Isolated central nervous system involvement by lymphomatoid granulomatosis in an adolescent: a case report and review of literature.

Lymphomatoid granulomatosis is an Epstein-Barr virus-associated multisystem disease that combines granulomatous inflammatory process with lymphoproliferative potential. It predominantly affects lungs, skin, and brain and is characterized by multifocal, transmural, angiocentric, and angiodestructive pleomorphic lymphoid infiltrate in a perivascular distribution. Lymphomatoid granulomatosis is generally considered to be a neoplastic B-cell proliferation that has traditionally been associated with poor prognosis, evolving as a progressive multisystem disease transforming into B-cell lymphoma, with a median survival of 14 to 16 months only. Its lymphomatous nature explains prompt response to steroids and systemic chemotherapy, although appropriate optimal management still remains to be defined. The authors report on a young boy who presented with features of raised intracranial tension and sudden onset seizures. Neuroimaging showed 2 space-occupying lesions, larger in the left frontoparietal region with heterogeneous enhancement, moderate perifocal edema, compression, and mass effect. He underwent surgical decompression of the dominant lesion with prompt relief of symptoms. The diagnosis of lymphomatoid granulomatosis was confirmed on light microscopy and immunohistochemistry. An extensive systemic work-up ruled out other site(s) of involvement. He was successfully treated with aggressive systemic chemotherapy and moderate dose of whole-brain radiotherapy. Awareness of disease spectrum in the central nervous system may permit early diagnosis and thus allow institution of timely appropriate therapy.