Cases reported "Paraneoplastic Syndromes"

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1/8. Cancer polyarthritis resembling rheumatoid arthritis as a first sign of hidden neoplasms. Report of two cases and review of the literature.

    Recent onset arthritis reminiscent of rheumatoid arthritis (RA) may be an early manifestation of an occult malignancy. In this report, we present two patients with cancer-associated polyarthritis. Both suffered from symmetric polyarthritis when initially visiting their physicians and did not achieve relief when treated with non-steroidal anti-rheumatic drugs (NSAIDs). In both patients, subsequent work-up led to the diagnosis of an underlying malignancy. One patient suffered from small cell lung cancer (SCLC), while the other was diagnosed with adenocarcinoma of the colon. In both, the arthritis spontaneously disappeared after successful treatment of the malignancy, i.e. chemotherapy and tumor resection, respectively. We discuss these cases in view of the existing literature, since awareness of the entity of cancer polyarthritis is necessary for its timely treatment and may potentially be life-saving.
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2/8. Remitting seronegative symmetrical synovitis with pitting oedema: a study of 12 cases.

    Twelve patients with remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) were analysed. Eight of them had typical RS3PE without underlying disease, and four presented associated neoplasia. The first patients experienced an excellent response to low doses of prednisone, and they all achieved complete and permanent remission. The mean treatment duration was 18 months and the mean follow-up was 4.4 years. During the follow-up, none of these patients relapsed, had fever or general health deterioration, and hand and foot radiographs did not show erosion. One of them developed a panarteritis nodosa 6 years later. Four RS3PE patients had associated neoplasia. Two were with solid malignancies, and the other two presented haematological malignancies. In one of them RS3PE preceded the diagnosis of malignancy. The diagnosis of RS3PE in the other patients was subsequent to cancer. The first patients presented clinical characteristics suggestive of paraneoplastic RS3PE, and they had a poor response to corticosteroid therapy. Two patients died, and the rest of them had a complete response to surgical resection of the tumour or to chemotherapy. In general, idiopathic RS3PE patients do not show either general health deterioration or fever and they do respond to low doses of steroids (10 mg/day). We observed strong contrasts with the results obtained when treating RS3PE patients with associated neoplasia. In patients with RS3PE the presence of systemic symptoms along with resistance to low doses of corticosteroid therapy should alert the physician to the possible presence of malignancy.
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3/8. Successful treatment of B cell chronic lymphocytic leukemia-associated severe paraneoplastic pemphigus with cyclosporin A.

    Since the first description of paraneoplastic pemphigus, several cases have been described in the literature. However, curative therapy is usually a challenge to the physicians treating this disease. Several publications are available discussing the efficacy of steroids, cyclophosphamide and cyclosporin A. Recently, a report of the successful use of rituximab was also published. However, the use of cyclosporin A is controversial in the case of B cell malignancies, as there are reports showing the cytotoxic effect of this drug on B cells. However, other authors report no effect, or even unwanted effects resulting in B cell proliferation. We report the case of a 50-year-old Caucasian male. He developed a B cell lymphoma consisting of CD5/CD20-double-positive cells, and 2 months later, it was followed by a very severe paraneoplastic pemphigus affecting the mucosa and the skin. The lymphoma was well managed with CHOP and CVP polychemotherapy, followed by oral chlorambucil; however, the bullous eruptions did not disappear. Oral steroids, cyclophosphamide, plasmapheresis and IVIG therapy were only partially successful, so we decided to use oral cyclosporin A. Starting with 7 mg/kg and maintaining a steady plasma level of no less then 110 ng/l, the bullae completely disappeared within 6 weeks, and the patient has been in remission for 17 months now, taking the oral cyclosporin A continuously. The underlying B cell disorder did not relapse during the therapy.
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4/8. A middle-aged woman with an anterior mediastinal mass and respiratory failure.

    The discovery of an anterior mediastinal mass presents a challenge to the physician. In addition to distinguishing between benign and malignant conditions, it is important to recognize the potential for associated paraneoplastic phenomena. We present a case of a malignant thymoma associated with myasthenia gravis.
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5/8. Rheumatoid arthritis-like syndrome: a presenting symptom of malignancy. Report of 3 cases and review of the literature.

    Recent onset arthritis might be an early manifestation of an occult malignancy. Three patients are described: one with carcinoma, primary site unknown; one with oat cell carcinoma of the bronchus; and one with breast cancer. The presenting symptom of their disease was polyarthritis. Two of the patients were seropositive and in two patients the arthritis regressed following the removal of the tumor. awareness of paraneoplastic arthritis, especially if its appearance is explosive or in relatively old age, should caution the physician of the possibility of a potentially curable, but hidden neoplasm.
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6/8. Erythematous papules in a patient with cardiac myxoma: a case report and review of the literature.

    A 35-year-old woman was followed up by a physician for one and a half years as a case of transient ischemic attach (TIA) due to thrombosis. She had a few erythematous papules on the dorsal side of her right forearm and on the right side of her neck. From the history of TIA and the transient erythematous papules, we suggested the possibility of cardiac myxoma. Echocardiogram demonstrated a large mass in her left atrium. An immediate operation was carried out, and the cardiac myxoma was excised surgically. Histological examination of the erythematous papule revealed myxoma emboli.
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7/8. Paraneoplastic sensorimotor neuropathy associated with breast cancer.

    Paraneoplastic sensorimotor neuropathy occurs in association with many different types of cancer. The clinical findings are heterogeneous, and the pathogenesis is unknown. We have encountered 9 women with breast cancer and shared neurological features that suggest a distinct paraneoplastic syndrome. The syndrome is characterized by upper and lower extremity paresthesias and numbness, itching, muscle weakness and cramps, and in some, radicular symptoms and signs. serum and CSF inflammatory changes suggested an immune pathogenesis but none had detectable antibodies directed at nervous system elements. Six patients presented with neuropathy 2 months to 8 years before the discovery of the breast cancer. In 7 the neoplastic disease was localized to the breast and axillary lymph nodes. The neurologic course was chronic in all, and while symptoms were annoying, disability was minimal until late. One improved transiently with plasmapheresis, and three had mild transient improvement with treatment of the cancer. Recognition of this paraneoplastic syndrome may forewarn the physician of an underlying breast malignancy.
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8/8. paraneoplastic cerebellar degeneration. Case report and literature review.

    paraneoplastic cerebellar degeneration (PCD) presents with acute or subacute onset of ataxia, dysarthria, and intention tremor. In patients older than 50 years, acute or subacute cerebellar degeneration is paraneoplastic in origin in 50% of cases. paraneoplastic cerebellar degeneration most often precedes a potentially curable remote malignancy. Less often, PCD occurs in a patient with a known malignancy or heralds the onset of a recurrence. The presence of specific antibodies in serum samples helps to guide identification of the occult underlying malignancy. physicians should entertain the diagnosis of PCD when older patients present with signs of cerebellar degeneration without an obvious cause. A systematic evaluation, including the selection of appropriate imaging and laboratory studies, will often enable physicians to identify the responsible cancer. However, because PCD can precede a cancer by months to years, periodic reevaluation is needed when the cancer remains occult.
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