Introduction: Primitive cutaneous neuroendocrine carcinoma (PCNC) is a rare tumor with poor prognosis. Surgery is the treatment of choice, but radiotherapy is taking a larger place in the management of these patients.
Methods: The files of 22 patients were studied retrospectively over a period of 14 years.
Results: The study included 17 women and 5 men with a mean age of 75.5 years. The localization of the tumor was the cephalic extremity in 12 cases. At the initial stage, the tumor in 3 of the 22 patients was of glandular or visceral localization. Thirteen stage I patients were treated surgically. Adjuvant radiotherapy was performed in 10 of these patients and only one relapsed (incomplete initial exeresis). The other three relapsed. Exclusive radiotherapy led to complete response in 4 patients who could not undergo surgery and in 2 with local relapses. Seven patients exhibited glandular involvement and 6 of them died (median 7 months) after the adenopathy had been discovered.
Discussion: Our series illustrates the clinical characteristics of this tumor of the elderly, which is predominantly cephalic and of rapid progression. From a therapeutic point of view, our results underline the benefit of radiotherapy as adjuvant to surgery. When surgery is impossible, and in the case of local relapse, radiotherapy gives good results.