Case 668 -- A 6 year-old girl with a large left neck mass

Contributed by Jennifer Picarsic, MD and Miguel Reyes-Mugica, MD


The patient is a 6-year old girl with past medical history significant for biliary cirrhosis secondary to extra hepatic biliary obstruction (biliary atresia) who, at one year of age, underwent an cadaveric, orthotopic liver transplant (OLTx). The recipient's EBV status prior to transplantation was not available in the clinical records. Post-transplant serology was positive for EBV. Around 40 months post-transplantation, she had an episode of early EBV-associated PTLD of the adenoids which clinically responded to decreased immunosuppression. At 63 months post-transplantation, she presented with an enlarging left neck nodal mass that was surgically excised.

At admission significant laboratory findings included:


Gross examination revealed two partially effaced lymph nodes matted together (4.5 x 2.5 x 1.5 cm) that on cut surface displayed partially hemorrhagic tissue with the majority of the nodal tissue composed of firm, tan-white parenchyma. On histologic H&E sections, the lymphoid tissue was largely replaced by a proliferation of monotonous, medium-sized, lymphoid cells having finely granular chromatin, 1 to 4 small, irregular, amphophilic nucleoli and a small rim of cytoplasm. On low power there is a "starry-sky" pattern imparted by tingible body macrophages (cellular debris-laden macrophages) engulfing apoptotic debris of the rapidly proliferating cells (Figures 1 and 2). On paraffin-immunohistochemical staining, these proliferating cells are strongly positive for CD20 in a membranous staining pattern (Figure 3), weak-moderate membranous staining for CD10 (Figure 4), moderate bcl-6 nuclear staining (Figure 5) and moderate lambda light chain staining with negative kappa staining (light chain IHC not shown) and negative CD3 (not shown). The Ki-67 proliferation index was >95% (Figure 6) and most of the proliferating cells were positive with the EBER probe for EBV in situ hybridization (Figure 7). This specimen did not have diagnostic tissue sent for cytogenetic testing, including fluorescence in situ hybridization studies.


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