A Merkel Cell Carcinoma of the Lacrimal Gland

Abstract

Merkel cell carcinoma is a rare neuroendocrine carcinoma that most often arises in sun-exposed skin. Primary Merkel cell carcinoma of the lacrimal gland is exceedingly rare, with only one prior case reported in the literature. The authors present a 49-year-old woman with a 6-month history of painless swelling of the left upper eyelid. MRI showed a lacrimal gland mass. Surgical excision was performed, and histologic evaluation revealed a Merkel cell carcinoma. Virology testing was positive for Merkel cell polyomavirus. The patient was treated with local radiation following surgical excision. She has had no recurrence after 1 year of follow up.

18F-Fluorodeoxyglucose Positron Emission Tomography–Computed Tomography Imaging in the Management of Merkel Cell Carcinoma: A Single-Institution Retrospective Study

“…Twenty patients (9 male, 11 female; median age 60, range 34–80) with the diagnosis of MCC underwent a total of 39 PET/CT scans (Table ). Seventeen (85%) primary tumors occurred in sun‐exposed anatomic locations, one occurred on the lower back, one appeared as an unknown primary in a cervical lymph node, and one occurred within the lacrimal apparatus . All patients were Caucasian, with the exception of one who was Hispanic.…”

“…Seventeen (85%) primary tumors occurred in sun-exposed anatomic locations, one occurred on the lower back, one appeared as an unknown primary in a cervical lymph node, and one occurred within the lacrimal apparatus. 29 All patients were Caucasian, with the exception of one who was Hispanic. Three (15%) patients were chronically immunosuppressed: two renal transplant recipients and one patient with Crohn's disease taking azathioprine and prednisone.…”

Primary Neuroendocrine Carcinoma of Ocular Adnexa

“…Furthermore, the negative signals of immunostaining with TTF-1 suggest that it is unlikely to be a primary lung cell carcinoma. Two cases of primary Merkel cell carcinoma of the lacrimal gland have been reported [ 5 , 6 ]; however, there is no established classification of NECs of the lacrimal gland. Thus, the histopathological diagnosis in this case strictly differentiated it from a Merkel cell carcinoma.…”

“…On the other hand, Case 2 is a typical primary Merkel cell carcinoma of the upper eyelid but Merkel cell polyomavirus was negative in the tissue samples. Although Merkel cell carcinomas usually develop in elderly individuals or more frequently in immunocompromised patients, younger and healthy patients can also develop Merkel cell carcinoma in tissues that are Merkel cell polyomavirus positive [ 4 , 6 , 7 ]. Although Merkel cell polyomavirus was negative in the sample tissues of our case, we diagnosed the patient as having a typical Merkel cell carcinoma because of the elderly onset, the characteristic clinical features, and histopathological findings.…”

“…Merkel cell carcinomas are also very rare and the estimated incidence is about 1/500,000 for Caucasians [ 4 ]. Only two cases of primary Merkel cell carcinoma of the lacrimal gland have been reported [ 5 , 6 ].…”