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.
"Low-grade myxoid neoplasm with recurrent potential" (cellular myxoma) is a term recently used to describe a subset of soft tissue lesions with histology intermediate between intramuscular myxoma and low-grade myxofibrosarcoma or myxoid malignant fibrous histiocytoma (MFH), while resembling a deeper counterpart of superficial angiomyxoma. Their distinctive biological behavior is characterized by the potential to recur locally, in contrast to intramuscular myxoma, while having no potential to advance in grade or metastasize when compared to low-grade myxofibrosarcoma. We present a cytohistological correlation for an intramuscular location of such a tumor in the lower extremity of a 49-yr-old male.
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