This rare small cell malignancy of the skin is characterized by its potentially aggressive behaviour. Locally it is capable of producing satellite metastases whilst regional nodes are commonly involved. Conversely the appearance of systemic metastases is often delayed, thus opening a window for the possibility of curative therapy.

Staging

Liver function tests and a CT scan of the regional nodes should be obtained. Metastatic small cell carcinoma of the bronchus must be excluded; chest X-ray and CT scan of the chest should be performed. If either is abnormal the patient should be referred for bronchoscopy.

Treatment

• Primary tumour. Either a biopsy followed by radiation therapy with a wide margin (3 cm.) or wide excision with a similar margin. The choice will depend on the potential morbidity and cosmetic result of either treatment.
• Regional lymph nodes. Prophylactic or, in the case of those with established metastases, therapeutic irradiation of the regional nodes is recommended. This is considered preferable to surgical node dissection.
• Systemic therapy. Adjuvant chemotherapy is not recommended. There may be a role for palliative chemotherapy for systemic metastases.