Cystic synovial sarcoma.

A case of synovial sarcoma of the paraspinal region showing massive cystic changes is described. The tumor presented in a 53-year-old white woman who noticed a lump on the upper aspect of her back. Magnetic resonance imaging of the cervical spine showed a heterogeneous paraspinal mass with well-defined margins and a multilocular quality with foci of hemorrhage. Fine needle aspiration of the mass showed clusters of polygonal cells admixed with a scattered spindle cell component. Surgical excision of the mass showed a well-circumscribed but nonencapsulated tumor that showed multiple small cystic structures on cross-section. Histologic examination showed a biphasic neoplasm characterized by bland-appearing glandular elements embedded in a moderately cellular spindle cell stroma. The tumor contained multiple cysts of varying size. Immunohistochemical studies showed the glandular component to be positive for cytokeratin and epithelial membrane antigen. The spindle cell component was immunoreactive for cytokeratin, vimentin, bcl-2, and CD99. Stains for muscle-specific actin, smooth muscle actin, S-100 protein, and CD34 were negative. Cytogenetic analysis showed a balanced reciprocal translocation involving chromosomes X and 18, in addition to other clonal abnormalities. Synovial sarcoma should be considered in the differential diagnosis of cystic lesions involving the soft tissues. Magnetic resonance imaging is considered the procedure of choice for the evaluation of soft tissue tumors because of its superior soft tissue contrast and multiplanar imaging capability. While the imaging features of soft tissue tumors are often nonspecific, magnetic resonance imaging may provide helpful clues, thus narrowing the differential diagnosis. Immunohistochemical studies and cytogenetic analysis may be very helpful for establishing the correct diagnosis in cases with this unusual presentation. Ann Diagn Pathol 5:48-56, 2001.

Cardiac manifestations of Weber-Christian disease: report and review of the literature.

A patient with chronic systemic Weber-Christian disease who was treated with hydroxychloroquine developed cardiac dilatation with congestive heart failure. Endomyocardial biopsy demonstrated myocyte degeneration and interstitial fibrosis, but no typical features of chloroquine induced cardiomyopathy. Clinical symptoms of congestive heart failure also were recognized in 7 of the 11 reported autopsy cases of Weber-Christian disease having cardiac involvement. This involvement can affect the pericardium and the myocardium.

Azathioprine induced hepatic veno-occlusive disease in rheumatoid arthritis.

A patient with rheumatoid arthritis developed hepatic veno-occlusive disease following the use of azathioprine. Although azathioprine induced veno-occlusive disease is suspected to occur more frequently in patients with autoimmune dysfunction, it has not previously been reported as a complication of treatment in rheumatoid arthritis. The mechanism responsible for this condition remains unknown.

Therapeutic strategies in autoimmune disease: cyclosporin A as a model agent.

Cyclosporin A is the derivative of the cyclic undecapeptide which is produced by 2 strains of fungi, Cylindrocapon lucidum Booth and Tolypocladium inflatum Gams. The drug has achieved widespread use in the treatment of organ transplant recipients as a vanguard against rejection, and currently is being evaluated as an immunosuppressive agent for the therapy of primary autoimmune phenomena as well. Its mechanism of action, and application to the treatment of rheumatologic disorders will be discussed.

Granulomatous musculoskeletal disease: sarcoidosis versus tuberculosis.

A patient with systemic sarcoidosis insidiously developed tuberculous arthritis. The concurrence of these diagnoses in any individual patient is rare. Musculoskeletal features of sarcoidosis and tuberculosis are reviewed, and the importance of obtaining appropriate cultures to differentiate these granulomatous disorders is illustrated.

Sterile splenic abscesses in systemic Weber-Christian disease. Unique source of abdominal pain.

A patient with Weber-Christian disease and relapsing abdominal pain due to sterile splenic abscesses is presented. Histologically, acute and chronic inflammation with focal suppurative infiltrates of phagocytosed fat was detected in the absence of vascular or embolic disease, infection, or other apparent cause. Abdominal discomfort, an uncommon manifestation of Weber-Christian disease, usually is related to mesenteric panniculitis. Sterile splenic abscesses represent a previously undescribed complication of Weber-Christian disease, and another source of abdominal pain in this disorder. Although the precise pathophysiology of sterile splenic abscess formation in Weber-Christian disease is unknown, splenectomy is an effective means of alleviating pain.

Rheumatoid-like arthritis presenting as idiopathic pulmonary hemosiderosis: a report and review of the literature.

Idiopathic pulmonary hemosiderosis, an unusual form of the alveolar hemorrhage syndromes, has been reported in association with several connective tissue disorders. Rheumatoid arthritis (RA), however, rarely presents in this manner. We discuss a young adult with recent onset RA following idiopathic pulmonary hemosiderosis, and review the 3 cases previously reported.

Bacterial meningitis in Navojo Indians.

An analysis of 219 confirmed cases of bacterial meningitis among Navajo Indians during a 5-year period, July 1, 1968, through June 30, 1973, revealed that 56 percent were caused by Haemophilus influenzae, 26 percent by Neisseria meningitidis, 6 percent by Mycobacterium tuberculosis, and 6 percent by other organisms. The annual incidence of H. influenzae meningitis (17.7 per 100,000 persons) and that of pneumococcal meningitis (8.0 per 100,000) were much higher than the rates for these diseases reported from other population groups. The annual incidence of meningococcal meningitis (2.0 per 100,000) was similar to that found elsewhere. There was an ususual concentration of cases during the first year of life; 78 percent of H. influenzae, 64 percent of pneumococcal, and 50 percent of meningococcal meningitis occurred during this time. However, bacterial meningitis during the first month of life was not frequent (0.29 per 1,000 live births). Case fatality rates were similar to those reported for other population groups.