Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Jan 1;27(1):56-67.
doi: 10.1097/LGT.0000000000000712. Epub 2022 Oct 24.

Small Cell Carcinoma of the Vagina: First Systematic Review of Case Reports and Proposal of a Management Algorithm

Sira Capote  1 , Marta Domènech  2 , Lorena Valdivieso  3 , Victoria Tuset  4 , Myriam Sanchez  1 , Elvira Carballas  5 , Iris Teruel  2 , David Durany  6 , Gloria Moragas  7 , Carlos Molina  1 , Judith Lleberia  1 , Sergio Martínez-Roman  5 , Margarita Romeo  2
Affiliations

Small Cell Carcinoma of the Vagina: First Systematic Review of Case Reports and Proposal of a Management Algorithm

Sira Capote et al. J Low Genit Tract Dis. .

Abstract

Objectives: Small cell carcinoma of the vagina (SmCCV) is an extremely rare disease. Evidence-based data and specific guidelines are lacking. We conducted the first systematic review of case reports to provide the most overall picture of SmCCV.

Materials and methods: Literature search in PubMed and Scopus was performed using the terms "small cell carcinoma" and "vagina." English-language case reports of primary SmCCV up to January 2022 were included.

Results: Twenty-nine articles describing 44 cases met our inclusion criteria. We report a new case of our hospital. The global median overall survival (mOS) was 12.00 months (95% CI = 9.31-14.69). The mOS was not reached for stage I, and it was 12.00, 12.00, 9.00, and 8.00 months for stages II, III, IVA, and IVB, respectively (statistically significant differences between stage I and stages II, III, or IVA [log rank p = .003-.017]). Thirty-five cases received local treatments (77.8%). The mOS of patients treated with surgery ± complementary chemotherapy, radiotherapy ± complementary chemotherapy, chemoradiation ± complementary chemotherapy, and surgery + radiotherapy ± complementary chemotherapy were 11.00, 12.00, 17.00, and 29.00 months, respectively. The use of adjuvant or neoadjuvant chemotherapy (64.5%, mostly platinum + etoposide) showed longer mOS (77.00 vs 15.00 months). Four of 5 tested cases presented human papillomavirus infection, 3 of them presenting type 18.

Conclusions: Small cell carcinoma of the vagina shows dismal prognosis. Multimodal local management plus complementary chemotherapy seems to achieve better outcomes. Human papillomavirus could be related to the development of SmCCV. A diagnostic-therapeutic algorithm is proposed.

PubMed Disclaimer

Conflict of interest statement

M.D. has received honoraria from advisory board (Novartis) and travel grants for attending to medical congresses (Seagen, Roche, Bristol Meyers Squibb, Novartis, MDS, AstraZeneca, Lilly). I.T. has received honoraria for speaking (Novartis) and inscription grant for attending medical congresses (AZ) in the last year. S.M.R. has received honoraria from advisory boards AstraZeneca, TESARO, and ROCHE. M.R. has received honoraria from advisory boards (AstraZeneca/MSD, GSK, Merck and Clovis) and travel grants for attending medical congresses (Pfizer) in the last year. The other authors have declared they have no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Pathological study of vaginal tumor. Histologic examination shows (A) a bluish submucosal proliferation growing in a solid fashion close to the resection margins. Molding and smudging phenomena, especially in the periphery of the lesion and around the vessels, are a distinctive feature (hematoxylin-eosin [H&E], 2×). Diffuse areas and (B) well-defined nests are observed (H&E, 20×). C, A relatively monotonous population of small round cells with finely stippled “salt and pepper” nuclear chromatin, inconspicuous nucleoli, and scant cytoplasm constitutes the lesion. A thin delicate fibrovascular stroma is also noted (H&E, 40×). D, Immunohistochemistry reveals the neuroendocrine nature of the tumor cells with a diffuse membrane expression of synaptophysin. E, The neoplasm is also positive for chromogranin A with a perinuclear dot-like pattern. F, An intense nuclear p16 expression is identified. Cytokeratin positivity was demonstrated with (G) CAM.5, CK7, and CK20 stains.
FIGURE 2
FIGURE 2
Flow chart of studies retrieved and finally included in the meta-analysis.
FIGURE 3
FIGURE 3
Overall survival curve of our series according to the FIGO staging.
FIGURE 4
FIGURE 4
Proposal of staging and management algorithm for patients with SmCCV. BGLND, bilateral groin lymph node dissection; BPLND, bilateral pelvic lymph node dissection; BT, brachytherapy; CDDP, cisplatin; EBRT, external beam radiation therapy; ETO, etoposide; IMRT, intensity-modulated radiation therapy; M, metastases; N, ganglionar status; RH, radical hysterectomy; T, tumor. *The FIGO 2009 stage. **If possible 6 cycles of chemotherapy.
See this image and copyright information in PMC

References

    1. Frazier SR, Kaplan PA, Loy TS. The pathology of extrapulmonary small cell carcinoma. Semin Oncol 2007;34:30–8. - PubMed
    1. van Meerbeeck JP, Fennell DA, De Ruysscher DKM. Small-cell lung cancer. Lancet 2011;378:1741–55. - PubMed
    1. Crowder S, Tuller E. Small cell carcinoma of the female genital tract. Semin Oncol 2007;34:57–63. - PubMed
    1. He Y Zhao H Li XM, et al. . A clinical analysis of small-cell neuroendocrine carcinoma of the gynecologic tract: report of 20 cases. Arch Gynecol Obstet 2019;299:543–9. - PubMed
    1. Scully RE, Aguirre P, DeLellis RA. Argyrophilia, serotonin, and peptide hormones in the female genital tract and its tumors. Int J Gynecol Pathol 1984;3:51–70. - PubMed

Publication types