Metastatic adeoid cystic carcinoma of the salivary gland

Background: Adeoid cystic carcinomas of the salivary gland account for approximately 10% of parotid gland tumors. Well-differentiated tumors are characterized by a slow growth rate, a low recurrence rate after complete surgical excision and rare metastatic potential. High-grade tumors are more aggressive, local reccurence rate after surgery approaches 60% and 30% develop distant metastases. We are presenting small group - 6 cases with longer survival after targeted, biological therapy Methods: 6 patients ( 4 males and 2 female) with median age: 50 years. Multiple lung and liver dissemination with all of them. Histology: adenoid cystic carcinoma, KRAS wt, ckit wt, BRAF wt with all. In one women PDGFR mutation on exon 10. No comorbidities, all tumors was clinically symptomatic. Treatment: Combination Cisplatin 50mg/m2 day 1 +Fluorouracil 100mg/m2 48 h continual and docetaxel 60mg/m2 day 1 was used on the first line , after progression in the second line patient was treated with Mitoxantron monotherapy . Third line we indicated imatinib mesylate to patients ckit wt and sunitinib undervent female patient with PDGFR mutation. Results: Therapy using docetaxel and platinum and fluorouracil was effective ( PR 4 patient , median TTP 10month ) The treatment with Mitoxantron was effective :partial regression 5 patients TTP 13 months we have documentated. The effect of the treatment with Imatinib mesylate: stabilization of the dissease 4 patient without mutation TTP longer than 5 months in all cases. Female patient with mutation was treated 8 months only,Sunitinib was ineffective.Patients PDGFR wt are alive more than 2 years with metastatic dissease Conclusions: In our group was PDGFR mutation negative prognostic factor for treatment effect and progression.Patients with ckit wt and PDGFR wt have documentated significantly better prognosis and chemotherapy response.