Management of Skin Cancer of the Head and Neck

History

• 1.

  History of present illness

  • a.

    How long has the lesion been present? Lesions that have not undergone change for many years tend to be benign.

  • b.

    Has the lesion changed in size? Increasing size or growth is a signal to consider malignancy and biopsy.

  • c.

    Are there associated symptoms? Bleeding commonly indicates malignancy. Dysethesias, paresthesias, anesthesia, or loss of motor function (uncommon) may indicate perineural invasion.

  • d.

    Has the lesion been treated previously? Previous treatment is a signal that the original treatment was inadequate, usually secondary to unrecognized positive margins. Routine pathologic examination only samples the surgical margins, therefore a report of clear margins is only an estimate. In the face of recurrence, retreatment with routine surgery is associated with much higher recurrence rates. Consider Mohs surgery.

• 2.

  Past medical history

  • a.

    Medical illness

    Predisposing syndromes that influence management

    • 1)

      Basal cell nevus syndrome

      • a)

        Features: multiple BCCs, palmoplantar pits, frontal bossing, hypertelorism, odontogenic keratocysts, skeletal abnormalities, and calcification of the falx cerebri

      • b)

        Relevance: Tumors often appear before puberty and number in the hundreds over a lifetime. Patients typically become discouraged by the need for frequent treatment, are often lost to follow-up, and ultimately reappear with multiple difficult-to-treat lesions.

      • c)

        Management: Counsel to prevent discouragement and lack of regular follow-up. Plan treatments to minimize loss of work/school and quality time. Select treatments with a high cure rate to prevent repeat treatments and minimize deformity.

      • d)

        Adjunctive therapy: Chemoprophylactic treatment with retinoids (topical or systemic) has no long-term benefit. The smoothened receptor inhibitor, vismodegib, is food and drug administration (FDA) approved for advanced cases but is typically limited by intolerable side effects at standard dosing (dysgeusia, gastrointestinal disturbance, weight loss, myalgias, alopecia).

    • 2)

      Muir-Torre syndrome (Subset of Lynch syndrome)

      • a)

        Features: at least one cutaneous sebaceous neoplasm (usually a sebaceous carcinoma/adenoma/epithelioma; sometimes a keratoacanthoma) and one visceral malignancy (most commonly colorectal or urogenital)

      • b)

        Relevance: A single cutaneous sebaceous neoplasm warrants further investigation. Truncal lesions are most commonly associated with the syndrome; periocular are more likely sporadic. Testing the pathology specimen for defective mismatch repair proteins via immunohistochemistry is a sensitive screen (detects loss of protein expression of MLH1, MSH2, MSH6, or PMS2 within the tumor) and should be requested. If loss is noted, refer the patient for colonoscopy and genetic counseling.

      • c)

        Management: Most patients have only a few cutaneous cancers (curable if treated early) and should be screened regularly during their lifetime (professional and self-examination). Routine gastrointestinal and primary care appointments for visceral malignancy screening are key. Family members should also be tested.

    • 3)

      Syndrome of multiple trichoepitheliomas

      • a)

        Features: Trichoepitheliomas, benign neoplasms of the hair follicle, appear as small papules on the medial aspect of the cheeks, nose, and upper lip (also, ears and elsewhere); these lesions increase in number and size over the patient’s lifetime. Cylindromas and spiradenomas may also be present.

      • b)

        Relevance: Management difficulties arise.

        • i)

          Trichoepitheliomas resemble BCC clinically and histologically; misdiagnosis may occur if an accurate clinical description is not given or if an expert pathologist is not available.

        • ii)

          BCCs often develop within benign trichoepitheliomas and require adequate treatment.

        • iii)

          Pathology examination is complex because of confusion between benign trichoepithelioma and malignant BCC at the margin.

      • c)

        Management: A lesion that is enlarging, ulcerated, or looks different from the others should be biopsied and sent to a dermatopathologist.

    • 4)

      Dysplastic nevus syndrome; atypical mole syndrome

      • a)

        Arises spontaneously or as an autosomal dominant trait with incomplete penetrance

      • b)

        Features: dozens of clinically atypical nevi, usually greater than 6 mm in diameter, and often with nonuniform pigmentation. Borders appear to diffuse into the surrounding skin (normal nevi have clearly distinct borders). Highest density of nevi on the trunk, head, neck and scalp. Biopsied lesions are labeled as dysplastic nevi (DN) based on pathologic architecture.

      • c)

        Relevance: associated with an increased risk for melanoma

        • i)

          5% lifetime risk (1% for the average population)

        • ii)

          A single DN may increase the risk.

        • iii)

          Having multiple nevi and a family history of melanoma confers the highest risk, approaching 100%.

      • d)

        Management: Controversy exists regarding diagnosis and treatment.

        • i)

          DN resemble melanoma in some ways (clinically and histologically).

        • ii)

          Biopsies are common because of the irregular clinical appearance.

        • iii)

          Pathologists may have difficulty distinguishing the morphologic and cytologic atypia characteristic of DN from melanoma.

        • iv)

          Margins are often positive.

        • v)

          Ambiguous pathology reports leave management decisions to the clinician, who may be confused about the unresolved controversy.

        • vi)

          DN with mild atypia are benign and do not require excision, regardless of biopsy margin status.

        • vii)

          DN with moderate atypia are also benign. If removal was attempted on biopsy and pathology reports clear margins in the plane examined, we do not recommend re-excision. If biopsy margins are positive, the patient may be followed clinically or re-excision may be performed.

        • viii)

          DN with severe atypia should be conservatively re-excised as one pathologist’s DN with severe atypia may be another’s melanoma or melanoma in situ (MIS).

      • e)

        Importantly:

        • i)

          DN may be found in patients with or without DN syndrome.

        • ii)

          Melanomas may appear in pre-existing nevi but often develop in normal skin.

        • iii)

          Elective removal of DN does not decrease the risk for melanoma.

        • iv)

          A recurrent nevus may falsely resemble a melanoma on histopathology; a history of previous biopsy or lesion at the biopsy site should be noted in the pathology requisition.

        • v)

          Counsel patients to minimize lifetime exposure to ultraviolet light, to perform regular skin self-examinations, to have yearly checkups by dermatologists, and to understand the genetics for children and close family members.

    Diseases associated with skin cancer

    • 5)

      Oral lichen planus

      • a)

        Chronic inflammatory condition of skin and mucous membranes

      • b)

        Whitening of the vermilion lip, ulceration, thickening of the epidermis, and induration caused by the inflammatory response can simulate SCC clinically and histologically.

      • c)

        Predisposes to development of true invasive SCC

      • d)

        Clinicopathologic correlation is imperative.

    • 6)

      Solid organ transplant

      • a)

        Skin cancer (majority are SCC) is a serious, nearly universal long-term sequela.

      • b)

        Emphasizes the important role of immune surveillance in the prevention of skin cancer

      • c)

        Patients often become discouraged with the overwhelming number of skin cancers, the need for frequent treatment, and the time and effort required to keep up with therapy.

      • d)

        Neglected or recurrent disease may be life-threatening and is often the cause of death in transplant patients.

      • e)

        Coordinating medical management with the transplant team to optimize the immunosuppressive drug regimen is often advisable.

      • f)

        Early effective treatment, constant surveillance, follow-up, and patient education are imperative.

    • 7)

      Chronic immunosuppression

      • a)

        Human immunodeficiency virus–positive patients with low CD4 lymphocyte counts have a higher risk for SCC (and other skin cancers). Chronic human papillomavirus infection plays an important role.

      • b)

        Compromises the prognosis of patients with known metastases or those at high risk for metastases

  • b.

    Surgery

  • c.

    Family history

  • d.

    Medications

    • 8)

      Many medications increase the risk of postoperative bleeding.

      • a)

        Antiplatelet drugs

      • b)

        Vitamin K antagonists

      • c)

        Direct thrombin inhibitors

      • d)

        Factor Xa inhibitors

      • e)

        High dose Vitamin E

      • f)

        Herbal products (garlic, gingko, feverfew, ginger, and saw palmetto)

    • 9)

      The risk of postoperative bleeding or hematoma (<1%) is a minor concern in comparison to consequences of myocardial infarction or cerebral vascular accidents; therefore it is ill-advised to stop physician-prescribed anticoagulants for skin cancer surgery.

    • 10)

      Self-prescribed supplements should be stopped 10 to 14 days prior to surgery.

  • e.

    Social history

    • 1)

      Alcohol

      • a)

        Increases risk of postoperative bleeding

      • b)

        Advise abstinence for 24 hours leading up to and 48 hours postprocedure.

    • 2)

      Tobacco

      • a)

        Increases risk of flap/graft failure

      • b)

        Adversely effects cosmesis

      • c)

        Patients should be counseled to quit (or decrease consumption as feasible).

    • 3)

      Living situation/support system

      • a)

        One repair option may be favored over another based on postsurgical needs.

      • b)

        May determine ability to pursue adjuvant therapies

Physical Examination

• 1.

  Crucial to preoperative planning and decision making

• 2.

  Evaluating tumor size and margins

  • a.

    Nonmelanoma skin cancers:

    • 1)

      Tightly stretch the skin under light-emitting diode (LED) examination or operating room lights.

    • 2)

      Clues to tumor extension of BCC and SCC

      • a)

        Subtle pink color (vascular response induced by inflammation)

      • b)

        Scar-like white or yellow color (fibrosis and tumor infiltration)

      • c)

        Shiny or pearly appearance (underlying tumor lobules obscuring normal fine skin wrinkling)

      • d)

        Dermatitis-like appearance

        • i)

          Extension of superficial BCC or SCC in situ beyond invasive disease may simulate dermatitis, appearing totally different from the primary tumor (must distinguish from dermatitis due to bandaging).

    • 3)

      Tumor depth

      • a)

        Difficult to estimate beyond simple palpation and movement over underlying structures (Mobile? Attached to bone?)

  • b.

    Melanoma of the head and neck

    • 1)

      Margin assessment is more difficult compared to the trunk/extremities.

    • 2)

      Lentigines, freckles, seborrheic keratoses, and actinic keratoses (AKs) may all camouflage the margin.

    • 3)

      In situ extensions at the edge are often amelanotic, simulate dermatitis, or may be totally invisible.

• 3.

  Evaluating for local, regional, or metastatic cancer

  • a.

    Palpate the regional skin, soft tissues, and lymph nodes to determine the presence of satellite metastases, in transit metastases, or lymph node metastases.

  • b.

    Important in SCC, melanoma, MCC, undifferentiated pleomorphic sarcoma (UPS) (previously MFH), and sebaceous carcinoma

Imaging

• 1.

  Adjunctive testing to evaluate lymph nodes for metastatic cancer has limited value.

• 2.

  Imaging may be useful in obese patients in whom palpation of early clinical disease is difficult.

• 3.

  Preoperative imaging to determine the extent of invasion is minimally helpful.

  • a.

    Only estimates the likelihood of bone invasion, orbit invasion, or involvement of vital structures of the neck

  • b.

    Estimates the extent of gross tumor involvement but cannot detect microscopic extensions of skin cancer in soft tissue that often extend many millimeters from the main tumor mass

Indications

• 1.

  Most are surgical candidates.

• 2.

  Most skin cancers are small (<2 cm) and can be treated under local anesthesia in the office, ambulatory surgery center, or hospital outpatient setting with or without sedation.

• 3.

  Common contraindications to surgery often do not apply to smaller procedures.

  • a.

    Anticoagulants may be safely continued (see [CR] above).

  • b.

    Age is generally not an issue.

    • 1)

      Most tumors require simple, low-risk operative procedures.

    • 2)

      A short, 1-day procedure is less disruptive to fragile, elderly patients than managing chronic, nonhealing ulcers from untreated skin cancer.

Contraindications

• 1.

  True contraindications to skin cancer surgery are rare.

• 2.

  Life expectancy is more important than age.

  • a.

    Consider the biologic behavior of the skin cancer and the patient’s health and life expectancy.

    • 1)

      BCC is a slowly growing cancer that may be asymptomatic for years. There may be no need to treat a small, nonulcerated BCC in a patient with other metastatic disease and a life expectancy of less than 1 year.

    • 2)

      Treatment of nodular melanoma in an independent 95-year-old patient may be curative and allow the family to celebrate the potential 100-year mark.

• 3.

  Extent of disease and the impact of surgery

  • a.

    Very rarely, extensive or invasive cancers may require surgery that has a significant mortality rate, impacts quality of life after surgery, or is unlikely to achieve cure. The balance of risks and benefits must be weighed carefully.

    • 1)

      Is it worthwhile to remove one eye because of a slowly growing BCC in an elderly patient who has little or no vision in the other eye?

    • 2)

      Is temporal bone resection for management of a “close positive margin” after excision of SCC worth the mortality risk?

• 4.

  Informed consent must include the probability of results from treatment versus no treatment, alternative forms of therapy, and palliative treatment options.

Preoperative Preparation: Determining Surgical Margins by Histologic Tumor Type