Review article: the biology, diagnosis and management of gastrointestinal stromal tumours.

BACKGROUND: Gastrointestinal stromal tumours (GIST) are the most common mesenchymal tumours of the gastrointestinal tract with an increasing incidence. AIMS: To review the biology, diagnosis and treatment of gastrointestinal stromal tumours. METHODS: A PubMed search using the phrases 'Gastrointestinal stromal tumor', 'imatinib', 'c-kit'. RESULTS: The diagnosis of GIST is established by histology supplemented by the immunohistochemical marker CD117, which is positive in 95% of cases. The most common site of the tumour is the stomach. Most GIST are benign with 20-30% malignant. Five-year survival for malignant GIST ranges between 35% and 65% and depends primarily on tumour size, mitotic index and location. The malignant behaviour of GIST is best assessed by invasion of adjacent structures and distant metastases. The gold standard for treatment is surgical resection. Imatinib, a tyrosine kinase inhibitor, is the primary therapy for unresectable, recurrent or metastatic disease. CONCLUSIONS: Gastrointestinal stromal tumours are rare tumours of the gastrointestinal tract and they vary in presentation. When surgical resection is not achievable, imatinib is the treatment of choice.

Use of the Montreal global definition as an assessment of quality of life in reflux disease.

According to the Montreal Consensus Group's classification, gastroesophageal reflux disease develops when the reflux of stomach contents causes troublesome symptoms and/or complications such as esophagitis. The characteristic gastroesophageal reflux disease symptoms included in this statement are retrosternal burning and regurgitation. Troublesome is meant to imply that these symptoms impact on the well-being of affected individuals; in essence, quality of life (QOL). Whether heartburn and regurgitation symptoms would be characterized as more troublesome in those with confirmed pathologic acid reflux was determined. A second purpose was to assess how well troublesome scores correlated with the results of a validated, disease-specific QOL instrument. Subjects who underwent esophagogastroduodenoscopy (EGD) with 48-hour wireless esophageal pH testing off proton pump inhibitor therapy were interviewed. Esophagitis on EGD or pH < 4.0 for ≥4.5% of time over the 2-day period was considered positive for acid reflux. Assessment of how troublesome their symptoms of heartburn and regurgitation were made using separate 0-100 visual analog scales (VAS). Subjects were then asked to complete the Quality of Life in Reflux and Dyspepsia (QOLRAD) 25-item questionnaire. Sixty-seven patients (21 males, 46 females) with mean age 47.8 ± 15.6 years were identified. Forty (59.7%) had an EGD or pH study positive for acid reflux. Overall 35/40 (87.5%) complained of either heartburn or regurgitation. There was no difference (P= 0.80) in heartburn VAS troublesome ratings for those with (54.0 ± 43.9) and without (56.7 ± 37.6) confirmed acid reflux. The same was true for regurgitation VAS troublesome ratings (P= 0.62). Likewise, mean QOLRAD scores did not differ between those with and without confirmed acid reflux by pH or EGD (4.5 ± 1.7 vs. 4.3 ± 1.7; P= 0.61). There was a moderately strong inverse correlation between patient self-rated VAS troublesome scores for both heartburn and regurgitation with each dimension (emotional distress, sleep disturbance, eating problems, physical/social functioning, and vitality) of the QOLRAD (P < 0.05 for all comparisons). In regression analysis, both heartburn and regurgitation troublesome ratings were associated with the overall QOLRAD score independent of pH data, frequency of reflux episodes, age, and gender. Use of the term troublesome in the Montreal Consensus Group classification is supported by our findings. It correlates well with the results of a validated disease-specific QOL instrument. Use of heartburn and regurgitation VAS may serve as accurate measures of the burden of reflux disease on patients. It is likely that these scales will not have sufficient discriminate value to identify individuals with pathologic acid reflux from those with negative studies.

Early neurologic complications following coronary bypass surgery.

BACKGROUND: Coronary artery bypass surgery is associated with central and peripheral nervous system complications in the period following surgery. Recognising these complications may help in their prevention or early treatment. METHODS: We reviewed medical records of all the patients who underwent coronary artery bypass surgery at our institution over a period of two years. We studied their risk factors, reasons for surgery, operative variables, and post operative neurologic complications. RESULTS: Of the 587 coronary artery bypass surgeries performed at our centre over a two year period. We found that 2.04% of these patients developed neurologic complication in the two weeks following the surgery. Fifty percent of these patients suffered from cerebrovascular insults and 50% suffered from cognitive decline. No patients in this group developed seizures or peripheral nerve lesions. Patients with renal failure, carotid stenosis, history of cerebral strokes, and redo coronary bypass surgery were more predisposed to develop neurologic complications after bypass surgery. Furthermore, a longer stay in the coronary care unit and the development of arrhythmias predisposed patients to neurologic complications. Mortality for patients who developed neurologic complications post bypass surgery ranged between 16.7% and 33.4%. CONCLUSIONS: Around 2% of patients who undergo coronary artery bypass surgery develop neurologic complications in the period directly after the surgery. Patients with previous history of cerebral, coronary, or carotid disease are more predisposed for such complications, as well as patients who spend more time in the intensive units after the surgery.

Seroprevalence of toxocariasis in Lebanon: a pilot study.

Toxocariasis is a common helminthic infection that has a worldwide distribution. However, data from Lebanon about the prevalence of this infection are non-existent. We conducted a Toxocara seroprevalence study with 150 subjects attending the outpatient clinics at the American University of Beirut Medical Center between May and June 2004. Serum specimens were tested for anti-Toxocara antibodies by enzyme-linked immunosorbent assay and confirmed by Western blot. Multivariate analysis was performed to identify risk factors for infection. The seroprevalence rate of toxocariasis was 19%. Male gender and below high school education were significantly associated with a positive serological test (odds ratios = 3.1 and 2.8, respectively). Higher numbers of persons in the household, and low family income during childhood, were significant on bivariate analysis only. Toxocariasis is common in Lebanon. A large population-based survey is needed to confirm these results.

Carpal tunnel syndrome: modern diagnostic and management techniques.

Carpal tunnel syndrome is a common disorder characterised by the classical symptoms of numbness and paraesthesiae along the distribution of the median nerve. Thenar muscle weakness is a late manifestation of advanced disease. Tinel's and Phalen's signs are helpful in suggesting the diagnosis. The symptoms and signs arise from entrapment of the median nerve. Electrophysiological tests are helpful in confirming the diagnosis and magnetic resonance imaging may be used in the diagnosis of atypical cases. Ergonomic manoeuvers and steroid injections may alleviate symptoms in mild cases. Surgery is reserved for severe cases and those who do not respond to conservative therapy. Open carpal tunnel release is the classical surgery with usually excellent results. Endoscopic carpal tunnel release surgery was introduced to decrease the morbidity of open surgery. This latter technique also has its complications and is still being refined.

Astrocytes give rise to oligodendrogliomas and astrocytomas after gene transfer of polyoma virus middle T antigen in vivo.

The cells of origin for oligodendrogliomas and astrocytomas are not known but are presumed to be oligodendrocyte and astrocyte precursors, respectively. In this paper we report the generation of mixed gliomas from in vivo transformation of glial fibrillary acidic protein (GFAP)-positive cells (differentiated astrocytes) with polyoma virus middle T antigen (MTA). MTA is a powerful oncogene that activates a number of signal transduction pathways, including those proposed to be involved in gliomagenesis, and has been shown to induce tumors in many cell types. We have achieved transfer of MTA expression specifically to GFAP(+) cells in vivo using somatic cell gene transfer, and find resultant formation of anaplastic gliomas with mixed astrocytoma and oligodendroglioma morphological features. We conclude that GFAP- expressing astrocytes, with appropriate signaling abnormalities, can serve as the cell of origin for oligodendrogliomas, astrocytomas, or mixed gliomas.

Trigeminal neuralgia associated with sinusitis.

When a patient presents with trigeminal neuralgia, one usually thinks of a vascular loop at the root entry zone of the nerve and consequently of vascular decompression. An image of sinusitis on the MRI may be considered an incidental finding. We present a case of an elderly woman who experienced severe neuralgic pain in the distribution of the trigeminal nerve on the left side following a mild upper respiratory tract infection. Routine MRI revealed severe sinusitis with no pathology in the brain. Following antibiotic treatment for the sinusitis, the symptoms of the neuralgia resolved completely and no other therapy was necessary. A review of the literature reveals a wide variety of etiologies for trigeminal neuralgia. A vascular loop compressing the nerve may be the most frequent cause of trigeminal neuralgia. Nevertheless, other etiologies must be considered prior to decompressive surgery since some can be treated medically.

Adrenoleukodystrophy associated with vitiligo and ulcerative colitis.

Adrenoleukodystrophy (ALD) is an X-linked inherited disorder of lipid metabolism usually presenting in childhood or early adolescence. It is a progressive disease with symptoms of adrenal insufficiency and central nervous system demyelination. The pathology results from the accumulation of very long-chain fatty acids and an inflammatory reaction in the brain white matter. We report a case of ALD associated with adrenal insufficiency and two autoimmune diseases: vitiligo and ulcerative colitis.

Idiopathic sciatic mononeuropathy.

Sciatic nerve lesions are usually painful and secondary to compression, trauma, infarction or part of a systemic illness. The etiology is usually defined by radiographic or blood studies, or by exploratory surgery. In rare cases, as the one being presented, there is clear clinical and electrophysiological evidence for a lesion of the sciatic nerve, but no morphological correlate or defined etiology. These idiopathic sciatic mononeuropathies seem to occur in the nerves of the legs in young adults.

Foramen magnum meningioma presenting as amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the nervous system. It affects adults in all age groups and leads to death few years after the diagnosis. The etiology is unknown, and there no convincing medical or surgical therapy Some diseases can mimic ALS. If diagnosed early these may be treatable, but if misdiagnosed as ALS and left untreated they may be fatal. We present a case of foramen magnum meningioma presenting clinically as ALS. After surgery, the symptoms disappeared and the neurological deficits improved.