst is a saclike growth that is present at birth. It contains structures such as hair, fluid, teeth, or skin glands that can be found on or in the skin.

Dermoid cysts grow slowly and are not tender unless ruptured. They usually occur on the face, inside the skull, on the lower back, and in the ovaries. Superficial dermoid cysts on the face usually can be removed without complications. Removal of other, rarer dermoid cysts requires special techniques and training. These rarer dermoid cysts occur in four major areas:  

Dermoid cysts in the brain: Dermoid cysts occur very rarely here. A neurosurgeon may need to remove them if they cause problems.

Dermoid cysts in the nasal sinuses: These are also very rare. Only a handful of cases involving dermoid cysts located here are reported each year. Removal of these cysts is extremely complicated.

Ovarian dermoid cysts: These growths can develop in a woman during her reproductive years. They can cause torsion, infection, rupture, and cancer. These dermoid cysts can be removed with either conventional surgery or laparoscopy (surgery that uses small incisions and specially designed instruments to enter the abdomen or pelvis).

Dermoid cysts of the spinal cord: A sinus tract, which is a narrow connection from a deep pit in  the skin, usually connects these very rare cysts to the skin surface. This type of dermoid cyst can become infected. Removal is often incomplete, but the outcome is usually excellent.

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Dermoid Cyst Causes

Dermoid cysts are caused when skin and skin structures become trapped during fetal development. Their cell walls are nearly identical to those of the outer skin and may contain multiple skin structures such as hair follicles, sweat glands, and sometimes hair, teeth, or nerves.

When to Seek Medical Care

A doctor should be contacted in the following situations:

A cyst becomes painful or inflamed. A cyst grows or changes color.

Removal is desired for cosmetic reasons.

Typically, removing a dermoid cyst is not an emergency procedure. If a dermoid cyst ruptures, becomes inflamed, or causes pain or fever, a person should seek immediate medical advice. Depending on the severity of pain or discomfort, a person might also consider visiting a hospital’s emergency department.

Exams and Tests for Dermoid Cysts

Prior to removal of superficial dermoid cysts on the face, a person should know the difference between cysts and other facial growths.

Because dermoid cysts stem from birth and grow slowly, a person usually notices them during childhood or early adulthood.

Dermoid cysts are firm and painless unless ruptured.

Dermoid cysts are not attached to the overlying skin.

In rare cases, a dermoid cyst extends into a structure deeper than skin, such as a facial cavity or an orbit. Some doctors recommend a CT scan or other imaging studies for these cases. This decision depends on the doctor’s suspicion of a deep-level cyst and after a determination of risk versus benefit.

Dermoid Cyst Home Remedies

Self-removal of facial cysts at home is not recommended because the cyst will grow back if not completely removed. Chances of infection, bleeding, and other complications increase for people who remove dermoid cysts themselves, especially since the person may not be able to differentiate between a harmless growth and other, more serious skin growths.

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Medical Treatment for Dermoid Cysts

To remove a dermoid cyst, the doctor will clean the area over which the cyst is located, inject a local anesthetic, and make an incision directly over the cyst and attempt remove it completely.

Outlook for Dermoid Cysts

Barring the possible complications associated with any operation, removal of a dermoid cyst usually results in complete recovery.