1/57. Multifocal polyradiculoneuropathy and carcinoma of the thymus.We studied a patient with polyradiculoneuropathy with anaplastic carcinoma of the thymus. Motor manifestations dominated. Postmortem examinations indicated that the primary changes were in the spinal nerve roots, peripheral nerves and, possibly, the spinal anterior horn cells. The posterior funiculi and posterior root ganglia were also affected, implying multifocal and multiphasic degeneration. This unusual polyradiculoneuropathy is a form of carcinomatous neuropathy.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
2/57. Morphometric evaluation of paraneoplastic neuropathies associated with carcinomas, lymphomas, and dysproteinemias.Paraneoplastic peripheral neuropathies are caused by indirect effects of carcinomas, mainly small cell bronchogenic carcinomas, lymphoproliferative disorders (lymphomas, myelomas, polycythemia vera), and dysproteinemias (benign monoclonal paraproteinemia, Waldenstrom's macroglobulinemia) including cryoglobulinemias. Those associated with carcinomas are usually considered as severe, those associated with benign gammopathies (monoclonal gammopathies of unknown significance, MGUS) as mild, and those with cryoglobulinemias as of variable severity. In a larger series of 104 autopsy and biopsy cases, we noted a wide range of severity concerning various morphometric parameters of peripheral nerve fibers by evaluating sural nerves. There were no apparent morphometric differences between the groups of disorders. The most valuable parameter of optic-electronic evaluation and comparison turned out to be the myelin area expressed as a percentage of the endoneurial area because this measure comprises the relative number, size, and myelin thickness of the myelinated nerve fibers. In the 104 cases of the three disease groups, most of the cases (38 cases; 36.5%) showed a moderate reduction of the myelin sheath area per endoneurial area of sural nerves. This was followed by 34 cases (32.7%) with severe and very severe reduction. Twenty-nine cases (27.9%) presented with mild reduction. It is concluded that the severity of the neuropathy depends largely on the stage of the disease and the time of progression rather than on the type of the underlying disorder.- - - - - - - - - - ranking = 151.27633364198keywords = paraneoplastic (Clic here for more details about this article) |
3/57. 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.- - - - - - - - - - ranking = 37.819083410494keywords = paraneoplastic (Clic here for more details about this article) |
4/57. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments.We identified the IgG autoantibody ANNA-2 ("anti-Ri") in 34 patients in a 12-year period by immunofluorescence screening of sera from approximately 75000 patients with subacute neurological disorders that were suspected to be paraneoplastic. Detailed clinical information was available for 28 patients (10 men, 18 women). Cancer was diagnosed in 24 patients (86%); 21 had histologically proven carcinoma (10 lung, 9 breast, 1 cervical, 1 bladder), and 3 had an intrathoracic imaging abnormality. Cancer anteceded neurological symptoms in 4 of 28 patients. Cancer detection frequency increased with continued surveillance. Neurological disorders, in decreasing frequency, were brainstem syndrome (including opsoclonus, myoclonus, or both), cerebellar syndrome, myelopathy, peripheral neuropathy, cranial neuropathy, movement disorder, encephalopathy, Lambert-Eaton syndrome, and seizures. Four patients had laryngospasm and four had jaw opening dystonia (two with neck dystonia). Nine (32%) were wheelchair-bound 1 month after neurological symptom onset. Most improved neurologically after immunomodulatory or tumor-directed therapy. Accompanying autoantibodies, found in 73% of sera, included ANNA-1, ANNA-3, CRMP-5-IgG, P/Q-type and N-type Ca(2 ) channel antibodies, and muscle-type acetylcholine receptor antibody. Some neurological accompaniments of ANNA-2 may reflect potentially pathogenic humoral or cell-mediated responses to coimmunogenic tumor antigens, for example, Lambert-Eaton syndrome (P/Q-type Ca(2 ) channel antibody) and peripheral neuropathy (ANNA-1 effector T cells).- - - - - - - - - - ranking = 189.09541705247keywords = paraneoplastic (Clic here for more details about this article) |
5/57. A case of paraneoplastic nephrotic syndrome in a patient with ovarian carcinoma.nephrotic syndrome is a rare manifestation of malignancy associated with paraneoplastic syndrome. Paraneoplastic nephrotic syndrome has been reported in various malignancies: malignant lymphoma, colon cancer, lung cancer and prostate cancer. However, an ovarian carcinoma associated with nephrotic syndrome has rarely been reported. Only six cases of ovarian carcinoma associated paraneoplastic nephrotic syndrome has been reported worldwide, but no cases have been reported in korea. Here, we report a case of paraneoplastic nephrotic syndrome in a patient with an ovarian carcinoma. The patient presented with ascites, proteinuria and hypoalbuminemia. An initial computed tomography (CT) scan and ultrasonography evaluations showed no specific findings suggestive of an ovarian tumor. Despite treatment for nephrotic syndrome, the symptoms became more aggravated. There after, follow up evaluation at Yonsei University Medical Center, including serum CA 125, pelvis MRI and peritoneal fluid examination were performed. On the pelvis MRI, a left ovarian mass was detected with an ascitic fluid collection. The serum CA 125 level was elevated to 2211 U/ml. The peritoneal fluid cytological examination showed malignant cells suggestive of an ovarian carcinoma. Combination chemotherapies including paclitaxel plus carboplatin, topotecan plus gemcitabine and oxaliplatin plus capecitabine were administered to the patient, and complete remission was achieved on image and tumor marker studies. There was complete recovery from the nephrotic syndrome with no evidence of ascites and proteinuria. These findings suggest that nephrotic syndrome caused by paraneoplastic syndrome can be resolved only after the complete control of the underlying malignancy.- - - - - - - - - - ranking = 302.55266728395keywords = paraneoplastic (Clic here for more details about this article) |
6/57. Ductal adenocarcinoma of the pancreas with huge cystic degeneration: a lesion to be distinguished from pseudocyst and mucinous cystadenocarcinoma.Cystic neoplasms of the pancreas are rare and often mistaken for pseudocyst by imaging studies and macroscopic examination. We describe an unusual tumor of the pancreas composed of a mural nodule of anaplastic carcinoma arising from a huge ductal adenocarcinoma undergoing cystic degeneration. The cyst measured 27 x 13 x 4 cm. light microscopy showed that the cyst was partly lined by a single layer of cuboidal to columnar tumor cells with focal mucin production and was surrounded by hyalinized connective tissue. Most lining epithelial cells were absent owing to extensive degenerative process. Immunohistochemical studies showed positive staining of cytokeratin and vimentin for pleomorphic giant tumor cells, which were negative for leukocyte common antigen (CD45), KP-1 (CD68), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The ductal adenocarcinoma stained strongly positive for cytokeratin and EMA, and negative for vimentin, CD45, CD68, and CEA. The clinical course of the current case was extremely poor and the prognosis resembled that of an anaplastic carcinoma. Therefore, we like to emphasize the importance of complete excision and extensive sampling of any cystic neoplasms in the pancreas including those with large cystic component to avoid missing the malignant elements.- - - - - - - - - - ranking = 5keywords = degeneration (Clic here for more details about this article) |
7/57. Rheumatoid-like polyarthritis as a presenting feature of metastatic carcinoma: a case presentation and review of the literature.The case of a 69-year-old woman who presented with abrupt onset of rheumatoid-like symmetrical polyarthritis but who was subsequently diagnosed to have disseminated abdominal carcinomatosis is reported. The temporal relationship between the joint symptoms and the discovery of carcinoma suggested that the arthritis was a paraneoplastic manifestation of the latter. An underlying malignancy should always be looked for in elderly patients who present with explosive polyarthritis for the first time, particular if unexplained anaemia or constitutional symptoms persist after treatment.- - - - - - - - - - ranking = 37.819083410494keywords = paraneoplastic (Clic here for more details about this article) |
8/57. A new anti-neuronal antibody in a case of paraneoplastic limbic encephalitis associated with breast cancer.Paraneoplastic neurological syndromes are rare remote effects of different types of cancer. Paraneoplastic limbic encephalitis is a specific syndrome, most often associated with small cell lung carcinoma. This report describes the case of a pure limbic encephalitis in association with breast cancer. An anti-neuronal antibody was found in the serum and CSF of the patient which has not been reported so far.- - - - - - - - - - ranking = 151.27633364198keywords = paraneoplastic (Clic here for more details about this article) |
9/57. Malignant transformation of intracranial epidermoid cyst with leptomeningeal carcinomatosis: case report.INTRODUCTION: Although rare, the malignant degeneration of epidermoid cysts is well documented. Its occurrence with leptomeningeal dissemination and malignant cells in CSF is scarce. To the best of our knowledge only four cases have been reported. CASE REPORT: The authors report the case of malignant transformation of an epidermoid cyst with leptomeningeal carcinomatosis (which simulates aseptic meningitis) in a 54 year-old woman. Changes in CSF and radiological features are not correlated with the clinical course. Despite improvement of both the lesions on the scan images and the malignant cells in the CSF under chemotherapy the patient died. CONCLUSION: The differential diagnosis of malignant degeneration of epidermoid cyst, and leptomeningeal carcinomatosis are discussed. The diagnosis of malignant transformation should be retained when MRI (or CT scan) shows contrast enhancement at the epidermoid site, and malignant cells are detected in CSF The prognosis is generally poor.- - - - - - - - - - ranking = 2keywords = degeneration (Clic here for more details about this article) |
10/57. Paraneoplastic immunobullous disease with an epidermolysis bullosa acquisita phenotype: two cases demonstrating remission with treatment of gynaecological malignancy.Two cases of paraneoplastic immunobullous disease occurring in women with gynaecological malignancies are reported. Both cases demonstrated mechanobullous mucocutaneous blistering as is typically seen in epidermolysis bullosa acquisita. Their immunopathology, however, favoured a dermal-binding mucous membrane pemphigoid (MMP) (or possibly bullous pemphigoid) for patient 1 and laminin-5 MMP for patient 2. Both patients showed resolution of blistering within 1 year of treatment of their malignancies; uterine and ovarian carcinoma, respectively. These cases are of interest because of their paraneoplastic nature; as well as overlapping clinicoimmunopathological features. In addition, patient 2 is, as far as we are aware, the first report of ovarian-carcinoma-associated laminin-5 MMP.- - - - - - - - - - ranking = 75.638166820988keywords = paraneoplastic (Clic here for more details about this article) |
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