Giant gastric trichobezoar

Bezoars are relatively common foreign body lesions of the stomach and intestine. Small bezoars can be treated using non-surgical methods, but large bezoars usually require open surgery. Occasionally they are giant-sized and may mimic an intra-abdominal mass clinically. We present the case of a 24-year-old woman with a giant gastric trichobezoar treated by open surgery; treatment methods are discussed.     

Solitary juxtacortical neurofibroma of the humerus

A neurofibroma as a discrete, superficial tumor of a long bone has been described only once in the world literature. We report the radiologic findings of a case of a juxtacortical neurofibroma.     

Serum IgD concentrations in immunodeficiency diseases

Serum IgD levels were determined in 66 patients with well-defined primary immunodeficiency diseases. The two major groups of patients consisted of those with ataxia-telangiectasia (38 patients) and those with selective IgA deficiency (11 patients). The ataxia-telangiectasia patients tended to have higher serum IgD levels while no significant difference was found in the serum IgD levels of the selective IgA deficiency patients when compared with the controls. No correlation was found between the IgD levels and the presence of frequent infections in both patient groups or the associated disorders present in the selective IgA deficiency patients. The percentage of low serum IgD phenotype in the normal subjects was similar to that described in the literature.     

Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency

Mutations of the Activation-Induced Cytidine Deaminase (AID) gene have been found in patients with autosomal recessive hyper-IgM (HIGM) syndrome type 2. We retrospectively analyzed clinical, immunologic and genetic characteristics of 29 patients from 22 families with AID deficiency. Patients' median age at diagnosis and at last evaluation was 4.9 years (range: 0 to 53) and 14.2 years (range: 2.7 to 63), respectively. Most patients had suffered from recurrent and severe infections, however, intravenous immunoglobulin (IVIG) replacement therapy resulted in a dramatic decrease in the number of infections. Lymphoid hyperplasia developed in 22 patients and persisted in 7 at last follow-up. It is striking to note that six patients developed autoimmune or inflammatory disorders including diabetes mellitus, polyarthritis, autoimmune hepatitis, hemolytic anemia, immune thrombocytopenia, Crohn's disease and chronic uveitis. Fifteen distinct AID mutations were found but there was no significant genotype-phenotype correlation. In conclusion, AID-deficient patients are prone to infections and lymphoid hyperplasia, which may be prevented by early-onset IVIG replacement, but also to autoimmune and inflammatory disorders.     

Carpal bone cysts: MRI,gross pathology,and histology correlation in cadavers

PURPOSE

Intraosseous cysts of carpal bones are frequently observed on routine imaging examinations of the wrist. There is controversy regarding the underlying pathogenesis of these cysts. In this study, we aimed to investigate the magnetic resonance imaging (MRI) appearance of intracarpal bone cysts in correlation with histologic analysis, using cadaveric wrists.

METHODS

Five freshly frozen cadaveric wrist specimens (from three women and two men; mean age at death, 80 years) were studied. Imaging was performed with T1-weighted fast spin-echo, and proton density-weighted fast spin-echo with and without fat-suppression. The existence of cysts was confirmed by comparing MRI and histology findings. Hematoxylin and eosin stain was performed on tissue slices of 3 mm thickness to analyze the structure of cysts and their communication with the joint cavity.

RESULTS

Ten cysts were observed. In all cases, cysts were eccentrically located either in the subchondral bone or beneath the cortex. On histologic examination, there were regions of fat necrosis without inflammation or increased vascularity, surrounded by fibrous walls. There were no giant cells, cholesterol granules, or a true synovial lining. Mucoid change was rare. Fibrous component of cysts varied from small fibrous septa to well-formed walls. Some cysts communicated with the joint cavity. Two cysts were adjacent to ligamentous attachments. Those cysts with fibrous tissue demonstrated variable hypointensity on T2.

CONCLUSION

In contrast to previous reports that described a mucoid composition of intracarpal bone cysts with occasional foamy macrophages, our observations support the concept that these lesions reflect a spectrum of fat necrosis and fibrous changes, without inflammation or hypervascularity. These cysts are typically surrounded by fibrous walls without a true synovial lining.Intraosseous cysts of the carpal bones are commonly identified as an incidental finding on routine imaging examinations of the wrist, particularly in older patients. Although the clinical significance of these cysts is variable, the majority are asymptomatic. In contrast, “intraosseous ganglion cysts”, as described by previous investigators, are painful, with histopathologic features that are identical to those of soft-tissue ganglion cysts; and they are surrounded by a sclerotic rim without a synovial lining or overlying cartilage pathology (1–3). In several case reports, these intraosseous ganglion cysts have been described to have a soft-tissue component. Some of these cysts have been characterized as mucoid in nature, filled with mucoid material and located in close proximity to the carpal insertion of the joint capsule and intrinsic or extrinsic ligaments. Other authors have considered true ganglion cysts of the carpus to be rare and pathologically distinct from degenerative cysts (2).This has let to inconsistencies in the nomenclature, with various terms being used to describe them such as intraosseous ganglion, intraosseous ganglion cysts, intraosseous bone cysts, juxta-articular bone cysts, subchondral bone cysts, and subchondral synovial cysts (1, 4–6). There are multiple hypotheses regarding the underlying pathogenesis of these cysts. Potential causes include intramedullary metaplasia of mesenchymal cells into synovium-like cells, ischemic bone necrosis with resultant resorption of the necrotic material, or cortical penetration of a previously existing soft-tissue ganglion cyst.In this study, the magnetic resonance (MR) imaging (MRI) appearance of carpal bone cysts is correlated with histologic analysis using cadaveric wrist specimens derived from persons who were elderly at the time of death, to further characterize the imaging as well as histologic features of these controversial, yet frequently encountered, lesions.     

Does fibrin glue cause foreign body reactions?

The study was performed in 20 Wistar rats. Approximately 4 mm perforations were created in the left colon of ten rats to which fibrin glue was applied (Group D). The control group had the same injury without any therapeutic or surgical measures (Group K). Of the 20 rats in both groups none died during the postoperative period. All animals sacrificed on the 11th postoperative day. All ten rats of Group D displayed inflammatory foreign body reactions. The control group showed no such reaction.     

Cyclic neutropenia complicated by renal AA amyloidosis

Cyclic neutropenia is a rare disease characterized by regular cyclic fluctuations in the numbers of neutrophils. Patients with the disease suffer from recurrent infections at regular intervals of nearly three weeks. Recently, recombinant human granulocyte colony-stimulating factor (rhG-CSF) was reported to be an effective treatment for this disease. here we describe 17-year-old cyclic neutropenic female patient with a very rare association of renal amyloidosis of AA type who was under intermittent rhG-CSF treatment for the previous one and a half years. We conclude that although the disorder is usually benign, reactive amyloidosis may rarely develop in cases who remain untreated for a long period of time. However familial Mediterranean fever (FMF) type II should also be born in mind, particularly in predisposed populations.     

Coexistence of pleomorphic xanthoastrocytoma with Sturge-Weber syndrome: MRI features

Pleomorphic xanthoastrocytoma is a rare, cortically based, partially cystic astroglial tumour with a strongly enhancing solid mural nodule. We report an 11-year-old boy with a pleomorphic xanthoastrocytoma in the contralateral hemisphere to that affected by Sturge-Weber syndrome. This unique case supports the association between Sturge-Weber syndrome and astrocytomas. Patients with Sturge-Weber syndrome who experience unexpected neurological symptoms should be evaluated with MRI.