1.1. Rationale: Salivary gland cancer is a rare malignant tumor of the head and neck. We provide a rare case, a malignant and poorly differentiated adenocarcinoma with no clear primary lesion and multiple regional lymph node metastasis. In terms of treatment, chemotherapy, targeted therapy and immunotherapy were explored and positive effects were achieved.
Patient concerns:A 48-year-old female who has been present with upper abdominal pain for more than 2 years. Imaging studies revealed multiple regional lymph node enlargement. There were no abnormal masses or nodules in other organs.
Diagnoses:The patient was clinically diagnosed as an aggressive, poorly differentiated adenocarcinoma with multiple lymph node metastases. Histologically, the lymph node tissue has ductal and myoepithelial (strongly calponin-positive, focal smooth muscle actin-positive) components and a prominent biphasic proliferation of epithelial structure. The pathological diagnosis was epithelial-myoepithelial carcinoma. Whole exome sequencing was identified as FGFR amplification.
Interventions:The patient received cisplatin in combination with fluorouracil chemotherapy for 2 cycles, docetaxel in combination with amlotinib for 4 cycles and maintenance therapy with amlotinib for 10 months, finally received programmed cell death protein 1 (PD-1) for 3 cycles. 1.5. Outcomes: The patient was followed up 14 months after amlotinib therapy without disease progress. This patient got extensive progressive disease and death occurred 32 months after initial treatment.1.6. Lessons: Whole exome sequencing may provide an effective treatment for rare metastatic tumors with unknown primary lesion.