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Presented by Peter Illei, M.D. and prepared by ChanJuan Shi, M.D., Ph.D.
Case 6: 50 y.o. white male who works as a wrestling coach and in the setting of a chest injury.
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1. Question
Week 353: Case 6
50 y.o. white male who works as a wrestling coach and in the setting of a chest injury, had a chest x-ray that revealed a left lower lobe mass, which had been entirely asymptomatic. PET CT scan performed revealed low-density lobulated masses at the left lung base and one at the anteromedial right lung base near the cardiophrenic angle.images/illei060908-6a.jpg
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images/illei060908-6e.jpgCorrect
Answer: Lymphomatoid granulmatosis
Histology: none provided
Discussion: Histologically there is extensive necrosis surrounded by a vaguely granulomatous mixed chronic inflammatory infiltrate. Scattered enlarged atypical lymphoid cells are noted that are predominantly seen in vessel walls and perivsacularly. The lymphoid infiltrate is largely composed of CD3 positive T-cells and also contains CD20 positive B-cells as well as CD68 positive histiocytes and plasma cells. The scattered atypical lymphoid cells are CD20 positive B-cells. In situ hybridization for EBER is positive in a subset of small B-cells and in a subset of the atypical B-cells. The large areas of zonal necrosis appear to be in areas of necrotic B-cells. Special stains for fungi (GMS) and acid fast bacilli (AFB) are negative. There are only few atypical large cells present consistent with grade 1 lymphomatoid granulomatosis (LYG).
LYG also known as midline lethal granuloma, is a clonal proliferation of EBV infected B-cells in a background of reactive T-cells and necrosis. It is a vasocentric process that lacks neutrophils. LYG is graded (grade I-III where grade III LYG has the features of a high grade non-Hodgkin’s lymphoma) based on the cytologic atypia and number of atypical large B-cells, as well as extent of necrosis. The bulk of the lymphoid cells in the background are CD2+, CD3+, CD4+ T-helper cells and a lower number of CCD8+ T-killer cells and CD16/CD56+ natural killer cells.
Incorrect
Answer: Lymphomatoid granulmatosis
Histology: none provided
Discussion: Histologically there is extensive necrosis surrounded by a vaguely granulomatous mixed chronic inflammatory infiltrate. Scattered enlarged atypical lymphoid cells are noted that are predominantly seen in vessel walls and perivsacularly. The lymphoid infiltrate is largely composed of CD3 positive T-cells and also contains CD20 positive B-cells as well as CD68 positive histiocytes and plasma cells. The scattered atypical lymphoid cells are CD20 positive B-cells. In situ hybridization for EBER is positive in a subset of small B-cells and in a subset of the atypical B-cells. The large areas of zonal necrosis appear to be in areas of necrotic B-cells. Special stains for fungi (GMS) and acid fast bacilli (AFB) are negative. There are only few atypical large cells present consistent with grade 1 lymphomatoid granulomatosis (LYG).
LYG also known as midline lethal granuloma, is a clonal proliferation of EBV infected B-cells in a background of reactive T-cells and necrosis. It is a vasocentric process that lacks neutrophils. LYG is graded (grade I-III where grade III LYG has the features of a high grade non-Hodgkin’s lymphoma) based on the cytologic atypia and number of atypical large B-cells, as well as extent of necrosis. The bulk of the lymphoid cells in the background are CD2+, CD3+, CD4+ T-helper cells and a lower number of CCD8+ T-killer cells and CD16/CD56+ natural killer cells.
