1/7. radiation-induced inflammatory malignant fibrous histiocytoma of the ileum.A case of inflammatory malignant fibrous histiocytoma of the ileum seemingly induced by radiation is described. A 50-year-old female with a past history of uterine cervical carcinoma and postoperative radiation therapy presented with abdominal pain, fever and leukocytosis. The subserosa of the distal part of the ileum showed a diffuse dense, neutrophilic and lymphocytic infiltrate with dispersed atypical, short spindle- or plump oval-shaped histiocyte-like cells. Pleomorphic mono- or multinucleated giant cells with bizarre nuclei were also intermingled in the lesion. Immunohistochemically, the tumorous atypical cells were positive for vimentin, alpha-smooth muscle actin, alpha-1 antitrypsin and granulocyte colony-stimulating factor. No EBV genomic sequences were detected by in situ hybridization. flow cytometry showed an aneuploid dna content with high S-phase fraction. The patient was well with no evidence of tumor at 5 months after surgery. It is important to include this type of tumor in the differential diagnosis of small intestinal lesions accompanied by fever and leukocytosis following radiation.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/7. Genetic characterization of angiomatoid fibrous histiocytoma identifies fusion of the FUS and ATF-1 genes induced by a chromosomal translocation involving bands 12q13 and 16p11.This case report documents the first karyotypic, fluorescence in situ hybridization, and genetic analysis of an angiomatoid fibrous histiocytoma that arose and recurred in the arm of a 5.5-year-old girl. Complex rearrangements between chromosomes 2, 12, 16, and 17 were noted, as well as deletion in the long arm of chromosome 11. flow cytometry revealed a normal cell population. The t(12;16) site was further investigated using reverse transcriptase-polymerase chain reaction. We found that the FUS (also known as TLS) gene from 16p11 combined with the ATF-1 gene from 12q13 to generate a chimeric FUS/ATF-1. The FUS gene is rearranged in the t(12;16)(q13;p11) that characterizes myxoid liposarcoma and in acute myeloid leukemia with t(16;21)(p11;q22), while the ATF-1 gene is rearranged in the t(12;22)(q13;q12) found recurrently in clear cell sarcomas (malignant melanoma of soft parts). Thus, the FUS/ATF-1 gene in angiomatoid fibrous histiocytoma is predicted to code for a protein that is very similar to the chimeric EWS/ATF-1 found in clear cell sarcoma.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/7. HHV-8 dna sequences in the peripheral blood and skin lesions of an hiv-negative patient with multiple eruptive dermatofibromas: implications for the detection of HHV-8 as a diagnostic marker for Kaposi's sarcoma.BACKGROUND: Multiple eruptive dermatofibroma (MEDF) is a rare disorder seen in immunocompromised patients, simulating Kaposi's sarcoma (KS). Whereas KS is strongly associated with human herpesvirus 8 (HHV-8), the virus has never been detected in MEDF until now. OBJECTIVE: To present a patient with MEDF who showed no signs of immunodeficiency but was seropositive for HHV-8 antibodies and demonstrated HHV-8 dna both in the peripheral blood and lesional skin of MEDF. methods: Clinical, histological and serological investigations were performed as well as polymerase chain reaction (PCR) studies and in situ hybridization (ISH). RESULTS: A 35-year-old white man with suspected KS was referred for evaluation of multiple pigmented nodules and patches. Biopsies revealed features of dermatofibroma, superficial fibrosing dermatitis and scar. One of the nodular lesions harbored HHV-8 dna sequences. A faint amplification product was detected in the superficial fibrosing dermatitis lesion, while no HHV-8 sequences were found in normal skin and scar. Whole-blood samples and serum were positive for HHV-8. None of the skin lesions shown to harbor HHV-8 dna sequences by nested PCR displayed a signal for HHV-8 rna by ISH. Repetitive peripheral blood examinations did not reveal any serum antibodies against or antigens of hiv. serum antibodies against the HHV-8 capsid antigen orf 65.2 were detected. CONCLUSION: Results of PCR studies and ISH indicate that the presence of HHV-8 in the lesional tissue was probably blood-borne due to viremia and not due to viral replication in tumor cells. The presence of HHV-8 is not fully restricted to KS. The differential diagnosis of KS and its simulators should be based on an integrative analysis of all available clinicopathological and molecular data and should not rely exclusively or predominantly on the presence or absence of HHV-8.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/7. Establishment of a new human malignant fibrous histiocytoma cell line, FU-MFH-1: cytogenetic characterization by comparative genomic hybridization and fluorescence in situ hybridization.Although a number of malignant fibrous histiocytoma (MFH) cell lines have been reported, their characterization at a molecular cytogenetic level has not been fully established. In this study, we established a new human cell line, designated as FU-MFH-1, from a storiform-pleomorphic MFH arising in the retroperitoneum of a 61-year-old woman, and applied comparative genomic hybridization (CGH) and fluorescence in situ hybridization (FISH) with chromosome painting probes for the characterization of chromosome alterations. FU-MFH-1 cells were spindle, round, or polygonal in shape with oval nuclei, and were maintained continuously in vitro for over 50 passages for more than 12 months. G-banding analysis was performed and FU-MFH-1 revealed a complex karyotype with an abnormal chromosome 19 containing a homogeneously staining region (hsr). CGH analysis showed a high-level amplification of 12q13-->q21. The high-level amplification detected by CGH was refined by FISH. These results showed that the hsr was composed of amplified dna sequences from 12q. Our study emphasizes the usefulness of CGH as a powerful tool for chromosomal localization of amplified sequences. The FU-MFH-1 cell line should be useful for biologic and molecular pathogenetic investigations of human MFH.- - - - - - - - - - ranking = 10keywords = hybridization (Clic here for more details about this article) |
5/7. Cutaneous aneurysmal fibrous histiocytoma with a t(12;19)(p12;q13) as the sole cytogenetic anomaly.We report a case of a 39-year-old woman with a recurrent aneurysmal fibrous histiocytoma of the right lower leg. cytogenetic analysis of the tumor specimen disclosed a 46,XX,t(12;19)(p12;q13) karyotype. Multicolor fluorescence in situ hybridization (M-FISH), followed by conventional FISH analysis, confirmed the reciprocal translocation as the sole cytogenetic anomaly, and allowed for the positioning of chromosomes 12 and 19 breakpoints proximal to the BCL3 gene and between ETV6 and PIK3C2G gene loci, respectively. Our case highlights the importance of distinguishing this recurrent but benign lesion from similarly appearing malignant skin tumors. cytogenetic analysis may contribute to the diagnosis of this uncommon but distinctive clinicopathological entity.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/7. A rare mediastinal tumour presenting with systemic effects due to IL-6 and tumour necrosis factor (TNF) production.patients presenting with prolonged systemic illnesses with no specific clinical or serological defining features may be diagnosed as having atypical systemic vasculitides, but often turn out to have occult malignancies. cytokines have been implicated in causing many of the systemic effects in such cases. In this study we describe a patient presenting after 2 years of a severe systemic illness with a marked acute phase response, due to an occult mediastinal angiomatoid malignant fibrous histiocytoma. Tumour resection was curative. We evaluated in detail the local and systemic production of cytokines induced by this tumour. blood samples were taken pre- and post-operatively for cytokine studies. in vitro production of IL-2, IL-2R, IL-1 beta, IL-6 and TNF-alpha by cultured monocytes from the patient, as well as plasma cytokine levels, were measured by ELISA. Tumour cytokine production was also evaluated immunocytochemically, and by in situ hybridization with specific cDNA probes. plasma IL-2R and IL-6, and IL-6 and TNF-alpha production by peripheral blood monocytes were markedly elevated before tumour resection, normalizing post-operatively. Most tumour cells and infiltrating lymphocytes stained with antibodies to IL-6, IL-6R and TNF-alpha, and expressed HLA class II. IL-6 and TNF-alpha mRNA production in the tumour was confirmed by in situ hybridization studies. We have described the first case of an occult angiomatoid malignant fibrous histiocytoma in the mediastinum. Studies of cytokine expression suggested that chronic TNF, IL-6, and IL-2 production by leucocytes and tumour cells in this patient was responsible for the severe systemic illness with which she presented.- - - - - - - - - - ranking = 2keywords = hybridization (Clic here for more details about this article) |
7/7. Identification of a ring chromosome in a myxoid malignant fibrous histiocytoma with chromosome microdissection and fluorescence in situ hybridization.We investigated the origin of a ring chromosome in a myxoid malignant fibrous histiocytoma (MFH) by microdissection and fluorescence in situ hybridization (FISH) analyses. Cytogenetically, only two ring chromosomes were observed; the smaller ring was seen more frequently. The latter was microdissected, and the material used for FISH. Hybridization of the microdissected labeled dna to normal metaphase cells revealed that the signal localized only to 20q. Three signals were seen in the tumor cells using either the microdissected 20q probe or chromosome 20 centromeric probe, indicating the involvement of both the long arm and the centromere in the ring chromosome. The short arm of chromosome 20 did not appear to be involved in the formation of the ring chromosome.- - - - - - - - - - ranking = 5keywords = hybridization (Clic here for more details about this article) |