1/30. Wegener's granulomatosis triggered by infection?Wegener's granulomatosis is a systemic disease of unknown origin, although recent studies suggest that auto-immune mechanisms and infection play a role in the pathogenesis of this disease. Wegener is characterized by a necrotizing vasculitis involving the lungs (pulmonary infiltrates), the upper airways and the kidneys (rapidly progressive glomerulonephritis). We present a case of a male patient admitted because of progressive deterioration of the general condition with weight loss, a unilateral neck mass, unilateral purulent rhinorrea and fever. CT-scan evaluation demonstrated a unilateral expanding mass in the sing-nasal cavity, obliterating the ethmoid complex. MRI revealed signs of intracranial inflammatory reaction and onset of absedation. A malignancy was suspected but a diagnosis of Wegener's granulomatosis was established based on histologic criteria (nasal biopsy) and a positive titer for anti-cytoplasmic antibodies (cANCA). During follow-up, nasal carriage of Staphyloccocus Aureus could be documented. An overview of Wegener's granulomatosis will be provided with emphasis on the potential role of acute infections as a trigger for Wegener's granulomatosis and the head and neck manifestations.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/30. Differential diagnosis of midline facial granulomas.The list of differential diagnoses in the case of midline facial granulomatous lesions is long. Intensive investigation into the true origin of disease must be done before definitive treatment can be begun. Extensive and repeated biopsies are necessary. The diagnosis may narrow down to the nonspecific midline lethal granuloma. Evidence in the literature coupled with our experience strongly indicates that this disease does not exist as a pathologic entity but merely as a clinical syndrome which upon further evaluation will reveal either Wegener's granulomatosis or a reticulum cell neoplasm. Treatment of the syndrome consists of corticosteroid drugs, irradiation, and chemotherapy until a definitive diagnosis is made. Midline granulomas have been and will continue to be a challenging diagnostic and therapeutic problem for the otolaryngologist.- - - - - - - - - - ranking = 1.204292460786keywords = neoplasm (Clic here for more details about this article) |
3/30. Atypical squamous cells as a diagnostic pitfall in pulmonary Wegener's granulomatosis. A case report.BACKGROUND: Wegener's granulomatosis (WG) is characterized by systemic, necrotizing, granulomatous inflammation accompanied by vasculitis. It classically involves the triad of the upper respiratory tract, lungs and kidneys. Isolated pulmonary lesions of WG may present in some patients as pulmonary masses, simulating neoplasms. The features of WG can be suggested by cytologic study. Atypical epithelial cells associated with WG have previously been reported as a cause of a false positive diagnosis of bronchoalveolar carcinoma. CASE: In this case the cytologic findings included atypical squamous cells in a background of acute, chronic and granulomatous inflammation. In several respiratory specimens the atypical squamous cells were incorrectly interpreted as diagnostic of squamous cell carcinoma. The correct diagnosis of WG was confirmed with open lung biopsy, which demonstrated necrotizing granulomatous inflammation with geographic necrosis and associated vasculitis. CONCLUSION: Markedly atypical squamous cells mimicking squamous cell carcinoma can be found accompanying the inflammatory process associated with WG and are a possible diagnostic pitfall. The possibility of WG as well as other inflammatory processes should always be considered in the differential diagnosis of squamous cell carcinoma of the lung. This case is the only reported case of WG in which atypical squamous cells were a diagnostic pitfall, initially suggesting a diagnosis of squamous cell carcinoma.- - - - - - - - - - ranking = 1.204292460786keywords = neoplasm (Clic here for more details about this article) |
4/30. Aggressive destructive midfacial lesion from cocaine abuse.Since the first reported case in 1912 of cocaine-induced perforation of the palate, an additional 7 cases have been reported describing extensive palatal destruction. The clinical presentation shares similarities with nasal-type natural killer/T-cell lymphoma, Wegener's granulomatosis, and infectious diseases. We describe a 50-year-old woman with a progressively destructive midfacial process that initially appeared as a small, localized palatal defect. Over time, the lesion caused bilateral deformity of the ala, extensive loss of the palate, maxillary and sinonasal complexes, ethmoids, and ulceration of adjacent tissue. Clinical laboratory tests showed elevated cytoplasmic-antineutrophil cytoplasmic antibodies, but the histopathology did not support the diagnosis of Wegener's granulomatosis. Special stains and cultures were negative for infectious organisms. flow cytometry and T-cell gene rearrangement studies ruled out lymphoma. Because of the inability to diagnose this worrisome process, the presence of polarizable foreign material in the original biopsy, and the patient's admission to past cocaine use, a urine drug screen was performed, which was positive for cocaine and marijuana.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/30. Orbital inflammatory pseudotumor due to hypersensitivity vasculitis and mononeuritis multiplex in a patient with atypical, cANCA-positive Wegener's granulomatosis.OBJECTIVE: We report on a 60-year-old woman with a retro-orbital pseudotumor and polyneuropathy. The retro-orbital inflammation was histologically diagnosed as hypersensitivity vasculitis (HV). As cytoplasmatic antineutrophilic cytoplasmatic antibody (cANCA) and anti-proteinase-3 antibody were detected, the differential diagnosis also included atypical Wegener's granulomatosis. hypersensitivity vasculitis is defined as small-vessel vasculitis mediated by the deposition of immune complexes (arthus reaction) after exposure to various agents such as drugs, toxins, and infections. Since an inflammatory retro-orbital pseudotumor due to HV has not previously been reported, the following case is presented. methods AND MAIN OUTCOME MEASURES: magnetic resonance imaging (MRI) revealed retro-orbital infiltrate without granuloma. histology from an orbital biopsy confirmed HV. electromyography was used for the diagnosis of polyneuropathy. Serum investigation indicated erythrocyte sedimentation rate (ESR) >100 mm/h, c-reactive protein (CRP) 223 mg/l, antinuclear antibodies 1:80, and cANCA 100 U/ml. RESULTS: The bilateral orbital pseudotumor, polyneuropathy, and serum levels of inflammation reactants (ESR and CRP) improved from therapy with corticosteroids (1 g of methylprednisolone initially) and azathioprine (150 mg/day). CONCLUSIONS: Because of cANCA and anti-proteinase-3 antibody positivity, this case can be viewed more as an atypical Wegener's granulomatosis than a systemic HV. The causal variety of inflammatory orbital pseudotumor, including HV and different therapeutic consequences, requires histological differentiation from usual orbital pseudotumors.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/30. leiomyosarcoma of urinary bladder following cyclophosphamide therapy: report of two cases.leiomyosarcoma of urinary bladder is rare, although it is the most common mesenchymal tumor in adults. We report two cases of this tumor following cyclophosphamide therapy. The first case is from a 53-year-old man with Wegener's granulomatosis treated for 6 years with cyclophosphamide. He presented with painless hematuria, and the initial biopsy of the bladder tumor revealed a malignant spindle cell neoplasm. A final diagnosis of leiomyosarcoma was made on radical cystoprostatectomy. The second example is from a 21-year-old man who had received cyclophosphamide in early infancy for a bilateral retinoblastoma. He also presented with painless hematuria, and a bladder tumor was resected transurethrally and diagnosed as leiomyosarcoma. He underwent partial cystectomy two months later. cyclophosphamide, when used for a neoplastic or non-neoplastic condition, is associated with an increased risk of developing bladder cancer. The distribution of histologic subtypes differs from that seen in spontaneous bladder tumors. A review of the literature shows an increased proportion of squamous cell carcinomas and sarcomas, especially leiomyosarcomas in cyclophosphamide exposed patients. acrolein, a cytotoxic metabolite of cyclophosphamide excreted in urine, is regarded as the most likely causative agent.- - - - - - - - - - ranking = 1.204292460786keywords = neoplasm (Clic here for more details about this article) |
7/30. Wegener's granulomatosis in a patient with a rheumatoid arthritis.A 38-year-old woman with rheumatoid arthritis who developed Wegener's granulomatosis is described. Wegener's granulomatosis appeared with saddle nose, perforation in her nasal septum, and granuloma in the nasal cavity. Laboratory evaluation showed a positive rheumatoid factor and circulating immune complex. Radiographic examination revealed ankylotic changes in both wrist and elbow joints. Bilateral anosmia and other disease manifestations completely responded to treatment with oral cyclophosphamide and prednisolone.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
8/30. carcinosarcoma of the urinary bladder following cyclophosphamide therapy: evidence for monoclonal origin and chromosome 9p allelic loss.We report a case involving a 45-year-old man with a 12-year history of wegener granulomatosis, who developed a carcinosarcoma of the urinary bladder after long-term cyclophosphamide therapy. cyclophosphamide is well recognized as an etiologic agent for urothelial carcinoma of the urinary bladder. However, only 5 cases of carcinosarcoma of the urinary bladder following cyclophosphamide therapy have been reported. We used loss of heterozygosity studies and microsatellite markers to define the molecular basis of this rare neoplasm. These studies revealed evidence supporting a monoclonal origin for the 2 components of this tumor. We also demonstrated allelic loss of chromosome 9p. This loss associated with carcinosarcoma of the urinary bladder is in agreement with previous studies, suggesting a possible role for the tumor suppressor gene p16 in the pathogenesis of this tumor.- - - - - - - - - - ranking = 1.204292460786keywords = neoplasm (Clic here for more details about this article) |
9/30. Current status of outcome measures in vasculitis: focus on Wegener's granulomatosis and microscopic polyangiitis. Report from OMERACT 7.The complexity of assessing disease activity, disease status, and damage in the vasculitides reflects the multisystemic pathologic manifestations of these often chronic illnesses. Major progress has been made in the past decade in the development of validated and widely accepted outcome measures for use in clinical trials. Over time, these tools have been regularly revised, expanded, and supplemented with new measures of disease prognosis and damage. As a result clinical research in this area has become increasingly complex. This article critically reviews the current status of tools for assessing disease activity and damage in "ANCA-associated" vasculitides (Wegener's granulomatosis and microscopic polyangiitis), summarizes the current level of validation of each measure, addresses central problems and controversies to be considered during development of new vasculitis assessment tools, and proposes a series of research agendas for consideration by the vasculitis research community.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
10/30. Pulmonary complications of combination therapy with cyclophosphamide and prednisone.Oral cyclophosphamide and prednisone are standard treatment for some neoplasms and necrotizing systemic vasculitis and are advocated with increasing frequency for idiopathic interstitial lung disease. During a 15-month period, we observed four cases of acute respiratory failure from pneumocystis carinii pneumonia (PCP) in patients treated with oral cyclophosphamide and prednisone. One patient each had polyarteritis nodosa, Wegener's granulomatosis, bronchiolitis obliterans with organizing pneumonia, and chronic lymphocytic leukemia with red blood cell aplasia. hypoalbuminemia (serum albumin level less than 3.0 g/dl) and daily therapy were associated with increased risk for development of PCP (p less than 0.05). None of the patients had leukopenia (less than 3,500/cu mm) or neutropenia (less than 1,000/cumm) at diagnosis. All were negative for the human immunodeficiency virus. patients receiving oral cyclophosphamide and prednisone may be at higher or increasing risk for PCP. A high index of suspicion and aggressive evaluation for opportunistic infection are needed in these patients; consideration for trimethoprim-sulfamethoxazole prophylaxis and development of more quantitative measures of immunosuppression are needed.- - - - - - - - - - ranking = 1.204292460786keywords = neoplasm (Clic here for more details about this article) |
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