1/48. Rapid evolution of chronic viral hepatitis into hepatocellular carcinoma after beta-interferon treatment.A 62-year-old man, affected by Chronic Active hepatitis (discovered in 1993) and treated with interferon, referred to our department with increased abdominal volume, persistent abdominal pain, continuous-remittent fever and jaundice. CT scan of the liver revealed a hypodense, not capsulated, infiltrative, solid formation in the right lobe. US guided biopsy showed multinucleated giant cells, with eosinophilic cytoplasm and pleomorphism of the nuclei, arranged in several thick trabecula lined by endothelial cells or formed bile containing acini. In our case, the rapid evolution of chronic viral hepatitis towards HCC calls for a careful evaluation of the role of IFN therapy, since this drug is widely used in chronic liver diseases.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/48. Unilateral proptosis resulting from giant-cell arteritis.BACKGROUND: Giant-cell arteritis (GCA) is a systemic, inflammatory vasculopathy that affects small- to medium-sized arteries. Arterial wall inflammation results in reduction of blood flow and subsequent ischemia. Arteries of the head and neck are particularly susceptible, including the ophthalmic and posterior ciliary arteries. The eye care provider is in a position to assist with the ultimate diagnosis of GCA. CASE REPORT: A 79-year-old black man was referred to the eye clinic for evaluation of exophthalmos of the left eye. The patient reported increasing proptosis over the previous 6 months; a history of sudden, permanent vision loss of the affected eye (approximately 2 years earlier); and generalized malaise and chronic frontal headache. Examination did reveal an exophthalmic eye of approximately 8-mm difference by Hertel exophthalmometry. Fundus examination revealed optic nerve pallor O.S. CT scan revealed chronic inflammatory changes of orbital tissue, including the extraocular muscles. No compressive lesions were present. Laboratory testing indicated an elevated erythrocyte sedimentation rate. A tentative diagnosis of giant-cell arteritis was made, which was confirmed with temporal artery biopsy. CONCLUSIONS: patients with ocular complications secondary to GCA manifest several different ocular symptoms, including unilateral and bilateral intermittent blur, sudden complete vision loss, double vision, etc. This was an unusual case of GCA because the initially manifested ocular sign was unilateral proptosis. The patient probably had initial ocular complications of GCA 2 years previously, with sudden loss of vision in the left eye. The patient never sought medical attention at that time, and the unilateral exophthalmic eye resulted from chronic inflammatory orbital changes associated with GCA.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
3/48. Progressive confluent circumpapillary multiple evanescent white-dot syndrome.PURPOSE: To describe a morphologic variant of the multiple evanescent white-dot syndrome that can mimic other conditions. methods: We examined three patients with severe cases of unilateral multiple evanescent white-dot syndrome characterized by an atypical progressive circumpapillary discoloration of the fundus. RESULTS: The confluent circumpapillary lesion progressed toward or beyond the equator of the fundus, raising initial concern of a viral retinitis. However, pinpoint dots at the leading edge evolved into the typical wreath-like spots of multiple evanescent white-dot syndrome, which then coalesced into the advancing edge of a geographic retinitis or retinal pigment epitheliitis, before spontaneous resolution. CONCLUSION: Progressive geographic circumpapillary discoloration, appearing as a giant white spot, occurs rarely in severe cases of multiple evanescent white-dot syndrome. The distinctive appearance may suggest a disorder other than multiple evanescent white-dot syndrome, which can make initial diagnosis more difficult and lead to unnecessary or inappropriate testing and treatment.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/48. Sarcomatoid carcinoma of the prostate: progression from adenocarcinoma is associated with p53 over-expression.BACKGROUND: The pathogenesis of sarcomatoid metaplasia of prostatic adenocarcinoma is uncertain. The histologic features of sarcomatoid carcinoma arising in two patients with previously irradiated prostatic adenocarcinoma are reported and the relationship between prostatic adenocarcinoma and subsequent sarcomatoid carcinoma is investigated by immunohistochemical detection of epithelial and soft tissue tumor markers, and p53 protein. methods AND RESULTS: Two patients, aged 72 and 67 years, underwent localized radiotherapy for prostatic adenocarcinoma and re-presented with sarcomatoid carcinoma 41 months and 60 months later, respectively. In both cases the tumor consisted of anaplastic spindle cells with occasional osteoclast-like giant cells. The initial tumors showed immunohistochemical staining typical of prostatic adenocarcinoma with absence of expression of p53 protein. The subsequent sarcomatoid carcinomas were positive for vimentin and negative for epithelial cell markers. In both cases serial biopsies showed a temporal increase in tumor expression of p53 protein. CONCLUSIONS: The development of sarcomatoid carcinoma in prostatic adenocarcinoma is associated with progressive accumulation of p53. This is suggestive of increasing clonal dominance of dedifferentiated tumor cells carrying p53 mutations.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/48. Malignant pilocytic astrocytoma in the medulla oblongata: case report.A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/48. Deterioration of giant cell arteritis with corticosteroid therapy.BACKGROUND: Failure of response of giant cell arteritis (GCA) to corticosteroid therapy has invariably been attributed to the delay in diagnosing the disease or the use of inadequate corticosteroid dosage. Following our observation of progressive deterioration following the introduction of prednisolone use in a patient, we examined the possibility that worsening of the condition might be due to corticosteroid therapy rather than coincidence. OBJECTIVE: To determine whether corticosteroid therapy may exacerbate GCA. DESLGN: Case report and an analysis of similar cases reported in the medical literature. PATIENT: A 64-year-old man had a 3-month history of headache, night sweats, malaise and general weakness, and anorexia and weight loss and a more recent history of jaw claudication, dysphagia, and hoarseness. Clinical findings included prominent temporal arteries with absent pulsation, abnormal saccades to the right, and eyelid retraction. Laboratory findings included an elevated erythrocyte sedimentation rate and platelet count. Results of a biopsy of the temporal artery confirmed GCA. magnetic resonance imaging scans showed ischemic cerebellar lesions and a mature infarct in the left anterior occipital, posteroparietal region. Following corticosteroid therapy commencement, the patient's condition deteriorated steadily for 5 days with clinical signs suggestive of an evolving vertebrobasilar stroke. Following treatment with high-dose intravenous dexamethasone sodium phosphate and heparin sodium, his symptoms improved. DATA SOURCES: The review included analysis of autopsy-based reports in which clinical details are provided and clinical reports in which major visual or cerebral complications are described. Significant complications occurred in many cases shortly following the introduction of corticosteroid therapy. In many of these cases, the symptoms indicated that GCA had been present for a significant period prior to corticosteroid therapy. CONCLUSIONS: Progressively evolving occlusive strokes may occur following corticosteroid therapy in patients with GCA. In cerebrovascular complications, vascular occlusion occurs at sites of active vasculitis, usually within the vertebrobasilar system. It is not certain that the worsening of the condition following corticosteroid therapy is always coincidental, and an alternative possibility, namely a functional relationship between the initiation of corticosteroid therapy and clinical deterioration, should be borne in mind.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
7/48. Scleroderma-like indurations involving fascias: an abortive form of congenital fascial dystrophy (Stiff skin syndrome).Four patients are described with stone-hard indurations of the skin and subcutaneous tissue, predominantly on the buttocks and thighs, in the areas of the thickest fascia lata and glutealis. All cases were sporadic, started in early infancy, were only slightly or not progressive, and showed no visceral involvement or immunologic abnormalities. In all, the hallmark of the disease was strikingly enlarged fascia. In one patient, typical features developed progressively for 9 years, and in two patients the changes remained abortive, limited to some areas, and not symmetrical. The fourth patient showed some similarity to profound morphea with no cutaneous involvement. Recognition of atypical or abortive cases of congenital fascial dystrophy, which is probably a variant of heterogeneous stiff skin syndrome involving exclusively fascia, is of practical importance, since no therapy is required. However, intensive rehabilitation should start in early infancy and continue throughout life. The genetic defect of molecular organization of collagen in the fascia results in formation of giant amianthoid-like collagen fibrils.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
8/48. Features of severe periodontal disease in a teenager with chediak-higashi syndrome.BACKGROUND: chediak-higashi syndrome (C-HS) is a rare congenital disease characterized by defective neutrophil function with abnormal lysosomal inclusions, neutropenia, and reduced chemotaxis. The complete syndrome includes oculocutaneous albinism with photophobia, neurologic features, recurrent infections, and enterocolitis. methods: A 14-year-old male C-HS patient was referred to us because of serious periodontal destruction with acute inflamed gingiva and ulcers. Clinical and biological investigations were performed, leading to the diagnosis of C-HS. RESULTS: Laboratory findings included neutropenia and hypergammaglobulinemia. Peripheral blood smears showed giant granules in neutrophils, eosinophils, and granulocytes. bone marrow smears showed giant inclusions in leukocyte precursor cells. These granules and inclusions were characteristic of chediak-higashi syndrome. Oral radiographic status showed extensive loss of alveolar bone leading, in most cases, to tooth exfoliation. bacteria often associated with periodontitis were detected in subgingival plaque samples, including fusobacterium nucleatum, campylobacter rectus, prevotella melaninogenica, peptostreptococcus anaerobius, and clostridium sp. Biopsies of periodontal tissues for light and electronic microscopic examinations revealed massive bacterial invasion of the epithelial tissue, epithelial cells, and connective tissue. Ultrastructural observations of periodontal polymorphonuclear leukocytes showed defective granulation, with abnormal granules not discharging their lysosomal content against engulfed bacteria. Viable dividing bacteria were found in the cytoplasm. CONCLUSIONS: In this case, early-onset periodontitis seems to be the expression of C-HS granulocyte deficiency. Periodontal treatment of these patients is often unsuccessful. This case report illustrates the importance of the dentist in initiating clinical and biological investigations in such early aggressive periodontitis in young patients.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/48. Longterm follow-up of electroencephalographic and clinical findings of a case with Gaucher's disease type 3a.Among three recognised clinical phenotypes, type 3a Gaucher's disease is characterised by mild to severe systemic disease, neurological manifestations and myoclonic seizures. We report the long term clinical and electrophysiological follow-up of a 27-year old man with a diagnosis of type 3a Gaucher's disease, which was confirmed by bone marrow biopsy examination and leukocyte glucocerebrosidase level measurement. His neurological examination was normal throughout the follow-up period. EEG examination, recorded five days after the first seizure, revealed generalised nonrhythmic paroxysmal rapid spikes with occipital predominance increased by photic stimulation and normal background activity. The frequency of seizures increased from 3-4/year to 1-2/month within a follow-up period of 12 years and a repeat EEG examination on the eight year of diagnosis revealed additional background slowing. A giant potential was obtained in somatosensory evoked potential (SEP) examination. EEG findings of this case demonstrate a specific pattern with rapid spike activity, photosensitivity, eye closure sensitivity and gradual background slowing.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/48. Serial neuroimaging of a growing thrombosed giant aneurysm of the distal anterior cerebral artery--case report.An 81-year-old female presented with a giant aneurysm of the distal anterior cerebral artery (A3) which grew from a small saccular aneurysm to a huge aneurysm within 36 months before manifesting as a mass lesion. The thrombosed portion of the aneurysm showed growth, whereas the aneurysmal cavity did not change in size. Computed tomography and magnetic resonance imaging showed new bleeding in the thrombosed portion. hemorrhage into the thrombus and/or aneurysmal wall might have caused the aneurysmal growth. She refused surgery and was discharged with no deficits. Distal anterior cerebral artery aneurysm which shows neuroimaging signs of growth requires regular follow up as such lesions may become giant before manifesting clinical symptoms.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
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