Lung
Lymphohistiocytic tumors
Lymphomatoid granulomatosis
Author: Deepali Jain, M.D.
Last author update: 1 September 2012
Last staff update: 12 July 2023
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: lymphomatoid granulomatosis
Table of Contents
Definition / general | Clinical features | Treatment | Microscopic (histologic) description | Positive stains | Differential diagnosisCite this page: Jain D. Lymphomatoid granulomatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungtumorlymphomatoidgran.html. Accessed April 16th, 2024.
Definition / general
- Also called angioimmunoproliferative lesion
- Lymphoproliferative disorder that is malignant per se, or likely to become malignant, resembles post-transplant lymphoproliferative disorders
Clinical features
- Middle aged men and women with well defined, bilateral rounded mass densities on chest X-ray
- Associated with transplant-related immunosuppression, Sjogren's syndrome, HIV
- 80% of cases have extrapulmonary involvement (skin, CNS, kidneys, liver, spleen, adrenal glands, heart, GI tract); rarely, there is only extrapulmonary involvement
- Usually no hilar or mediastinal lymph node involvement
- May be an EBV related, B-cell proliferation; T-cells present are usually reactive; may have reversal of T-helper / suppressor ratio
- Large number of atypical lymphoid cells is a poor prognostic factor
- Median survival 14 months; death due to sepsis, destruction of lung tissue
Treatment
- Chemotherapy
Microscopic (histologic) description
- Nodular inflammatory infiltrate of large atypical lymphoid cells (prominent nuclei, mitotic activity), plasma cells, immunoblasts, involving walls of pulmonary vessels (angioinvasive, angiocentric, angiodestructive)
- No multinucleated giant cells
- Similar infiltrates found in other organs
Positive stains
- EBV (50 - 70%)
Differential diagnosis
- Granulomatosis with polyangiitis (Wegener's): giant cells, necrosis
