Composite cervical adenocarcinoma composed of adenoma malignum and gastric type adenocarcinoma (dedifferentiated adenoma malignum) in a patient with Peutz Jeghers syndrome

ObjectivesTo 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.MethodsA 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.ResultsA 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.ConclusionsGastric-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 numberCRD42019118151

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

10.21203/rs.3.rs-150876/v2 ◽

2021 ◽

Author(s):

Tong Tong ◽

Qiong Fan ◽

Shu Shi ◽

Yuhong Li ◽

Yudong Wang

Keyword(s):

Human Papilloma Virus ◽

Mucinous Adenocarcinoma ◽

Clinical Stage ◽

Young Patients ◽

Hpv Infection ◽

Sexual Life ◽

Autosomal Dominant Disorder ◽

Papilloma Virus ◽

Gastric Type ◽

Peutz Jeghers Syndrome

Abstract Background: Peutz-Jeghers syndrome(PJS) is a very rare autosomal dominant disorder with predisposition to multiple neoplasms. Gastric-type adenocarcinoma (GAS) is a less common carcinoma of the cervix than squamous cell carcinoma, which is more aggressive and has lower 5 year survival rate than usual type endocervical adenocarcinoma (ECA), and unrelated to human papilloma virus(HPV) infection as well. We present a 32 year-old patient with Peutz-Jeghers syndrome who was found to have gastric-type adenocarcinoma of the cervix.Case presentation: A 32-year-old woman without sexual life ever who was diagnosed Peutz-Jeghers syndrome when she was two years old presented with watery discharge for more than 6 months. A tumor around 6cm was found on the cervix and she was diagnosed gastric-type mucinous adenocarcinoma of the cervix clinical stage IB3. She was treated with artery intervention chemotherapy for one course followed by radical surgery and then systematic chemotherapy.Conclusions: The case suggests more thorough cancer screening for patients with PJS as the disorder is rare and has high risk of malignancies. Young patients with Peutz-Jeghers syndrome, including those without sexual life, who have watery discharge or bleeding should be screened for cervical carcinoma even if cytologic results or human papilloma virus (HPV) is negative.

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