Childhood Cervical and Vaginal Cancer Treatment (PDQ®)–Health Professional Version

Adenocarcinoma of the cervix and vagina is rare in childhood and adolescence.[1,2] Two-thirds of cases in previous reports have been associated with exposure to diethylstilbestrol (DES) in utero.[3] However, the few case reports of vaginal cancer in children in the last decade have not been associated with exposure to DES in utero.[4]

The median age at presentation is 15 years, with a range of 7 months to 18 years. Most patients present with vaginal bleeding. Adults with adenocarcinoma of the cervix or vagina present with stage I or stage II disease 90% of the time.[1] In children and adolescents, there is a high incidence of stage III and stage IV disease (24%). This difference may be explained by the practice of routine pelvic examinations in adults and the hesitancy to perform them in children.

References

1. McNall RY, Nowicki PD, Miller B, et al.: Adenocarcinoma of the cervix and vagina in pediatric patients. Pediatr Blood Cancer 43 (3): 289-94, 2004. [PUBMED Abstract]
2. You W, Dainty LA, Rose GS, et al.: Gynecologic malignancies in women aged less than 25 years. Obstet Gynecol 105 (6): 1405-9, 2005. [PUBMED Abstract]
3. Huo D, Anderson D, Palmer JR, et al.: Incidence rates and risks of diethylstilbestrol-related clear-cell adenocarcinoma of the vagina and cervix: Update after 40-year follow-up. Gynecol Oncol 146 (3): 566-571, 2017. [PUBMED Abstract]
4. Fernandez-Pineda I, Spunt SL, Parida L, et al.: Vaginal tumors in childhood: the experience of St. Jude Children's Research Hospital. J Pediatr Surg 46 (11): 2071-5, 2011. [PUBMED Abstract]

The Fédération Internationale de Gynécologie et d’Obstétrique (FIGO) system is used to stage cervical and vaginal cancer. For more information, see the Staging Information for Cervical Cancer section in Cervical Cancer Treatment and the Staging Information for Vaginal Cancer section in Vaginal Cancer Treatment.

Treatment options for childhood carcinoma of the cervix and vagina include the following:

The treatment of choice is surgical resection,[1] followed by radiation therapy for residual microscopic disease or lymphatic metastases. The role of chemotherapy in management is unknown. However, drugs commonly given for the treatment of gynecological malignancies, such as carboplatin and paclitaxel, have been used.[2,3]

In a retrospective report, 37 patients with cervical clear cell adenocarcinoma or cervical mesonephric adenocarcinoma were treated with various modalities (surgery, radiation therapy, and/or chemotherapy). The 3-year event-free survival rate was 71% (± 11%) for patients with all stages of tumors, 82% (± 11%) for patients with stage I and stage II tumors, and 57% (± 22%) for patients with stage III and stage IV tumors.[4]

References

1. Abu-Rustum NR, Su W, Levine DA, et al.: Pediatric radical abdominal trachelectomy for cervical clear cell carcinoma: a novel surgical approach. Gynecol Oncol 97 (1): 296-300, 2005. [PUBMED Abstract]
2. Baykara M, Benekli M, Erdem O, et al.: Clear cell adenocarcinoma of the uterine cervix: a case report and review of the literature. J Pediatr Hematol Oncol 36 (2): e131-3, 2014. [PUBMED Abstract]
3. Singh P, Nicklin J, Hassall T: Neoadjuvant chemotherapy followed by radical vaginal trachelectomy and adjuvant chemotherapy for clear cell cancer of the cervix: a feasible approach and review. Int J Gynecol Cancer 21 (1): 137-40, 2011. [PUBMED Abstract]
4. McNall RY, Nowicki PD, Miller B, et al.: Adenocarcinoma of the cervix and vagina in pediatric patients. Pediatr Blood Cancer 43 (3): 289-94, 2004. [PUBMED Abstract]

Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website.

Cancer in children and adolescents is rare, although the overall incidence has slowly increased since 1975.[1] Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the following pediatric specialists and others to ensure that children receive treatment, supportive care, and rehabilitation to achieve optimal survival and quality of life:

For specific information about supportive care for children and adolescents with cancer, see the summaries on Supportive and Palliative Care.

The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of children and adolescents with cancer.[2] At these centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate is offered to most patients and their families. Clinical trials for children and adolescents diagnosed with cancer are generally designed to compare potentially better therapy with current standard therapy. Other types of clinical trials test novel therapies when there is no standard therapy for a cancer diagnosis. Most of the progress in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI website.

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[3-5] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer.

Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[6] The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people in the United States. Therefore, all pediatric cancers are considered rare.

The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[7,8] In children and adolescents, the designation of a rare tumor is not uniform among international groups, as follows:

These rare cancers are extremely challenging to study because of the relatively few patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the small number of clinical trials for adolescents with rare cancers.

Information about these tumors may also be found in sources relevant to adults with cancer, such as Cervical Cancer Treatment and Vaginal Cancer Treatment.

References

1. Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [PUBMED Abstract]
2. American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. Also available online. Last accessed August 23, 2024.
3. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014. [PUBMED Abstract]
4. National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed August 23, 2024.
5. Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed September 5, 2024.
6. Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr. [PUBMED Abstract]
7. Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017. [PUBMED Abstract]
8. DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017. [PUBMED Abstract]
9. Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019. [PUBMED Abstract]
10. Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010. [PUBMED Abstract]

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

This summary was comprehensively reviewed.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.