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PubMed1992

Nervous system dysfunction in children with paraneoplastic syndromes.

Paraneoplastic syndromes are complexes of symptoms and signs that occur in association with cancer and that are unexplained by the known anatomic and physiologic characteristics of the tumor. Many of these syndromes are neurologic in nature or have consequences for the central or peripheral nervous system. These syndromes have been well characterized in adults. With the exception of opsoclonus-myoclonus, little has been written about the occurrence of such syndromes in children. This review looks at published reports of paraneoplastic syndromes in children and concludes that paraneoplastic syndromes in childhood differ from those seen in adulthood because of differences in both the host and the kinds of neoplasms most prevalent in each age group. Paraneoplastic syndromes may be underreported in childhood because of the difficulty in eliciting specific neurologic complaints from children and because a thorough neurologic examination is often not undertaken as a matter of routine.

Child↗

PubMed1988

Bilateral Baker's cyst as the presenting symptom of paraneoplastic syndrome.

Paraneoplastic syndrome is defined as a systemic malignancy producing prostaglandins or other substances that lead to various manifestations, syndromes or diseases. In the following report we present a case of a young patient complaining of bilateral Baker's cysts who ultimately was diagnosed as suffering from gastric lymphoma. Following subtotal gastrectomy the Baker's cysts disappeared with no specific treatment.

Adult↗

PubMed1996

[Optimal fixation for the detection of anti-neuronal antibody by immunohistochemistry in the paraneoplastic syndrome].

Paraneoplastic syndrome(PS) associated anti-neuronal autoantibodies are characterized by their antigen molecular weight determined by Western blot and immunostaining pattern recognized through immunohistochemistry. We investigated immunohistochemical fixatives for their sensitivity in the detection of anti-neuronal nuclear antibody(Hu). Serum used for this study was taken from a patient with anti-Hu antibody seropositivity, which was ascertained by recombinant Hu protein. Western blot analysis produced 37kDa band. We examined six fixative conditions: immersion fixed with acetone, Bouin's solution, Sakura rapid fixative("Ufix'), and perfusion fixed with 2%, 4%, 8% paraformaldehyde (PFA) on the basis of each immunoreactivity in a rat cerebellum, brain stem and liver. The optimal fixation for detecting anti-Hu antibody was perfusion fixed with 2% PFA, that reacted conspicuously with nucleus but not nucleolus of neurons. The perfusion method proved superior to immersion in immunostaining intensity. With immersion fixation, specific immunostaining pattern lessened and differentiation from cytoplasm decreased. With various concentrations of PFA, immuno-reactivity with nucleus at 2% PFA was similar to 4%, although serum optimal dilution at 2% was slightly greater than 4%. The variety of staining patterns of anti-Hu antibody is closely related to the vulnerability of neuronal antigens to the fixatives. The detection of anti-neuronal antibodies is important for early diagnosis and treatment of occult tumors. The immunostaining method is a useful and sensitive way to research these antibodies. Therefore, it is essential to consider the selection of fixation and the preservation of the antigenicity in evaluating immunohistochemical hallmarks.

Animals↗

PubMed1997

Mucocutaneous paraneoplastic syndromes.

Mucocutaneous paraneoplastic syndromes represent a group of dermatoses of variable morphology, pathology, and etiology that can occur as idiopathic conditions or in association with a visceral malignancy. These conditions can be categorized with respect to their predominant pathologic change: dermal "depositions," neutrophilic dermatoses, papulosquamous disorders, proliferative reactions, reactive erythemas, vacuolar degeneration of the basal layer, vasculitis, and vesiculobullous disorders. Some of these dermatoses occur more frequently in patients with hematologic malignancies whereas others are more prevalent in patients with solid tumors. The major clinical characteristics and commonly associated malignancies in patients with mucocutaneous paraneoplastic syndromes are reviewed. Suggested workups to evaluate for cancer in patients with these dermatoses are summarized. The appearance of a mucocutaneous paraneoplastic syndrome can either precede, occur concurrently with, or follow the detection of an associated neoplastic process. Therefore, the dermatosis can be the presenting feature of a previously unsuspected neoplasm or the earliest sign of recurrent cancer in an oncology patient. When the possibility of a mucocutaneous paraneoplastic syndrome is entertained, the diagnosis of the dermatosis should be confirmed either based on the clinical morphology of the lesions, the pathologic changes observed after lesional biopsy, or both. Once the diagnosis of a malignancy-associated dermatosis has been established, either an appropriate evaluation for an asymptomatic neoplasm in a cancer-free individual or an investigation for recurrence of malignancy in an oncology patient can be initiated.

Hematologic Neoplasms↗

PubMed1996

Paraneoplastic syndromes.

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.

Humans↗

PubMed1994

Nephrotic syndrome as an unusual paraneoplastic syndrome of Hodgkin's disease demonstrated on gallium-67 scan.

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.

Adolescent↗

PubMed1994

Paraneoplastic syndromes.

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.

Adult↗

PubMed1994

Cutaneous paraneoplastic syndromes.

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.

Humans↗

PubMed1995

Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes.

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.

Autoantibodies↗

PubMed1990

[Paraneoplastic syndromes in internal medicine].

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.

Adult↗

PubMed1992

Paraneoplastic syndromes in lung cancer.

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.

Cachexia↗

PubMed1997

Paraneoplastic syndromes affecting the nervous system.

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.

Autoantibodies↗

PubMed1991

Paraneoplastic syndromes in thoracic malignancies.

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.

Humans↗

PubMed1976

[Stauffer syndrome, paraneoplastic hepatic dysfunction syndrome associated with renal cell carcinoma (author's transl)].

In two patients with renal cell carcinoma, the following biochemical changes were found independently of hepatic metastases: increased alkaline phosphatase activity, rise in bromsulfothalein retention, hypoalbuminemia, increase in alpha2-globulin, and prolonged prothrombin time (Stauffer syndrome). In both cases the syndrome was found to be the first sign of the renal cell carcinoma. In one patient liver function returned to normal after removal of the neoplasm, in correlation with the good recovery. In the other case the abnormal laboratory findings persisted after total renal surgery. Clinically, diffuse pulmonary metastases occurred. Both case histories show the high significance in knowing Stauffer syndrome and its value for early diagnosis and operative success.

Adenocarcinoma↗

PubMed1988

Opsoclonus, myoclonus, ataxia, and encephalopathy in adults with cancer: a distinct paraneoplastic syndrome.

The clinical and pathological findings in 4 adults with cancer and opsoclonus were compared with those of 15 other patients described elsewhere. The clinical syndrome of paraneoplastic opsoclonus is characterized by the acute onset of opsoclonus and truncal ataxia, often accompanied by encephalopathy, myoclonus and a cerebrospinal fluid pleocytosis. Unlike most other paraneoplastic syndromes, the course is often remitting and relapsing. Neuropathological examination in 3 of our patients showed lymphocytic cuffing of occasional blood vessels throughout the central nervous system, associated with a mild, diffuse proliferation of microglia in 1 patient. Apart from a mild, patchy loss of Purkinje cells in 1 patient, there was no loss of neurons from the cerebellum, brainstem, cerebral hemispheres, or spinal cord. These patients differ from those with the more common paraneoplastic cerebellar degeneration by the predominance of truncal over limb ataxia, the presence of myoclonus, the absence of severe dysarthria, a tendency for remission, and the preservation of Purkinje cells.

Ataxia↗

PubMed1991

[Paraneoplastic syndromes in urology].

The paraneoplastic syndromes in patients with malignant urological tumors are reviewed herein. Some general aspects of these syndromes are briefly described and discussed. Similarly, the different urological tumors in which paraneoplastic syndromes have been observed are briefly reviewed. These are very common in renal cancer and cancer of the prostate, uncommon in bladder cancer and rare in testicular cancer.

Female↗

PubMed1993

[Paraneoplastic syndromes (2)].

Although not rare, paraneoplastic syndromes develop in a minority of cancer patients. Their exact frequency seems to be under estimated. These syndromes are hereby described. Their clinical usefulness is of great importance: they can be the first sign of a malignancy and can be used as tumor markers. The diagnosis of paraneoplastic syndromes is often difficult. A "diagnosis score" is proposed to establish this diagnosis easier.

Amyloidosis↗

PubMed1993

[Paraneoplastic syndromes (1)].

Although not rare, paraneoplastic syndromes develop in a minority of cancer patients. Their exact frequency seems to be underestimated. These syndromes are hereby described. Their clinical usefulness is of great importance: they can be the first sign of a malignancy and can be used as tumor markers. The diagnosis of paraneoplastic syndromes is often difficult. A "diagnosis score" is proposed in order to make the diagnosis easier.

Carcinoid Heart Disease↗