Table 1.
Literature Review of Lacrimal System Epithelial–Myoepithelial Carcinomas.
| Study | Age/Sex | Clinical Presentation | Imaging | Treatment | Gross | Histopathology | Immunohisto chemistry | Final Stage | Outcome | Comments |
|---|---|---|---|---|---|---|---|---|---|---|
| Ostrowski et al.11 | 63/M | 8 years of slowly progressive, painless proptosis followed by 2 months of rapid progression | CT: Left 2.5 × 1.5 cm oblong mass of LG with irregular borders and central focal calcification; no bony erosion | Left orbital exenteration. No RT | 3.2 × 2.8 × 1.9 cm firm, tan mass | Biphasic pattern. (+) Atypia. No LVI, PNI, or necrosis. Positive margins with capsular invasion | Myoepithelial:(+) S-100, alpha-actin Epithelial: (+) EMA, cytokeratin, AE1/AE3 |
∼T2N0M0 | No recurrence at 2.5 years | LG EMC in context of PA |
| Chan et al.9 | 80/M | 9 months of painless, subcutaneous mass in left upper outer eyelid | CT: Left 2.4 × 1.5 × 1.8 cm mildly enhancing mass of LG | Left translid lateral orbitotomy with gross total resection. RT advised but refused | 2.3 × 1.8 × 1.8 cm firm, well-circumscribed encapsulated tumor; no bony erosion | (+) Myoepithelial anaplasia. No LVI, PNI, or necrosis. Positive margins and capsular invasion | Myoepithelial: (+) S100, SMA Epithelial: (+) cytokeratin AE1/AE3, EMA |
∼T2N0M0 | No recurrence at 2 years | LG EMC |
| Wiwatwongwana et al.13 | 86/M | 6 months of painless diplopia | CT: Left 2.5 × 1.8 cm moderately homogenous, hyperattenuating mass with area of scalloping in the lacrimal fossa | Left lateral orbitotomy with gross total resection followed by RT due to positive margins (60 Gy) | Not reported. Mass adherent to periosteum with irregularity of adjacent bone | (+) Myoepithelial anaplasia and necrosis. No LVI or PNI. Positive margins, no bony invasion | Myoepithelial: (+) p63a | Reported as T2N0M0, however likely T4aN0M0 | Death at 15 months postoperatively with no clinical evidence of recurrent disease | LG EMC |
| Singh et al.7 | 62/F | 4 years of right orbital swelling with 6 months of rapid progression; painless; vision unaffected | CT: Right 2.7 × 2.4 × 2.0 cm heterogeneously enhancing lobulated LG mass with expansion and thinning of the overlying bone MRI: hypointense on T1 and heterogeneously hyperintense on T2 |
Right fronto-orbital craniotomy with gross total resection followed by RT due to positive margins (dose not reported) | 4 × 2 × 2 cm multinodular, circumscribed mass invading through periorbita without dural invasion | (+) Atypia, PNI. No necrosis or LVI. Positive margins. | Myoepithelial: (+) S100, BCL2, SMA, MIC3 Epithelial: (+) cytokeratin AE1/AE3, c-kit/CD117, MIC3 |
∼T4aN0M0 | No recurrence at 3 months | LG EMC |
| Venkatesulu et al.12 | 47/M | 3 months of progressive vision loss and painless swelling of right orbit. History of previously excised pleomorphic adenoma of the right lacrimal gland 2 years ago | CT: Right well-circumscribed heterogeneously enhancing mass with inferior displacement of globe, expansion of orbit with destruction and remodeling of the lateral wall of orbit involving all oblique as well as medial and superior rectus muscles | Right radical orbital exenteration with anterior skull base resection and temporalis muscle reconstruction followed by RT due to concern for possible recurrence (60 Gy) | 3 × 3 cm tumor grossly involving extraocular muscles | LVI, necrosis, PNI, atypia not reported. All margins negative | Myoepithelial: (+) SMA, and S-100 Epithelial: (+) EMA |
∼T4aN0M0 | No recurrence at 6 months | LG EMC in the setting of previously excised PA |
| Goncalves et al.10 | 61/F | 6 months of painless progressive proptosis in left orbit. History of previously excised PA of the left LG 14 years ago | CT: Left well-circumscribed, heterogeneously enhancing, and mostly hyperattenuating LG mass MRI: Hypointense on T1; heterogeneously isointense on T2 weighted and enhancement with gadolinium |
Left combined anterior and lateral orbitotomy with complete excision followed by RT (dose not reported) | Not reported | (+) PNI. LVI, necrosis, atypia not reported. All margins negative. | Myoepithelial: (+) p63, SMA Epithelial: (+) cytokeratin 7 |
Unknown staging. Low-grade behavior reported | No recurrence at 36 months | LG EMC in the setting of previously excised PA |
| Avdagic et al.8 | 53/F | 1 month of painless decreased vision, progressive left periorbital fullness | CT: Left 2.0 × 2.5 cm enhancing, circumscribed LG mass with optic nerve and globe compression | Left lateral orbitotomy with complete excision followed by RT (60 Gy) | 3.5 × 2.0 × 1.5 cm well-encapsulated tan mass | Biphasic pattern. (+) Necrosis, PNI, atypia. No LVI. Positive lateral margin. | Myoepithelial: (+) p63, calponin Epithelial: (+) Cam5.2, CEA-M |
∼T3N0M0, intermediate grade | No recurrence at 24 months | LG EMC in the setting of a carcinoma ex PA |
| Li et al.6 | 41/F | Right orbital mass excision 10 years prior, several days of right eye pain and blurred vision. Presented as a VLM with radiographic and histopathologic evidence of intralesional cysts. Initially treated with bleomycin with minimal resolution. | MRI: Right 3.6 × 2.1 × 3.5 cm extraconal, multilobulated lacrimal gland mass with cystic fluid-fluid levels | Right orbitotomy with complete excision. Radiation advised but not reported if completed. | 5.1 x 4.3 x 2.6 cm homogenous mass | Biphasic pattern. No necrosis, LVI, or anaplasia. PNI not reported. All margins negative. | Myoepithelial: (+) p63 Epithelial: (+) cytokeratin AE1/AE3 Both (+) Sox-10 |
∼T3N0M0, low-grade | No recurrence at 4 months | LG EMC in the setting of a VLM |
| Current Case, 2019 | 72/M | History of DCR 8 years ago. 4 years of worsening epiphora. Mass found incidentally on work-up of lung nodules | CT: Right 2.1 × 1.6 × 2.4 cm mass centered in the nasolacrimal sac | Right orbitotomy and dacryocystectomy; right medial maxillectomy, anterior ethmoidectomy, inferior turbinectomy, partial middle turbinectomy | 4.5 × 4.0 × 0.5 cm gray-tan, homogenous mass | Biphasic pattern. No necrosis, PNI, LVI, or anaplasia. All margins negative | Myoepithelial: (+) SMA, Calponin, p63, keratin 5/6; Epithelial: (+) Keratin 7 |
T3N0M0, low-grade | No recurrence at 20 months | Lacrimal Sac EMC |
Abbreviations: CEA-M, carcinoembryonic antigen–monoclonal; CT, computed tomography; EMA, epithelial membrane antigen; EMC, epithelial-myoepithelial carcinoma; DCR, dacryocystorhinostomy; IHC, immunohistochemistry; LG, lacrimal gland; LVI, lymphovascular invasion; MRI, magnetic resonance imaging; PA, pleomorphic adenoma; PNI, perineural invasion; RT, radiotherapy; SMA, smooth muscle actin.
aDoes not report epithelial markers.