Proptosis Revealing a Rare Lacrimal Gland Tumor: A Case of Chondroid Syringoma in a 35-year-old Patient

Lacrimal gland chondroid syringoma is a very rare tumor with classic clinico-radiological symptoms that should be familiar to clinicians for appropriate patients’ management as the tumor has potential for recurrence and malignant transformation. We report herein a case of chondroid syringoma in a 35-year-old patient presenting with progressive painless proptosis for 2 years. He underwent complete surgical removal of the tumor, with subsequent clinical improvement of his symptoms.

Invasive Carcinoma Ex-Pleomorphic Adenoma of the Lacrimal Gland with a Cystadenocarcinoma Component: A Case Report and Review of the Literature

Lacrimal gland neoplasms comprise up to 18% of all orbital masses clinically and histologically. Much of our current core knowledge regarding lacrimal gland tumors stems from prior study of their more common counterparts, the salivary glands. The prognosis for each lacrimal gland tumor is contingent upon proper clinical evaluation and ultimately the histopathologic diagnosis. We describe a case of an invasive carcinoma ex-pleomorphic adenoma (Ca-ex-PA) with a cystadenocarcinoma component arising from the lacrimal gland in the absence of any previously diagnosed pleomorphic adenoma (benign mixed tumor) or prior incisional surgery. This case illustrates the importance of the histopathologic assessment including immunohistochemistry and genetic testing to narrow a differential diagnosis and potentially aid or guide therapy in the future. Our finding suggests that carcinoma of the lacrimal gland may be derived from previously undiagnosed and perhaps even subclinical pleomorphic adenoma.

Loss of BRCA1 Spontaneously Induces the Tumorigenesis in Lacrimal Gland

Environmental and genetic factors exert important influences on lifespan and neoplastic transformation. We have previously shown that spontaneous tumors form frequently in mice homozygous for a full-length Brca1 deletion. In general, mutations of BRCA1 are closely associated with induction of breast and ovarian cancers but are also known to contribute to the incidence of other cancers at a low frequency. Female Brca1-mutant mice (Brca1co/coMMTV-cre) were generated by crossing Brca1 conditional knockout mice and MMTV-cre mice, and the occurrence of lacrimal gland abnormalities and tumors was followed until mice reached 18 months of age. Lacrimal gland tumors, which occur at a very low frequency in the human population (1 per 1,000,000 per year), were detected in 7 cases of Brca1co/coMMTV-cre mice (2.75%) older than 9 months of age. None of seven mice exhibited any abnormality in the mammary gland including neoplasia, suggesting lacrimal gland tumor is spontaneously and independently formed. These tumors, which were detected in seven mutant mice that displayed exophthalmoses, were malignant, originated from epithelial cells, and were identified as acinic cell carcinoma by pathological analysis. Further analysis revealed that tumorigenesis was accompanied by the accumulation of cyclin D1 and decreased expression of the cellular oncogenes, c-Myc, c-Jun, and c-Raf. Tumors also exhibited rearrangement of cytoskeletal proteins, including β-catenin, keratin 5, and vimentin, depending on tumor progression. These results suggest that BRCA1 is involved in genetic stability of the lacrimal gland, providing new insight into genomic instability in organism maintenance and tumorigenesis of the lacrimal gland.

POTENTIAL OF COMPUTED TOMOGRAPHY IN THE DIAGNOSTICS OF THE LACRIMAL GLAND EPITHELIAL TUMORS

Objective. Discussion of the X-ray data in patients with epithelial tumors of the lacrimal gland.Material and methods.We examined CT scans of 20 patients with lacrimal gland epithelial tumors (20 orbits). Pleomorphic adenoma was diagnosed in 9 patients, pleomorphic adenocarcinoma was diagnosed in 11 patients. The computed tomogtaphy was performed according to the standard method with obtaining axial and frontal slices. The slicethickness was 1.0 mm, sliceinterval – 1.0 mm.Results. The article presents data on the structure of lacrimal gland epithelial tumors, considering their volume, dimension and density. Based on the investigation results, the criteria for the differential diagnosis between pleomorphic adenoma and adenocarcinoma have been established.Conclusion.Computed tomography is an essential diagnostic technique for a suspected lacrimal gland tumor. Pleomorphic adenocarcinoma is characterized by a greater density and more heterogeneous structure compared to pleomorphic adenoma. Nearly 3/4 of patients with pleomorphic adenocarcinoma present changes in their orbital walls. The pleomorphic adenoma can’t be ruled out in patients with the thinning of the orbital wall.