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Langerhans cells are specialized cells that participate in the initiation of an immune response. These and similar cells are also found in other epithelia within the body. The pathological proliferation of Langerhans cells is called Langerhans cell histiocytosis. This condition, most often benign, is also called histiocytosis X. Langerhans cells contain a characteristic structure in their cytoplasm, the Birbeck granule. These granules are sometimes called X-bodies, hence the name 'histiocytosis X'. (The condition should not be confused with histiocytosis, which involves cell types other than Langerhans cells.) Generally, histiocytosis X follows a chronic, indolent course. Some pathologists are unsure if the condition can ever be truly malignant. In contrast to classic histiocytosis, which affects men and women with equal frequency, this disease affects males versus females six to one. The authors present data from 31 cases of histiocytosis X; among them are cases which support the existence of malignant histiocytosis X as a distinct disease. In 19 cases, the disease followed an indolent course, and examination of the involved tissues revealed cells with a benign appearance. The lymph nodes were the most commonly involved tissue, followed by bone. Skin, liver, and lung were also involved in some of these patients. In three patients of the 31 patients, the microscopic appearance of the cells was benign, but the disease followed an aggressive course, resulting in death between one and seven months. Conversely, two other patients had a benign course of disease, despite malignant appearance of cells in the involved tissues. The one patient for whom long-term follow-up is available has survived 18 years. Seven patients were seen in which the malignant appearance of the cells was matched by a malignant disease course. These patients all died after periods ranging from eight days to two years. The authors suggest that this last group of patients represents a distinct syndrome, malignant histiocytosis X. The observations made in this study also indicate that the pathological appearance of cells are imperfect indicators of the prognosis of histiocytosis X. (Consumer Summary produced by Reliance Medical Information, Inc.)

Angiosarcomas, also called hemangiosarcomas, are cancers that arise from the endothelial cells and fibroblast cells which normally make up the inside of blood vessels. These cancers are not common, and only between one and two percent of all sarcomas affecting soft tissues are angiosarcomas. Furthermore, the majority of angiosarcomas arise in the skin of the face and scalp and the underlying subcutaneous tissue. Two cases were recently seen, however, in which patients developed angiosarcomas within the abdominal cavity. The two female patients, aged 69 and 80, had suspected bowel obstruction (blockage) and underwent exploratory surgery. Surgical examination of the abdominal cavity revealed the presence of widely dispersed masses of cancer which proved to be angiosarcoma upon microscopic examination. Both patients died of complications while in the hospital. Both patients had previously undergone radiation therapy for gynecologic cancer, one 20 years previously and the other 7 years previously. Although it can not be demonstrated with certainty that the patients' angiosarcomas were caused by the exposure to radiation, there is precedent in the medical literature for the relationship between angiosarcoma and radiation. Most cases of angiosarcoma following radiotherapy involve the skin. There have, however, been previous reports of angiosarcomas arising in the abdomen years after radiotherapy, and it is suspected that in these cases, as well as in the two recent cases, this rare cancer most likely resulted from the use of radiation to treat a previous cancer. (Consumer Summary produced by Reliance Medical Information, Inc.)

It is very unusual for tumors growing in the lungs to form cavities. Consequently, there is a risk of failing to properly identify cystic tumors in the chest. This problem is illustrated by two case histories of patients with cystic metastatic sarcoma in the lungs. The cancers could not be identified by chest X-ray. The lesions could be seen on computed tomography (CT) of the chest, but they had the appearance of benign bullous disease, which involves the formation of large vesicles or bullae. Upon removal of the cysts, the lack of any solid cancerous tissue for examination, and the variability of the tissue comprising the cyst wall, made histopathological analysis difficult. The definitive diagnosis was made on the basis of specific immunochemical staining of the tumors, which were identified as a smooth muscle tumor, or leiomyosarcoma, in one patient and a sarcoma of the synovium in the other. It is clear that thin-walled bullae, which appear benign on CT scan, must be considered potentially malignant and removed surgically when appropriate. (Consumer Summary produced by Reliance Medical Information, Inc.)

Alpha-1-antitrypsin (AAT) is one of several serpin molecules which functions to balance the action of various endogenous proteolytic enzymes. A deficiency of functional levels of this enzyme is most commonly inherited in an autosomal recessive fashion. Greater than 95% of AAT-deficient persons are homozygous for the non-functional Z-variant of the enzyme. ZZ-homozygosity is relatively common, affecting 1 in 2500 persons of northern European decent. Known complications of AAT-deficiency include early-onset emphysema, childhood liver disease, cirrhosis, and increased incidence of hepatocellular carcinoma1. Although AAT-deficiency is frequently diagnosed in early childhood, it is not uncommon for persons to go undiagnosed for many years until otherwise unexplained lung or liver disease become evident. We report a case of AAT-deficiency in a 44-year-old female which became evident post-operatively after unexplained, uncontrollable ascites and respiratory failure. Keywords: Alpha-1-antitrypsin deficiency: serpinopathy: panacinar emphysema: ascites, Introduction Alpha-1-antitrypsin (AAT) is produced primarily in hepatocytes and functions to counteract neutrophil elastase and various other proteolytic enzymes in the lung. The discovery of early emphysematous changes in young, [...]