1/3. Exclusive CNS involvement by lymphomatoid granulomatosis in a 12-year-old boy: a case report.BACKGROUND: lymphomatoid granulomatosis [LYG] is an angiocentric, angiodestructive disease at the higher grade end of the spectrum of angiocentric immunoproliferative lesions. It primarily involves the lungs, but it may also involve several extrapulmonary sites including the central nervous system (CNS), skin, and kidneys. CASE DESCRIPTION: Clinical presentation, radiology and treatment of LYG in a 12-year-old male child with multiple intracranial extraaxial lesions is described. A 12-year-old boy presented with sudden onset of left focal motor seizures with associated history of headache and vomiting. Computerized tomographic scan of the brain suggested high-density, bilateral, parietal extraaxial lesions. On magnetic resonance imaging, the lesions were iso- to hyperintense on T1-weighted images and hyperintense on T2-weighted images. The lesions were excised in two stages and histopathological examination confirmed the diagnosis. CONCLUSION: LYG seldom involves the CNS exclusively. The present case demonstrates exclusive CNS involvement by LYG in a young boy.- - - - - - - - - - ranking = 1keywords = lymphomatoid granulomatosis, granulomatosis (Clic here for more details about this article) |
2/3. Successful treatment of systemic and central nervous system lymphomatoid granulomatosis with rituximab.lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder with a mortality rate approaching 60% in the first year. The median survival is 14 months from the time of diagnosis. Although a variety of chemotherapeutic regimens have been utilized, there is no standard treatment. Studies have shown that in most cases the malignant cells are B-cells, which induce massive infiltration of reactive t-lymphocytes in the background. The disease is therefore considered as a T-cell rich B-cell lymphoproliferative disorder. We report a case of LYG with pulmonary, hepatic, central and peripheral nervous system involvement that was successfully treated with the anti-CD20 (B-cell) monoclonal antibody, Rituximab.- - - - - - - - - - ranking = 1keywords = lymphomatoid granulomatosis, granulomatosis (Clic here for more details about this article) |
3/3. Lyphomatoid granulomatosis causing central diabetes insipidus: case report and review of the literature.We report a patient with pulmonary and cutaneous lymphomatoid granulomatosis with central nervous system (CNS) involvement manifesting as central diabetes insipidus and review other cases reported in the literature with attention to presence of endocrine manifestations and response to therapy. Imaging of the pituitary in our patient demonstrated a thickened hypophyseal stalk and empty sella appearance. CHOP chemotherapy proved to be an effective treatment for both the systemic and CNS involvement in our patient, but diabetes insipidus has persisted. We postulate that there was localized involvement of the stalk of the hypophysis by lymphomatoid granulomatosis, which led to a permanent lesion causing diabetes insipidus. We conclude that lymphomatoid granulomatosis may cause endocrine complications that may not resolve despite systemic remission, and while the optimal regimen for CNS involvement with lymphomatoid granulomatosis is unknown, certain patients can have long-term survival after treatment with systemic chemotherapy. Endocrine complications of malignant diseases often present diagnostic and therapeutic challenges to the internist and neuro-oncologist. lymphoproliferative disorders such as lymphomatoid granulomatosis and malignant lymphomas may cause generalized illness, which may complicate the recognition of neurological and endocrine abnormalities. Once central nervous system (CNS) involvement is documented, there is controversy regarding which therapeutic approaches are indicated to treat the CNS lesions and the systemic disease. We present a patient with lymphomatoid granulomatosis involving the pituitary hypophysis and review the literature to search for a consensus on the best treatment of CNS involvement.- - - - - - - - - - ranking = 1.5191916645242keywords = lymphomatoid granulomatosis, granulomatosis (Clic here for more details about this article) |