Abstract Neuroendocrine small cell carcinoma of the uter- ine cervix (SCC) is a rare disease that mixes clinical and biological characteristics of both cervical neoplasms and neuroendocrine small cell cancer. The prognosis is poor and the optimal treatment has not yet been clarified. Mul- timodality treatment, with surgery and concurrent chemo- radiation has recently been shown to improve local control and survival rates. Keywords Neuroendocrine small cell carcinoma · Radiochemotherapy Introduction Neuroendocrine small cell carcinoma (SCC) of the uter- ine cervix is a rare and aggressive entity [1, 2]. It mixes clinical and biological characteristics of both cervical neo- plasms and neuroendocrine SCC of any site, making it an original nosologic entity [3]. SCC accounts for 1–3% of all cervical neoplasms [3, 4]. The diagnosis of neuroendocrine SCC requires the presence of small round or fusiform cells with scanty cytoplasm, frequent mitoses, hyperchromatic nuclei having finely granular chromatin, and absent or inconspicuous nucleoli, with or without positive staining of neuroendocrine markers [3, 5]. Indeed, the main charac- teristic of this tumour is its poor prognosis [3]. The optimal treatment has not yet been completely clarified [3]. Multi- modality treatment, with surgery and concurrent chemora- diation, has recently been shown to improve local control and survival rates [3]. Case report A 38-year-old woman presented with a history of vaginal bleeding. Initial physical examination revealed a cervical mass. Cervical biopsy demonstrated neuroendocrine SCC of the uterine cervix. Computed tomography (CT) scan and magnetic resonance imaging (MRI) re- vealed a 6  5  5.4-cm cervical tumour, with parametrial involve- ment. Clinical staging according to the International Federation of Gynecology and Obstetrics (FIGO) classification was stage IIB. The patient received pelvic radiotherapy to a total dose of 45 Gy/1.8 Gy daily/25 fr concurrent with the first cycle of chemotherapy for six courses of a combination of intravenously administered cisplatin 40 mg/m 2 day 1 and etoposide 100 mg/m 2 days 1, 3 and 5; then, a boost of 10 Gy with low-dose-rate brachytherapy was given. After this, a hysterectomy was done, and histology revealed the presence of a 2  2.5  2-cm small cell tumour with hyperchromatic nuclei and high mitotic index, chromogranin positive, involving the endocervical canal and with vascular invasion. The patient remained disease free for 24 months after diagnosis. Two months later, she presented with headache and hemiparesis, and CT scan revealed cerebral metastases. The patient died 3 months after diagnoses. Discussion Neuroendocrine SCC of the cervix is a rare and aggressive subtype of cervical cancer [1]. Up to 5% of all SCC arise in extrapulmonary sites. SCC of the female genital tract is rare, constituting <2% of all gynaecologic malignancies [1], and occurs most frequently in the cervix but can also occur in the endometrium and other sites [1]. As with other primary sites, it is associated with early distant metastases [5] and poor prognosis with the current therapeutic modali- ties [1]. Abeler et al. [6] concluded that SCC of the cervix is one of the most aggressive tumours of the female genital tract. Large tumour size, lymph node metastases, stage, A. Reig Castillejo () · I. Membrive Conejo · P. Foro Arnalot · N. Rodríguez de Dios · M. Algara López Institut d’Oncologia Radioteràpica Hospital de l’Esperança Sant Josep de la Muntanya, 12 ES-08024 Barcelona, Spain e-mail: areig@parcdesalutmar.cat Clin Transl Oncol (2010) 12:512-513 DOI 10.1007/s12094-010-0546-0 CASE REPORTS Neuroendocrine small cell carcinoma of the uterine cervix Anna Reig Castillejo · Ismael Membrive Conejo · Palmira Foro Arnalot · Nuria Rodríguez de Dios · Manuel Algara López Received: 20 July 2009 / Accepted: 1 November 2009