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21/26. lymphomatoid granulomatosis with isolated involvement of the brain.

    A patient with biopsy-proven lymphomatoid granulomatosis of the brain as the sole manifestation of the disease is described. During 14 months of follow-up since surgical excision of the cerebral mass, no evidence of recurrent brain involvement or of extracranial lesions has been found. This case is unusual in the confinement of the disease to the central nervous system and in the favorable outcome following surgical treatment alone.
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ranking = 1
keywords = nervous system
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22/26. lymphomatoid granulomatosis. Clinicopathologic study of four cases and literature review.

    lymphomatoid granulomatosis (LYG) is a poorly understood inflammatory angiitis of uncertain pathogenesis with histological similarities to Wegener's granulomatosis (WG) and atypical lymphoma. Pulmonary, constitutional, cutaneous, and central nervous system manifestations predominate, but clinical signs are extraordinarily diverse. Clinical, roentgenographic, and laboratory abnormalities are nonspecific and the diagnosis is frequently missed or delayed. We describe four cases of LYG and review the previous literature with emphasis on clinical and histological features, course, and therapy. The histological characteristics of LYG are described, and compared and contrasted with WG and atypical lymphoma. In addition to reviewing typical clinical features, we report rare findings noted in two of our cases, e.g., retroperitoneal fibrosis and lytic and blastic bone lesions which expand the spectrum of the disorder and emphasize its protean nature. Our four cases reconfirm the frequency of diagnostic delay, high mortality, and poor responsiveness to therapy. In the review of our cases and previously published data, no therapeutic regimen consistently altered the course of the disease. The efficacy of corticosteroids, immunosuppressive and cytotoxic agents remains uncertain due to variable drug and dosage schedules, lack of comparable patient populations, and retrospective nature of previous reports. However, it appears that corticosteroids may transiently alter manifestations of LYG, but do not prevent ultimate progression. cyclophosphamide may have contributed to arrest of the disease in our Cases 3 and 4, as well as in sporadic cases from the literature, but its overall efficacy appears to be limited. Multiple-agent chemotherapy has been associated wih occasional responses, but generally has been unsuccessful. Surgery and radiation therapy may be helpful in selected patients with localized disease. The role of corticosteroids, cytotoxic agents, and radiation therapy, alone or in combination, needs to be clarified by prospective cooperative trials.
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ranking = 1
keywords = nervous system
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23/26. Cutaneous manifestations of lymphomatoid granulomatosis. Report of 44 cases and a review of the literature.

    A review of records of 44 patients who had lymphomatoid granulomatosis (LYG) with skin lesions showed that cutaneous manifestations are protean and generally transient. Subcutaneous or dermal nodules were present in more than 60% of cases. A maculopapular rash or macular erythema was less common, and ulceration was rare. skin lesions were widely distributed. Frequently, in patients who had early cutaneous involvement with LYG, associated involvement of the nervous system and joints occurred. Findings from laboratory studies were generally inconclusive. lymphomatoid granulomatosis progressed to lymphoma in 18% of the cases, and death from respiratory failure was common. Theories on the possible cause and the pathogenesis of LYG are presented.
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ranking = 1
keywords = nervous system
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24/26. Multiple giant intracranial aneurysms associated with lymphomatoid granulomatosis. A magnetic resonance imaging and angiographic study.

    lymphomatoid granulomatosis is an uncommon lymphoproliferative disorder that frequently has central nervous system manifestations. lymphomatoid granulomatosis has clinical features similar to both vasculitis and lymphoma. The pathological hallmarks of this disease include necrotic angiocentric and angiodestructive infiltrations of premalignant or malignant lymphoid cells. There are, to the authors' knowledge, only a few magnetic resonance imaging reports and no magnetic resonance angiographic reports of this disorder. Presented here is a case of lymphomatoid granulomatosis producing multiple giant fusiform and saccular aneurysms throughout the major intracerebral arteries, along with patterns of vascular beading typically seen with vasculitis demonstrated by magnetic resonance angiography.
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ranking = 1
keywords = nervous system
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25/26. Angiocentric T-cell lymphoma presenting with multiple cranial nerve palsies and retrobulbar optic neuropathy.

    Angiocentric T-cell lymphoma (lymphomatoid granulomatosis) may present with prominent central nervous system (CNS) findings with variable radiographic features. We describe a patient who presented with multiple cranial nerve palsies involving the left optic nerve, left facial nerve, left ocular motor nerves, and bilateral acoustic nerves. Enhancement of the right temporal meninges and a cavernous sinus mass were noted on magnetic resonance (MR) scan. A right temporal craniectomy and meningeal biopsy were performed. Meningeal biopsy revealed atypical angiocentric granulomatous lymphoid infiltrates without associated necrosis, giant cells, or granuloma formation. Morphologic and T-cell, receptor gene rearrangement findings were diagnostic of an angiocentric T-cell lymphoma. Retrobulbar optic neuropathy and multiple cranial nerve palsies may be the presenting features of angiocentric T-cell lymphoma. The neurologic and unique radiographic changes in our case expand the previously reported findings in CNS angiocentric T-cell lymphoma.
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ranking = 1
keywords = nervous system
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26/26. 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.
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ranking = 2
keywords = nervous system
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