23. Neuroendocrine cervical carcinoma – Cervical Carcinoma
Major changes in the chapter on neuroendocrine cervical carcinoma
The chapter has undergone few changes. It has been extracted from the previous Chapter 8, Foundations of therapy and updated as a separate chapter.
T. Fehm, A. Bartens, F. Martogini, A. K. Dietl, D. Gantert
Neuroendocrine cervical carcinoma (NECC), representing 1–1.5% of cervical carcinomas, is a particularly rare but high-risk form of cervical carcinoma [828], [829]. It frequently occurs in younger women (mean age of onset 45 years) [830], [831]. Detection of HPV, particularly HPV-16 and -18, appears to be a risk factor [832]. NECC is usually an exophytically growing tumor [143].
NECC is divided by the WHO into two main types, depending on the degree of differentiation: NECC with low differentiation (small cell and large cell neuroendocrine cervical carcinoma), and NECC with high differentiation (typical and atypical carcinoid) [393], [833]. Small cell NECC is the most common subtype, accounting for 80% of cases [393] (section 7.1, Classification of invasive cervical carcinomas).
NECC has a generally poor prognosis, independently of the treatment, and also a poorer prognosis in comparison with adenocarcinoma and squamous cell carcinoma [138], [139], [834], [835], [836], [837]. The prognosis of the disease is mainly influenced by the FIGO stage and lymph-node status [838], as well as infiltration depth [834], smoking, age, and purely small cell histology [142], [837]. Even in the early stages (I to IIA), regional lymph-node or hematogenous distant metastases occur in 40–60% of the patients. Recurrence or metastasis to the lung, liver, bone, brain, or lymph nodes is common within 1 year [139], [143], [835]. The 5-year survival rate ranges from 34% to 37%, with a median overall survival of 40 months [839], [840]. The median recurrence-free survival is 16 months [839].
In early stages (FIGO I–IIA), radical hysterectomy, optionally followed by adjuvant chemotherapy (or with a primary neoadjuvant approach), is most common and appears to be associated with the best survival rates [839], [840]. By analogy with the histologically similar small cell lung cancer (SCLC), chemotherapy consisting of etoposide and cisplatin/carboplatin (PE) or vincristine, Adriamycin (doxorubicin), and cyclophosphamide (VAC) is often administered [839], [840]. In locally advanced NECC (IIB–IVB) or with recurrences, combined radiochemotherapy or chemotherapy is administered [839], [840]. With recurrences after platinum-containing primary therapy, a combination of topotecan, paclitaxel, and bevacizumab is possibly superior in comparison with PE or VAC [839]. Few studies of radiochemotherapy are as yet available. However, brachytherapy in addition to teletherapy appears to improve the median survival (48.6 vs. 21.6 months) and should be discussed when planning radiotherapy [839]. For treatment planning, the case can be presented at a specialized tumor conference for neuroendocrine tumors.
In the clinical follow-up, it should be noted that a total of 68% of the patients die in the second and third year after the disease [839].
References
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