Orbital lymphoma with concomitant sarcoid-like granulomas.
A 41-year-old man presented with chronic eyelid swelling, conjunctival injection, and decreased ocular motility in all gaze directions. MRI showed bilateral enlarged extraocular muscles, including the tendons. Laboratory tests revealed elevated levels of angiotensin-converting enzyme. An orbital biopsy showed collections of monotonous small lymphocytes, and granulomatous inflammation that included multinucleated giant cells, predominantly Langhans type. Flow cytometric analysis of tissue demonstrated a light chain-restricted clonal population of B cells, a finding that confirmed the morphologic impression of lymphoma. This case demonstrates that elevated angiotensin-converting enzyme and granulomatous inflammation can occur in lymphoma. Careful histopathologic examination and flow cytometric analysis are essential to avoid an erroneous diagnosis that could lead to inappropriate management.
Behbehani
Raed S
RS
Oculoplastic and Orbital Surgery Service, Wills Eye Hospital, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA. r_behbehani@hotmail.com
Bilyk
Jurij R
JR
Haber
Marian M
MM
Savino
Peter J
PJ
D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006101:Granuloma, Giant Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy; D008279:Magnetic Resonance Imaging; D008297:Male; D009918:Orbital Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy; D049268:Positron-Emission Tomography; D018714:Radiotherapy, Adjuvant; D000069283:Rituximab; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab