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Gastric-type adenocarcinoma of the cervix in patients with Peutz-Jeghers syndrome: a systematic review of the literature with proposed screening guidelines
  1. Sushmita B Gordhandas1,
  2. Ryan Kahn1,
  3. Dib Sassine2,
  4. Emeline M Aviki1,3,
  5. Becky Baltich Nelson3,
  6. Amanda Catchings4,
  7. Ying L Liu3,4,5,
  8. Yuliya Lakhman6,
  9. Nadeem R Abu-Rustum1,3,
  10. Kay J Park7 and
  11. Jennifer J Mueller1,3
  1. 1 Gynecology Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  2. 2 Department of Obstetrics and Gynecology, Columbia University Vagelos College of Physicians and Surgeons, New York, New York, USA
  3. 3 Joan & Sanford I. Weill Medical College of Cornell University, New York, New York, USA
  4. 4 Clinical Genetics Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA
  5. 5 Gynecologic Medical Oncology, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  6. 6 Body Imaging Service, Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  7. 7 Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  1. Correspondence to Dr Jennifer J Mueller, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA; muellerj{at}mskcc.org

Abstract

Objectives To perform a systematic review of gastric-type adenocarcinoma of the cervix and lobular endocervical glandular hyperplasia (a possible precursor lesion) in Peutz-Jeghers syndrome, and to analyze data from the literature, along with our institutional experience, to determine recommendations for screening and detection.

Methods A comprehensive literature searc and retrospective search of pathology records at our institutio were conducted. Articles were screened by two independent reviewers. Case reports/series on lobular endocervical glandular hyperplasia/gastric-type adenocarcinoma of the cervix in Peutz-Jeghers syndrome were included. Demographic, clinical, and radiologic information was collected.

Results A total of 1564 publications were reviewed; 38 met the inclusion criteria. Forty-nine were included in the analysis (43 from the literature, 6 from our institution). Forty-three reported on gastric-type adenocarcinoma alone, 4 on lobular endocervical glandular hyperplasia alone, and 2 on concurrent lobular endocervical glandular hyperplasia/gastric-type adenocarcinoma. Median age at diagnosis was 17 (range, 4–52) for patients with lobular endocervical glandular hyperplasia alone and 35 (range, 15–72) for those with gastric-type adenocarcinoma. The most common presenting symptoms were abdominal/pelvic pain and vaginal bleeding/discharge. Imaging was reported for 27 patients; 24 (89%) had abnormal cervical features. Papanicolaou (Pap) smear prior to diagnosis was reported for 12 patients; 6 (50%) had normal cytology, 4 (33%) atypical glandular cells, and 2 (17%) atypical cells not otherwise specified. Patients with gastric-type adenocarcinoma (n=45) were treated with surgery alone (n=16), surgery/chemotherapy/radiation (n=11), surgery/chemotherapy (n=9), surgery/radiation (n=5), or radiation/chemotherapy (n=4). Twelve (27%) of 45 patients recurred; median progression-free survival was 10 months (range, 1–148). Twenty patients (44%) died; median overall survival was 26 months (range, 2–156). Thirteen patients (27%) were alive with no evidence of disease.

Conclusions Gastric-type adenocarcinoma in Peutz-Jeghers syndrome is associated with poor outcomes and short progression-free and overall survival. Screening recommendations, including pathognomonic symptom review and physical examination, with a low threshold for imaging and biopsy, may detect precursor lesions and early-stage gastric-type adenocarcinoma, leading to better outcomes in this high-risk population.

PROSPERO registration number CRD42019118151

  • adenocarcinoma
  • uterine cervical neoplasms
  • pathology
  • surgical oncology

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Footnotes

  • KJP and JJM are joint senior authors.

  • Contributors SG: Conceptualization, methodology, formal analysis, Investigation, data curation, writing-original draft, writing-review and editing, visualization, project administration; final approval of version to be published; agrees to be accountable for all aspects of the work. RK: Conceptualization, methodology, investigation, data curation, writing-original draft, writing-review and editing; final approval of version to be published; agrees to be accountable for all aspects of the work. DS: Writing-original draft, writing-review and editing; data interpretation; final approval of version to be published; agrees to be accountable for all aspects of the work. EA: Conceptualization, writing-review and editing; data interpretation; final approval of version to be published; agrees to be accountable for all aspects of the work. BBN: Methodology, software, resources, data curation, writing-review and editing; final approval of version to be published; agrees to be accountable for all aspects of the work. AC: Resources, writing-review and editing; data interpretation; final approval of version to be published; agrees to be accountable for all aspects of the work. YLL: Writing-review and editing; data interpretation; final approval of version to be published; agrees to be accountable for all aspects of the work. YL: writing-review and editing; data interpretation; final approval of version to be published; agrees to be accountable for all aspects of the work. NRA-R: Writing-review and editing; data interpretation; final approval of version to be published; agrees to be accountable for all aspects of the work. KP: Conceptualization, methodology, data curation, writing-original draft, writing-review and editing, visualization, supervision, project administration; final approval of version to be published; agrees to be accountable for all aspects of the work. JM: Conceptualization, methodology, data curation, writing-original draft, writing-review and editing, visualization, supervision, project administration; final approval of version to be published; agrees to be accountable for all aspects of the work.

  • Funding This study was funded in part through the NIH/NCI Support Grant P30 CA008748.

  • Disclaimer NRA-R reports the following, outside the submitted work: grant from Stryker/Novadaq (paid to institution); grant from Olympus (paid to institution); grant from GRAIL (paid to institution). Memorial Sloan Kettering Cancer Center (MSK) has financial interests relative to GRAIL. As a result of these interests, MSK could ultimately potentially benefit financially from the outcomes of this research.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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