Adenocarcinoma, Not Otherwise Specified, Arising in Accessory Lacrimal Gland: A Diagnostic Challenge

Abstract

Neoplasms arising in accessory lacrimal glands are rare. We describe a 33-year-old man with adenocarcinoma arising in the left lower eyelid accessory lacrimal gland. Microscopic evaluation demonstrated an infiltrative neoplasm composed of mildly to moderately pleomorphic cells with abundant eosinophilic cytoplasm and focal intracytoplasmic vacuoles, arranged predominantly in ductules. Foci of luminal and intracytoplasmic eosinophilic secretory material and occasional mucin were noted. An in situ component was identified in the gland of Wolfring. Though perineural invasion was present, high-grade nuclear features, brisk mitotic activity, and comedonecrosis were not identified. Immunohistochemical studies were notable for immunoreactivity of the tumor cells for CK7, carcinoembryonic antigen, BRST-2, androgen receptors, and HER2/neu (2+). The neoplastic cells were negative for CK20, estrogen and progesterone receptors, S-100, p63, calponin, thyroid transcription factor-1, and prostate-specific antigen. Fluorescence in situ hybridization studies for ETV6 and MAML2 rearrangements and for HER2/neu amplification were negative. Because of the absence of unifying morphologic, immunophenotypic, and molecular genetic findings, the diagnosis of adenocarcinoma, not otherwise specified, was rendered. The patient underwent comprehensive oncologic workup, which was negative for another primary tumor and metastases. He remains disease free with a follow-up of 4 years. This case illustrates the challenges encountered in applying salivary gland tumor classification to the accessory lacrimal gland neoplasm.

Adenocarcinoma NOS arising from accessory lacrimal glands: A case report and a review of the literature

“…The first and the only case of adenocarcinoma NOS arising from ALGs was described by Milman et al 7 However, the malignant tumour had a different clinical presentation, appearing as a firm, vascular and erythematous mass in the central lower eyelid, associated with distortion of the eyelid margin architecture and focal madarosis; histologically, it showed ductules-arranged pleomorphic cells with eosinophilic cytoplasm, focal intracytoplasmic vacuoles and foci of luminal eosinophilic secretory material. Differently from our case, a perineural invasion was found.…”

“…Primary epithelial tumours of the lacrimal gland are rare, but those arising in the accessory lacrimal glands (ALGs) are rarer still, with only few cases described in literature. 2–9…”

“…1 Primary epithelial tumours of the lacrimal gland are rare, but those arising in the accessory lacrimal glands (ALGs) are rarer still, with only few cases described in literature. [2][3][4][5][6][7][8][9] The ALGs of Krause and Wolfring derive from the palpebral lobe of the main gland and are more abundant in the upper palpebral conjunctiva than in the lower one.…”

Adenocarcinoma of Lacrimal Gland Non Otherwise Specified - A Case Report

“…Adenocarcinoma NOS is used to distinguish nonspecitic carcinomas, because of the undistinguished histomorphology features characterize for the other, previously mentioned, more specific carcinoma [9].According to recent data adenocarcinoma NOS of lacrimal gland is very rare, therefore we have limited clinical trials, treatments, follow ups and prognosis [12].Only two cases with this diagnosis are represented; adenocarcinoma of the lacrimal gland presenting an abduction deficit [12] and adenocarcinoma NOS of the lower eyelid accessory lacrimal gland [4], diagnosis that also questioned the accessory lacrimal gland neoplasm classification [4].Our patient underwent orbital exenterating under general anesthetize and was sent to oncology department for further treatment with radiotherapy and chemotherapy.…”

“…In the group of malignant tumors there is also included an unspecified group that numbers about 5 to 10 percent of very rare malignant tumors called adenocarcnimoa not otherwise specified (NOS) [3].In the absence of clear definition, special morphologic features, histopathological and imunohistochemical fenotipisation as in our paper not othervise specified adneocarinoam was determined. In this case we present a report and update on these different tumor types, withregard to their clinical presentation,histolopathology findings, immunohistochemical results and the best state treatment strategies for this rare tumor and more heterogeneous group of diseases [4]. past 5 months (Figure 1).…”