A Rare Pediatric Case of Lacrimal Gland Pleomorphic Adenoma

Abstract

Summary: Pleomorphic adenoma of the lacrimal gland is a rare benign finding in adults, and extremely uncommon in children. These tumors often present with increased propotosis or hypoglobus, and they invariably require operative excision. While many of these lesions can be removed through a subcranial or transorbital exposure, larger and more posterior tumors occasionally necessitate an intracranial approach. This report describes a large lacrimal gland pleomorphic adenoma in a 16 year-old patient that required use of an expansive intracranial exposure to ensure complete excision including the capsule. We contrast this case and its management to other pediatric cases described in the literature.

Pleomorphic Adenoma of the Eyebrow: An Unexpected Finding and Review of Unusual Periorbital Soft Tissue Lesions

“…In this paper, we present the case of a 35-year-old male with an asymptomatic eyebrow soft tissue mass that was postoperatively identified as PA. Traditionally, most PAs have salivary gland origins, and only a few cases have identified PAs arising from the lacrimal or sweat glands 2,3,8 . In the present report, the eyebrow lesion was relatively distant from the lacrimal gland, and the specimen did not resemble lacrimal or sweat glands on histology.…”

“…Biopsy or fine needle aspiration play a limited role due to the potential for capsular disruption and seeding of tumor cells; few studies recommend incisional biopsy or FNA in cases suspicious for malignancy where imaging is not diagnostic (Supplementary Digital Content, Table 1, http://links.lww.com/ SCS/D520). 1,5,6,7,8 In the case of our patient, presurgical imaging and FNA or biopsy was not performed due to the low level of suspicion for malignancy.…”

“…Because PAs are true neoplasms of epithelial and myopeithelial tissue with stromal components, in rare instances they can undergo malignant transformation 2,3,4,8 as carcinoma ex-PA. Carcinoma ex-PA is an aggressive tumor with poor prognosis and a 5-year disease specific survival of less than 50%. Treatment involves surgical excision followed by radiotherapy and/or chemother- apy 10,11,12 .…”

“…In general, PAs of the lacrimal gland present as a slow growing, firm, non-tender mass in the lid margin, central lid, or brow like in the case of our patient. 1,5,6 They can occur in adults and, less commonly, in children, with a reported mean age of presentation of 46 years old for adults, and 11 years old for children 8,9 (Supplementary Digital Content, Table 1, http://links.lww. com/SCS/D520).…”

Pleomorphic Adenoma of Lacrymal Gland: Report an Unusual Case

“…Surgical approach chosen depends upon tumor location, size, as well as surgeon experience [1]. Long term follow up is highly recommended because of the high rate of recurrence, the possibility of malignant transformation, or in case of incomplete excision [9].…”