1/120. Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour.A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/120. dermatomyositis in association with transitional cell carcinoma of the bladder.We report a case of dermatomyositis occurring in association with transitional cell carcinoma of the bladder. The case illustrates the importance of a thorough search for neoplasms in elderly patients with dermatomyositis and is a reminder that bladder cancer may be a rare cause of dermatomyositis. The case also shows that successful treatment of an underlying tumour may lead to resolution of paraneoplastic dermatomyositis, and relapse of cutaneous and muscle symptoms and signs may indicate recurrence of tumour.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/120. Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/120. Constrictive bronchiolitis obliterans and paraneoplastic pemphigus.Constrictive bronchiolitis obliterans is rare, and the pathogenesis of the disease often remains unknown. This study reports on the case of a 38 yr-old female with constrictive bronchiolitis obliterans and paraneoplastic pemphigus associated with malignant lymphoma. The patient developed progressive obstructive lung disease. The chest radiograph showed almost normal lungs. Paraneoplastic pemphigus is a newly described syndrome in which patients have autoantibodies binding to some epithelia, including in the respiratory tract. The disease develops in association with non-Hodgkin's lymphomas or other malignant neoplasms. The case presented here suggests that constrictive bronchiolitis obliterans associated with paraneoplastic pemphigus may be one of the facets of autoimmune responses in this context.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/120. Paraneoplastic pemphigus: a case of long-term survival associated with systemic lupus erythematosus and polymyositis.A 35-year-old man presented with a generalized bullous eruption and oral ulceration. He had been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, direct and indirect immunofluorescence and immunoprecipitation studies confirmed a diagnosis of paraneoplastic pemphigus (PNP). Initially several medical treatments were tried unsuccessfully. The pelvic tumor was surgically removed and the mucocutaneous lesions slowly regressed. Four years after presentation, he developed polymyositis which was completely controlled with short courses of corticosteroids. There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clinical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients with benign-neoplasm-associated PNP may undergo complete remission of the autoimmune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lupus erythematosus and polymyositis.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/120. Paraneoplastic vasculitis associated with esophageal carcinoma.We report a case of esophageal carcinoma associated with paraneoplastic vasculitis. A 69-year-old man suffered from low-grade fever and numbness of the lower limbs for 3 months before esophageal and gastric carcinomas were detected. Concurrent infection or collagen disease was ruled out following clinical and laboratory examinations. In April 1996, the gastric carcinoma was completely removed by endoscopic mucosal resection, but the symptoms remained. Three weeks later esophagectomy was performed for esophageal carcinoma after which time the fever and numbness disappeared. The esophageal carcinoma was a well-differentiated squamous cell carcinoma invading into the submucosal layer. Twenty-two lymph node metastases were found in 68 resected lymph nodes. Latent thyroid cancer was found. Histologically, vasculitis was detected in the esophagus, stomach and serratus anterior muscle. The distribution and degree of vasculitis were most pronounced in the esophagus. The concurrent onset and spontaneous resolution of fever and numbness after the removal of the esophageal carcinoma suggested a paraneoplastic origin. The majority of patients with malignant neoplasm-associated vasculitis had hematologic neoplasms. Cases of esophageal carcinoma associated with paraneoplastic vasculitis are extremely rare.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
7/120. adult-onset angiofibroma and multiple endocrine neoplasia type I.multiple endocrine neoplasia type 1 (men 1) predisposes affected persons to neoplasms of the parathyroid glands, the endocrine pancreas, the anterior pituitary, and the duodenum. We report the first case of adult-onset multiple angiofibromas of the central face associated with men 1. Seven siblings also developed adult-onset angiofibromas, none with evidence of tuberous sclerosis. Basic fibroblast growth factor (BFGF) is elevated in many patients with men 1 and may play a pathogenetic role.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/120. Metastatic paraganglioma and paraneoplastic-induced anemia in an adolescent: treatment with hepatic arterial chemoembolization.Mediastinal paragangliomas are rare neoplasms in children. anemia, as a paraneoplastic syndrome, has been described in adults with metastatic paraganglioma. The management of paraneoplastic anemia from metastatic paraganglioma has been problematic, with no reports in the literature describing successful treatment. This article describes a 17-year-old Jehovah's Witness with a mediastinal paraganglioma, hepatic metastases, and severe anemia. The patient and his family refused blood products and the anemia was refractory to erythropoietin and elemental iron therapy. Serial chemoembolization of the hepatic lesions resulted in resolution of the anemia, allowing subsequent debulking of the mediastinal paraganglioma.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/120. Paraneoplastic pemphigus associated with bronchiolitis obliterans.Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. autoantibodies of PNP bind to the plakin family of cytoplasmic proteins and desmogleins of cell-surface target antigens. We describe a 36-year-old female patient with PNP who had non-Hodgkin's lymphoma, and who developed bronchiolitis obliterans and died of respiratory failure. autopsy findings confirmed luminal narrowing of bronchioles by scarring, which is a histopathologic features of bronchiolitis obliterans. After the onset of respiratory failure, the reaction of autoantibodies against the plakins detected by immunoprecipitation at the onset of PNP disappeared with negative immunofluorescence within the bronchial epithelium. It is thought that autoantibodies against some of these antigens play a role in causing acute inflammation of the respiratory epithelium. In treating PNP, the possibility of the patient developing the lethal complication bronchiolitis obliterans should be kept in mind. Furthermore, prevention of the initial autoantibody-mediated injury to the respiratory epithelium should be an important treatment goal.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/120. Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure.A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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