Orbital Metastatic Carcinoma

Rebecca T Leibowitz, MD - Case Coordinator
John Walter Millstine, MD - Radiology Discussion
Kimberley M Springer, MD - Pathology Discussion
Russell A Blinder, MD - Attending Radiologist
Steven E Seltzer, MD - Attending Radiologist

December 8, 2003

Presentation

Imaging Findings

MR images through the orbits show a relatively low T2 signal mass arising from the medial aspect of the left orbit. Post-contrast, the lesion demonstrates heterogeneous enhancement. The lesion is extraconal and there may be a portion of the lesion extending into a left anterior ethmoid air cell. The eyeball is deviated laterally by the mass. The underlying bone is hard to assess.

Differential Diagnosis

Diagnosis

Discussion

The specimen is an orbital resection. The conjuctiva looks normal. On cross-section, the white-yellow tumor is visible in the inferior aspect of the orbit. Low-power microscopy of the eye (band at the top, left) and conjunctiva (rippled area on the right) demonstrates a blue, infiltrative process (lower right corner and bottom of the slide). At higher power, this process is incredibly pleomorphic and hyperchromatic. Some of the cells are multinucleated. At higher power still, it is clear that the tumor does involve the conjunctiva. In fact, the tumor involves subcutaneous tissue, skeletal muscle, and dermis and extends into and through lateral nasal bone into sinonasal mucosa; the tumor does not invade the globe. There is extensive lymphovascular invasion (3 of 10 Level 1 lymph nodes were positive for metastatic carcinoma), but it is not possible to positively identify the primary from this specimen. Adenocarcinoma of the lacrimal sac is suspected.

Radiology Discussion

Although the diagnosis in this case is uncertain, the lesion did ultimately involve the lower-medial aspect of the eye and it is not unreasonable to speculate that it arose in the lacrimal sac. The most common lesion of the lacrimal sac is nongranulomatous inflammation (85.1%), followed by granulomatous inflammation (sarcoid; 2.1%), lymphoma (1.9%), papilloma (1.1%), lymphoplasmacytic infiltrate (1.1%), and transitional cell carcinoma (0.5%). Adenocarcinoma, undifferentiated carcinoma, granular cell tumor, plasmacytoma, and leukemia can also arise in this location but are quite rare. In fact, tumors arising from the lacrimal sac are very rare. Because they are often misdiagnosed for some time as chronic dacryocystitis, and there is a 55% rate of malignancy, there is typically a poor prognosis for these patients.

The typical presentation of a lacrimal gland tumor includes medial canthal swelling, nasolacrimal duct obstruction and mucocele. The differential diagnosis includes diseases originating in the sinuses (e.g., squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, lymphoma, infection), skin cancer, extraocular / intraorbital masses (e.g., rhabdomysocarcoma, lymphoma, orbital pseudotumor), metastatic disease (e.g., intraoculartumors [86%], breast, lung [adults], neuroblastoma, Ewing sarcoma, leukemia, Wilms tumor [children]), cystic lesions (e.g., abscess, hematoma, dermoid cyst, lacrimal cyst, lymphangioma), primary bone lesions, and vascular tumors (e.g., hemangioma, hemangiopericytoma).

References

Anderson NG, Wojno TH, Grossniklaus HE. Clinicopathologic findings from lacrimal sac biopsy specimens obtained during dacryocystorhinostomy. Ophthal Plast Reconstr Surg 2003;19(3):173-6.

Dahnert W. Radiology Review Manual, 4th ed. Baltimore: Williams and Wilkins, 1999.

Stefanyszyn MA, Hidayat AA, Pe’er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994;10(3): 169-84.

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