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[The incidence and clinical significance of paraneoplastic syndromes in patients with hepatocellular carcinoma].

BACKGROUND/AIMS: Patients with hepatocellular carcinoma (HCC) may manifest paraneoplastic syndromes such as hypercholesterolemia, hypoglycemia, hypercalcemia and erythrocytosis. This study was aimed at evaluating the incidence and clinical significance of paraneoplastic syndromes in Korean HCC patients. METHODS: The medical records of 165 HCC patients who were diagnosed and died in the Kyung Hee University Hospital, were reviewed retrospectively. The following variables were analyzed: age, gender, hepatitis markers, platelet, liver function test, alpha-fetoprotein (AFP), Child-Pugh score, tumor features, and the duration of their survival. RESULTS: In total, paraneoplastic syndromes were presented in 43.6% of the HCC patients during the course of their disease. Hypercholesterolemia was solely presented in 14.5%, hypoglycemia in 12.7% and hypercalcemia in 7.8%. The patients who presented with more than 2 syndromes were 8.5%. While 80% of erythrocytosis (4/5) and 51.6% of hypercholesterolemia (16/31) was presented at the time of HCC diagnosis, hypoglycemia and hypercalcemia mainly occurred as terminal events. The patients with paraneoplastic syndromes were younger and had higher rates of portal vein thrombosis, bi-lobar tumor involvement and tumor more of more than 10 cm in diameter, compared to those patients without them. The proportion of patients with a serum AFP more than 400 ng/mL tended to be higher in the patients with paraneoplastic syndromes. The HCC patients with paraneoplastic syndromes, except for erythrocytosis, had a shorter survival than those patients without them. CONCLUSIONS: Paraneoplastic syndromes are not infrequently presented in HCC patients, especially at an advanced stage, and the survival of these patients is relatively shorter.

Aged↗

FDG-PET imaging in patients with paraneoplastic syndromes and suspected small cell lung cancer.

Paraneoplastic syndromes may be the presenting clinical manifestation of small cell lung cancer. In some cases, however, confirming the diagnosis can be difficult because findings on conventional imaging studies can be subtle or nonspecific. This study examined the utility of fluoro-2-deoxy-glucose positron emission tomography (FDG-PET) in identifying clinically suspected small cell lung cancer in patients with paraneoplastic syndromes. FDG-PET appears to be very useful in localizing suspected small cell lung cancer in patients presenting with paraneoplastic syndromes.

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Paraneoplastic syndromes: a challenge for neuroscience nursing.

Neurologic paraneoplastic syndromes are medical and nursing challenges. Although neurologic paraneoplastic syndromes are relatively rare in the total cancer population, they may be more commonly seen in the acute neurologic setting. O'Neill et al estimate that 60% of persons with Eaton-Lambert syndrome and 50% of persons with subacute cerebellar degeneration have an underlying cancer. The slow and subtle development of neurologic paraneoplastic disorders underscores the need for careful evaluation of persons with neurologic deficits. Once the diagnosis is made, nursing care must be based on the problems associated with paraneoplastic manifestations of the disease, as well as effects of the cancer and its treatment.

Aged↗

Paraneoplastic syndromes affecting the central nervous system.

Paraneoplastic syndromes affecting the nervous system are rare neurologic syndromes caused by cancer but not ascribable to metastases. Any portion of the nervous system may be involved in a paraneoplastic syndrome. The pathogenesis of these disorders appears to be an immune reaction against antigens shared by the cancer and the nervous system. Some disorders, such as the Lambert-Eaton myasthenic syndrome, are effectively treated by removal of autoantibodies directed against the presynaptic cholinergic synapse. In other disorders, such as paraneoplastic cerebellar degeneration or paraneoplastic sensory neuronopathy, neither removal of the autoantibody nor treatment of the cancer is effective.

Autoantibodies↗

Paraneoplastic syndromes in patients with hepatocellular carcinoma in Taiwan.

BACKGROUND: Hepatocellular carcinoma (HCC) is the most common malignancy in Taiwan. Some patients may manifest paraneoplastic syndromes during the clinical course of the disease. In this study, the authors evaluated the clinical significance of these paraneoplastic syndromes, compared the prevalence of these syndromes between cases of hepatitis B virus (HBV)-related and hepatitis C virus (HCV)-related HCC, and estimated significant predictors associated with the syndromes. METHODS: Clinical data on 1197 HCC patients, including age, gender, Child-Pugh score, survival time, laboratory data (including liver biochemistry, hepatitis markers, and serum alpha-fetoprotein [AFP]), and tumor features (including tumor size, portal vein thrombosis, and histologic pictures), were retrospectively reviewed. RESULTS: A total of 232 of 1197 patients (19.4%) had paraneoplastic syndromes. HCC patients with paraneoplastic syndromes had significantly higher serum AFP; higher rates of initial main portal vein thrombosis, metastasis, and bilobal tumor involvement; larger tumor volume; and shorter survival than those without these syndromes. Patients with HBV-related HCC had a significantly higher prevalence of paraneoplastic syndromes than patients with HCV-related HCC (20.1% vs. 11.2%, P = 0.005). In a stepwise multivariate logistic regression analysis, AFP >50,000 ng/mL and tumor volume >30% were significant predictive variables associated with the presence of paraneoplastic syndromes in HCC patients. CONCLUSIONS: HCC patients with paraneoplastic syndromes usually had higher levels of serum AFP and larger tumor volumes than those without. Patients with HBV-related HCC had a significantly higher prevalence of paraneoplastic syndromes than those with HCV-related HCC.

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Hodgkin's disease associated with neurologic paraneoplastic syndrome.

In this case of neurologic paraneoplastic syndrome in a patient with Hodgkin's disease, both the Hodgkin's disease and the associated neurologic syndrome were cured by surgical intervention and postoperative adjuvant chemotherapy. The patient's tumor was removed en bloc. Postoperative chemotherapy consisted of doxorubicin (Adriamycin), etoposide, and vinblastine. At 5-year follow-up in August 1996, the patient was active and tumor-free, without evidence of recurrent Hodgkin's disease or paraneoplastic syndrome. The possibility that this patient's syndrome was the result of a cross-reacting monoclonal idiotype produced by his tumor is suggested by the rapid recovery associated with tumor debulking. The role of surgery in the treatment of patients with Hodgkin's disease is limited, well defined, and even more infrequent in recurrent disease. Systemic chemotherapy is the treatment of choice for these patients, but surgery may play an important role in the initial cytoreduction of the recurrent tumor, especially in cases of localized bulky disease.

Adult↗

[Paraneoplastic syndromes--the endocrine system and the lungs].

By the term "paraneoplastic syndrome" is meant a constellation of symptoms and findings that can be traced back to a tumor, but which are not caused by local or metastatic mechanisms. Such paraneoplastic syndromes can suggest the diagnosis of a tumor, or serve to chart the course of known tumor disease. When the pathophysiology is known, the mediators (hormones) may replace the clinical syndrome as useful parameters. With respect to the pathobiology of tumors, an analysis of paraneoplastic syndromes would seem to be highly promising, and might possibly form the basis for the development of new therapeutic concepts.

Biomarkers, Tumor↗

Neuro-ophthalmology and paraneoplastic syndromes.

PURPOSE OF REVIEW: To describe the neuro-ophthalmological manifestations of paraneoplastic syndromes and their immunological associations. RECENT FINDINGS: Neuro-ophthalmological signs and symptoms are usually present in paraneoplastic syndromes of the central nervous system. Unlike opsoclonus, less characteristic eye movement abnormalities are difficult to recognize as presenting symptoms of paraneoplastic syndromes. In this setting, the detection of several antibodies, including anti-Hu, Yo, Ma2, Ri, Tr, CV2/CRMP5 or voltage-gated calcium channel antibodies may help to establish that the neuro-ophthalmological disorder is paraneoplastic. Among the recently characterized antibodies, those against the Ma proteins often associate with brainstem encephalitis and vertical gaze paralysis. A small subset of patients with opsoclonus and ataxia harbor anti-Ri antibodies. In other patients, there is preliminary evidence that the autoantigens of opsoclonus reside in the postsynaptic density, but no dominant antibody marker has been identified. Uveitis and optic neuritis are rare accompaniments of paraneoplastic encephalomyelitis; some of these patients harbor anti-CV2/CRMP5 in association with other antibodies. Studies on paraneoplastic retinopathy indicate that immunity to retinal proteins other than recoverin can result in a similar syndrome to that associated with recoverin antibodies, and that melanoma-associated retinopathy may associate with several retinal antibodies. SUMMARY: There is increasing recognition of an extensive variety of neuro-ophthalmological abnormalities as manifestations of paraneoplastic syndromes and of several antineuronal antibodies as clinical markers of these disorders. Basic immunological studies support the pathogenic role of some of these antibodies, and are elucidating the pathogenic mechanisms that underlie these and other antibody-associated paraneoplastic syndromes.

Autoantibodies↗

[Metabolic disturbance as paraneoplastic syndrome].

Metabolic disturbances of Na, K, Ca and glucose as paraneoplastic syndrome were reviewed on the basis of recent progress of such areas. These abnormalities usually occur due to the production of hormones or other physiologically active substances by tumor tissues. Hyponatremia is the most common abnormality of Na metabolism in patients with cancers such as lung cancer, malignant lymphoma, thymoma and so on. Usual cause of hyponatremia as paraneoplastic syndrome is inadequate secretion of Antidiuretic Hormone (SIADH), which brings dilution hyponatremia associated with water intoxication. Recently hyponatremia due to abnormal secretion of atrial natriuretic peptide has been noted. Ca metabolism disturbance associated with cancer is usually observed as hypercalcemia and it is said that such hypercalcemia is seen in about 10% of cancer patients. Main cause of hypercalcemia associated with cancer is local osteolytic hypercalcemia (LOH) due to bone metastasis or humoral hypercalcemia of malignancy (HHM). The most common etiology of HHM is the production of Parathormone (PTH) related peptide (PTH-rP) massively secreted from cancer tissues. PTH-rP has been recently well investigated and its molecular, mRNA and gene structure have been already determined. The progress of this area is very rapid and PTH-rP will be assayed in the clinical laboratory in near future. As for glucose metabolism disturbance as paraneoplastic syndrome, hypoglycemia is the most common abnormality. This type of hypoglycemia has been noted in relation with excessive production of somatomedin.

Atrial Natriuretic Factor↗

Negative-antibody paraneoplastic syndrome complicating small cell carcinoma.

The neurological paraneoplastic syndromes represent nonmetastatic complications of cancer and may affect several levels of the nervous system. They are thought to be immunologically-mediated. The syndrome predates the diagnosis of cancer by months to years in two thirds of cases. We report the case of a female patient presenting with a cerebellar syndrome and a sensory neuronopathy on a background of severe weight loss. We searched for occult malignancy and later diagnosed her to be suffering from a paraneoplastic syndrome secondary to small cell carcinoma of the lung. Paraneoplastic antibodies were negative. She was subsequently treated with chemotherapy.

Antibodies, Neoplasm↗

Progressive overlap syndrome due to small cell lung cancer as a paraneoplastic syndrome.

The association between rheumatic diseases and malignancy has been documented in many studies. Polymyositis and especially dermatomyositis are associated with a higher risk of malignant disease. Furthermore, lung cancer usually develops in patients with a prolonged history of systemic sclerosis accompanied by pulmonary fibrosis. Though overlap syndrome is rarely reported in the associated cancer, this is the first report describing progressive overlap syndrome in small cell lung cancer as a paraneoplastic syndrome.

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Paraneoplastic syndromes: what we know and what we should know.

Paraneoplastic syndromes are a group of clinical disorders associated with malignant diseases that are not directly related to the physical effects of primary or metastatic tumor. Those that are associated with the secretion of circulating peptides may be used as a useful tool in monitoring patients after the start of chemotherapy. Most of the paraneoplastic syndromes represent an interesting tool of internal medicine. Moreover, they also represent a starting point for a work up in patients with an occult neoplasia, even though the clinical presentation may be very multifaceted. As consequence of all these considerations, the present editorial represents a useful tool for a rational approach to paraneoplastic syndromes.

Humans↗

Advanced heart block as a manifestation of a paraneoplastic syndrome from malignant thymoma.

Malignant thymoma is a rare tumor that is associated with paraneoplastic syndrome. Myocarditis as a paraneoplastic syndrome has been rarely described. Reported herein is a young male patient with malignant thymoma and myocarditis as part of a paraneoplastic syndrome. This resulted in high-degree heart block and an asystolic cardiac arrest despite placement of a permanent pacemaker.

Adolescent↗

Life-threatening paraneoplastic cutaneous syndromes.

Paraneoplastic syndromes are diseases or symptom complexes associated with malignancy, usually internal. In dermatology, we modify the definition to refer to dermatoses associated with internal malignancy. In this article, we discuss the link between malignancy and such dermatologic disorders as acanthosis nigricans, acrokeratosis paraneoplastica of Bazex, dermatomyositis, erythema gyratum repens, necrolytic migratory erythema (glucagonoma syndrome), and paraneoplastic pemphigus and discuss, where such information is known, the mechanism by which these paraneoplastic diseases occur.

Critical Illness↗