kin Cancer Disorder

Definition: It is a cancer of the T-lymphocytes and most often occurs in people aged between 40 and 60.  Unlike other forms of non-Hodgkin lymphoma, CTCL mainly affects the skin. It is caused by the uncontrolled growth of a type of white blood cell within the skin, called a T-cell.  The most common types of CTCL are mycosis fungoides and Sezary syndrome. Sezary syndrome is a specific type of CTCL in which large areas of skin, or lymph glands, are affected, and abnormal T-lymphocytes are also found in the blood. Mycosis fungoides is the general name given to the other types of CTCL when the blood is not affected.
Etiology: The causes of CTCL are unknown. CTCL, like other cancers, is not infectious and cannot be passed on to other people.
Clinical Features: The stage of CTCL indicates how the disease is affecting the skin. There are three recognisable stages of early CTCL, although the disease will not necessarily progress through all three stages.

1) Premycotic or pre-tumour stage Small, raised, red patches appear on the skin, commonly on the breast and buttocks, although they can appear anywhere. At this stage, the disease often looks like common skin conditions such as eczema or psoriasis.

2) Plaque or infiltrative stage Irregularly shaped red patches (plaques) form. Any part of the body may be affected, but the buttocks, skin folds, and face are particularly common places.

3) Tumour stage Only a small proportion of people progress to this stage, as most people never progress beyond the first stage. Raised lumps (tumours) appear on the skin. The tumours and the red plaques may become deep sores (ulcerate).

Clinical Approach: The diagnosis is made through biopsy of affected areas.
Treatment: If the CTCL is in the premycotic and patch-phase, bland emollients, gentle skin care, topical antipruritic agents and gradual exposures to sunlight will help.  In the infiltrative plaque phase, topical mustard applications (mechlorethamine HCl) is applied daily or if PUVA photochemotherapy is suggested. PUVA therapy involves taking a drug called psoralen and then being exposed to ultraviolet A light. The drug makes the cancer cells sensitive to light and in turn, the light kills the cancer cells.

Definition: The most common skin cancer in the white population.  It can be destructive and disfiguring.  It is rarely life-threatening, but if left untreated, can be disfiguring, cause bleeding, and produce local destruction (e.g., eye, ear, nose, lip).  Seen less in black skin, due to high melanin higher content allowing greater UV protection.
Etiology: The main trigger appears to be UVB rays.  However there is a genetic component.  About 2/3 of basal cell carcinomas occur on sun-exposed areas of the body. 1/3 occur on areas of the body that are not exposed to sunlight, more common in Blacks, emphasizing the genetic susceptibility of the basal cell cancer patients.
Clinical Features: Basal cell carcinomas present as a firm nodule, clearly growing within the skin and below it, rather than on the surface. Color varies from that of normal skin to dark brown or black, but there is a characteristic "pearly white" translucent quality on the periphery. Basal cell carcinomas display a characteristic "rolled edge". Various forms recognized.

• Nodular: flesh-colored papule with telangiectasis; if it ulcerates, it becomes a "rodent ulcer" (an ulcerating nodule with a pearly border).
• Cystic: rarer and difficult to distinguish from the nodular form, which has a central cavity with fluid
• Pigmented: a variant of the nodular form that may be confused with melanoma. This is the most common seen in Blacks!
• Sclerosing/cicratising: a scar-like lesion
• Superficial: a red scaling patch.

Clinical Approach: Clinical examination and biopsy.  Dermoscopy is very helpful for pigmented or atypical BCC’s.  Fortunately BCC is a straight forward pathological diagnosis, even when clinical questions exist.
Treatment:  The aim is complete removal and thus a 100% cure.  Removal can be done by surgical excision, curettage & electrodessication, laser ablation, or radiation therapy.

 Definition: Dermatofibrosarcoma protuberans is a rare type of malignant tumor that occurs in the dermis, the second layer of the skin.
Etiology: The cause of dermatofibrosarcoma protuberans is unknown.
Clinical Features: Dermatofibrosarcoma protuberans is a slow-growing tumor that is most commonly seen on the trunk of the body (chest, abdomen, and back), but can also occur on the arms, legs, head, neck, hands, or feet. The tumor begins as a very small bump on the surface of the skin, sometimes having a reddish-brown or reddish-blue color. The tumor then undergoes a period of very rapid growth where the many small growths in the dermis form a larger, more noticeable mass. This mass swells and bulges outward, hence the term “protuberans.” The area becomes tender and may bleed because the top layer of the skin is stretched so tight over the mass. Dermatofibrosarcoma protuberans usually does not spread to other parts of the body (metastasize).
Clinical Approach: Because the initial growth phase is so slow, it can be a few years before the tumor is noticeable and causes enough discomfort for the patient to seek a diagnosis. To diagnose, a biopsy is required.
Treatment: Dermatofibrosarcoma protuberans has commonly been treated with wide excision, which means the tumor and a large amount of the tissue surrounding it are removed. Mohs’ microscopically controlled surgery (also called Mohs’ micrographic surgery) is another option that has been very effective. Dermatofibrosarcoma protuberans has a high recurrence rate because the tumor has many projections beyond the main mass that may be left behind when the tumor is removed. However, Mohs’ surgery has proven very effective in reducing the recurrence rate. Chemotherapy is not an effective treatment because the tumor grows so slowly. Radiation therapy may be recommended for some patients.

 Definition: ALM is an enlarging maligmant black macule with a prolonged noninvasive stage, occurring chiefly on the palms and soles; it is the most common type of melanoma in persons of African origin.
Etiology: Cause is unknown. However, unlike other forms of melanoma, acral lentiginous melanoma does not appear to be linked to sun exposure.
Clinical Features: Typical symptoms of acral lentiginous melanoma include longitudinal tan, black, or brown streak on a finger or toe nail (melanonychia striata), pigmentation of proximal nail fold, or areas of dark pigmentation on palms of hands or soles of feet.

Clinical Approach: The diagnosis is made through biopsy of affected areas. Diagnosis of ALM during the earlyphase is often difficult, and it may not be recognized initially, but treatment in this phase offers an excellent prognosis.
Treatment: Treatment of (ALM) depends on the stage of cancer.  The list of treatments mentioned in various sources for Acral lentiginous melanoma includes chemotherapy, radiation therapy, and  surgery.

Squamous Cell Carcinoma (SCC)

Definition: Cutaneous squamous cell carcinoma (SCC) is the second most common form of skin cancer and frequently arises on the sun-exposed skin of middle-aged and elderly individuals. SCC is much more common in geographic areas where patients have a high frequency of sun exposure.
Etiology: Most SCC’s are a result of the damage to DNA in skin cells from exposure to UV radiation from sunlight and commercial tanning lamps and beds. The damage is cumulative, so the more time you spend in the sun or in a tanning booth, the greater your chance of developing skin cancer. Your risk increases even more if most of your outdoor exposure occurs at times of the day or in locations where the sun is strongest.
Clinical Features: Squamous cell carcinomas usually appear as crusted or scaly patches on the skin with a red, inflamed base, a growing tumor, or a non-healing ulcer. They are generally found in sun-exposed areas like the face, neck, arms, scalp, backs of the hands, and ears. The cancer also can occur on the lips, inside the mouth, on the genitalia, or anywhere on the body.
Clinical Approach: Diagnosis made from history & clinical examination.  Can be confirmed by biopsy.
Treatment: Most squamous cell carcinomas can be completely removed with relatively minor surgery or occasionally with a topical medication. The type of squamous cell carcinoma treatment usually depends on the size, location and aggressiveness of the tumor and may include one or more of the following:

• Freezing
• Simple excision

• Laser therapy
• Mohs' surgery
• Radiation therapy
• Chemotherapy