Orbital Tumor in a 62-year-old Patient
A 62-year-old man presented with progressive ptosis of the left eyelid, which he had been experiencing for the past year. He had no other symptoms or significant medical history. Physical examination revealed a firm mass below the left supraorbital rim, with secondary mechanical ptosis (Figure 1). Magnetic resonance imaging (MRI) revealed an extraconal, expansile lesion with well-defined edges at the level of the left upper eyelid, along with predominantly hypointense homogeneous signal intensity in T1 and T2 sequences showing restricted diffusion (Figure 2). Analysis of the biopsy specimen revealed a diffuse pattern of small lymphocyte proliferation and monomorphic cytology consisting predominantly of centrocyte-like cells of B-cell lineage (CD20+). Cyclin D1 and SOX11 expression were detected, although the specimen was negative for BCL6, CD5, CD10, and CD23 expression (Figure 3). The neoplastic cells exhibited restricted expression of kappa light chain, an unremarkable pattern of p53 expression (wild-type pattern), and a low Ki67 proliferation index. Fluorescence in situ hybridization detected chromosomal rearrangement involving CCND1 at 11q13.
Figure 1. Mechanical ptosis at the level of the left orbit.
Figure 2. Axial MRI showing a lesion in the extraconal compartment of the left orbit.
Figure 3. Biopsy findings. A: Magnified image of hematoxylin and eosin (H&E) staining; B: CD20+ staining; C: Reaction of the tumor cells with anti-cyclin D1; D: Low Ki67 index.
Drs. Suárez, Morillo, and Piris indicated no relevant conflicts of interest.
Correct!
The findings were diagnostic of MCL. Bone marrow biopsy results indicated an absence of lymphoma involvement, and no other lesions were observed on PET-CT. While classical nodal MCL is often associated with multisite involvement, isolated extranodal disease at the time of diagnosis is rare based on rates observed for other lymphomas. In most cases, orbital and periorbital lymphomas are extranodal marginal zone B-cell lymphomas of the mucosa associated lymphoid tissue (MALT) type. The disease appears to exhibit an aggressive clinical course, often leading to disseminated lymphoma at stage IVE, with frequent development of bilateral tumor masses involving the orbit. Isolated extranodal disease has been associated with a more favorable prognosis and more indolent course than typical cases with lymph node involvement. The optimal treatment for isolated extranodal MCL depends on individual risk factors and the presence of signs or symptoms of organ dysfunction, although options include watch-and-wait, immunochemotherapy, radiotherapy, and/or participation in a clinical trial.
Choice A (Mucosa-associated lymphoid tissue (MALT) lymphoma) is incorrect.
Choice C (Small lymphocytic lymphoma (SLL)) is incorrect as well.
Choice D (Follicular lymphoma (FL)) is incorrect.
Sorry, that was not the preferred response.
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