Presentation
Shortness of breath and hypoxia.
Patient Data
There is a prominent right perihilar and basilar nodular/rounded opacity.
There is circumferential soft tissue density surrounding the mid-lower trachea and proximal mainstem bronchi with resultant narrowing of the mid-lower trachea. There is a soft tissue nodule in the non-dependent mid-trachea and defects of the distal left mainstem bronchial wall with surrounding foci of pneumomediastinum. There is occlusion of the left lower lobe bronchus.
There is a mildly enlarged right hilar lymph node. There is irregular nodular consolidation in the medial right upper lobe which abuts the mediastinum without a clear fat plane between it and the circumferential tracheal soft tissue. There are two solid noncalcified soft tissue masses at the right lung base.
There are multiple soft tissue nodules identified throughout the chest and abdominal wall and
an ill-defined mass in the posterior left breast.
Diffuse soft tissue lesions are seen throughout the chest, abdomen, and pelvis in the subcutaneous regions, as well as within the right nasal cavity, mediastinal/right hilum, and right lung base.
Case Discussion
This is a case of lymphomatoid granulomatosis initially presenting as pulmonary masses. The patient underwent a tracheal biopsy which showed geographic necrosis with atypical lymphoid cells that were of medium to large size. These lymphoid cells were positive for EBER (in situ hybridization) and are of B-cell lineage (positive for CD20). AFB and GMS were negative for microorganisms. The lymphoid proliferation was CD20 and EBER-ISH positive.
The patient also underwent skin biopsies. Histopathology revealed superficial to deep perivascular, interstitial, and periadnexal infiltrate of atypical intermediate to large-sized cells with vesicular nuclei, variably prominent nucleoli, and identifiable mitoses with areas of necrosis. There was also evident epidermal necrosis. Immunostains showed lesional cells positive for CD20, bcl-2, MUM-1, and INI-1 (expression retained). In-situ hybridization study for EBER was positive in the lesional cells. In-situ hybridization studies for immunoglobulin kappa and lambda light chains showed a predominance of kappa staining cells. Ki-67 shows approximately 50-60% proliferation rate. No fungal, bacterial, or acid-fast bacilli were identified using GMS and PAS, Gram, or AFB stains, respectively. An immunostain for Treponema was negative. The overall appearance suggests an aggressive B-cell lymphoma that is positive for Epstein-Barr virus.
The patient underwent an additional core biopsy for hematopathology. Fluorescence in situ hybridization (FISH) analysis was performed using a dual color ALK and MALT1 break-apart probe set to detect rearrangement, but these results were within normal limits. Lastly, FISH analysis using a dual color, dual fusion CCND1(BCL1)/IGH probe set used to detect the (11;14) translocation was within normal limits.
The histologic differential diagnosis includes lymphomatoid granulomatosis or Epstein-Barr virus-positive diffuse large B cell Lymphoma, NOS. With regard to the former, the atypical B cell infiltrate showed evidence of angioinvasion and angiodestruction (immunostains for SMA and desmin, and elastic stains that were performed). Combined with pulmonary involvement, the overall findings are most consistent with lymphomatoid granulomatosis. Given the extent of the B cell infiltrate with necrosis and conspicuous Epstein-Barr virus positivity, the lesions are further characterized by Grade 3 lymphomatoid granulomatosis.
Co-author:
Camille Dumas, DO
Unable to process the form. Check for errors and try again.