Once the stage of disease has been determined, a treatment plan is formulated. As mentioned above, because cervical cancer is clinically staged, some of the findings on the initial workup are not included in the official stage, but do alter treatment recommendations. Because the disease is so rare, there are no completed prospective trials to date establishing the standard of care for the treatment of this disease. While treatment plans are often extrapolated from treatment of more common types of cervical cancers, given the aggressive nature of this particular disease, a multimodal approach to treatment is more often employed. In 2011, the Society of Gynecologic Oncology issued a clinical document summarizing available literature on NET of the female reproductive tract . The treatment algorithms below are based on that document . Additionally, there is extensive data for treatment of high-grade lung and extrapulmonary NEC provided by the North American Neuroendocrine Tumor Society (NANETS) and their published consensus guidelines . Knowledge gained from treatment of NEC affecting other sites of origin has been extrapolated to the treatment of cervical NEC.
For early stage disease that is confined to the cervix and has not spread to the lymph nodes or other organs, the initial treatment depends on tumor size and involvement of local tissue.
For tumors less than 4cm in size that do not appear to locally invade other pelvic structures based on physical exam, initial treatment frequently includes surgery in the form of radical hysterectomy and removal of appropriate lymph nodes. Ideally, this should be performed by a gynecologic oncologist. Pathological information from surgery will dictate additional care, although usually further therapy is recommended, even if the tumor has been completely resected with negative margins. Usually chemotherapy with a combination of cisplatin and etoposide (EP) is recommended. Frequently this is given at the same time a radiation therapy, and additional chemotherapy is given following completion of chemoradiation. An acceptable alternative approach for initial treatment of women in this category may be with chemoradiotherapy followed by chemotherapy alone, without surgery.
For early stage disease with bulky tumors (>4cm), chemoradiation is recommended. There is limited data about the use of neoadjuvant chemotherapy then proceeding with surgery, followed by more chemotherapy with consideration for radiotherapy as well.
For locally advanced disease including those with lymph node metastatsis, the recommended approach includes a combination of chemotherapy and radiation.
When disease has spread to other organs beyond the pelvis and lymph nodes, chemotherapy is the recommended treatment. This can include the two drugs mentioned above, cisplatin and etoposide. Another regimen that has been used effectively and was borrowed from treatment of lung cancer is VAC/PE (vincristine, adriamycin and cyclophosphamide, alternating with cisplatin and etoposide).
Unlike NEC of the lung, given the uncommon incidence of spread NEC of the cervix to the brain, prophylactic cranial irradiation (PCI) is not recommended at this time.
For patients without evidence of disease at the completion of their primary treatment, frequent routine follow-up with their physician is recommended. They may choose to perform imaging such as a CT or PET/CT scan at regular intervals, and imaging is recommended for any patient with symptoms that might indicate recurrence.