Cases reported "Paraneoplastic Syndromes"

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1/25. Palmar fasciitis and arthritis: association with endometrial adenocarcinoma.

    A 74-year-old woman was referred because of rheumatic symptoms consisting of pain, swelling of the hands, contracture and flexion of the fingers and severe palmar erythrosis. One year earlier she had undergone a total abdominal hysterectomy (TAH) for uterine adenocarcinoma. A paraneoplastic syndrome with palmar fasciitis and arthritis was then suspected and an evolutive peritoneal carcinomatosis was confirmed by abdominal CT scan. The patient was first treated with hormonal therapy (progestagen) and then with chemotherapy. This, associated with calcitonin, corticosteroids and physiotherapy, allowed a temporary recovery, but the patient died 10 months later from progressive peritoneal carcinomatosis.
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2/25. Painless erythema of the hands associated with non-Hodgkin's lymphoma in a lung transplant recipient.

    Acral erythema limited to the fingers or the hands has been described in systemic disease. We report the case of a 17-year-old patient who had undergone lung transplantation for cystic fibrosis. Six weeks after transplantation, rapidly growing large-cell immunoblastic non-Hodgkin's lymphoma of the lungs and mediastinum was diagnosed. Sharply demarcated, painless, glovelike erythema was found on both hands. Therapy was reduction of immunosuppression and 12 cycles of extracorporeal photophoresis. After 4 months, lymphoma was in clinical and radiologic remission. Bilateral erythema of the hands also had disappeared. Acral erythema has been reported in association with chemotherapy, collagen vascular disease, infections as with hiv, hepatitis c virus, parvovirus B19, or cytomegalovirus. None of the described associations were detected in this case.
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3/25. Polyarteritis associated with hypopharyngeal carcinoma.

    A rare case of polyarteritis associated with a solid tumor is presented. A 66-year-old man was referred to our hospital, because of gangrene in the bilateral fingers and toes, right pleural effusion, and an abnormal sensation in the throat. A diagnosis of polyarteritis was made based on pleuritis, digital gangrene and the arteriography findings. He also had a hypopharyngeal carcinoma. After being treated with intermittent intravenous cyclophosphamide, oral corticosteroid, alprostadil and aspirin, the pleural effusion rapidly disappeared, while the digital gangrene gradually improved. For the treatment of hypopharyngeal carcinoma, radiation therapy was initiated and resulted in complete disappearance.
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4/25. Hypertrophic osteoarthropathy associated with metastatic phyllodes tumor.

    Hypertrophic osteoarthropathy (HOA) is a paraneoplastic syndrome consisting of digital clubbing, polyarthralgias, and periostosis that sometimes accompanies primary bronchogenic carcinoma and other pulmonary malignancies. We report a case of HOA as the initial manifestation of pulmonary metastases in a 42-year-old woman with malignant phyllodes tumor of the breast. Since the treatment for malignancy-associated HOA is targeted at the underlying neoplasm, it is important to make the diagnosis in a timely fashion so that appropriate therapy may be initiated without delay. HOA symptoms generally improve, as they did in our patient, if the cancer responds to treatment. The pathophysiology of HOA is poorly understood, but a role for tumor-associated humoral mediators has been postulated. The hypothesized mechanisms underlying HOA are reviewed, and evidence for a prominent role for platelet-derived growth factor in mediating this syndrome is examined. This unusual case illustrates the importance of suspecting HOA in a patient with a history of cancer who presents with otherwise unexplained polyarthralgias.
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keywords = osteoarthropathy
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5/25. Palmar fascial thickening and contractures of fingers resembling arthritis--a paraneoplastic symptom?

    A 59-year-old women presented with contractures of the fingers of both hands 11 months before a diagnosis of an ovarian carcinoma with paraaortic lymph node metastases was made. We suggest that the contractures, which were associated with palmar fascial thickening and which clinically resembled arthritis, might have been a paraneoplastic sign.
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6/25. Carcinoma of the pancreas with neuroendocrine differentiation and nodular panniculitis.

    BACKGROUND: On rare occasions tumours of the pancreas produce high amounts of pancreatic lipase. The enzyme activity in the blood and in different tissues causes a syndrome called nodular panniculitis by focal necrosis of lipids and a concomittant inflammatory reaction. CASE REPORT: A 72-year-old man was admitted to the dermatology clinic with the diagnosis of erythema nodosum. The patient had been well until 3 months earlier when painful red nodes developed on the skin of both shanks. He complained of profuse night sweating and a weight loss of 10 kg within that time but did not have fever. He also had noticed a painful swelling of his right index finger, left middle finger and the third toe on his left foot. biopsy of the nodes revealed a focal necrosis of fatty tissue. Laboratory examinations showed a highly elevated concentration of serum pancreatic lipase. Further investigations showed a tumour in the pancreas and several osteolytic lesions. Tumour biopsy revealed a neuroendocrine carcinoma. After tumour resection serum lipase level immediately fell to almost normal values, and all skin and bone manifestations disappeared quickly. CONCLUSION: Due to its clinical appearance the panniculitis syndrome is most often mistaken for either erythema nodosum or rheumatoid arthritis. A resection of the tumour after correct diagnosis should always be considered because the widespread manifestations in the skin and bones do not represent distant metastasis and have a very good chance to dissolve completely.
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7/25. hand involvement in paraneoplastic syndrome: a case report.

    Cutaneous paraneoplastic syndromes are skin and mucous membrane changes that are associated with cancer. We report a previously healthy 76-year-old man who developed marked finger and thumb contracture, pain, and hypersensitivity of both hands who was diagnosed subsequently as having gastric carcinoma with colonic metastasis. After the gastrointestinal tumors were resected the finger and thumb contracture lessened and the pain eased. Both the temporal relationship between the changes in the hand and the neoplasm and the improvement after resection suggest a paraneoplastic syndrome.
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8/25. Hypertrophic osteoarthropathy associated with metastatic melanoma.

    Hypertrophic osteoarthropathy (HOA) is one of the paraneoplastic syndromes most commonly associated with non-small-cell lung cancer. Although pulmonary metastasis is the second most common initial site of melanoma metastasis, HOA is rarely detected in patients with metastatic melanoma in the lung. We report a case of a 45-year-old woman with advanced melanoma who developed HOA after her disease had progressed through first-line systemic therapy. The patient's diagnosis of HOA was made on the basis of digital clubbing, arthralgia, pain, joint effusion and periosteal bone formation on X-ray with negative rheumatologic laboratory studies. Only six cases of HOA in metastatic melanoma have been reported previously. This diagnosis should be considered with lung metastases and the presentation of polyarthralgia with appropriate laboratory and imaging findings. Interestingly, the patient responded to bisphosphonates and second-line chemotherapy with carboplatin and paclitaxel, which is commonly used for lung cancer, not advanced melanoma. As with many paraneoplastic syndromes, successful treatment of the underlying disease was associated with a rapid resolution of the symptoms.
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ranking = 2982.0460140037
keywords = osteoarthropathy
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9/25. Presymptomatic diagnosis of bronchogenic carcinoma associated with bilateral diffuse uveal melanocytic proliferation.

    A 62-year-old man presented with bilateral diffuse uveal melanocytic proliferations (BDUMP) and painful flexor contractures of the fingers of both hands. All these features were considered paraneoplastic but extensive and repeated investigations revealed no underlying malignancy. Oral steroids and orbital radiotherapy were ineffective. The diagnosis was confirmed by trans-scleral biopsy of the right choroid. Rapidly progressive cataracts were treated by phacoemulsification. Severe exudative retinal detachment with rubeosis and neovascular glaucoma in the left eye were treated successfully by partial choroidectomy. Fifteen months after presentation, investigations detected a 22 mm, poorly differentiated adenocarcinoma, which was resected without complication. The ocular tumours in both eyes regressed, without improvement in vision of light perception, and the palmar fasciitis also improved. The patient remained free of tumour recurrence until sudden death from myocardial infarction five years after he first presented.
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10/25. Antibody to a zinc finger protein in a patient with paraneoplastic cerebellar degeneration.

    In some patients with paraneoplastic cerebellar degeneration (PCD), autoantibodies against neural components have been identified. Here, we demonstrate a major 58 kd protein antigen in an immunoblot of human cerebellum by serum from a patient with PCD. Immunohistochemically, the serum recognized neural cells especially purkinje cells in a human brain. To identify the details of the target antigens for the antibody, we isolated a cDNA clone from a human cerebellar library. Homology searches revealed a similarity with the zinc finger proteins. PCD related proteins reported here may be important to maintain neural cells especially those in the cerebellum, and further studies on this molecule may help us elucidate the causes of degenerative or autoimmune diseases in the cerebellum.
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