Gastric-type adenocarcinoma of the cervix in patients with Peutz-Jeghers syndrome: A systematic review of the literature with proposed screening guidelines Review
| Authors: | Gordhandas, S. B.; Kahn, R.; Sassine, D.; Aviki, E. M.; Baltich Nelson, B.; Catchings, A.; Liu, Y. L.; Lakhman, Y.; Abu-Rustum, N. R.; Park, K. J.; Mueller, J. J. |
| Review Title: | Gastric-type adenocarcinoma of the cervix in patients with Peutz-Jeghers syndrome: A systematic review of the literature with proposed screening guidelines |
| 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. © IGCS and ESGO 2022. No commercial re-use. See rights and permissions. Published by BMJ. |
| Keywords: | adenocarcinoma; pathology; uterine cervical neoplasms; surgical oncology |
| Journal Title: | International Journal of Gynecological Cancer |
| Volume: | 32 |
| Issue: | 1 |
| ISSN: | 1048-891X |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2022-01-01 |
| Start Page: | 79 |
| End Page: | 88 |
| Language: | English |
| DOI: | 10.1136/ijgc-2021-002997 |
| PUBMED: | 34903560 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Review -- Export Date: 1 February 2022 -- Source: Scopus |
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95Lakhman -
888Abu-Rustum -
305Park -
186Mueller -
81Aviki -
105Liu -
20Catchings -
47Gordhandas -
41Kahn
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