Abstract
BACKGROUND: Retroperitoneal high-grade serous carcinoma (HGSC) of unknown origin is a sporadic tumor that can originate from ovarian cancer. Herein, we report the case of a woman with retroperitoneal HGSC of unknown origin and describe how she was diagnosed and treated. CASE SUMMARY: A 71-year-old female presented with the tumor marker CA125 elevated to 1041.9 U/mL upon a regular health examination. Computed tomography revealed retroperitoneal lymph node enlargement. Subsequently, positron emission tomography scanning revealed lesions with increased F-18 fluorodeoxyglucose uptake at the nodes. As a result, she underwent laparoscopic lymph node resection, and pathology revealed metastatic adenocarcinoma with CK7(+), PAX8(+), WT1(+), PR(-), and p53 mutational loss of expression, indicating that the origin may be from the adnexa. The patient was admitted to our ward and underwent laparoscopic staging; however, the pathological results were negative. Under the suspicion of retroperitoneal HGSC of unknown origin, chemotherapy and targeted therapy were initiated. Tumor marker levels decreased after treatment. CONCLUSION: We present a case of HGSC of unknown origin managed using retroperitoneal lymphadenectomy, staging surgery, chemotherapy, and targeted therapy.