Patients’ demographics and baseline characteristics
| ACC (n=46) | Non-ACC (n=52) | |
| Sex | ||
| M (%) | 26 (56.5%) | 29 (55.8%) |
| F (%) | 20 (43.5%) | 23 (44.2%) |
| Median age (range) | 59 (36–80) | 63 (29–81) |
| ECOG | ||
| 0 | 23 (50.0%) | 19 (36.5%) |
| 1 | 23 (50.0%) | 32 (61.5%) |
| 2 | 0 (0.0%) | 1 (1.9%) |
| Classification at initial diagnosis; n (%) | ||
| T | ||
| 0 | 1 (2.2) | 1 (1.9) |
| 1 | 4 (8.7) | 6 (11.5) |
| 2 | 11 (23.9) | 7 (13.5) |
| 3 | 10 (21.7) | 12 (23.1) |
| 4 | 9 (19.6) | 12 (23.1) |
| X | 11 (23.9) | 14 (26.9) |
| N | ||
| 0 | 24 (52.2) | 15 (28.8) |
| 1 | 2 (4.3) | 9 (17.3) |
| 2 | 7 (15.2) | 14 (26.9) |
| 3 | 1 (2.2) | 0 (0) |
| X | 12 (26.1) | 14 (26.9) |
| M | ||
| 0 | 33 (71.7) | 43 (82.7) |
| 1 | 13 (28.3) | 9 (17.3) |
| Primary site of cancer | ||
| Major glands; n (%) | 32 (69.6) | 44 (84.6) |
| Parotid | 12 (26.1) | 34 (65.4) |
| Sublingual* | 3 (6.5) | 2 (3.8) |
| Submandibular* | 18 (39.1) | 8 (15.4) |
| Minor glands; n (%) | 14 (30.4) | 8 (15.4) |
| Base of tongue† | 2 (4.3) | 3 (5.8) |
| Floor of mouth‡ | 2 (4.3) | 0 (0) |
| Lacrimal gland | 1 (2.2) | 0 (0) |
| Larynx | 2 (4.3) | 1 (1.9) |
| Paranasal sinuses‡ | 6 (13.0) | 1 (1.9) |
| Peritonsillar area† | 0 (0) | 1 (1.9) |
| Posterior pharyngeal walls | 0 (0) | 0 (0) |
| Retromolar trigone | 0 (0) | 0 (0) |
| Soft palate | 2 (4.3) | 3 (5.8) |
| Metastatic disease at inclusion | ||
| Yes§ | 42 (91.3%) | 49 (94.2%) |
| No | 4 (8.7%) | 3 (5.8%) |
| Locoregional relapse at inclusion | ||
| Yes | 11 (23.9%) | 16 (30.8%) |
| No | 35 (76.1%) | 36 (69.2%) |
| Prior treatments | 45 (97.8%) | 51 (98.1%) |
| Surgery | 39 (84.8%) | 47 (90.4%) |
| Radiotherapy | 42 (91.3%) | 47 (90.4%) |
| Prior chemotherapy | 21 (45.7%) | 34 (65.4%) |
| 1 line | 13 | 12 |
| 2 lines | 2 | 9 |
| >2 lines | 6 | 13 |
| Histology for non-ACC (as per local review¶) | ||
| Mucoepidermoid carcinoma | 6 (11.5%) | |
| Adenocarcinoma, NOS** | 28 (53.8%) | |
| Salivary duct carcinoma | 2 (3.8%) | |
| Other | 16 (30.8%) | |
| Acinic cell carcinoma | 3 | |
| Carcinoma ex-pleomorphic adenoma | 1 | |
| Epidermoid carcinoma | 3 | |
| Hyalinising clear cell carcinoma | 2 | |
| Myoepithelial carcinoma | 4 | |
| Myoepithelioma†† | 1 | |
| Oncocytic carcinoma | 1 | |
| Undifferentiated carcinoma | 1 | |
*One patient in the ACC cohort had tumour on submandibular and on sublingual.
†One patient in the non-ACC cohort had tumour on base of tongue and on peritonsillar area.
‡One patient in the ACC cohort had tumour on floor of mouth and on paranasal sinuses.
§Metastatic sites at inclusion are described in online supplemental table 1.
¶Local reviews were realised by REFCOR (Réseau d'Expertise Français des Cancers ORL Rares) members.
**Not otherwise specified.
††This patient was first diagnosed with myoepithelioma of the left vocal cord then developed lung metastasis and had locoregional recurrence with the involvement of lymph node.
ACC, adenoid cystic carcinoma; NOS, not otherwise specified.