paraneoplasic syndrome

Chronic intestinal pseudo-obstruction (CIPO) is a heterogeneous group of rare disorders characterised by symptoms of intestinal obstruction with no mechanical evidence of obstruction. It is caused by ineffective intestinal contractions due to visceral neuropathy and/or neuropathy. In adults, CIPO is mostly secondary. The most common causes are metabolic disorders, connective tissue disorders, neuropathic drug related injuries, paraneoplasic and post-infectious syndromes and amyloidosis. Secondary forms of CIPO have been reported with anti-Hu antibodies...

Paraneoplastic cerebellar degeneration (PCD) is a rare condition which is characterised by global cerebellar dysfunction. Patients may present with these syndromes months or years before the diagnosis of underlying malignancy is established. Less often, PCD occurs in patients with a known malignancy or heralds the onset of a recurrence. The presence of specific antibodies in serum simples helps to guide identification the occult malignancy. We report here the case of a PCD in 74-year-old lady underwent a left mastectomy for breast cancer 5 years ago...

The cancer associated retinopathy (CAR) is a paraneoplasic retinopathy in which an antigen-antibody reaction, due to retinal antigens, also expressed in tumours, leads to degeneration of retinal photoreceptor cells. We observed in CHL-Luxembourg, 2 clinical cases of non-Hodgkin's lymphoma with severe prognosis in whom we described the presence of anti-recoverin antibodies. The CAR is most frequently associated with small cell lung and ovarian carcinomas. Clinical symptoms (phosphenes, progressive loss of eyesight) sometimes, occur before the diagnostics of primary cancer...

Chronic intestinal pseudo-obstruction (CIPO) is a syndrome characterized by the presence of recurrent episodes of clinical intestinal obstruction in the absence of obstructive lesions. Although this syndrome is rare, it causes a high morbidity. It is caused by a disturbance of the intestinal motility, that results in a failure of the progression of the intestinal content. Basically, the failure of the intestinal motility is a consequence of muscular disorder, neurological disorder or both. Usually, CIPO is secondary to other systemic disease; however, in the last years, many cases of primary CIPO have been described...

Intracraneal manifestations of Hodgkin's Disease (HD) are extremely rare, with an estimated incidence rate of approximately 0.5%. They can be classified as: 1) treatment-related leucoencephalopathy, 2) central nervous system infections, 3) paraneoplasic syndromes and 4) intracraneal lymphomas, which could be sub-classified into intraparenchymal or intradural masses. We describe a case of a 40 year-old male with mixed cellularity type HD who developed neurological manifestations as relapsed disease. Magnetic resonance imaging suggested leptomeningeal metastases and atypical cells were found in cerebrospinal fluid...

Cushing paraneoplasic syndrome is a rare cause of hypercorticism. We report a case of 35 year-old man presenting with Cushing's syndrome characterized by severe signs of hypercorticism and hypokalemia. Endocrine investigations were suggestive of an hypercortisolism linked to an ectopic adrenocorticotropic (ACTH) secretion, both at baseline (mean ACTH levels=275 pg/ml, urinary free cortisol excretion=3.898 mmol/24 h) and after pharmacodynamic testing (lack of inhibition of ACTH by dexamethasone). Thoracic tomodensitometric examination revealed a 15 mm tumor corresponding to a neuroendocrine pulmonary carcinoid with positive immunostaining for chromogranin A...

Lambert-Eaton myasthenic syndrome is a paraneoplasic syndrome which can reveal a primitive tumor. Frequently, the first diagnosis is myasthenia gravis. This disease is extremely rare in children. Only 10 cases have been reported in the last 35 years. We report 2 new observations occurring in very young patients, aged 2 and 3 years, with a ganglioneurobastoma as primitive tumor.

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We report the case of a 24 years old, nineteen weeks pregnant that develops pictures of severe hypoglycemia as first manifestation of primary hepatocarcinoma by hepatitis B. The great increase of the alpha fetoprotein was diagnostic. We reviewed the Literature and comment the hypoglycemic effect as a paraneoplasic manifestation by the increase of the growth-factor similar to insulin II of tumor origin, with low insulin levels.

A 35-year-old male with an 11-year history of intestinal pseudo-obstruction associated with an idiopathic inflammatory insult of the myenteric plexus and the presence of circulating anti-Hu antibodies developed a neurological syndrome characterized by bilateral hearing loss, deteriorating balance, an unsteady gait and difficulty in estimating distances. A similar neurological syndrome has previously been described in older patients among the paraneoplasic syndromes associated with small-cell lung carcinoma and the presence of circulating anti-Hu antibodies, but never in the rare cancer-free patients with anti-Hu-associated chronic idiopathic intestinal pseudo-obstruction...

Early ovarian cancers account for 25 to 30% of cases. They constitute the only curable cases. There is no specific clinical symptom of early ovarian cancer. Paraneoplasic syndroms, especially phlebitis, are of major importance when they occur in young women. In most of cases, the diagnosis is performed during the management of a complex ovarian cysts. Quality of the preoperative work-up, especially sonography, and management by a specialised team are the best factors to provide an early diagnosis and a comprehensive staging...

BACKGROUND: Inflammatory pseudotumor (IPT) is the most frequent pulmonary mass in childhood. It is histologically benign but locally aggressive. Atelectasis and recurrent airway infections are the most frequent presenting findings. We present two children in whom first clinical signs were paraneoplasic syndromes. MATERIAL AND METHODS: Retrospective study of two cases of IPT treated in our clinic from 1998 to 2002. Age, clinical presentation, preoperative diagnosis, treatment, histological diagnosis and postoperative outcome were reviewed from clinical chart...

A case of Guillain-Barré with unusual autonomic dysfunction at its onset, that consisted of constipation and hypertension, followed by adynamic ileus and flaccid paraparesis with areflexia limited to the lower limbs, is presented. Inappropriate secretion of antidiuretic hormone and hyperprolactinemia were demonstrated, which resolved spontaneously afterwards. The adynamic ileus resolved and the paraparesis improved with gastric aspiration and intravenous immunoglobulin administration, only to worsen eighty days later...

Paraneoplastic neurological syndromes consist of a dysfunction of any part of the nervous system, in isolation or in combination, caused by a malign neoplasia, but not by the direct tissular or metastasic invasion of the tumour. Their pathogeny is explained by immunological mechanisms and they are characterised by the presence of high rates of antibodies in serum and cerebrospinal fluid. We present the case of a patient with a sensitive neuropathy that produced ataxia, and who suffered from a poorly differentiated adenocarcinoma of the lung, in whom the search for antineuronal antibodies was positive for antiamphiphysin antibodies, supporting the diagnosis of paraneoplastic polyneuropathy...

A 69-year-old man under long-term spironolactone therapy (16 years) was hospitalized with spontaneous hematoma on the trunk and extremities. Coagulation studies disclosed an acquired hemophilia that was successfully treated with human factor VIII for a few days and immunosuppressive agents for several months. Physical examination revealed bilateral gynecomastia and an upper left quadrant breast nodule. Complete staging was unremarkable. Complete left mastectomy was performed. Histopathology showed invasive ductal carcinoma, expressing positivity for estrogen and progesterone receptors...

A 74-year-old man developed pandysautonomia with severe orthostatic hypotension. Search for a paraneoplastic etiology was confirmed with the positivity of anti-Hu antibodies. [18] Fluorodeoxyglucose positron emission tomography (PET) enabled recognition of a lung adenocarcinoma. We emphasize the usefulness of PET scans for early diagnosis of cancer.

AIMS: To review the classification and the clinical and pathological characteristics of the most common dysautonomias. METHOD: Primary dysautonomia includes neurodegenerative diseases of unknown causes that are characterised by the intracytoplasmic accumulations of alpha synuclein that manifest with four different phenotypes: pure autonomic failure, Parkinson s disease, dementia with Lewy bodies and multiple system atrophy. Of the secondary dysautonomias, diabetes mellitus is the most common cause of autonomic neuropathy in developed countries...

INTRODUCTION: Opsoclonus myoclonus is a rare neurological syndrome affecting children and adults, and which is characterised by a sudden onset of chaotic eye movements and myoclonias. In children it generally appears before the age of three as a parainfectious or paraneoplasic process; the type of tumour most frequently associated with this syndrome is the neuroblastoma. CASE REPORT: We report the case of a 22 month old girl who, after a febrile syndrome probably caused by a virus, began to present myoclonias in the upper and lower limbs, opsoclonus, a marked ataxic gait and extreme irritability...

INTRODUCTION: Dermatopolymyositis is an inflammatory disorder of an unknown origin. Twenty to thirty percent cases of this disease are associated with a cancer. Glomerular lesions in dermatopolymyositis are rare. EXEGESIS: We describe the case of a patient who presented a dermatopolymyositis together with a glomerulopathy and then a non Hodgkin's lymphoma. A treatment by corticoid and secondary intravenous immune globulins because of corticoid-resistance of the dermatopolymyositis and oesophagus injury led to a significant improvement of the cutaneous signs and of the muscular weakness...

A clinical case of the "Paraneoplasic thrombosis of subclavian vein and right internal jugular vein", is presented at a young patient, with right lung carcinomatous lymphangitis, clinically confirmed by BAL with probable prostatic point of start.