1/198. Pulmonary lymphangiomyomatosis: three new cases studied with electron microscopy.Three cases of pulmonary lymphangiomyomatosis are described, with emphasis on the ultrastructural changes. The clinicopathologic features corresponded to those previously described. Each patient was a female in the reproductive years; breathlessness and recurrent pneumothoraces were the predominant clinical features. Histologically, the lungs showed a focal interstitial infiltrate of short, spindle-shaped mononuclear cells compatible with primitive smooth muscle, which was associated with irregular emphysema and hemosiderosis. Electron microscopy confirmed the smooth muscle nature of the pulmonary infiltrate and showed the presence of cells intermediate between smooth muscle and fibroblasts. Abnormalities were also noted in the pulmonary connective tissue that are possibly related to the fragility of the lung in this condition.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/198. Epstein-Barr virus-associated leiomyosarcoma of the thyroid in a child with congenital immunodeficiency: a case report.We report an unusual case of multifocal leiomyosarcoma involving the thyroid gland, liver, and right lung in a child with congenital immunodeficiency disease. The smooth muscle nature of these neoplasms was confirmed by immunohistochemistry and electron microscopic studies. in situ hybridization showed large amounts of Epstein-Barr virus messenger rna within the tumor cells. Although Epstein-Barr virus-associated smooth muscle tumors have been reported in children with AIDS and after organ transplantation, we are unaware of any case report in congenital immunodeficiency disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/198. Pulmonary lymphangitis carcinomatosa and acute pancreatitis: a rare presentation of choledochal cyst.Pulmonary lymphangitis carcinomatosa is an unusual cause of death in a young adult. This case describes an apparently healthy young woman who presented with severe acute pancreatitis, which is a recognized complication of a choledochal cyst. autopsy examination revealed advanced malignancy with poorly differentiated adenocarcinoma penetrating the wall of the choledochal cyst and metastatic adenocarcinoma in the lymph nodes, lungs and kidneys. This case emphasises the unusual presentation of a choledochal cyst with acute pancreatitis and the aggressive nature of malignancy associated with this congenital anomaly.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/198. Malignant triton tumor of the head and neck: A case report and review of the literature.BACKGROUND: Malignant triton tumor (MTT) is a relatively rare, aggressive tumor comprised of both malignant schwannoma cells and malignant rhabdomyoblasts. Because MTT frequently arises in the head and neck, the otolaryngologist must be aware of the nature of the tumor and its response to various treatment modalities. METHOD: This article reviews the treatment and outcome of all reported cases of MTT arising in the head and neck. CONCLUSIONS: Although statistical analysis is limited by the short duration of follow-up of many patients, complete tumor resection appears to carry an improved chance of survival. Adjuvant radiation and chemotherapy may also improve survival, although a benefit of these therapies was not well demonstrated in this small series.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/198. Pulmonary angiomyolipoma and multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis.A 36-year-old woman with a long-standing diagnosis of tuberous sclerosis was found dead. A yellow-tan 0.4 cm-diameter pulmonary tumor was identified at autopsy which had typical microscopic features of an angiomyolipoma (AML). Immunohistochemical stains showed reactivity for actin, but not HMB-45, Melan-A, and tyrosinase (despite reactivity of the patient's renal AML for HMB-45 and Melan-A), perhaps owing to the small size of the lesion and the sometimes focal nature of the reactivity for these markers. Additional lung nodules proved to be multifocal micronodular pneumocyte hyperplasia. This report highlights the first occurrence of a pulmonary angiomyolipoma in the setting of tuberous sclerosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/198. Angiofollicular lymph node hyperplasia of the lung simulating carcinoma: pseudolymphoma with inflammatory changes.Angiofollicular lymph node hyperplasia of the lung developed in a 45-year-old man who was a heavy smoker. Slow growth of the lesion 3 years after biopsy and the finding of atypical cells in the sputum and bronchial washings strongly suggested a malignant lesion. There were no systemic manifestations other than dyspnea, which may have been related to allergy. The lesion was angiofollicular lymph node hyperplasia of the hyaline-vascular type. Invasion by lymphoid cells of both blood vessels and bronchioles suggested that the pathogenesis was probably infectious or inflammatory; also, the nature of the infiltrate indicated an immune mechanism. The course of the disease, as in other cases reported, has been benign, though a patchy density has developed in contralateral lung 4 years after lobectomy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/198. Low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes): report of a case that metastasized."Hyalinizing spindle cell tumor with giant rosettes" is a tumor recently described by Lane et al. and thought by them possibly to represent a form of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. We report an example of this tumor on the arm of a 28-year-old woman. The ultrastructural study of the tumor confirmed the fibroblastic nature of the lesion, which subsequently metastasized to the lung. Histologically, the giant rosettes simulated palisaded granulomas. Our findings warrant classifying the tumor as a sarcoma, and we suggest the designation low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes).- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/198. The multiple sclerotic osteogenic sarcoma of early childhood.Two cases of sclerosing osteogenic sarcoma that occurred in early childhood are reported. They and the similar cases reviewed are unusual in their development at an unusually early age, their multicentric presentation and their densely sclerotic nature. These are uncommon manifestations of osteogenic sarcoma and are considered due to proliferative metastatic osteoid formation in areas of rapid bone growth.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/198. Concurrence of sarcoidosis and lung cancer. A report of four cases.Although sarcoidosis and lung cancer are both frequently encountered conditions, their simultaneous occurrence in the same patient is unusual. In this report, we describe 4 cases of their concurrence and discuss the possible pathogenic mechanisms of their concurrent appearance. In particular, in 2 of the cases, both diseases had coexisted for a long period (more than 6 and 4 years, respectively), showing a surprisingly slow growth of cancers. Although the chest computed tomography showed hilar and mediastinal lymphadenopathy, the histopathological findings of the excised lymph nodes of both cases revealed no metastasis. The causal relationship between sarcoidosis and lung cancer remains uncertain, but cases such as these may be helpful in elucidating its precise nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/198. New Purkinje cell antibody (PCA-2): marker of lung cancer-related neurological autoimmunity.Neuron-restricted autoantibodies are important markers of neurological autoimmunity related to cancer. We identified a new paraneoplastic IgG, PCA-2 (Purkinje cell cytoplasmic antibody type 2), in 10 patients. Nine had mixed subacute neurological presentations (5 brainstem or limbic encephalitis, 3 cerebellar ataxia, 2 lambert-eaton myasthenic syndrome, 1 autonomic neuropathy, and 1 motor neuropathy). All 9 were smokers, and 8 had definite or probable lung cancer (7 with biopsy-confirmed small cell lung carcinoma [SCLC]; 1 imaged only). One patient had no follow-up information. A 10th patient was among 58 with uncomplicated SCLC. PCA-2 binds to a cytoplasmic antigen in neurons and SCLC cells. Its immunostaining pattern in mouse tissues is distinct from that of the paraneoplastic autoantibodies PCA-1 (anti-Yo, marker of immune response initiated by ovarian or breast carcinoma) and PCA-Tr (anti-Tr, immune response marker of Hodgkin's lymphoma). PCA-2 binds to cerebellar Purkinje somata and dendrites, neurons in internal granular layer and dentate nucleus, and neuronal elements in gut and kidney. Western blots of reduced/denatured cerebellar and SCLC proteins reveal a common antigenic band, of approximately 280 kd. PCA-2 is the seventh IgG neuronal autoantibody marker of paraneoplastic autoimmunity identifiable unambiguously by standardized immunofluorescence criteria.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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