Dramatic case of an acinic cell carcinoma

Case description A 29-year-old male presented with progressive left neck swelling since four weeks. Ultrasound of his neck revealed multiple pathological nodes bilaterally without demonstrable primary salivary gland lesion and otherwise normal ENT examination. Diagnostic lymph node excision (Level 1b) reported nodal and soft tissue metastasis from an acinic cell carcinoma. MRI neck, CT chest, PET-CT showed systemic cervical, thoracic and abdominal lymphadenopathy without primary tumour. Additional, malignant pleural effusion was confirmed by cytology. Two cycles of chemotherapy (Cetuximab, Cisplatin, Docetaxel) were given. Nevertheless, due to rapid disease progression the patient deceased two month after first presentation.

Discussion  Acinic cell carcinomas are rare tumours, which originate commonly in the parotid gland and more rarely in pancreas or lung. Mostly these are low-grade tumours with good prognosis. Distant metastases are reported in 0-13% of cases but soft tissue metastases are very rare in the literature. Clinical picture and histopathology suggested a high-grade carcinoma in this case. No primary was identified here and we assume the disease to be originating from the small salivary glands. As conventional surgical treatment was not feasible any more, chemotherapy was delivered. Unfortunately, there are no standard protocols for these tumours and the young patient died very soon.

Conclusion  Despite its rarity the case underscores the relevance of further studies about systemic therapies in salivary gland malignancies.

Publication History

Article published online:
12 May 2023