Gastric-type mucinous adenocarcinoma of the cervix in a woman with Peutz-Jeghers syndrome: a case report

Abstract

Background

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by a predisposition to the development of multiple neoplasms. Gastric-type mucinous adenocarcinoma (GAS), a new variant of carcinoma of the cervix according to 2014 WHO classification, is less common compared with squamous cell carcinoma, is more aggressive and has a lower 5-year survival rate compared with the usual-type endocervical adenocarcinoma, and is also unrelated to human papillomavirus (HPV) infection.

Case summary

We herein present the case of a 32-year-old patient with PJS who was diagnosed with GAS of the cervix. The patient was not sexually active and had been diagnosed with PJS at 2 years of age. A tumor ∼6 cm was found on the cervix and was diagnosed as GAS of the cervix of clinical-stage IB3. The patient was treated with intra-arterial chemotherapy for one course, followed by radical surgery and then systematic chemotherapy.

Conclusion

The present case highlights the need for more thorough cancer screening for patients with PJS, as this disorder is rare and is associated with a high risk of malignancies. Young patients with PJS, including those who are not sexually active, who present with watery vaginal discharge or bleeding should be screened for cervical carcinoma, even if the cytological results or HPV tests are negative.

Keywords:

• Peutz-Jeghers syndrome
• gastric-type adenocarcinoma
• cervix
• HPV
• case report

Acknowledgements

We thank Professor Huijuan Zhang for providing pathology information and guidance on the case.

Ethics approval and consent to participate

The Institutional Review Board (International Peace Maternity and Child Health Hospital) approved this work.

Consent for publication

Informed consent for publication of clinical data/details/images was obtained from the patient. A copy of consent is available for review by the Editor of this journal.

Author contributions

TT: writing of the manuscript. FQ: providing the case details. SS: providing the case details. LYH: writing and editing of the manuscript. WYD: editing of the manuscript. The authors read and approved the final manuscript.

Disclosure statement

The authors declare that they have no competing interests.

Data availability statement

There is no dataset as this is a case report. Data/details of the patient available upon request.

Additional information

Funding

This research did not receive any specific grants or funding.