TABLE 1.
Patient No. | Age/Gender/Affected Side | ACC Subtype/Perineural/Bone Infiltration | IACC Cycles/Total Cycles | Prior Tumor Resection | Radiographic Tumor Response/Tissue Margins | Treatment | Time (mo) of and Status at Last Follow-Up | Time (mo) of Local Recurrence/Met | Remarks |
---|---|---|---|---|---|---|---|---|---|
Group 1 | |||||||||
1 | 29/M/OD | Basa/P/B | 3/4 | NO | Yes/− | Exent + RT | 288; Alive, no D | No | Intracranial extension |
3 | 73/F/OS | Basa/P | 2/6 | NO | Yes/− | Exent + RT | 193; Alive, no D | No | Kidney transplant |
4 | 58/M/OS | Crib/P | 2/6 | NO | Yes/− | Exent + RT + GK | 156; Alive, no D | No | Intracranial, ethmoid sinus ACC |
5 | 35/M/OS | Crib/P | 3/6 | NO | Yes/+ | Exent + RT | 149; Alive, no D | No | Non-healing apex; free flap |
7 | 30/M/OS | Basa/P | 2/4 | NO | Yes/− | Exent + Bone + RT | 116; Alive, no D | No | |
10 | 29/F/OD | Basa | 2/6 | NO | Yes/− | Exent + RT | 96; Alive, no D | No | |
17 | 53/F/OD | Basa | 2/6 | NO | Yes/− | Exent + RT | 29; Alive, no D | No | Neck dissection negative |
18 | 44/M/OS | Basa/Crib/P/B | 3/6 | NO | Yes/+ | Exent + RT | 20; Alive, no D | No | Lung Bx negative |
Group 2 | |||||||||
2 | 32/M/OD | Crib/P/B | 3/6 | YES | Yes/− | Exent + Bone + RT | 167; Died, no D | No | HIV. Died of oral SCC |
6 | 42/F/OD | Crib/P/B | 3/6 | YES | Yes/− | Exent + Bone + RT | 127; Alive, with D | 50; Lung | Bone necrosis, hearing deficit; 7th N palsy |
8 | 36/F/OS | Basa/P/B | 2/2 | NO | Yes/+ | Exent + RT | 28; Died of D | 18; LR/Liver | Delayed exent, refused final 4 cycles |
9 | 64/F/OD | Crib/B | 2/2 | YES | Yes/+ | Exent orbit/sinuses + Bone + RT | 112; Alive, no D | 13; LR/Sinus | Delayed exent, refused final 4 cycles, ACC in sinus mucosa resected |
11 | 54/M/OS | Crib/Basa | 2/6 | YES | Yes/+ | Exent + Bone + TM +RT | 87; Died of D | 20; LR/Lung/Brain | Extensive disease at presentation |
12 | 32/F/OD | Crib | 2/5 | YES | Yes/+ | Exent + Bone + RT + GK | 85; Alive, no D | No | |
13 | 34/M/OD | Crib | 2/6 | YES | Yes/− | Exent + Bone + RT | 89; Alive, no D | No | Ophthal artery infusion, vessel thrombosis |
14 | 49/M/OS | Crib/Basa | 2/5 | YES | Yes/− | Exent + Bone + RT | 82; Alive, no D | 60; Lung | NED after lung nodule resection |
15 | 20/M/OD | Crib/Basa/P/B | 3/5 | YES | Yes/− | Exent + Bone + RT | 73; Alive; no D | No | |
16 | 38/F/OS | Basa | 2/6 | YES | Yes/+ | Exent + RT | 52; Alive; no D | No | |
19 | 56M/OS | Basa/P | 5/5 | YES | Yes/+ | Exent orbit/sinuses + RT | 20; Alive; no D | No | Intracranial and ethmoid sinus ACC, free flap |
Alternate Histology | |||||||||
20 | 43/F/OS | Adenocar/P/B | 2/6 | YES | Yes/− | Exent + RT | 41; Died; no D | No | No ACC detected in exenteration specimen; AML 3 years post IACC; 11q23 translocation + margin at superior orbital fissure |
21 | 45/F/OD | Adenocar/P/B | 2/2 | NO | Yes/+ | Exent + RT | 30; Died of D | 26; Brain | |
Conventional Treatment Group | |||||||||
1 | 37/M/OS | Basa | N/A | N/A | Exent + RT | 22; Died of D | 3; LR/Sinus | ||
2 | 57/F/OD | Crib/P/B | N/A | N/A | Resect + RT | 94; Died of D | 36; LR/Lung | ||
3 | 48/M/OD | Basa | N/A | N/A | Resect + Exent | 157; Died of D | No | ||
4 | 42/F/OD | Basa | N/A | N/A | Resect | 18; Died of D | 7; Brain | ||
5 | 69/M/OS | Basa | N/A | N/A | Exent + RT | 198; Died of D | 84; Brain | ||
6 | 53/F/OS | Crib | N/A | N/A | Exent + RT | 8; Died of D | 5; Brain | ||
7 | 37/M/OD | Crib/P/B | N/A | N/A | Exent + Bone + RT | 44; Died of D | 16; Lung |
ACC = adenoid cystic carcinoma; Basa = basaloid subtype; Crib = cribriform subtype; B = bone infiltration; P = perineural infiltration; Radiographic Tumor Response/Tissue Margins = radiographic evidence of tumor shrinkage after IA chemotherapy cycles/presence of tumor cells at soft tissue surgical margins; No D = No ACC; LR = local recurrence; Met = distant metastases; D = disease (local or metastatic ACC); Exent + bone + RT = exenteration with removal of lateral orbital wall fragment plus postoperative radiation therapy; TM = temporalis muscle resection; Exent + RT = exenteration plus postoperative radiation therapy; GK = gamma knife therapy; NED = no evidence of disease; N/A = not applicable; Resect = primary resection of mass; SCC = squamous cell carcinoma; 7th N = facial nerve palsy; Adenocar = adenocarcinoma; AML = acute myelocytic leukemia.