WORKUP	Section 5 of 9
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Procedures:

• A skin biopsy always is required for definitive diagnosis, despite the fact that the diagnosis is suspected strongly on clinical grounds.

• Biopsy is performed routinely in the physician's office with a local anesthetic.

• All skin biopsies obtained to diagnose VC must reach at least the depth of the mid dermis to allow for determination of the presence or absence of invasive disease.

• A deep (scoop) shave biopsy, a punch biopsy, an incisional biopsy, or an excisional biopsy may be employed.

• Pathology readings preferably are made by a dermatopathologist who has extensive experience with VC.

Staging: Most VCs are nonmetastatic and are staged based on size, as follows:

• T0 lesions - In situ

• T1 lesions - Less than 2 cm in diameter

• T2 lesions - Between 2 and 4 cm in diameter

• T3 lesions - Greater than 4 cm in diameter

• T4 lesions - Invasive of muscle or bone

	TREATMENT	Section 6 of 9
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Surgical Care: Most physicians treat patients with VC in their offices. Complete tumor extirpation should be performed at first presentation because VC is a lesion that can recur, metastasize, and ultimately cause death. Recurrent VC carries a relatively poor prognosis.

• Cryosurgery

• Cryosurgery using liquid nitrogen is a safe and low-cost procedure for the ablation of selected VC and is well tolerated by patients.

• Cryotherapy has provided a high cure rate for select well-circumscribed superficial VC. Because of no histologic control, close follow-up is necessary.

• This procedure is the least likely to result in cure and is the least preferred intervention.

• Curettage and electrodesiccation

• Cure rates of 96-99% have been quoted in several large studies for destruction of T0 and T1 VC (ie, in situ lesions and invasive lesions <2 cm in diameter). This high cure rate was affected by careful patient selection.

• The main disadvantage of curettage and electrodesiccation is a lack of margin control; nonetheless, the procedure is minimally invasive, well tolerated, and effective for in situ lesions without deep involvement.

• Curettage and electrodesiccation is most appropriate for slow-growing lesions of the trunk and extremities.

• Excision with conventional margins

• Simple excision is most valuable in the treatment of small VCs of the trunk and extremities and in areas in which tissue sparing is not essential.

• Cure rates following simple excision of well-defined T1 lesions may be as high as 95-99%.

• A 4-mm margin of normal tissue is recommended for straightforward lesions.

• Standard excision with permanent conventional sections is a highly effective treatment for many VCs. The depth of the excision should include the subcutaneous fat because even small VCs may extend into the subcutaneous fat.

• The disadvantages of excision with an arbitrary margin are that, in some cases, the pathology reveals a subclinical positive margin, requiring further surgery. Additionally, more normal tissue may be excised than is necessary.

• Mohs surgery

• A dermatologic surgeon usually offers Mohs micrographic surgery (MMS). The main advantage of MMS over simple excision in the extirpation of cutaneous VC is the ability to examine all excision margins (deep and lateral) and to carefully map residual foci of invasive carcinoma.

• MMS provides a cure rate for VC of 94-100% and has been of particular value in curing VC with perineural invasion. MMS offers the added benefit of preserving normal tissue, thus facilitating reconstruction.

• MMS is performed routinely in an outpatient setting under local anesthesia.

• MMS is not widely available outside the United States.

• A multidisciplinary approach employing MMS performed in conjunction with plastic surgery, otolaryngology, and radiation oncology may allow for the complete removal of deeply invasive VC, preserve vital structures, and facilitate the reconstruction of a large operative defect.

• Because of its many advantages, MMS is the procedure of choice for VC where tissue preservation is needed. Furthermore, surgery for VC using MMS may be an integral component in the management of certain VCs that otherwise would be beyond the experience of the cutaneous surgeon.

• Radiation therapy

• Radiation therapy offers the potential advantage of avoiding the trauma and deformity of a surgical procedure.

• Radiation therapy for patients with VC commonly is administered 5-12 times over a 5- to 6-week period with fractionated doses of 400-800 cGy.

• Currently in the United Kingdom and the United States, ionizing radiation therapy is used mainly as a treatment for primary cutaneous carcinoma in patients who cannot tolerate or wish to avoid surgery (eg, elderly patients).

• Cure rates for T1 lesions range from 85-95%.

• Although the initial cosmetic result following radiation often is good, the long-term result frequently is poor, with atrophy, hypopigmentation, and telangiectasia. Some patients treated with radiation also develop radiation necrosis. This risk increases over time.

• Radiation therapy is not advocated for use over bony structures because of the risk of osteoradionecrosis. Radiation therapy is not advocated for patients who are young or middle-aged because of the small, but real, risk of a radiation-induced cutaneous carcinoma or sarcoma later in life.

• Radiation therapy is expensive and requires multiple visits. The procedure is blind to histologic margin control. For these reasons, the use of radiation as primary therapy for VC generally is restricted to older patients who cannot tolerate or refuse surgery.

	FOLLOW-UP	Section 7 of 9
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Further Outpatient Care:

• VC usually is cured with appropriate therapy; however, patients at risk for additional VC and squamous cell carcinoma should be evaluated with a skin examination at 3- to 12-month intervals.

Prognosis:

• Most patients with VC have a good prognosis. Local recurrence following definitive treatment is not uncommon, but distant metastasis is rare. Patients with oral VC may be at an increased risk of a second primary oral squamous cell carcinoma, which carries a poor prognosis.

Patient Education:

• Advise patients about the importance of receiving effective treatment for areas of chronic skin inflammation or trauma (eg, leg or decubitus ulcers) to prevent these problems from developing malignancies within them. Improved oral, genital, and perianal hygiene may help to prevent inflammatory conditions that predispose patients to VC. Cessation of chewing tobacco use may help to prevent oral VC.

	MISCELLANEOUS	Section 8 of 9
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Medical/Legal Pitfalls:

• The main pitfall in the diagnostic evaluation is an inadequate biopsy. Additionally, in some cases, the carcinoma is so well differentiated that the pathologist may read the tissue as pseudoepitheliomatous hyperplasia. VC is a lesion with the potential to cause substantial morbidity and even mortality, and physicians who diagnose and treat VC are held legally accountable for their actions.

• Failure to ensure adequate patient follow-up care is a pitfall because primary treatment of VC is not a guarantee of cure.

• Not informing patients of the potential morbidity associated with VC may lead to the lesion being regarded as trivial and not requiring follow-up care. (The courts hold the physician, not the patient, responsible for appropriate follow-up care.)

• Missed appointments for patients with VC before or following surgery may indicate a worried or angry patient and should be followed up with a phone call to reschedule and, if necessary, with a certified letter.

• Failure to outline all possible risks prior to surgery is another pitfall.

• Surgery for patients with VC may cause bleeding, infection, scar formation, deformity, and nerve damage.

• Removal of deeply invasive lesions may lead to substantial morbidity, including pain syndromes and paralysis.

• If a surgical complication develops, the physician who performed the primary procedure will be held legally responsible, regardless of who handles the complication.

• Any patient with lesions that are outside the realm of comfort of an individual physician should be referred to another physician.