Carcinoma Expleomorphic Adenoma of the Lacrimal Gland.

AIMS

To report the characteristics and long-term outcomes of patients presenting with carcinoma ex-pleomorphic adenoma (CXPA) or malignant mixed tumor of the lacrimal gland.

METHODS

A retrospective case-note review for patients with CXPA, seen at Moorfields Eye Hospital between 1985 and 2018, was performed for demographics, presentation, imaging, histopathology, management, and outcome.

RESULTS

Twenty-six patients (11 male; 42%) presented at a mean age of 46 years (median 46.4; range 24-81), with average symptom(s) duration of 67 months (median 24 months; range 6 weeks-53 years). The commonest symptoms were proptosis (13 patients; 50%), orbital ache or pain (11/26, 42%), having noted a lump in the upper eyelid (8/26, 31%), and diplopia (7/26, 27%). About 90% of patients had nonaxial globe displacement, over a half had a palpable mass and 3-quarters had reduced motility. Adenocarcinoma was the commonest malignant component of the tumor, occurring in 16 (62%) of the patients, followed by adenoid cystic carcinoma in 5 patients (19%); 10/26 (28%) tumors showed significant invasion on histological examination. A quarter of the patients had tumor-related death at an average of 2.8 years after diagnosis (median 2.5; range 1.5-4.4 years), and the overall probability of survival for the whole cohort was 63% at both 5 years and 10 years after treatment. The prognosis for CXPA was significantly worse with a history of prior incompletely excised pleomorphic adenoma (p < 0.001), a markedly invasive malignant component (p = 0.001), evidence of perineural invasion (p = 0.042), and an American Joint Committee on Cancer tumor size of T3 or greater (p = 0.024).

CONCLUSION

Intact excision of CXPAs that do not display histological evidence of extension beyond the tumor pseudocapsule have a good outcome, similar to that for intact excision of benign pleomorphic adenomas. In contrast, our patients with markedly invasive CXPA had a tendency to local recurrence or distant metastasis, and tumor-related death occurred in about two-thirds.