Neuroendocrine small cell carcinoma of the uterine cervix: What disease? What treatment? Report of ten cases and a review of the literature

Neuroendocrine small cell carcinoma of the uterine cervix (NESCCC) is an entity with very aggressive behaviour. The optimal initial therapeutic approach to this rare disease has not yet been clearly defined. We reviewed our experience of this disease over the past 10 years with regard to chemosensitivity. Since 1988, ten patients (mean age 33 years; range 24-47) have been diagnosed with NESCCC and treated in our institutions. Disease stage at presentation was IA (one), IB (five, two with lymph node involvement), IIB (one), IIIB (one), and IV (two). One patient had metastatic disease at presentation; three developed metastases during initial treatment. Eight patients underwent surgery and eight received radiation therapy. Six patients received pre- or postoperative cisplatinumvepeside (PE) combination chemotherapy, either alone or concurrently with radiation therapy. PE alone as primary chemotherapy led to disease stabilization in the two patients so treated; concurrent PE and radiation therapy resulted in a pathological complete response in one patient. Eight patients relapsed within 16 months and died of their disease within 29 months from the initial diagnosis. Two patients are alive with no evidence of disease at 13 and 53 months. Our series confirms the previously described very poor prognosis of NESCCC, despite initial aggressive multidisciplinary treatment. It may be that the introduction of chemotherapy, especially combined primary chemoradiotherapy, might allow patients to do a little better, although at the moment there is no good evidence one way or the other.