1/17. High mitotic index associated with poor prognosis in gastrointestinal autonomic nerve tumour.AIMS: Three gastrointestinal autonomic nerve tumours (GANT) were characterized by immunohistochemistry and flow cytometry. Two of the three cases occurred in the small intestine, while the third was found in the stomach. Besides the immunohistochemical and ultrastructural description, the aim of this study was to examine the relation between the known and accepted predictive factors (ploidy data, the S-phase fraction, the mitotic and MIB-1 index and the size of the tumour) and the survival of the patients. methods AND RESULTS: The immune profile showed that 3/3 cases were vimentin and NSE, 2/3 were synaptophysin and PGP 9.5 positive, while 1/3 also showed S100 positivity. Ultrastructurally, all the cases had dense core granules, one of them contained skenoid fibres. The flow cytometry revealed diploid dna in all cases, however, significant differences could be seen in the proliferative activity of the individual neoplasms. CONCLUSIONS: In spite of the published data of gastrointestinal stromal tumours (GIST) generally, neither the MIB-1 index and the ploidy data nor the size of the primary tumour helped to predict the clinical progression of the examined GANTs. However, the high proliferative activity (57 mitoses/10 HPF) and the elevated S-phase fraction (24%) was associated with advanced, metastatic and recurring disease in case 3. On the basis of these three cases, high mitotic activity is the most reliable factor in predicting aggressive clinical behaviour.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/17. Malignant granular cell tumour of the cervical sympathetic nerve trunk.Granular cell tumour is a rare neoplasm that can occur in various sites. This report describes a 48-year-old female with a malignant granular cell tumour originating from the cervical sympathetic nerve trunk, who presented with hyperaesthesia of the left shoulder. The tumour had a maximum diameter of 86 mm and involved adjacent tissues directly. Histopathologically, it showed considerable variation in the size and shape of nuclei, with occasional mitosis and an abundant granular cytoplasm. Immunohistochemically, the tumour reacted positively for S-100 protein and neuron-specific enolase, indicating its neural origin. Following total local excision of the lesion, the patient has been well without recurrence.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
3/17. Rasmussen syndrome: multifocal spread of inflammation suggested from MRI and PET findings.BACKGROUND: A 6-year-old girl with Rasmussen syndrome (RS) showed multiple small high-signal-intensity areas independently in the right hemisphere by fluid-attenuated inversion recovery (FLAIR) imaging on magnetic resonance imaging (MRI) 1 year after the onset of epilepsy. methods: MRI performed 4 months later demonstrated a further increase in the number of these foci and enlargement in the size of the previous FLAIR lesions. RESULTS: An [18F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) study showed a strong, spotty uptake in the right temporooccipital regions, corresponding to the sites of continuous EEG seizure discharges. In contrast, [11C]methionine PET demonstrated multifocal uptake regions, which corresponded anatomically to the FLAIR lesions, suggesting sites of underlying chronic inflammation. CONCLUSIONS: These neuroimaging findings suggested that the inflammatory process in RS spreads either multifocally at the same time, as seen in this case, or from one discrete area to the adjacent region, as reported previously.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
4/17. Hypothalamic activation in trigeminal autonomic cephalgia: functional imaging of an atypical case.We report headache induced BOLD changes in an atypical case of trigeminal autonomic cephalgia (TAC). A 68-year-old patient was imaged using fMRI during three attacks of a periorbital head-pain with a average duration of 3 min. During the attacks, left sided conjunctival injection, rhinorrhea, lacrimation, facial sweating and hypersalivation were apparent. These attacks were usually partly responsive to oxygen administration but otherwise refractory to any drug. The patient described either attacks with a duration of one minute or less or longer attacks persisting for maximum of 20 min with headaches occurring up to 100 times a day. When considering the symptoms, frequency, duration and therapeutic response of the patient's headache, no clear-cut classification to one of the subtypes of trigeminal autonomic cephalgias (cluster headache, paroxysmal hemicrania, SUNCT) or trigeminal neuralgia was possible. The cerebral activation pattern was similar but not identical to those previously observed in cluster headache and SUNCT with a prominent activation in the hypothalamic grey matter. This case study underlines the conceptual value of the term TAC for the group of headaches focusing around the trigeminal-autonomic reflex. Our results emphasize the importance of the hypothalamus as key region in the pathophysiology of this entity.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
5/17. Gastrointestinal autonomic nerve tumors. 'Plexosarcomas'.Four plexosarcomas (gastrointestinal autonomic nerve tumors) characterized by light microscopic, immunocytochemical, and ultrastructural examination, including immunoelectron microscopy in one case, are described. The four neoplasms occurred in the small intestine (duodenum, two; jejunum, one; and ileum, one) and they had an aggressive course with either local or distant metastases. The light-microscopic patterns varied from epithelioid and organoid to spindle cells, mimicking endocrine and sarcomatous neoplasms. Ultrastructurally, these tumors exhibited interdigitating cytoplasmic processes that contained scattered aggregates of membrane-bound granules varying in size from 100 to 300 nm intermixed with empty vesicles and numerous diffusely distributed intermediate filaments. Basal lamina covering cell surfaces, attachment plaques, and myofilaments, as expected in smooth-muscle tumors, were not identified, and diffusely distributed membrane-bound granules, as seen in paragangliomas and carcinoid tumors, were also absent. By immunocytochemistry, the tumors were intensely positive for vimentin and neuron-specific enolase and focally positive for neurofilaments and synaptophysin. In addition, three tumors were S100 protein positive and one stained for vasoactive intestinal peptide. Similar positive immunocytochemical reactions were identified in normal enteric plexus. It is essential to recognize plexosarcomas, which are invariably accompanied by aggressive clinical behavior, in spite of a seemingly benign, mitotically inactive light-microscopic appearance in most instances. Ultrastructural examination can readily separate plexosarcomas from paragangliomas and other sarcomatous and endocrine neoplasms.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
6/17. Diffuse, multicentric neurogenic tumors in two macerated fetuses: a possible intrauterine form of neurofibromatosis.Two tiny macerated fetuses with a remarkably similar pattern of multicentric neurogenic neoplasms of both paravertebral autonomic structures and peripheral nerves are described. Maceration precluded further histologic classification of the neoplasms in either fetus. The first fetus had a Meckel's diverticulum, short attachment of the small bowel mesentery, pulmonary hypoplasia, and intrauterine growth retardation. The second had the sympus bipus variant of sirenomelia sequence. The multicentric neoplasms in these two fetuses are very similar to those previously described in a few neonates and one stillborn with well-documented or suspected neurofibromatosis. It is reasonable to hypothesize that these two fetuses may represent an early intrauterine expression of neurofibromatosis characterized by multicentric neurogenic neoplasms of autonomic structures and peripheral nerves. Detailed examination of early abortuses, especially those from families with neurofibromatosis, may help to confirm or disprove the hypothesis.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
7/17. Postganglionic cholinergic dysautonomia: report of muscle findings in 1 case.Heretofore unreported findings in skeletal muscle in a 4th case of pure postganglionic dysautonomia are described. They consist of variation in fiber size, Z line and myofilamentous disorganization, intracytoplasmic inclusions and honeycomb formations. The most striking alterations, however, was excess lipid accumulation without concomitant mitochondrial alterations. sural nerve histology and cytology were normal. The mechanism of lipid accumulation in skeletal muscle of patients with dysautonomia remains unexplained.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
8/17. Primary myenteric plexus alterations as a cause of megacolon in Von Recklinghausen's disease.This is a case report of a megacolon in a 26 years old male patient having typical von Recklinghausen's disease. Exploratory laparotomy shows no neurofibroma on digestive tract. myenteric plexus study of colic resection specimen following B. Smith technique reveals marked alterations. These alterations are both quantitative (hyperhypoganglionosis) and qualitative (ganglion cells are irregular in shape and size, dendritic processes are hypertrophic, irregular; schwannosis; no inflammatory infiltration). The patient suffers no more transit disorders after adequate resection of this megacolon.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
9/17. Cardiovascular autonomic insufficiency in a patient with metastatic malignancy.A case of cardiovascular autonomic insufficiency is described in a cachectic 70-year-old man with widely metastatic small-cell tumor. The patient experienced disabling syncopal episodes in association with severe postural hypotension. Tests of cardiovascular autonomic insufficiency were abnormal. The introduction of fludrocortisone and the use of elastic stockings produced palliation of the symptomatology, but his condition deteriorated and he died. autopsy demonstrated unexpectedly extensive tumor invasion of autonomic nervous tissue. Although cardiovascular autonomic insufficiency (CAI) has been described in association with malignancy and malnutrition, local tumor invasion of autonomic nervous tissue and radiation injury may have been other possible, albeit unusual, etiologic factors. This case illustrates the differential diagnosis of hypotension and CAI in patients with advanced metastatic malignancy and also emphasizes the need for autopsy studies when speculating as to the cause of CAI in this group of patients.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
10/17. Dysautonomia with acute sensory motor neuropathy. A new classification of acute autonomic neuropathy.OBJECTIVES: To characterize the dysautonomia associated with acute sensory motor neuropathy and to discuss the classification of acute autonomic neuropathy. DESIGN: Case series. methods: Sympathetic skin response. Local sweat response to acetylcholine. norepinephrine infusion test and acetylcholinesterase histochemistry of sural nerve biopsy specimens in addition to making conventional analyses of myelinated and unmyelinated fibers. RESULTS: In 12 patients with chronic neuropathy, acetylcholinesterase-positive fiber density and plantar sympathetic skin response size were well correlated, but in the two patients with acute autonomic sensory and motor neuropathy, there were discrepancies, acetylcholinesterase-positive fiber density being well preserved and sympathetic skin responses being absent. Histologic and electrophysiologic results indicated primary demyelination of the myelinated fibers. In contrast, previous studies of acute autonomic sensory and motor neuropathy reported dysfunction of the sympathetic postganglionic fibers and axonopathic change in myelinated fibers, poor recovery from dysautonomia. CONCLUSIONS: Dysautonomia with acute idiopathic neuropathy can be divided into two categories--postganglionic axonopathic and preganglionic demyelinating types of the sympathetic efferent pathways. The recovery from dysautonomia produced by the former lesion is poor, but recovery is better for that produced by the latter lesion.- - - - - - - - - - ranking = 0.5keywords = size (Clic here for more details about this article) |
| | Next -> |