PMC3640232_01
Pretreatment MRI: Coronal (a), axial (b) and sagittal (c) postcontrast T1demonstrate a mildly enhancing upper nasal cavity mass with extending through the cribriform plates. Associated abnormal retropharyngeal lymph nodes
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F1
MR imaging of the orbital and nasal region demonstrated a homogenous and well-circumscribed mass, with mild homogenous enhancement [Figure 1] and mild restricted diffusion [Figure 2a]. Endonasal biopsy of the mass yielded specimens that showed histologic and immunohistochemical findings diagnostic of an ALK(+) ALCL [Figure 3 and Table 1].
MR images important for the correct differential diagnosis: ADC map (a) demonstrating mild restricted-diffusion, suggesting hypercellularity. Axial T2 (b) at the level of the nasopharynx demonstrates enlarged retropharyngeal lymph nodes, suggesting either primary lymphoid-disease or typical nodal spread of esthesioneuroblastoma
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F2
MR imaging of the orbital and nasal region demonstrated a homogenous and well-circumscribed mass, with mild homogenous enhancement [Figure 1] and mild restricted diffusion [Figure 2a]. Enlarged retropharyngeal lymph nodes [Figure 2b] and upper level IB/II lymph nodes were observed.
Microphotographs of the lymphoma cells and immunohistochemistry. Diffuse lymphoid infiltrate composed of large-sized cells (a) (H and E, x100); "hall marker" cells (b) (H and E, x400); lymphoma cells are positive for CD30 (c), TIA-1 (d), CD4 (e) and ALK-1 (f) (C-E, x200; F, x400)
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F3
Endonasal biopsy of the mass yielded specimens that showed histologic and immunohistochemical findings diagnostic of an ALK(+) ALCL [Figure 3 and Table 1]. Hematoxylin and eosin (H&E) sections of the nasal cavity mass biopsy show a diffuse lymphoid infiltrate [Figure 3a] underneath the nonkeratinizing stratified squamous epithelium consistent with the nasopharyngeal location. Occasional nuclei demonstrate horseshoe-like shape, consistent with the so-called "hallmark cells" [Figure 3b]. To further delineate the nature of this lymphoma, a panel of immunohistochemistry was performed [Figure 3c-f]. T-cell intracellular antigen-1 (TIA-1) [Figure 3d]. The majority of the neoplastic cells are also positive for CD4 [Figure 3e] and CD5, partially positive for CD45, and rarely positive for CD2 [Table 1]. The neoplastic cells show diffuse nuclear and cytoplasmic staining of ALK-1 [Figure 3f]. City, Utah) showed monoclonal TCR gamma gene rearrangement. Chromosomal analysis of the rearrangements suggest that the positive ALK expression was most likely due to the translocation of ALK located on chromosome 2p23 and nucleophosmin located on 5q35 namely t(2;5)(p23;q35), based on the both nuclear and cytoplasmic granular staining pattern [Figure 3f].
Four month post treatment coronal (a), axial (b), and sagittal (c) postcontrast T1 MR images, demonstrate decreased size of the lymphoma mass in the nasal cavity, along the cribriform plates, and prepontine cistern
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F4
Repeated MR imaging 6 months after completion of the treatment regimen showed a dramatic decrease in tumor size, such that the optic nerve no longer showed signs of compression [Figure 4].