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CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES SECTION EDITOR: W. RICHARD GREEN, MD ing the supraorbital notch revealed High-resolution MRI scans of the Lymphoepitheliomalike lymphoepithelioma with perineural orbits were obtained with conven- Carcinoma of the Orbit invasion. tional pulse sequences. They re- The ophthalmologic exam- vealed a multilocular cystlike mass in Lymphoepitheliomalike carcinoma ination results revealed a best- the medial aspect of the right orbit (LELC) of the skin is an uncommon corrected visual acuity of 20/70 OD (Figure 1). Other imaging features cutaneous malignancy with the po- and 20/15 OS; the visual acuity had included fluid-fluid layers within the tential for distant metastasis.1 We de- been stable in the right eye since a lesion and peripheral enhancement. scribe a patient with LELC of the mid scleral buckle procedure for retinal A computed tomography scan of the forehead and an asymptomatic or- detachment was performed approxi- head and neck area that was ob- bital mass, which when biopsied mately 18 years prior to this presen- tained 6 weeks prior to the MRI scan proved to be a lymphoepithelioma- tation. The external examination re- did not show an orbital mass. like carcinoma (LELC). vealed quiet globes; the Hertel An orbital biopsy of the mass exophthalmometry measurement was performed through a modified Report of a Case. A 45-year-old man was 19 mm in each eye. Results of Lynch incision (superonasal orbi- was referred to the Ophthalmology the extraocular motility examina- totomy).2 The cystic mass was iden- Clinic at the University of Texas M. tion were normal. The pupils were tified in the superonasal orbit, ap- D. Anderson Cancer Center, Hous- equal, round, and reactive to light proximately 25 mm posterior to the ton, for the evaluation of an asymp- with no afferent pupillary defect. The anterior lacrimal crest. The mass ex- tomatic right-sided orbital mass that confrontation visual fields were nor- tended posteriorly toward the supe- had been identified on recent mag- mal in the left eye, but superior and rior orbital fissure, and there was no netic resonance imaging (MRI) scans. temporal field defects were found in clearly defined capsule. Partial exci- The patient denied any orbital symp- the right eye; it was not clear whether sion of the mass was performed, and toms or signs except for numbness the field deficit was new or due to the frozen-section diagnosis was con- and paresthesia in the area of the the previous retinal detachment in sistent with poorly differentiated right-sided supraorbital notch, which the right eye. The slitlamp exami- LELC. The patient underwent con- had been present for at least 2 years. nation and applanation tonometry current chemoradiotherapy, consist- He had a history of a skin nodule on measurements were normal in both ing of 2 cycles of cisplatin followed his mid forehead, of which a biopsy eyes. A dilated fundus examination by 3 cycles of cisplatin and 5-fluoro- specimen had been taken at another revealed a 0.4 cup-disc ratio in the uracil; external beam radiotherapy institution and diagnosed as LELC of right eye and a 0.2 cup-disc ratio in was administered in 33 fractions, at the skin. Subsequently, 2 wide-local the left eye. There was evidence of a total dose of 6600 rad (66 Gy). excisions with positive margins were a scleral buckle band with an at- The material from the fore- performed on the nodule, and bi- tached retina in the right eye; there head lesion that was provided by opsy specimens of the skin overly- were no choroidal striae. another institution consisted of A B C Figure 1. A, An axial T1-weighted magnetic resonance imaging (MRI) scan, tailored to the orbits, reveals a multilobular, slightly hyperintense lesion in the upper medial aspect of the right orbit (arrows). B, An axial T2-weighted MRI scan demonstrates a cystlike appearance, with a fluid-fluid layer (arrows) best seen in the anterior component of the tumor. C, An axial postcontrast fat-suppressed T1-weighted image shows peripheral enhancement and a lack of central enhancement. (REPRINTED) ARCH OPHTHALMOL / VOL 119, AUG 2001 WWW.ARCHOPHTHALMOL.COM 1206 ©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 A B C Figure 2. A, Deep nodular infiltrate (hematoxylin-eosin, original magnification 34). B, Island of atypical cells (pleomorphic nuclei with prominent nucleoli) surrounded by small lymphocytes (original magnification 340). C, Immunohistochemical study performed in the orbital lesion, which shows positive expression of cytokeratin (anti-cytokeratin stains: AE1/AE3, CAM 5.2, Zym 5.2, MNF 116; amino-ethylcarbazole, original magnification 340). a skin ellipse containing a dense, Swanson et al11 in 1988, at least 21 tures of the orbital mass were sug- nodular infiltrate of the deep der- cases have been described. The pa- gestive of a benign cystic lesion. Al- mis (Figure 2A). Islands of large tient described here is slightly though there were minimal to no cytologically malignant cells with younger than most previously re- orbital signs or visual symptoms of polygonal, pleomorphic nuclei and ported patients (older than 50 years) a mass in this patient, and the ra- prominent nucleoli were identified with LELC of the skin. diologic features suggested a be- (Figure 2B). These islands were sur- Lymphoepitheliomalike carci- nign process, the orbital biopsy was rounded by a very dense lympho- noma of the skin is microscopically undertaken to rule out metastasis. cytic infiltrate. distinct from other primary cutane- Performing the orbital biopsy was a The surgical specimen from the ous neoplasms. Although poorly crucial step, and it changed the stag- orbital biopsy performed at M. D. differentiated squamous cell carci- ing and treatment of this disease. Anderson Cancer Center consisted of noma could conceivably be associ- This case underscores the im- multiple fragments of pink, firm tis- ated with a prominent lymphoid portance of a thorough systemic sue with a combined measurement of infiltration, the latter is usually con- workup for patients with LELC of the 130.630.4 cm. The specimen was centrated in the superficial dermis skin. The orbital mass was identi- routinely processed, and additional and has connections with the epi- fied during an MRI scan of the head slides were analyzed by a standard dermis. Furthermore, squamous cell and neck, which was obtained to rule immunohistochemical method. His- carcinoma is usually associated with out involvement of the paranasal si- tologically, there was an infiltrate very at least focal evidence of cytoplas- nuses or the oropharynx. In gen- similar to that seen in the outside ma- mic keratinization. eral, because of the close histologi- terial. An immunohistochemical Cases of LELC of the skin asso- cal similarity to nasopharyngeal study showed expression of cyto- ciated with a metastatic or satellite le- lymphoepithelioma, patients with keratin (Figure 2C), which con- sion in the orbit are extremely rare. suspected LELC of the skin should firmed the epithelial nature of the ma- The only mention in the literature is have a thorough otolaryngological ex- lignant cells. This constellation of in a study of 1422 orbital tumors ana- amination, including indirect laryn- histologic features is indicative of lyzed at the eye pathology labora- goscopy, to rule out metastasis to the LELC. Standard in situ hybridiza- tory at the Shanghai Medical Univer- skin. The differential diagnosis also tion studies failed to reveal evidence sity, Shanghai, China.12 The authors includes Merkel cell tumor; lym- of infection by Epstein-Barr virus. list an LELC of the skin metastatic to phoma; pseudolymphoma; and meta- the orbit in one of the 1422 cases re- static lymphoepithelioma from the Comment. Lymphoepitheliomas are viewed in their article, but no fur- salivary gland, thymus, cervix, lung, malignant tumors of epithelial ori- ther description of the histologic find- vulva, stomach, or tonsil. These may gin with various amounts of reac- ings or the clinical findings is be excluded by history, physical ex- tive lymphocytic infiltrate. Al- provided. amination findings, and the results of though initially described in the In the case described here, the histological studies, including im- nasopharynx (World Health Orga- orbital mass was an incidental find- munohistochemical analysis. Lym- nization type 3 nasopharyngeal car- ing on an MRI scan after the diag- phoma and pseudolymphoma can be cinoma),3 these tumors have been nosis of LELC of the forehead skin excluded because of the expression identified in various locations with perineural invasion. It is likely of lymphoid markers and the lack of throughout the body.4-8 that the orbital mass represents a cytokeratin expression. Merkel cell Lymphoepitheliomalike carci- metastatic lesion secondary to the carcinoma is characterized by malig- noma of the skin is a rare cutane- forehead lesion, though the possi- nant epithelial cells with a lesser de- ous malignancy that is probably of bility of a direct extension into the gree of pleomorphism than is seen in adnexal origin.9,10 It is usually a flesh- orbit or a primary orbital tumor with LELC of the skin; also, the nuclei in colored or red, firm nodule or plaque subsequent cutaneous metastasis Merkel cell carcinoma characteristi- that appears most often on the face. cannot be completely ruled out. It cally have finely dispersed (“salt-and- Since the original description by is interesting that the radiologic fea- pepper”) chromatin. (REPRINTED) ARCH OPHTHALMOL / VOL 119, AUG 2001 WWW.ARCHOPHTHALMOL.COM 1207 ©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 We recommend an imaging 1. Dozier SE, Jones TR, Nelson-Adesokan P, Hruza magnetic resonance (MR) imaging GJ. Dermatol Surg. 1995;21:690-694. study of the head and neck in all pa- 2.

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