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The authors discuss the possible development of rheumatoid paraneoplastic syndrome in patients with benign tumours. They present three observations they made (cardiac myxoma, subpleural lipoma and pulmonary fibrosclerotic pseudotumour). Paraneoplasias in these cases are interesting from the pathogenetic aspect as regards their development, possibly malignant transformation of benign intrathoracic tumours.
The paraneoplastic syndromes are effects of cancer that occur at sites remote from the primary tumor and its metastases. Recognition of these disorders is important from both diagnostic and therapeutic viewpoints. The important paraneoplastic syndromes involving the endocrine, nervous, hematologic, and dermatologic systems are discussed in this article.
Nephrotic syndrome, although rare, is recognized as one of the paraneoplastic syndromes. A patient with documented Hodgkin's disease showed increased uptake of Ga-67 in the cortex of both kidneys in addition to multiple sites of lymphomatous involvement. The patient was not receiving chemotherapy or any nephrotoxic drugs, and no other reasons that may account for renal uptake of gallium could be identified. The final clinical diagnosis was nephrotic syndrome as an unusual paraneoplastic syndrome of Hodgkin's disease. Gallium imaging was able to detect multiple sites of lymphoma and the renal uptake due to the associated nephrotic syndrome.
The paraneoplastic syndrome does exist and its manifestations are as old as malignancy itself. Progress in molecular pathology will help us to understand its origin. At the same time, each investigator is obliged to develop therapeutic means that can be offered to the cancer patient to overcome its painful and troublesome disorders.
Neurological paraneoplastic syndromes are a rare group of disorders that occur in 1-2% of people with malignancy. They are usually caused by an immune response, triggered by and directed against a tumour, that cross-reacts with protein expressed by the peripheral or central nervous system. Any part of the nervous system can be affected and patients often develop severe and permanent disability. Diagnosis can be difficult as in two-thirds of patients the neurological problems appear up to 5 years before the tumour manifests. However, certain of these syndromes are often associated with specific serum autoantibodies that can be useful both in diagnosis of the neurological syndrome and in focusing the search for a particular tumour. Thus, these antibodies can allow earlier identification and treatment of cancer and, potentially, a reduction in morbidity and mortality. It was only in the 1980s that the first anti-neuronal autoantibodies were characterized and their associations with clinical syndromes and tumours defined. Further antibodies have been isolated over the past 20 years and novel pathogenic mechanisms for several syndromes have been recognized. For example, voltage-gate ion channels seem to be a common target for autoantibodies involved in peripheral nerve diseases such as the Lambert-Eaton myasthenic syndrome and neuromyotonia (Isaacs' syndrome). However, the place of most paraneoplastic antibodies in the pathogenesis of central syndromes is yet to be fully elucidated.
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Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman's disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive therapies. The patient died 1 year after presentation, as a result of complications of bronchiolitis obliterans. This case is unusual because of the young age of the patient.
Cutaneous paraneoplastic syndromes are a large group of dermatoses that may be associated with an internal malignancy. Among these dermatoses are acanthosis nigricans, tripe palms, dermatitis herpetiformis, dermatomyositis, extramammary Paget's disease, hypertrophic pulmonary osteoarthropathy, pemphigus vulgaris, pruritus, pyoderma gangrenosum, Sweet's syndrome and reactive erythemas. Once the diagnosis of the dermatosis has been confirmed, an appropriate work-up should be undertaken to search for an underlying asymptomatic neoplasm in a patient without known cancer or to detect recurrent or metastatic disease in a patient with an established history of malignancy.
Neurological paraneoplastic syndromes (NPS) affect only 0.01% of cancer patients, chiefly those affected by lung, breast, ovarian and stomach cancer. They frequently cause major disability and produce limitations in patients' daily activities; the character of the disease is irreversible. Clinical suspicion is fundamental for an early diagnosis and it must be backed up by the specification of certain antibodies both present in blood and in cerebrospinal fluid (CSF). Conventional treatments are very inefficient at the time of treating these disorders; at present, the administration of immunoglobulins, immunosuppressors, chemotherapy agents and corticoids are under study, but so far results are not promising. The aim of this review is to analyze the variety of NPS and describe the findings concerning autoimmunity and treatments used at present.
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BACKGROUND: Voltage-gated calcium channels in small-cell lung carcinomas may initiate autoimmunity in the paraneoplastic neuromuscular disorder Lambert-Eaton syndrome. The calcium-channel subtype that is responsible is not known. METHODS: We compared the effects of antagonists of L-type, N-type, and P/Q-type neuronal calcium channels on the depolarization-dependent influx of calcium-45 in cultured carcinoma cells. Serum samples from patients with various disorders were tested for reactivity with P/Q-type channels solubilized from carcinoma and cerebellar membranes and N-type channels from cerebral cortex. RESULTS: P/Q-type calcium-channel antagonists were the most potent inhibitors of depolarization-induced 45Ca influx in cultured small-cell carcinoma cell lines. Anti-P/Q-type calcium-channel antibodies were found in serum from all 32 patients with Lambert-Eaton syndrome and a diagnosis of cancer and in 91 percent of the 33 patients with Lambert-Eaton syndrome without cancer. Anti-N-type calcium-channel antibodies were found in 49 percent of the 65 patients with the Lambert-Eaton Syndrome. Lower titers of anti-P/Q-type and anti-N-type calcium-channel antibodies were found in 54 percent of 70 patients with a paraneoplastic encephalomyeloneuropathic complication of lung, ovarian, or breast carcinoma, 24 percent of 90 patients with cancer but no evident neurologic complications, 23 percent of 78 patients with sporadic amyotrophic lateral sclerosis, and less than 3 percent of 69 patients with myasthenia gravis, epilepsy, or scleroderma. CONCLUSIONS: The high frequency of P/Q-type calcium-channel antibodies found in patients with Lambert-Eaton syndrome implies that antibodies of this specificity have a role in the presynaptic pathophysiology of this disorder.
Paraneoplastic syndromes supplement the complex clinical picture of malignomas. Their knowledge improves early diagnosis. The development, course and regression of paraneoplastic syndromes indicates that they have a multifactorial origin and their manifestation depends on the biochemical, metabolic and immunological situation of the organism. The pathogenesis involves many obscure points and the clinical picture is often a surprise, an unexpected variant with regard to theoretical expectations. Sometimes paraneoplastic syndromes prove fatal in patients with a tretable tumour. There does not exist any paraneoplastic syndrome specific for a certain malignoma. In the authors' group of 1538 patients with malignant tumours belonging into the competence of internal medicine a paraneoplastic syndrome was found in 282 (18.3% patients). In 144 (9.4%) it was the first symptom of disease, incl. 74 (4.8%) where it was also an early sign of the disease. In the group thrombohaemorrhagic and rheumatological paraneoplastic syndrome was most frequently encountered. The relatively highest incidence of early paraneoplasias was observed in those malignomas which affect the immunocompetent apparatus (immunocytomas, haemoblastoses, and lymphomas). The authors recommend that suspect paraneoplastic syndromes should be dispensarized as precanceroses.
Paraneoplastic syndromes are common complications of lung cancer. Although most frequently associated with advanced disease, paraneoplastic syndromes may also occur at early stages. Occasionally, the paraneoplastic syndrome may be the presenting symptom of lung cancer. For most paraneoplastic syndromes, the best treatment is to treat the underlying malignancy. However, in many cases, treatment of moderate efficacy or urgent therapy is required. Specific recommendations for the management of the most common paraneoplastic syndromes, including cachexia, hypercalcemia, and hyponatremia, are provided.
Paraneoplastic syndromes are caused by factors produced by cancer cells that often act at a site distant from both the primary site and its metastases. These syndromes are estimated to occur in only 7% to 15% of patients with cancer and are diagnoses of exclusion. If the definition of paraneoplastic syndrome is broadened to include indirect effects of the tumor such as cachexia or the anemia of chronic disease, the incidence is much higher. Lung cancer, particularly small cell lung cancer, is the most common malignancy causing paraneoplastic syndromes. This review focuses on recently published literature on paraneoplastic syndromes associated with lung cancer, including humoral hypercalcemia of malignancy, autoimmune paraneoplastic neurologic syndromes, neuromuscular disorders, and cancer cachexia. It includes advances in both molecular biology and immunology, and in clinical investigation.
The importance of paraneoplastic syndromes is often underestimated in the horse. Clinically, paraneoplastic syndromes can cause greater morbidity than the actual physical presence of the malignant tumor. The appearance may be the first sign of a malignancy and may be so severe that appropriate therapy for the underlying cancer is not initiated. This article reviews some of the most common paraneoplastic syndromes that are likely to occur in the horse.
Paraneoplastic syndromes can affect virtually any portion of the nervous system. Most paraneoplastic syndromes are believed to be caused by an autoimmune reaction to an "onconeural" antigen shared by the cancer and the nervous system. The immune reaction may retard growth of the cancer, but it also damages the nervous system. Specific autoantibodies found in some individual paraneoplastic syndromes are usually associated with specific tumors. Neurological disorders, clinically and pathologically identical to paraneoplastic syndromes, may occur in some patients without cancer, but paraneoplastic antibodies are not found in these patients. The diagnosis of a paraneoplastic syndrome is based on its increased incidence in patients with cancer, the occasional response of the neurological syndrome to treatment of the underlying cancer, or the presence of specific autoantibodies. Some paraneoplastic syndromes respond to treatment of the underlying cancer or to immunosuppression but, for most syndromes, no effective treatment exists.
BACKGROUND/AIMS: Hepatocellular carcinoma patients with paraneoplastic syndromes usually have a larger tumor volume and a higher serum alpha-fetoprotein. Whether the presence of paraneoplastic syndromes, such as hypercholesterolemia, hypoglycemia, hypercalcemia, and erythrocytosis in hepatocellular carcinoma patients is a significant prognostic factor in patients' survival is of clinical interest. METHODOLOGY: We evaluated the occurrence of paraneoplastic syndromes in 903 hepatocellular carcinoma patients; 179 (20%) patients developed paraneoplastic syndromes either upon diagnosis or during follow-up, 179 age-sex-tumor volume-matched hepatocellular carcinoma patients without paraneoplastic syndromes were selected as controls. Serum liver biochemistry, alpha-fetoprotein, clinical Child-Pugh's score, tumor image studies, histological features, accessibility to treatment, and survival were recorded and compared between patients with and without paraneoplastic syndromes. Prognostic factors in patients' survival were analyzed. The clinical course and survival of hepatocellular carcinoma patients with different paraneoplastic syndromes were also compared. RESULTS: Multivariate Cox regression analysis revealed Child-Pugh's grade C, ineligibility for active treatment, serum alpha-fetoprotein > 10,000 ng/mL, main portal vein tumor thrombosis, and the presence of paraneoplastic syndromes were all independent unfavorable prognostic factors for survival. The median survival from the occurrence of paraneoplastic manifestation to death was only 36 days. Patients with different paraneoplastic syndromes had similar clinical characteristics in Child-Pugh's score, serum alpha-fetoprotein level, tumor volume, prevalence of main portal vein tumor thrombosis, and accessibility for active treatment upon diagnosis. However, erythrocytosis and hypercholesterolemia usually developed earlier in the clinical course, whilst hypoglycemia and hypercalcemia were usually terminal events. Hepatocellular carcinoma patients with erythrocytosis tended to have a longer survival than patients with other paraneoplastic syndromes. CONCLUSIONS: The presence of paraneoplastic syndromes in hepatocellular carcinoma patients is an unfavorable prognostic factor, excluding the occurrence of erythrocytosis.
Paraneoplastic syndromes are caused by factors produced by cancer cells that often act at a distance from both the primary site and its metastases. The most extensively characterized syndromes caused by cancer are those produced by polypeptide hormones, such as adrenocorticotropic hormone, and those produced by antibodies directed against tumor antigens that cross-react with neural tissue, such as in the Eaton-Lambert myasthenic syndrome. These syndromes develop in a minority of cancer patients, and are diagnoses of exclusion. Lung cancer, particularly small cell lung cancer, is the most common malignancy causing paraneoplastic syndromes. A large number of paraneoplastic syndromes have been described. This review focuses on the increased understanding of some of the well-documented syndromes that has occurred through recent advances principally in molecular biology and immunology.