Multimodal therapy in the management of lacrimal gland adenoid cystic carcinoma

Abstract

Background: To evaluate the prognosis of Chinese patients with lacrimal gland adenoid cystic carcinoma treated with eye-sparing surgery and adjuvant multimodal therapy.

Methods: The study included 24 consecutive patients with lacrimal gland adenoid cystic carcinoma treated at the Ninth People's Hospital of Shanghai from May 2008 to September 2017. All patients underwent eye-sparing surgical tumor resection and 20 (83.3%) of the 24 patients in the cohort received postoperative RT. Eight (41.7%) patients in the cohort received chemotherapy. Each patient's medical records were reviewed.

Results: The study included 13 male and 11 female patients. The median follow-up time after surgery was 33.5 months. Fifteen (62.5%) patients experienced local recurrence. The 1-, 3-, and 5-year recurrence rates were 27.9, 60.0, and 80.0%, respectively. Eleven (45.8%) patients developed metastasis. The 1-, 3-, and 5-year metastasis rates were 8.7, 48.5, and 66.9%, respectively. Eight (33.3%) patients died of lacrimal gland adenoid cystic carcinoma, with a median survival duration of 34.0 months. The 1-, 3-, and 5-year tumor-related mortality was 4.5, 28.1, and 58.0%, respectively. More advanced T stage (≥ T3a) was a risk factor for local recurrence (hazard ratio [HR]: 5.374, P = 0.02), distant metastasis (HR: 8.585, P < 0.01), and tumor-related survival (HR: 9.654, P < 0.01).

Conclusions: Eye-sparing tumor resection protocol followed by adjuvant therapy seems to be associated with high rates of local recurrence, metastases and death. In addition, greater attention should be paid to patients with lacrimal gland adenoid cystic carcinoma with ≥ T3a tumors.

Keywords: Eye-sparing surgery; Lacrimal gland adenoid cystic carcinoma; Local recurrence; Metastasis; Tumor-related mortality, 8th American joint committee on Cancer.

Fig. 1

CT scan of Case 9, who had adenoid cystic carcinoma of the lacrimal gland, and axial computed tomographic scan after surgical resection (a, b). Photos and CT scan of Case 20, who had adenoid cystic carcinoma of the lacrimal gland, and axial computed tomographic scan after surgical resection (c, d, e, f). Intraoperative photograph for eye-sparing tumor excision (g). Excised tumor (h)

Fig. 2

Histopathologic features of adenoid cystic carcinoma of the lacrimal gland. (a, b). Histologic section of mixed pattern of adenoid cystic carcinoma of lacrimal gland (c). Perineural invasion (d)

Fig. 3

The presence of T3a stage and prognosis in Chinese patients with lacrimal gland adenoid cystic carcinoma. Significantly lower recurrence-free (a), metastasis-free (b) and tumor-related (c) survival rates were observed in patients with < T3a and ≥ T3a tumors according to the 8th AJCC classification (all P < 0.05)