Epithelial lacrimal gland tumors: A comprehensive clinicopathologic review of 26 lesions with radiologic correlation

Abstract

Aim: To study the prevalence, clinicopathological and radiological correlations of epithelial lacrimal gland tumors and compare these with similar published literature. The study was also designed to look at the natural history of benign mixed tumors (BMT) in regard to recurrence and malignant degeneration.

Methods: THIS WAS A RETROSPECTIVE STUDY OF ALL SUSPECTED EPITHELIAL TUMORS OF THE LACRIMAL GLAND SURGICALLY EXCISED AT KING KHALED EYE SPECIALIST HOSPITAL (KKESH) FOR THE PERIOD: 1983-2008. Exclusion criteria included structural lesions (dacryops) and inflammatory lesions. We included 26 cases of epithelial lacrimal gland tumors (from 24 patients). The histopathologic slides and the radiologic findings were reviewed. The corresponding demographic and clinical data were obtained by chart review using a data sheet.

Results: BMT accounted for 12/26 of the lesions while malignant lesions including adenoid cystic carcinoma (ACC) were more common (14/26). The mean age was 44.27 years (range 12-75). Commonest clinical presentation was proptosis. Median duration of symptoms in the BMT cases was 30 months and 7 months in the ACC group. The 12 BMT cases were primary in 9 and recurrent in 3 patients. The 11 ACC cases showed mostly cribriform pattern and low histopathologic grade. We had 2 cases of malignant mixed tumor (MMT) one of which arising in a recurrent tumor. One case of primary mucoepidermoid carcinoma with histopathologic grade 2 was noted. Radiologically, a well-defined appearance with bone remodeling was observed in BMT in contrast to invasive appearance with destruction in malignant lesions.

Conclusion: Our series information indicated a different distribution of benign and malignant epithelial lesions with a slightly higher rate of malignancy. BMT was the commonest benign tumor where recurrence was a squeal of incomplete surgical excision. ACC was the commonest malignant tumor with shorter duration of symptoms and radiologic evidence of invasiveness that correlated with the histopathologic features.

Keywords: Adenocarcinoma; Adenoma; Carcinoma; Lacrimal gland; Tumor.

Graph 1

Frequency of 26 epithelial lacrimal gland tumors.

Graph 2

The presenting symptoms in each type of epithelial lacrimal gland tumors.

Figure 1

(a) Gross photo of a case of recurrent BMT with irregular outer surface, described as “with bosselations”; (b) The corresponding histopathologic appearance of the recurrent tumor with mixture of stromal and epithelial components. (Original magnification 100×. Hematoxylin and Eosin); (c) microscopic appearance of an intact capsule (red arrow) in a primary BMT. (Original magnification 100×. Hematoxylin and Eosin); (d) the epithelial component in another BMT forming several ducts (black arrows) within myxoid stroma. (Original magnification 100×. Hematoxylin and Eosin); (e) Microscopic appearance of the commonest classic myxoid stroma in BMT. (Original magnification 200×. Hematoxylin and Eosin); (f) chondroid-like stroma in a case of BMT. (Original magnification 200×. Hematoxylin and Eosin).

Figure 2

(a) A case of ACC with cribriform pattern. (Original magnification 100×. Hematoxylin and Eosin); (b) the same case with highlighted cribriform pattern stained with Alcian-Blue. (Original magnification 200×); (c) another ACC with solid pattern. (Original magnification 200×. Hematoxylin and Eosin); (d) a different ACC case of the solid type but showing evidence of palisading. (Original magnification 200×. Periodic acid Schiff).

Graph 3

Variable radiologic findings among benign versus malignant lesions.

Figure 3

(a and b) The preoperative clinical appearance of left eye proptosis in one of the cases in the benign group (BMT); (c and d) the postoperative clinical appearance of the same patient with completely resolved left eye proptosis.

Figure 4

(a) Histologic appearance of first recurrence of the BMT. (Original magnification 100×. Hematoxylin and Eosin); (b) the same BMT first recurrence showing focal atypia (Original magnification 400×. Hematoxylin and Eosin); (c) Ca ex PA malignant cystic areas of the second recurrence in the same patient. (Original magnification 40×. Hematoxylin and Eosin); (d) Ca ex PA showing capsular infiltration (Original magnification 100×. Hematoxylin and Eosin); (e) Ca ex PA showing bone invasion (Original magnification 100×. Hematoxylin and Eosin); (f) Ca x PA showing perivascular invasion (Original magnification 200×. Hematoxylin and Eosin).

Figure 5

(a) Initial CT scan of the patient at the time of his first recurrence in 1989 which was 9 years following primary BMT excision elsewhere showed homogeneously enhancing mass lesion involving the right lacrimal gland with molded appearance and rounded border; (b) Follow-up sequential contrast enhancing Axial CT scans with second recurrence in 2002; (c and d) follow up CT in 2003, 14 years post an incomplete resection showing irregular soft tissue mass with heterogeneous enhancement combined with aggressiveness of the recurrent, malignant mixed tumor (double head arrow).

Figure 6

(a and b) The clinical presentation in a case of primary pleomorphic adenocarcinoma with more profound left eye proptosis and globe displacement; (c) pleomorphic adenocarcinoma primary (microscopic appearance). (Original magnification 200×. Hematoxylin and Eosin); (d) the capsular infiltration in the same case (Original magnification 400×. Hematoxylin and Eosin).

Figure 7

(a) Mucoepidermoid carcinoma appearance with low grade histopathologic features. (Original magnification 200×. Hematoxylin and Eosin); (b) the same tumor appearance with Periodic acid Schiff stain. (Original magnification 100×); (c) Mucicarmine positive tumor areas (Original magnification 100×); (d) mucin producing cell in the tumor (Original magnification 400×. Alcian Blue); (e and f) the preoperative appearance of the patient with mucoepidermoid carcinoma (right lacrimal gland); (g and h) the postoperative appearance of the same patient with resolved proptosis after complete removal of the tumor.

Figure 8

CT scan of a case of BMT showing well-defined mass in the area of the lacrimal gland (blue curved lines).

Figure 9

MRI photos in the case with mucoepidermoid carcinoma: (A–C) coronal T1 WI, Axial T2 with fat suppression and post contrast axial T1 with fat suppression showed poorly enhancing bi-lobulated right lacrimal gland mass (black arrow) with hemorrhage (white arrow).