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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↗

PubMedDate not supplied

[Lambert-Eaton myasthenic syndrome--a rare manifestation of paraneoplastic syndrome in ovarian cancer--case report].

INTRODUCTION: Paraneoplastic neurological syndromes are rare non-metastatic complications of cancer that have an immune-mediated etiology. The Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disorder, often associated with small cell lung carcinoma (SCLC), which is characterized by reduced quantal release of acetylcholine from the motor nerve terminals. LAMBERT-EATON MYASTHENIC SYNDROME: The Lambert-Eaton Myasthenic Syndrome (LEMS) is characterized by proximal muscle weakness initially affecting gait, autonomic symptoms (dry mouth, constipation, erectile failure) and augmentation of strength during initial voluntary activation. Symptomatic treatment of the junctional disorder is based on cholinergic drugs, immunosuppression, immunomodulation and physical therapy useful in case of unsuccessful antineoplastic therapy. CASE REPORT: A rare case of ovarian cancer with Eaton-Lambert syndrome is reported. A 50-year-old woman was admitted to the gynecologic department, complaining of weakness and pain in her arms and shoulders. Physical therapy resulted in partial improvement. Treatment of paraneoplastic syndrome markedly improves the quality of life of cancer patients. Patients presenting with this syndrome should undergo a careful evaluation for the presence of an occult malignancy.

Female↗

PubMed2003

[Paraneoplastic syndromes in ENT surgery].

Paraneoplastic syndromes are extremely rare in otolaryngology.However, they can significantly influence both the therapy and outcome of patients with oncologic disease. This report deals with two cases of paraneoplastic syndromes associated with malignant tumors of the head and neck region. A 45-year-old male presented with a polymyalgia rheumatica, which was identified as a synchronous feature of a hypopharyngeal carcinoma. The symptoms disappeared after successful surgical treatment of the carcinoma. No analogous report has been published as yet. As demonstrated in a second case of a 54-year-old male, leukocytosis can be paraneoplastically associated with a squamous cell carcinoma of the tongue. The international literature is reviewed in order to analyze the incidence of different paraneoplastic syndromes related to malignancies of the upper aerodigestive tract including the rare neuroendocrine oat cell carcinoma of the larynx.

Carcinoma, Squamous Cell↗

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↗

PubMed2004

Bazex syndrome (paraneoplastic acrokeratosis).

Bazex syndrome (paraneoplastic acrokeratosis) is characterized by the presence of hyperkeratotic lesions on the nose, ears, palms, and soles that appear in association with malignancies of the upper aerodigestive tract, most often a squamous cell carcinoma. We present a case of Bazex syndrome and provide a review of the literature.

Acrodermatitis↗

PubMed1998

Neurologic paraneoplastic syndromes.

Several neurologic paraneoplastic disorders are believed to be caused by an autoimmune reaction against antigen(s) co-expressed by tumour cells and neurons. Of the paraneoplastic syndromes, the evidence for an autoimmune etiology is strongest for the Lambert-Eaton myasthenic syndrome, in which autoantibodies downregulate voltage-gated calcium channels at the presynaptic nerve terminal. For other syndromes, including cerebellar degeneration, multifocal encephalomyelitis, sensory neuronopathy, limbic encephalitis, opsoclonus-myoclonus, stiff person syndrome, and retinal degeneration, the autoimmune theory is supported by the presence of specific antineuronal antibodies. These antibodies serve as a useful diagnostic tool, but their actual role in causing neuronal injury and clinical disease remains unclear. Further understanding of immunopathogenesis awaits successful experimental models. Among different syndromes, a varied proportion of patients shows neurologic improvement with immunosuppressive treatments; it is likely that many patients have already suffered irreversible neuronal injury at the time of diagnosis.

Animals↗

PubMedDate not supplied

Cutaneous paraneoplastic syndromes: uncommon presentations.

Paraneoplastic syndromes are a group of clinical manifestations associated with a malignancy, but not directly related to the primary tumor itself or to its metastases. Characteristically, they follow a course parallel to the tumor, resolve with successful treatment of the primary tumor, and tend to recur with its relapse or the onset of metastases. The mechanism by which they occur is not well understood, but may be related to the production of bioactive substances by or in response to the tumor, such as polypeptide hormones, hormone-like peptides, antibodies or immune complexes, cytokines, or growth factors.

Biopsy, Needle↗

PubMed1997

[Paraneoplastic syndrome].

Treatment for the paraneoplastic syndrome associated with lung cancer was reviewed. The principle of the treatment of paraneoplastic syndrome is to control cancer as an underlying disease. Therefore, the standard therapy for Cushing's syndrome associated with lung cancer is surgical treatment if the tumor is operable. There is no standard therapy for Cushing's syndrome associated with advanced small-cell lung cancer. Metyrapone is used in combination with systemic chemotherapy. The effects of ketoconazole and octreotide are under investigation. To control hyponatremia due to the syndrome of inappropriate antidiuretic hormone secretion, fluid restriction is standard. When hyponatremia cannot be controlled with fluid restriction, demeclocycline can be used. For life-threatening hyponatremia, hypertonic saline with intravenous furosemide is administered under careful monitoring. Followed by hydration with saline, pamidronate is effective for the control of symptomatic hypercalcemia. Combined use of calcitonin facilitates rapid normalization of serum calcium for critically ill cases. Heparin is used for patients with recurrent episodes of thrombosis resulting from chronic disseminated intravascular coagulation, although the efficacy is controversial. Thrombocytes and coagulation factors are combined with heparin for patients with uncontrollable bleeding, although the efficacy is not established.

Alendronate↗

PubMed2007

Paraneoplastic syndromes in patients with primary head and neck cancer.

Paraneoplastic syndromes represent the clinical manifestations of the indirect and remote effects produced by tumor metabolites or other products. The clinical spectrum of the various paraneoplastic syndromes related to primary malignancies of the head and neck region is presented. A review of the literature on paraneoplastic syndromes in patients with primary head and neck cancer was carried out. Paraneoplastic syndromes related to primary malignancies of the head and neck region can be categorized as: endocrine, cutaneous or dermatologic, hematologic, neurologic, osteoarticular or rheumatologic, ocular syndromes. Sometimes, paraneoplastic syndromes can be more serious than the consequences of the primary tumor itself and can precede, follow or be concurrent to the diagnosis of a malignancy; moreover, they can dominate the clinical picture and thus lead to errors with respect to the origin and type of the primary tumor. Physicians who deal with cancer-associated syndromes should be able to differentiate the paraneoplastic syndromes from the benign disorders that mimic them. Patients with a suspected paraneoplastic disorder should undergo a complete panel of laboratory studies, in addition to imaging studies and endoscopy. Identification of paraneoplastic syndromes allow the clinician to make an early diagnosis and to provide adequate treatment of tumors, with a favorable oncologic outcome and improved life expectancy for the patient. These syndromes can follow the clinical course of the tumor and thus be useful for monitoring its evolution.

Chorionic Gonadotropin, beta Subunit, Human↗

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↗

PubMed2000

Paraneoplastic syndromes of the nervous system.

Paraneoplastic syndromes affecting the nervous system are unique among immune-mediated disorders in that the trigger of the immune response is known: tumor expression of proteins normally restricted to neurons (or other immunoprivileged sites, such as testis) but ectopically expressed in some cancers results in an immunological response characterized by high titers of antibodies targeting the "onconeuronal" antigen. A T-cell response is also elicited in some paraneoplastic syndromes and may be the cause of neuronal destruction. In some instances genes that code for the antigens recognized by the autoantibodies have been identified, cloned and sequenced. Some of the proteins so identified are RNA binding proteins but their specific function has not been identified. In some individuals with cancer but no paraneoplastic syndrome, low titers of antibody can be identified in the serum. Low titers of antibody are associated with a better prognosis of the cancer. Experimental animals immunized against a paraneoplastic antigen are partially protected against tumors that express that antigen.

Animals↗

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↗

PubMed1976

[The paraneoplastic syndrome].

The notion of the paraneoplastic syndrome contains a great number of signs which are not to be explained by the local growth of the tumour. The disturbances may express themselves in form of fever, anorexia and cachexia, or they may also affect selectively determined organs. As a cause for these tumour-associated changes, apart from unspecific and specific metabolic functions of the tumour cell, also the sequelae of immunological reactions between tumour and host organism are made responsible. The metabolic remote action of malignant neoformations proved as clearly manifest above all in the paraneoplastic endocrinopathies. Though most of the paraneoplastic syndromes are not strongly specific for the tumour their knowledge may be significant in the diagnostics and control of the course of neoplasias. This is also the case in emergency situations which can be evoked by such pathological processes. The most important paraneoplastic syndromes and problems of their pathogenesis are explained.

Anorexia↗

PubMed1998

Peripheral neurological paraneoplastic syndromes.

Peripheral neurological paraneoplastic syndromes include some of the most convincingly demonstrated autoimmune disorders. Though rare, they are an important cause of neurological morbidity and mortality, often signaling the presence of an occult tumor. Covered under this heading are disorders of peripheral nerves and nerve roots (neuropathies and radiculopathies), the neuromuscular junction (myasthenic syndromes) and muscle (myopathic syndromes). Electrophysiological studies, laboratory screening for circulating autoantibodies, cerebrospinal fluid analysis and muscle or sensory nerve biopsies in selected cases aid the clinician in making the neurological diagnosis. Discrete components of the nervous system such as the neuromuscular junction's calcium channels (Lambert-Eaton myasthenic syndrome) or acetylcholine receptors (myasthenia gravis) are the targets of immunoaggression. Treatment directed at the tumor and the demonstrable or presumed immunological pathophysiology, assisted by supportive medical care and symptomatic drug therapies, offers the best chance for clinical improvement.

Journal Article↗

PubMed1999

The neuropathology of paraneoplastic syndromes.

The term "paraneoplastic neurological syndromes" encompasses a number of uncommon disorders associated with systemic malignancies. In order to be classified a paraneoplastic neurological syndrome, the malignancies must not invade, compress, or metastasize to the nervous system. They can either focally or diffusely involve the central and peripheral nervous system or the neuromuscular junction. This paper reviews the neuropathology of the syndrome. It will first describe the clinical presentation and give an account of the systemic tumors most commonly associated with the various types of disorders. Then it will review the general pathological features that consist of an inflammatory process predominantly affecting the gray matter. Finally, it will describe in detail the main clinico-pathological types, including 1) encephalomyelitis, 2) cortical cerebellar degeneration, 3) peripheral neuropathy, 4) opsoclonus-myoclonus and 5) retinopathy. The Lambert-Eaton myasthenic syndrome will be dealt with separately in another paper in this symposium.

Autoantibodies↗

PubMed1991

Occurrence of hypercalcemia and leukocytosis with cachexia in a human squamous cell carcinoma of the maxilla in athymic nude mice: a novel experimental model of three concomitant paraneoplastic syndromes.

Hypercalcemia and leukocytosis may occur in conjunction as paraneoplastic syndromes associated with malignant disease. Here we describe a human squamous cell carcinoma of the maxilla that was associated with hypercalcemia and leukocytosis, and also cachexia. The primary tumor was surgically removed and established in permanent cell culture. When either primary tumors or cultured tumor cells were inoculated into nude mice, the nude mice developed the same paraneoplastic syndromes as those which occurred in the patient from whom the tumor was originally derived. The plasma calcium was increased two and one-half-fold and the WBC count 30-fold, and the body weight was decreased by 45% in tumor-bearing animals. Each of these paraneoplastic syndromes was alleviated by surgical excision of the tumor, indicating that the paraneoplastic syndromes were due to a factor or factors produced by the primary tumor. The development of each of these paraneoplastic syndromes in nude mice correlated positively with the other two syndromes. We examined the organs of tumor-bearing mice and found striking histopathologic abnormalities in the bones, spleen, and liver, but no infiltration with tumor cells. The bones showed marked evidence of osteoclastic bone resorption. This model of a human tumor associated with the hypercalcemia-leukocytosis paraneoplastic syndrome, together with cachexia, should make it possible to determine the mechanisms responsible for these paraneoplastic syndromes and their relationship to each other.

Animals↗

PubMed2002

Paraneoplastic syndromes associated with bronchogenic carcinoma.

Paraneoplastic syndromes associated with lung cancer are diverse in their presentation, pathophysiology, and implications. They can be seen as a diagnostic and therapeutic challenge or as an opportunity to detect an otherwise asymptomatic malignancy. Unraveling the mechanisms that produce these syndromes will lead to insight into tumor biology that will be translated into novel approaches for early detection and therapy.

Carcinoma, Bronchogenic↗