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.