CUTANEOUS T-CELL LYMPHOMAS (INCLUDING MYCOSIS FUNGOIDES)

•Mycosis fungoides is the most frequent of the cutaneous T-cell lymphomas.

•Erythematous scaling plaques and patches are seen in early localized forms (Figure 34-26).

•Plaques and patches may be hyperpigmented or hypopigmented (Figure 34-27).

•Can progress to diffuse erythema (erythroderma) in later disseminated stage—Sézary syndrome.

•May present as refractory or recurrent eczema.

•Itching and photosensitivity are common.

•May develop into raised fungating lesions (see nasal lesion in Figure 34-27).

FIGURE 34-26  Plaque-type mycosis fungoides on the extremities and trunk. Biopsy a few years before this was read as atopic dermatitis only. Lack of response to medications and the suspicious appearance led to a new biopsy and the correct diagnosis. (Courtesy of Deborah Henderson, MD.)

FIGURE 34-27  Cutaneous T-cell lymphoma causing large hyperpigmented patches and plaques on this Ethiopian woman. She also has a tumor under her left ala. (Copyright Richard P. Usatine, MD.)

Biopsy

•Perform a broad shave biopsy or a 4-mm punch biopsy of involved skin.

•Alert the pathologist that this is on your differential diagnosis so that special stains can be done.

•The first biopsy may not show the disease, so consider repeating the skin biopsies if the clinical suspicion remains high.

•Flow cytometry can be done on peripheral blood to look for abnormal lymphocytes.

•Palpate for lymph nodes and consider a lymph node biopsy if the skin biopsies and flow cytometry are not definitive.

•CT of the abdomen and pelvis can be used to look for enlarged lymph nodes and/or splenomegaly.

•Consult specialists if needed—this can be a hard diagnosis to make.

Treatment and Prognosis

•Early stages are treated locally with topical steroids, nitrogen mustards, psoralen and UVA, UVB, and others with excellent prognosis for remission and preventing progression of disease.

•More advanced disease carries a poor prognosis and is treated with a combination of topical medications as above, radiation therapy, and systemic immune system modulators and chemotherapy.

DERMATOFIBROSARCOMA

PROTUBERANS

Diagnosis

•Flesh-colored gradually enlarging nodule from one to several or more centimeters in diameter. It may be shiny with a look of multiple deep nodules (Figure 34-28).

•It is of different origin than a dermatofibroma and is not a dermatofibroma becoming malignant.

•May be erythematous or occasionally pigmented.

•Site distribution is typically 45% head and neck and 55% trunk and extremities.18

Biopsy

•Use a 4-cm punch biopsy or larger incisional biopsy to provide adequate tissue for diagnosis.

FIGURE 34-29  Merkel cell carcinoma on the ear. (Courtesy of Frank Miller, MD.)

Treatment and Prognosis

•The tumor can invade into adjacent deep structures, becoming locally invasive and highly recurrent.

•Mohs surgery is the preferred treatment to decrease the recurrence rate.

•Wide local excision with 2–4 cm margins is the alternative with lower cure rates.

•One study using pooled data from the literature reported a recurrence rate of 1.3% with Mohs surgery and 20.7% with wide local excision.19

•Frequent clinical follow-up is indicated for more than 5 years because 25% of recurrences occur after 5 years.18

MERKEL CELL CARCINOMA

Diagnoses

•Blue red nodular lesion most commonly of the head and neck (Figures 34-29 and 34-30).

•Mostly older patients and/or immunosuppressed persons.

•Rapidly growing.

•Usually in sun-exposed areas (Figure 34-30).

•Viral etiology with polyoma virus under inves­ tigation.20

Biopsy

• Perform a shave, 4-mm punch, or excisional biopsy.

Treatment and Prognosis

•High rate of nodal metastasis and distant metastasis even for small primary tumors.21

•Up to 30% have regional lymph node metastases.20

FIGURE 34-28  Dermatofibrosarcoma protuberans on the leg. (Copyright Richard P. Usatine, MD.)

FIGURE 34-30  Merkel cell carcinoma on the lip. (Courtesy of Jeff Meffert, MD.)

•Chest x-ray to check for lung cancer as primary site with cutaneous metastasis.

•Wide local excision with 2- to 3-cm margins.

•Best outcome is achieved with multidisciplinary management that includes local radiotherapy and chemotherapy for distant metastases.20–22

CODING AND BILLING PEARLS

•Bill for malignant lesion removals after pathology is determined because malignancies have different codes and higher reimbursements.

•When calculating the size of the excised lesion, it is important to include the necessary margins. Therefore, if you are excising a 1-cm basal cell carcinoma with 4-mm margins, you would bill for an excision at 1.8 cm. Do not forget to make these measurements so that you get paid for what you do.

CONCLUSION

There are choices among the methods of removal of malignant lesions. Small lesions that are not near important structures, have distinct margins, and are not high-grade cancers can usually be managed by clinicians with good basic skills in their office. For high-risk locations and lesions, the best results of complete lesion removal are typically obtained by Mohs micrographic surgery. This chapter has provided a general guideline for malignant lesion management, but as always providers must use their best clinical judgment and adjust the specific treatment based on their individual patient’s needs.

Resources

The American Cancer Society (www.cancer.org) provides information on skin cancers and brochures for patients and clinicians on the ABCDE rules for melanoma.

References

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