Mucoepidermoid carcinoma of the lacrimal gland in a patient with the CRTC1-MAML2 fusion gene

Kensaku Makino, MD; Kei Nakajima, DDS; Satoshi Tsutsumi, MD; Akane Toriyama, MD; Senshu Nonaka, MD; Hidehiro Okura, MD; Kenichi Matsuzaka, DDS; Toshitaka Nagao, MD; Hiroshi Izumi, MD; Shigeki Tomita, MD; Hisato Ishii, MD

Radiology Case Reports (Dec 2021)

Mucoepidermoid carcinoma of the lacrimal gland in a patient with the CRTC1-MAML2 fusion gene

Kensaku Makino, MD,
Kei Nakajima, DDS,
Satoshi Tsutsumi, MD,
Akane Toriyama, MD,
Senshu Nonaka, MD,
Hidehiro Okura, MD,
Kenichi Matsuzaka, DDS,
Toshitaka Nagao, MD,
Hiroshi Izumi, MD,
Shigeki Tomita, MD,
Hisato Ishii, MD

Affiliations

Kensaku Makino, MD: Department of Neurological Surgery, Juntendo University Urayasu Hospital, Chiba, Japan
Kei Nakajima, DDS: Clinical Pathophysiology, Tokyo Dental College, Tokyo, Japan
Satoshi Tsutsumi, MD: Department of Neurological Surgery, Juntendo University Urayasu Hospital, Chiba, Japan; Corresponding author.
Akane Toriyama, MD: Department of Pathology, Juntendo University Urayasu Hospital, Chiba, Japan
Senshu Nonaka, MD: Department of Neurological Surgery, Juntendo University Urayasu Hospital, Chiba, Japan
Hidehiro Okura, MD: Department of Neurological Surgery, Juntendo University Urayasu Hospital, Chiba, Japan
Kenichi Matsuzaka, DDS: Clinical Pathophysiology, Tokyo Dental College, Tokyo, Japan
Toshitaka Nagao, MD: Department of Anatomic Pathology, Tokyo Medical University, Tokyo, Japan
Hiroshi Izumi, MD: Department of Pathology, Juntendo University Urayasu Hospital, Chiba, Japan
Shigeki Tomita, MD: Department of Pathology, Juntendo University Urayasu Hospital, Chiba, Japan
Hisato Ishii, MD: Department of Neurological Surgery, Juntendo University Urayasu Hospital, Chiba, Japan

Journal volume & issue: Vol. 16, no. 12
pp. 3643 – 3646

Abstract

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Mucoepidermoid carcinoma (MEC) of the lacrimal gland (LG) is a rare entity. A 47-year-old woman was aware of periorbital swelling for 3 months. At presentation, the patient showed periorbital swelling in the right eye. CT scan showed an isodense mass in the anterior superolateral part of the orbit. MRI delineated the mass as enhancing, extra-conal tumor appearing isointense on T1-weighted sequences, and to be of mixed intensity on T2-weighted sequences. The tumor was totally resected. Microscopically, the tumor tissue was comprised of squamous, epithelioid cells, and cells with plump and clear cytoplasm. Necrosis, neural invasion, or mitotic figures were not observed. Immunohistochemical examination revealed intense staining for cytokeratin 7. A subset of the cells was positively stained with periodic acid–Schiff and mucicarmine stains. Genetic analysis revealed the presence of the CRTC1-MAML2 fusion. The CRTC1-MAML2 fusion may be a useful indicator for the prognosis and planning of adjuvant therapy.

Published in Radiology Case Reports

ISSN: 1930-0433 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Medicine (General): Medical physics. Medical radiology. Nuclear medicine
Website: https://www.sciencedirect.com/journal/radiology-case-reports

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