Objective: To explore the clinical characteristics, diagnostic approach, and multidisciplinary management strategies of cervical cancer complicated with Von Hippel-Lindau syndrome (VHL), and to enhance clinical awareness of rare and complex cases. Methods: A retrospective analysis was conducted on the clinical data of a patient with cervical cancer complicated by VHL syndrome. The patient’s medical history, family genetic background, laboratory tests, imaging findings, pathological results, diagnostic process, and multidisciplinary treatment were organized and analyzed in conjunction with relevant literature. Results: The patient was a 77-year-old female presenting with vaginal bleeding and lower abdominal distension. Cervical biopsy pathology indicated cervical squamous cell carcinoma, staged as IIIC1r. Further examination revealed abnormal changes in multiple systems, including the liver, pancreas, kidneys, and adrenal glands, along with a clear family genetic background of VHL. After comprehensive evaluation through imaging, pathology, and multidisciplinary consultation, the patient was ultimately diagnosed with cervical cancer complicated by VHL syndrome. This case was characterized by advanced age at onset, pronounced family clustering, multi-organ involvement, and complex diagnosis and treatment. Conclusion: Cervical cancer complicated by VHL syndrome is relatively rare in clinical practice. For patients with a special family history and multi-system involvement, it is essential to enhance awareness of hereditary tumor syndromes, strengthen multidisciplinary collaboration, and conduct individualized assessments to improve the diagnostic proficiency and clinical management capabilities for complex cases.
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