Contributor: Gordon K. Klintworth
Carcinoma of the lacrimal sac is rare and can occur as a primary malignant tumor as well as a secondary tumor arising in the adjacent eyelid, nose, paranasal sinuses, and orbit. Carcinoma of the lacrimal sac usually presents with epiphora, swelling, and inflammation due to obstructed outflow and pain. Primary carcinoma of the lacrimal lac is most frequently a transitional cell carcinoma [carcinoma - transitional cell] or squamous cell carcinoma [carcinoma - squamous cell]. The commonest malignant neoplasm of the lacrimal sac is squamous cell carcinoma. Some arise without a prior lesion; others follow a transitional cell [papilloma - transitional cell] or squamous cell papilloma [papilloma - squamous cell]. Lacrimal sac carcinoma is composed of either atypical transitional epithelial or squamous cells. Extremely rare carcinomas of the lacrimal sac are adenoid cystic carcinoma [carcinoma - adenoid cystic] and mucoepidermoid carcinoma [carcinoma - mucoepidermoid].
Transitional cell carcinoma is a carcinoma with recognizable stratified columnar epithelium. Mitotic figures are conspicuous, cellular and nuclear pleomorphism with nuclear irregularity, and ultimately, evidence of extension into the wall of the sac with penetration of the epithelial basement membrane. Local recurrence after surgery with spread into the orbit has complicated ~50% of reported cases and, exceptionally, widespread carcinomatosis has been recorded. Histological distinction between benign papilloma and a carcinoma can be very difficult and sometimes impossible, in which case the initial surgery should aim for complete excision. A state intermediate between benignity and unequivocal malignancy seems to exis and repeated recurrence correlates with increasing histological evidence of malignancy. The prognosis for carcinoma of the lacrimal sac is not encouraging. Human papillomavirus has been detected by PCR in many carcinomas of the lacrimal sac.