Patients with an inflammatory bowel disease and narcotic dependence.
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Adult↗
Biomedical subjects
D Present
Publications and source records attributed to D Present.
The IBD1 locus for susceptibility to Crohn's disease has a greater impact in Ashkenazi Jews with early onset disease.
OBJECTIVE: Recent studies have suggested that a susceptibility gene located on chromosome 16 and designated IBD1 may contribute to the development of Crohn's disease (CD). However, these findings were observed in predominantly non-Jewish populations; in the three studies where Ashkenazi Jews were included for analysis, the results have been widely divergent. Because Ashkenazi Jews are known to have a higher incidence of the disease than non-Jews, we sought to determine whether this previously reported linkage could be extended to the Ashkenazi population. In addition, we examined whether Ashkenazi Jewish patients with an early age of onset (< or = 21 yr) showed greater evidence of linkage to this locus. METHODS: Linkage analysis for the IBD1 region was performed on 123 Ashkenazi Jewish CD patients distributed among 53 families. Only patients with four Jewish grandparents were considered to be Jewish. Of the 123 Ashkenazi Jewish patients, 75 (61%) had an age of onset < or = 21 yr. RESULTS: Ashkenazi Jews showed only modest evidence of linkage (nonparametric linkage 1.63, p = 0.05) to the IBD1 locus. However, when the Ashkenazi population was subdivided on the basis of age of onset, there was a striking increase in linkage in families where affected individuals had an age of onset < or = 21 yr (nonparametric linkage 3.02, p = 0.002). In contrast, there was no evidence of linkage in the Jewish families where all affected individuals had an age of onset > 21 yr. CONCLUSIONS: The IBD1 gene plays a greater role in conferring susceptibility to CD in Jews with early onset disease than in Jews with late onset disease.
Adult↗
Epiphyseal extension of a unicameral bone cyst.
Epiphyseal extension of a unicameral bone cyst is rare. We report a case of a 13-year-old boy with three pathological fractures through a unicameral bone cyst with epiphyseal involvement in the proximal humerus. These lesions initially tends to expand the humeral epiphysis laterally and progress medially. They also commonly cause a slip of the epiphysis in a medical direction. They also have a greater association with growth retardation and lesser degree of recurrence than their metaphyseal counterpart.
Adolescent↗
Proliferation index and vascular density of giant cell tumors of bone: are they prognostic markers?
BACKGROUND: The proliferation index (PI) and vascular density (VD) in giant cell tumors (GCT) of bone were studied to assess then value as prognostic markers. METHODS: Formalin fixed, paraffin embedded tissue from 7 nonrecurrent (NR-GCT) and 13 recurrent (R-GCT) tumors were stained with MIB-1, a monoclonal antibody against Ki-67 (nuclear proliferation antigen), and QBEND-10, a monoclonal antibody against CD34 (endothelial antigen). A minimum of ten fields/case were analyzed on the SAMBA 4000 Image Analyzer. Only mononuclear cell nuclei were analyzed for MIB-1 staining. RESULTS: Mean values +/- 1 Standard deviation (SD) for PI (stained nuclear area/total nuclear area) were: NR-GCT, 10.98 +/- 3.70; R-GCT (initial presentation), 8.94 +/- 3.57; and R-GCT (first recurrence), 9.25 +/- 3.52. In addition, the mean PI +/- 1 SD for mononuclear round-ovoid cells was 8.71 +/- 3.47 and was 9.16 +/- 10 for mononuclear spindle cells. The mean values +/- 1 SD for VD (stained area/total area of structures) were: NR-GCT, 10.61 +/- 6.37; R-GCT (initial curettage), 9.40 +/- 4.94; and R-GCT, (first recurrence), 12.56 +/- 5.88. Comparing the means for PI and VD for both groups of tumors using Student's t test showed no statistically significant differences (P = 0.34). In one case of histologically benign GCT that metastasized to the lungs, the PI of the metastatic tissue was not significantly different from the primary tumor. CONCLUSIONS: This study presents evidence that the degree of tumor cell proliferation and vascularity are not useful parameters to predict the recurrence of GCT of bone and that there are no significant differences between the PI of mononuclear round-ovoid cells and mononuclear spindle cells.
Adolescent↗
Expression of metalloproteinases and tissue inhibitors of metalloproteinases in giant cell tumor of bone: an immunohistochemical study with clinical correlation.
The clinical behavior of giant cell tumors (GCTs) is unpredictable. To gain insight into this tumor's biological behavior, matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) were studied. These substances play essential roles in wound healing and neoplastic invasion and metastasis. Paraffin-embedded tissue was collected from 18 cases of histologically benign GCT, with 17 treated by curettage and 1 by resection. Eight cases showed no recurrence after a minimum of 2.5 years, and 10 had local recurrence. One showed metastasis. Antibodies to MMP-9, MMP-2, TIMP-1, and TIMP-2 were applied by immunohistochemical methods. In all cases, MMP-9 was strongly expressed in giant cells predominantly in a diffuse pattern and was strong but focal in stromal cells. MMP-2 decorated stromal cells and giant cells heterogeneously. TIMP-1 was variably expressed in giant cells of the nonrecurrent cases and was strongly present in a diffuse or patchy distribution in the stromal cells in 6 of 8 cases. However, in 9 of 10 recurrent cases, TIMP-1 was expressed weakly by both giant and stromal cells. TIMP-2 was variably expressed in the giant cells of the nonrecurrent cases, but 6 of 8 nonrecurrent cases showed strong stromal cell positivity for TIMP-2. Weak staining for TIMP-2 was observed in 7 of 10 recurrent cases in the stromal cells and 9 of 10 recurrent cases in the giant cells. These results indicate that expression of MMPs and TIMPs differs in giant cells and stromal cells in the same tumor. More significantly, in contrast to the nonrecurrent giant cell tumors, there is an imbalance in the MMPs and TIMPs in the recurrent tumors with a net excess of MMPs. This unopposed expression of MMPs in GCTs may play a role in breakdown of extracellular matrix and tissue invasion. Finally, these markers may prove useful in predicting behavior in these tumors.
Adolescent↗
Sarcoma in association with bone infarcts. Report of five cases.
Sarcoma associated with bone infarct is rare, and only 41 well-documented cases have been published. We describe five additional patients, three women and two men, aged 39 to 57 years. The tumors involved the femur (three patients), tibia (one patient), and humerus (one patient). In three patients, the infarcts were idiopathic. Radiologic evidence of malignancy was found in all patients, and bone infarcts were suspected in four. Four of the patients had malignant fibrous histiocytoma and one an osteosarcoma. Histologically, bone infarcts were seen in all patients, but in three they were mostly replaced by tumor. Portions of intact infarcts were seen adjacent to the tumor, indicating that they had preceded the development of the sarcoma. No hypercellular or atypical reparative tissue was found in the infarcted bones or in three additional uncomplicated infarcts studied from the same patients. The pathogenesis of sarcoma arising in bone infarct is unknown. The prognosis is poor; four of our five patients died within 2 years.
Adult↗
Ewing's sarcoma of humerus with epithelial differentiation.
We report an unusual case of Ewing's sarcoma of the humerus with epithelial differentiation. This rare finding has recently been documented in this type of tumor.
Adult↗
Intramuscular ossified hemangioma.
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Adult↗
Osteosarcoma: subtle, rare, and misleading plain film features.
Osteosarcoma is a malignant, mesenchymal, osteoid, and bone-forming tumor [1-3]. In most cases, typical radiographic features clearly illustrate the aggressive bone-forming nature of the lesion. These features include long-bone metaphyseal location, mixed areas of lysis and sclerosis, cortical destruction, periosteal new bone, and soft-tissue mass. However, numerous factors contribute to misleading radiographic patterns of osteosarcoma. They include histologic low-grade, lytic, or minimally sclerotic lesions, early detection, confinement to the intramedullary canal, benign-appearing periosteal reaction, rare intraosseous locations (subchondral, diaphyseal, intracortical), and rare skeletal sites (e.g., soft tissues, skull, ribs, tarsal bones). Secondary osteosarcomas, such as those arising from infarcts or fibrous dysplasia, may also produce a confusing radiologic picture. The purpose of this pictorial essay is to illustrate cases of osteosarcoma that proved to be difficult diagnostic dilemmas because of their subtle, rare, or misleading plain film features.
Adolescent↗
A specific protein found in human senescent fibroblasts.
The limited potential for proliferation of human fibroblasts in culture represents cell level senescence. Aging is a programmed process under genetic control, and at certain stage of the life-span of animal cells, some genes start to express. Studying the biomarkers of senescent cells is important to understanding the basic mechanism of aging which may be relevant to the normal cell growth control and tumor biology. A protein with 72,000 Dalton molecular weight was detected by the hybridoma method in our laboratory. The protein shows specificity to senescent or presenescent cells of several cell lines, including WI-38, K.D., U2OS, etc.
Animals↗
Tumors of the ankle and foot.
Although tumor and tumor-like conditions of the foot and ankle are unusual, certain bone and soft tissue lesions are more common than others. Conventional radiographs remain essential in all such cases and are especially specific for intraosseous tumors. MR imaging is more sensitive to the presence and extent of both bone and soft tissue lesions.
Ankle↗
Intracapsular and paraarticular chondromas.
Intracapsular and paraarticular chondromas are rare benign tumors. The authors report four such lesions, three of which occurred about the knee and one at the hip. Radiographs showed calcified soft-tissue masses in the infrapatellar region of the knee in three patients. In the hip, the lesion was not visible on conventional radiographs. Three lesions originated from the joint capsule and one was extracapsular. These lesions should be differentiated from synovial chondromatosis and other calcified lesions occurring about the joints. After local excision, the tumors usually do not recur.
Aged↗
Eosinophilic granuloma: MRI manifestations.
The appearance on magnetic resonance imaging (MRI) of 16 cases of pathologically proven eosinophilic granuloma were reviewed retrospectively and correlated with the radiographic appearance of the lesion. The most common MR appearance (ten cases) was a focal lesion, surrounded by an extensive, ill-defined bone marrow and soft tissue reaction with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, considered to represent bone marrow and soft tissue edema (the flare phenomenon). The MRI manifestations of eosinophilic granuloma, especially during the early stages, are nonspecific, and may stimulate an aggressive lesion such as osteomyelitis or Ewings sarcoma, or other benign bone tumors such as osteoid osteoma or chondroblastoma.
Adolescent↗
Clear cell chondrosarcoma of bone. A report of 8 cases.
We report 8 cases of clear cell chondrosarcoma from 350 cases of chondrosarcoma (2.2%) seen at Istituto Rizzoli. The lesion is always low grade in nature and occurs in an epiphyseal location, usually in the proximal femur or humerus. This relatively inaccessible location may hinder the exact diagnosis. Wide resection is the treatment of choice, although 3 of our patients underwent intralesional or marginal procedures. At present all patients are free of disease. However, a longer followup is essential because of the slow-growing nature of this sarcoma.
Adult↗
Transvaginal approach for repair of rectovaginal fistulae complicating Crohn's disease.
The management of rectovaginal fistulae complicating Crohn's disease is difficult and often unsatisfactory. Between December 1983 and November 1988, 13 patients with Crohn's disease underwent repair of rectovaginal fistulae via a transvaginal approach. All patients had a diverting intestinal stoma either as part of the initial step in the staged management of intractable perianal disease or concurrent with the repair of the rectovaginal fistula. Each of the patients had low or mid septal fistulae; high fistulae generally are treated transabdominally and are not the focus of this discussion. Fistulae were eradicated in 12 of the 13 women and did not recur during the follow-up period, which averaged 50 months (range, 9 to 68 months). The only treatment failure was a patient who had a markedly diseased colon from the cecum to the rectum and a very low-lying fistula. It is concluded that a modified transvaginal approach is an effective method for repair of rectovaginal fistulae secondary to Crohn's disease.
Adult↗
Malignant fibrous histiocytoma of the distal fibula: a case report.
A 25-year-old female with primary malignant fibrous histiocytoma of the distal fibula is presented. Malignant fibrous histiocytoma of bone is a rare primary bone tumor in this anatomic location. Careful preoperative staging studies and neoadjunct chemotherapy permitted an unusual resection to be performed resulting in successful limb salvage for the patient.
Adult↗
Intracapsular paraarticular chondroma of the knee.
Intracapsular or paraarticular chondromas are seldom mentioned in the literature. They generally occur in the area around the knee, and must be recognized and differentiated from other soft-tissue tumors that appear at this site such as sarcomas containing focal calcification or ossification, and from other cartilaginous lesions such as synovial chondromatosis. We present a case of intracapsular chondroma of the knee in a 63-year-old woman. A biopsy was performed to rule out the possibility of a soft-tissue sarcoma.
Bone Neoplasms↗
Disease-associated fractures: detection and management in the elderly.
Pathologic fractures in the elderly result from a variety of conditions, including malignancies and Paget's disease. Comprehensive laboratory and radiological evaluation is essential to analyze both the underlying etiology and the extent of the disease. Surgical intervention is critical in stabilizing these fractures so that pain is relieved and function is improved.
Aged↗