Gastric Adenocarcinoma of the Cervix with Peutz-Jeghers Syndrome: A Case Report and Literature ReviewXinmei Dang\*¹, Zhenjiang Wang¹, Zhaocheng Zhang¹, Lintao Bi¹¹Department of Gynecology, Gansu Provincial Maternity and Child-care Hospital, Lanzhou, ChinaEmail: dangxinmei@hospital.cnAbstractBackground:Gastric adenocarcinoma of the cervix (G-EAC), a rare HPV-independent malignancy, is associated with diagnostic challenges and poor prognosis. Its co-occurrence with Peutz-Jeghers syndrome (PJS), a genetic disorder predisposing to multiple tumors, is scarcely documented.Case Presentation: A 57-year-old woman with PJS presented with irregular vaginal discharge. Imaging revealed a 6.6 cm cervical mass, and histopathology confirmed gastric-type adenocarcinoma (MUC6+/CK7+/p16−). Staged as FIGO IIA2, she received neoadjuvant chemotherapy (paclitaxel/cisplatin) with minimal response, followed by radical hysterectomy and adjuvant chemoradiation. Despite initial remission, disease progression led to death 26 months post-diagnosis.Conclusion:This case underscores the aggressive behavior of G-EAC in PJS patients, emphasizing the importance of genetic testing, multidisciplinary management, and exploration of targeted therapies. Regular surveillance for PJS-associated malignancies is crucial.Keywords: Gastric-type cervical adenocarcinoma; Peutz-Jeghers syndrome; STK11 mutation; Chemoresistance; Genetic counselingIntroductionGastric adenocarcinoma of the cervix (G-EAC), defined by gastric differentiation and HPV-independence, accounts for 0.15%-0.45% of cervical cancers. Its poor prognosis stems from late diagnosis and resistance to conventional therapies. Peutz-Jeghers syndrome (PJS), caused by germline STK11 mutations, confers an 18-fold increased cancer risk. Few reports describe G-EAC in PJS patients, highlighting the need for clinical vigilance.Case PresentationClinical HistoryA 57-year-old woman (gravida 3, para 1) with PJS presented with 2 months of irregular vaginal discharge. She exhibited perioral and mucosal hyperpigmentation since age 12. Her son carried a pathogenic STK11 mutation.Diagnostic EvaluationImaging: Pelvic MRI demonstrated a 6.6 cm cervical mass invading the vaginal fornix (Figure 1).Histopathology: Cervical biopsy confirmed gastric-type adenocarcinoma (MUC6+/CK7+/p16−; Figure 2).Staging: FIGO IIA2 (tumor size 5×4×2.5 cm; lymphovascular space invasion present).Therapeutic Intervention1. Neoadjuvant Chemotherapy: Two cycles of paclitaxel (300 mg/m²) + cisplatin (50 mg/m²) yielded 9% tumor regression.2.Surgery:Radical hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy (4/10 nodes positive; upstaged to IIIC1p).3. Adjuvant Therapy: Pelvic radiotherapy (50.4 Gy/28 fractions) with concurrent cisplatin.Clinical CoursePost-treatment surveillance revealed elevated CA-199 (1,200 U/mL) and lower limb edema secondary to vascular thrombosis. The patient died 26 months post-diagnosis without radiographic evidence of recurrence.DiscussionKey Findings1.Genetic Association:PJS-related STK11 mutations drive tumorigenesis via disrupted cell cycle regulation.2. Therapeutic Challenges:G-EAC exhibits chemoradiation resistance; HER2/PD-L1-targeted therapies may improve outcomes.3. Diagnostic Pitfalls:Negative HPV testing and nonspecific symptoms delay diagnosis. MRI and immunohistochemistry (MUC6/HIK1083) are critical tools.Clinical Implications- Proactive screening (annual pelvic exams, CA-199 monitoring) for PJS patients starting at age 18.- Genetic counseling and STK11 testing for at-risk families.ConclusionG-EAC in PJS demands a high index of suspicion. 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