Abstract. Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Two cases of PNET of the cervix are presented herein. Two female patients, aged 48 and 43 years, presented with irregular uterine bleeding over the course of 1 year, and increased urinary frequency for 1 month, respectively. On gynecological examination, a mass in the cervix was palpated and a biopsy performed. The findings of the initial biopsy revealed small-cell carcinoma in both patients. Following neoadjuvant chemotherapy and radiotherapy, radical hysterectomy was performed in both patients. One patient received 5 courses of consolidation chemotherapy and postoperative radiotherapy, whereas the other patient received 1 course of consolidation chemotherapy. At the time of the article submission, both patients remained disease-free at 27 and 12 months, respectively, after their initial diagnosis. Only a limited number of cases of PNET of the cervix uteri have been reported in the literature to date. Multimodal therapies, including total excision, adjuvant chemotherapy and/or radiotherapy, have been adopted to treat patients with PNET of the cervix.
IntroductionThe term primitive neuroectodermal tumor (PNET) was first introduced by Hart and Earle to describe tumors composed of small round cells with different degrees of neural, glial and ependymal differentiation (1). PNETs have been classified according to the World Health Organization as central-and peripheral-type. Central PNETs usually involve the brain and spinal cord, whereas peripheral PNET involve the sympathetic nervous system, skeleton and soft tissues (2).PNETs of the cervix are extremely rare. To the best of our knowledge, only 14 cases have been described between 1987 and 2015 in the English literature (3-14) and there are currently no universally accepted standard treatment guidelines. Data on long-term follow-up are not available and the clinical outcome of PNET patients remains elusive (4-6). The aim of this study was to present two cases of primary PNET of the cervix and describe the diagnostic and treatment procedures.
Case reportsCase 1. A 48-year-old woman, gravida 3, para 2, presented with irregular uterine bleeding over the course of 1 year. On gynecological examination, a cervical mass rich in blood vessels was identified, measuring 6.0 cm in its greatest dimension (Fig. 1). The left parametrium was also involved, and the tumor was staged as IIb. A cervical biopsy was performed and the initial diagnosis was small-cell carcinoma.Histologically, the tumor comprised small blue-stained tumor cells with scant cytoplasm, arranged in dense sheets, without rosette or gland formation. Areas of necrosis were present. The neoplastic cells, some of which exhibited prominent nucleoli, infiltrated several capillaries. The cells uniformly expressed CD99 and vimentin, whereas synaptophysin (Syn), CD56, S-100 and epithelial membrane antigen were focally positive. Neuron-specific enolase (NSE), chromogranin A (CgA), cytokeratin (CK), CK5, CK8/18, P16, leukoc...