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Paraneoplastic Syndrome (paraneoplastic + syndrome)
Selected AbstractsHypothalamic dysfunction associated with neuroblastoma: Evidence for a new Paraneoplastic syndrome?PEDIATRIC BLOOD & CANCER, Issue 5 2003Nicolas Sirvent MD No abstract is available for this article. [source] Non-metastatic cholestatic paraneoplastic syndrome associated with soft tissue sarcomaASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 2 2009Amanjit BAL Paraneoplastic syndromes have been described in association with variety of malignant neoplasms. The non-metastatic cholestatic paraneoplastic syndrome first described as Stauffer's syndrome in association with renal cell carcinomas is also associated with other malignancies. We describe the autopsy findings of a patient with recurrent and metastatic leiomyosarcomas presenting with cholestatic liver dysfunction. The diagnosis requires the exclusion of all other possible causes of hepatitis and, where possible, resolution after the effective treatment of the underlying malignancy. [source] Hypocretin/orexin and narcolepsy: new basic and clinical insightsACTA PHYSIOLOGICA, Issue 3 2010S. Nishino Abstract Narcolepsy is a chronic sleep disorder, characterized by excessive daytime sleepiness (EDS), cataplexy, sleep paralysis and hypnagogic hallucinations. Both sporadic (95%) and familial (5%) forms of narcolepsy exist in humans. The major pathophysiology of human narcolepsy has been recently discovered based on the discovery of narcolepsy genes in animals; the genes involved in the pathology of the hypocretin/orexin ligand and its receptor. Mutations in hypocretin-related genes are rare in humans, but hypocretin ligand deficiency is found in a large majority of narcolepsy with cataplexy. Hypocretin ligand deficiency in human narcolepsy is probably due to the post-natal cell death of hypocretin neurones. Although a close association between human leucocyte antigen (HLA) and human narcolepsy with cataplexy suggests an involvement of autoimmune mechanisms, this has not yet been proved. Hypocretin deficiency is also found in symptomatic cases of narcolepsy and EDS with various neurological conditions, including immune-mediated neurological disorders, such as Guillain,Barre syndrome, MA2-positive paraneoplastic syndrome and neuromyelitis optica (NMO)-related disorder. The findings in symptomatic narcoleptic cases may have significant clinical relevance to the understanding of the mechanisms of hypocretin cell death and choice of treatment option. The discoveries in human cases lead to the establishment of the new diagnostic test of narcolepsy (i.e. low cerebrospinal fluid hypocretin-1 levels for ,narcolepsy with cataplexy' and ,narcolepsy due to medical condition'). As a large majority of human narcolepsy patients are ligand deficient, hypocretin replacement therapy may be a promising new therapeutic option, and animal experiments using gene therapy and cell transplantations are in progress. [source] Effect of parathyroid hormone-related protein on fibroblast proliferation and collagen metabolism in human skinEXPERIMENTAL DERMATOLOGY, Issue 4 2002Emanuela Maioli Abstract: The parathyroid hormone-related protein (PTHrp), structurally similar to the parathyroid hormone (PTH) in its NH2 -terminal part, was first identified as a tumour-derived peptide responsible for a paraneoplastic syndrome known as humoral hypercalcemia of malignancy. The PTHrp gene is expressed not only in cancer but also in normal tissues during adult and/or fetal life, where it plays predominantly paracrine and/or autocrine roles. In the skin PTHrp produced by keratinocytes acts on fibroblasts by complex cooperative circuits involving cytokines and growth factors. In this report, we studied the direct effects of synthetic PTHrp 1,40 on proliferation and collagen synthesis and matrix metalloproteinase-2 (MMP-2) activity in cultures of fibroblasts isolated from normal human skin. Fibroblasts exposure to varying doses of PTHrp for 48 h, significantly and dose-dependently inhibited proliferation evaluated by [3H]-thymidine incorporation into DNA. A dose-dependent stimulation of cAMP released into the medium was concomitantly observed. In contrast, PTHrp had no effect on collagen synthesis evaluated either by [3H]-proline incorporation or by radioimmunoassay (RIA) of the carboxyterminal fragment of type I procollagen (PICP). MMP-2 activity, evaluated by quantitative zymographic analysis, was significantly increased by PTHrp treatment at doses of 160 and 320 nM. These findings indicate that PTHrp may play a role in normal dermal physiology by controlling both fibroblast proliferation and extracellular matrix degradation. [source] Psychiatric disorder as a first manifestation of cancer: A 10-year population-based studyINTERNATIONAL JOURNAL OF CANCER, Issue 12 2009Michael E. Benros Abstract To investigate the possibility that psychiatric symptoms could be caused by a yet undetected cancer or be part of a paraneoplastic syndrome, nationwide population-based registers were linked including the Danish Psychiatric Central Register and the Danish Cancer Registry. Data were analysed as a cohort study using survival analysis techniques and incidence rate ratios (IRRs) were used as measures of relative risk. A total of 4,320,623 persons were followed in the 10-year period 1994,2003, resulting in 37,581,600 person-years at risk, 202,144 persons with a first-time psychiatric contact, and 208,995 persons diagnosed with cancer. During the first month after a first-time psychiatric contact, the incidence of all forms of cancer was elevated; IRR: 2.61 (95% CI, 2.31,2.95). Particularly the incidence of brain tumours was elevated; IRR: 18.85 (95% CI, 14.52,24.48), but also the incidence of lung cancer; IRR: 2.98 (95% CI, 2.16,4.12), and especially small-cell lung cancer; IRR: 6.13 (95% CI, 3.39,11.07) was elevated. The elevated IRR for most cancers decreased towards unity within the first 3 months, except for brain tumours, for which the IRR remained significantly elevated during the first 9 months. One of every 63 patients above 50 years of age was diagnosed with malignant cancer within 1 year of first-time psychiatric contact. These results indicate an increased incidence of cancer, especially for brain tumours and small-cell lung cancer, in the first months after a first-time contact to a psychiatric hospital. Clinicians should be aware that first-onset psychiatric symptoms could be a sign of a yet undetected cancer. © 2009 UICC [source] 14-3-3 protein in the CSF of inflammatory peripheral neuropathiesJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2004A Bersano 14-3-3 proteins are a highly conserved protein family of unknown function, although some authors suggested a role in cellular proliferation and differentiation, neurotransmitters biosynthesis and apoptosis. The expression of these proteins increases during development, in particular, in large projection neurons such as spinal motor neurons. Recently the protein was described in cerebrospinal fluid (CSF) of patients with spongiform encephalopathies, in particular Creutzfeld-Jacob disease, where the protein is considered a highly sensitive and specific marker. 14-3-3 protein has been also detected in CSF of other prion-unrelated dementias and other neurodegenerative (Parkinson disease, stroke and paraneoplastic syndromes) and inflammatory diseases like Multiple Sclerosis. The aim of our study was to evaluate whether the 14-3-3 protein is also present in the CSF of peripheral nervous system diseases. We studied by Western Blot the CSF of 120 patients including 38 with Guillain-Barré syndrome (GBS), 23 with chronic inflammatory demyelinating polyneuropathy (CIDP), 12 with multifocal motor neuropathies (MMN), 20 motor neuron disease (MND), 8 paraneoplastic syndrome, 14 other neuropathies or radiculopathies (OPN), and 5 normal subjects (NC). We found the 14-3-3 protein in the CSF of 21 (55%) patients with GBS, 13 (56%) with CIDP, 1 (5%) with MND, 3 (21%) with OPN and none with paraneoplastic syndrome, MMN or NC. Our results reveal that 14-3-3 protein can be detected not only in central but also in peripheral nervous system diseases where it is significantly associated (p < 0.0001) with GBS and CIDP. [source] Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 83JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003G Lauria We describe a 64-year-old patient complaining of progressive gait disturbance, referred to the compressive effect of multiple discal protrusions, for about 3 years. At the age of 62 he presented epileptic seizures during a febrile episode. Cerebral MRI showed bilateral frontobasal T2-weighted hyperintensity involving cortex and white matter. Partial seizures reappeared one year later and a MRI revealed a mild frontobasal atrophy. At the moment of our observation, neurological examination showed waddling gait with bilateral foot drop, muscular atrophy and weakness limited to the gluteal muscles and widespread deep tendon areflexia. Nerve conduction studies showed absent F-waves at both upper and lower limb examination, with normal distal sensorimotor nerve conduction. Needle EMG examination detected mild chronic partial denervation, predominant in proximal muscles of lower limbs. Somatosensory evoked potentials recorded from upper extremities showed bilateral increase in early latencies (N9). Overall, neurophysiological findings indicated a widespread radiculopathy. Serum exams revealed positive anti-nucleus (1:640, granular). CSF examination detected increased IgG level and several oligoclonal bands. Chest radiogram was normal. Soon after our first observation, the patient showed symptoms of respiratory insufficiency. A CT scans revealed a thoracic mass compatible with microcytoma, whereas anti-Hu (3 +) antibodies and increased NSE (neuronal specific enolase) titer were found. In the following two weeks, the patient showed a progressive worsening of the general clinical conditions and died. We interpreted this complex neurological picture, which included an atypical limbic encephalitis and a slowly progressive polyradiculopathy, as a paraneoplastic syndrome. The almost complete resolution of the encephalitic process and the subtle chronic involvement of the peripheral nervous system, characterized by a limited, though widespread, radicular impairment, are rather peculiar features. [source] Neocortical neurones may be targeted by immune attack in anti-Yo paraneoplastic syndromeNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 2 2008F. Aboul-Enein First page of article [source] Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2010Trista Stankowski-Drengler POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare paraneoplastic syndrome in which nearly all patients have a monoclonal lambda restricted plasma cell disorder. We investigated whether patients with POEMS have abnormal serum immunoglobulin free light chain (FLC) ratios. Fifty patients with newly diagnosed POEMS syndrome were assessed. Cystatin C levels were measured to discern whether subclinical renal insufficiency could account for FLC elevations in the presence of a normal FLC ratio. Forty-five patients (90%) had elevated lambda FLC; however, only nine (18%) had abnormal FLC ratios. The rise in serum FLC of POEMS patients appeared to be multifactorial,both a function of subclinical renal insufficiency and polyclonal activation of medullary and extramedullary plasma cells. Those patients expressing a clonal IgA were more likely to have clonal plasmacytosis observed on iliac crest biopsy than those with IgG. In summary, serum immunoglobulin profiles are unique in POEMS syndrome as compared with other plasma cell disorders. Am. J. Hematol. 2010. © 2010 Wiley-Liss, Inc. [source] Paraneoplastic gastro-intestinal anti-Hu syndrome in neuroblastomaPEDIATRIC BLOOD & CANCER, Issue 3 2009C.A.M. Drukker MD Abstract The anti-Hu syndrome is a well-known paraneoplastic syndrome and may be rarely seen in patients with neuroblastoma. However, it is relatively unknown that anti-Hu antibodies can cause gastro-intestinal signs and symptoms. We report on a child with neuroblastoma who presented with gastro-intestinal disturbances as a result of the anti-Hu syndrome and summaries two similar case reports reported in literature. Neuroblastoma patients with gastro-intestinal disturbances, ranging from constipation to a paralytic ileus, might suffer from the gastro-intestinal anti-Hu syndrome. The causative antibodies can be determined to diagnose or exclude this syndrome, and successful treatment is possible. Pediatr Blood Cancer 2009;52:396,398. © 2008 Wiley-Liss, Inc. [source] Non-metastatic cholestatic paraneoplastic syndrome associated with soft tissue sarcomaASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 2 2009Amanjit BAL Paraneoplastic syndromes have been described in association with variety of malignant neoplasms. The non-metastatic cholestatic paraneoplastic syndrome first described as Stauffer's syndrome in association with renal cell carcinomas is also associated with other malignancies. We describe the autopsy findings of a patient with recurrent and metastatic leiomyosarcomas presenting with cholestatic liver dysfunction. The diagnosis requires the exclusion of all other possible causes of hepatitis and, where possible, resolution after the effective treatment of the underlying malignancy. [source] Indurated reticulate palmar erythema as a sign of paraneoplastic palmar fasciitis and polyarthritis syndromeAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 3 2009Veronica A Preda ABSTRACT A 62-year-old woman presented with a 6-month history of polyarthritis. She had also noted a 2-month history of indurated palmar erythema and increasing bilateral hand swelling and stiffness. A biopsy from the area of palmar erythema showed interstitial fibroplasia within the dermis and subcutis representing a palmar fibromatosis. This presentation appears to belong to the spectrum of palmar fasciitis and polyarthritis syndrome. Rheumatologists have recognised this syndrome as a paraneoplastic disorder and subsequent investigations in our patient revealed an elevated cancer antigen 125 and an inoperable ovarian carcinoma. Indurated palmar erythema is a sign that is not widely recognised by dermatologists as a clue for this paraneoplastic syndrome, and skin biopsy demonstrating dermal and subcutaneous fibroplasia may help in diagnosis in the absence of advanced signs of palmar fasciitis. [source] Parathyroid hormone-related protein and its receptors: nuclear functions and roles in the renal and cardiovascular systems, the placental trophoblasts and the pancreatic isletsBRITISH JOURNAL OF PHARMACOLOGY, Issue 6 2001Thomas L Clemens The cloning of the so-called ,parathyroid hormone-related protein' (PTHrP) in 1987 was the result of a long quest for the factor which, by mimicking the actions of PTH in bone and kidney, is responsible for the hypercalcemic paraneoplastic syndrome, humoral calcemia of malignancy. PTHrP is distinct from PTH in a number of ways. First, PTHrP is the product of a separate gene. Second, with the exception of a short N-terminal region, the structure of PTHrP is not closely related to that of PTH. Third, in contrast to PTH, PTHrP is a paracrine factor expressed throughout the body. Finally, most of the functions of PTHrP have nothing in common with those of PTH. PTHrP is a poly-hormone which comprises a family of distinct peptide hormones arising from post-translational endoproteolytic cleavage of the initial PTHrP translation products. Mature N-terminal, mid-region and C-terminal secretory forms of PTHrP are thus generated, each of them having their own physiologic functions and probably their own receptors. The type 1 PTHrP receptor, binding both PTH(1-34) and PTHrP(1-36), is the only cloned receptor so far. PTHrP is a PTH-like calciotropic hormone, a myorelaxant, a growth factor and a developmental regulatory molecule. The present review reports recent aspects of PTHrP pharmacology and physiology, including: (a) the identification of new peptides and receptors of the PTH/PTHrP system; (b) the recently discovered nuclear functions of PTHrP and the role of PTHrP as an intracrine regulator of cell growth and cell death; (c) the physiological and developmental actions of PTHrP in the cardiovascular and the renal glomerulo-vascular systems; (d) the role of PTHrP as a regulator of pancreatic beta cell growth and functions, and, (e) the interactions of PTHrP and calcium-sensing receptors for the control of the growth of placental trophoblasts. These new advances have contributed to a better understanding of the pathophysiological role of PTHrP, and will help to identify its therapeutic potential in a number of diseases. British Journal of Pharmacology (2001) 134, 1113,1136; doi:10.1038/sj.bjp.0704378 [source] Choroidal neovascularization associated with cancer-associated retinopathyACTA OPHTHALMOLOGICA, Issue 5 2010Giuseppe Querques Abstract. Purpose:, To report an unusual association between cancer-associated retinopathy (CAR) associated with invasive thymoma and choriodal neovascularization (CNV), treated by photodynamic theraphy (PDT). Methods:, A 39-year-old man affected with thymoma and paraneoplastic syndrome (myasthenia gravis and diarrhoea) was observed between October 1997 and September 2007. The patient developed progressive visual dysfunction including bilateral visual acuity loss and concentric constriction of visual fields. Ophthalmological, immunological and systemic examinations were performed. Immunological evaluations included an assessment of antibody activity by indirect immunohistochemistry on sectioned rhesus monkey eye, and Western blot reactions upon an extract of pig retina. Results:, Fundus ophthalmoscopy and fluorescein angiography revealed retinal vessel attenuation and retinal pigment epithelium degeneration. Electroretinogram suggested both rod and cone dysfunction. Indirect immunohistochemistry identified antibody activity within the photoreceptor outer segments. Western blots on the retina revealed that most of the patient's antibody activity was focused upon a retinal protein antigen approximating 145 kD. These findings share the commonalities of size and retinal distribution of the interphotoreceptor retinoid-binding protein (IRBP), a recognized autoantigen. The surgically resected mediastinal tumour was diagnosed as invasive thymoma. No other malignancy has since been found throughout nearly 10 years of follow-up. In March 2006, the patient developed a subfoveal CNV in his left eye, which was treated by PDT. Conclusion:, We describe the third case of paraneoplastic retinopathy associated with invasive thymoma. This is the first example of CAR involving autoantibodies reactive with a retinal protein having the characteristics of the IRBP, and is also the first complicated by CNV treated by PDT. [source] Bilateral diffuse uveal melanocytic proliferation and uterine cancer.ACTA OPHTHALMOLOGICA, Issue 3 2000A case report ABSTRACT. Purpose: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP), a rare paraneoplastic syndrome causing visual loss in patients with systemic carcinoma. Results: A 70-year-old woman developed visual symptoms 13 months after surgery and local irradiation therapy for uterine cancer. Following bilateral external beam irradiation supplemented with subsequent drainage of subretinal fluid in the left eye, the visual acuity improved from 0.01 to 0.15 in this eye only. The visual acuity remained at this level until she died 4 1/4 years after the onset of eye symptoms. Conclusion: This is the fourth case that survived longer than 24 months after the onset of visual symptoms of the 22 previously reported cases with BDUMP. It demonstrates that radiotherapy may have a vision-preserving effect in this group of patients. The patient also developed two different paraneoplastic phenomena , a nephrotic syndrome before and BDUMP after treatment for uterine cancer. [source] Primary and secondary small cell neuroendocrine carcinoma of the larynx: A reviewHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 4 2008Alfio Ferlito MD, DPath, FASCP, FDSRCS, FHKCORL, FRCPath, FRCSEd, FRCSGlasg, FRCSI Abstract Primary laryngeal small cell neuroendocrine carcinoma (SCNC) is an unusual malignancy accounting for <0.5% of laryngeal carcinomas. To date, approximately 200 cases of primary and 5 of secondary SCNC of the larynx have been reported. This tumor most often presents in the sixth and seventh decades in men who are heavy cigarette smokers. The lesion may be associated with different paraneoplastic syndromes (ectopic adrenocorticotropic hormone syndrome, Schwartz,Bartter syndrome or syndrome of inappropriate secretion of antidiuretic hormone, and Eaton,Lambert myasthenic syndrome) or with ectopic hormone production. The diagnosis is based essentially on the histologic appearance of the tumor, confirmed by immunocytochemical investigations. Concurrent chemoradiotherapy regimens offer potential for long-term survival. This tumor is biologically aggressive, and the extent of the disease is the most significant independent prognostic factor of survival. The survival rate is similar to that with pulmonary SCNC. © 2008 Wiley Periodicals, Inc. Head Neck, 2008 [source] Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective studyINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 11 2002Nobuo Wakata MD Background Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear. Patients and Method During the 30-year period 1969,99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer. Results The risk of cancer is significantly higher in dermatomyositis and somewhat higher in polymyositis. An increased cancer risk was found in male patients with dermatomyositis who were older than 50 years. Cancer was diagnosed within 4 years before or after the diagnosis of polymyositis or dermatomyositis, and usually within 1 year. An operation was not possible in many of the patients with cancer because of the advanced stage of the disease. Conclusion Our findings suggest that early discovery of malignancy is critical in cases of polymyositis and dermatomyositis. [source] 14-3-3 protein in the CSF of inflammatory peripheral neuropathiesJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2004A Bersano 14-3-3 proteins are a highly conserved protein family of unknown function, although some authors suggested a role in cellular proliferation and differentiation, neurotransmitters biosynthesis and apoptosis. The expression of these proteins increases during development, in particular, in large projection neurons such as spinal motor neurons. Recently the protein was described in cerebrospinal fluid (CSF) of patients with spongiform encephalopathies, in particular Creutzfeld-Jacob disease, where the protein is considered a highly sensitive and specific marker. 14-3-3 protein has been also detected in CSF of other prion-unrelated dementias and other neurodegenerative (Parkinson disease, stroke and paraneoplastic syndromes) and inflammatory diseases like Multiple Sclerosis. The aim of our study was to evaluate whether the 14-3-3 protein is also present in the CSF of peripheral nervous system diseases. We studied by Western Blot the CSF of 120 patients including 38 with Guillain-Barré syndrome (GBS), 23 with chronic inflammatory demyelinating polyneuropathy (CIDP), 12 with multifocal motor neuropathies (MMN), 20 motor neuron disease (MND), 8 paraneoplastic syndrome, 14 other neuropathies or radiculopathies (OPN), and 5 normal subjects (NC). We found the 14-3-3 protein in the CSF of 21 (55%) patients with GBS, 13 (56%) with CIDP, 1 (5%) with MND, 3 (21%) with OPN and none with paraneoplastic syndrome, MMN or NC. Our results reveal that 14-3-3 protein can be detected not only in central but also in peripheral nervous system diseases where it is significantly associated (p < 0.0001) with GBS and CIDP. [source] | ||||||||||||||||||||||