1/23. Epstein-Barr virus-associated leiomyosarcoma of the thyroid in a child with congenital immunodeficiency: a case report.We report an unusual case of multifocal leiomyosarcoma involving the thyroid gland, liver, and right lung in a child with congenital immunodeficiency disease. The smooth muscle nature of these neoplasms was confirmed by immunohistochemistry and electron microscopic studies. in situ hybridization showed large amounts of Epstein-Barr virus messenger RNA within the tumor cells. Although Epstein-Barr virus-associated smooth muscle tumors have been reported in children with AIDS and after organ transplantation, we are unaware of any case report in congenital immunodeficiency disease.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/23. Epstein-Barr virus-associated multicentric leiomyosarcoma in an adult patient after heart transplantation: case report and review of the literature.Epstein-Barr virus (EBV)-associated smooth muscle tumors following solid organ transplantation are extremely rare, with only 12 cases reported in the literature thus far. The exact pathogenetic role of EBV infection in the oncogenesis of these soft tissue tumors in immunodeficient patients and the biologic behavior of such tumors is still unclear. We report a 26-year-old man in whom multiple smooth muscle tumors developed 36 to 51 months after heart transplantation. All tumors, two synchronous liver nodules, two subsequently occurring paravertebral tumors, and a single tumor in a vein at the left ankle were surgically resected. The tumor tissue was processed for routine histology and immunohistochemical (IHC) stains. Additionally, competitive polymerase-chain-reaction (PCR), reverse-transcriptase PCR (RT-PCR), as well as in situ hybridization (ISH) were used for EBV particle quantification and gene transcription analysis. The histologic features and immunohistochemical profiles were consistent with leiomyosarcoma in all tumor nodules. EBV infection was detected in >95% of tumor cell nuclei by EBER 1/2 ISH. Competitive PCR revealed 3105 EBV particles per milligram of tumor tissue. The EBV gene expression pattern analyzed by RT-PCR and IHC corresponded to the latency type III with specific expression of EBNA1, EBNA2, LMP1, and LMP2A genes. Under continuous antiviral therapy (famcyclovir) the patient currently shows no evidence of disease. Our data indicate that EBV infection plays a causal role in the development of smooth muscle tumors following organ transplantation. A latency type III, identical to EBV-associated posttransplant lymphoproliferative disorders, was identified and suggests a common pathogenetic mechanism in the development of these histogenetically distinct neoplasms. The fact that the patient currently shows no evidence of disease may be the result of the continuous administration of antiviral therapy because the soft tissue recurrences of the leiomyosarcoma occurred while the patient was not receiving antiviral prophylaxis.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/23. 19p deletion in recurring leiomyosarcoma lesions from the same patient.Ten leiomyosarcomas (LMS) affecting the same patient over a period of 3 years were cytogenetically studied to detect nonrandom chromosomal changes with a pathogenetic significance. All tumors, likely metastases of a previous LMS presentation, were classified as small, including eight that developed before chemotherapy; the diagnoses were based on standard immunohistochemistry methods for smooth muscle tumors. Scoring of 613 metaphases revealed monosomy of chromosome 22 in six LMS, monosomy of chromosome 19 in three, and deletion of chromosome 19p in all ten. interphase fluorescence in situ hybridization (FISH) of the 22-alphoid-specific probe allowed loss of the target chromosome to be detected in four tumors at higher frequencies than those detected by cytogenetics. Double-color FISH of the 19p- and 19q-specific YAC performed on one tumor made it possible to distinguish the monosomic and 19p deleted cells, the relative frequencies of which were found to be 10% and 20%, respectively. The deletion breakpoint could be mapped at 19p13 between YAC 957d12 and YAC 947g4. The recurrence of the 19p deletion in a subset of tumor cells from all of the analyzed LMS suggests that this structural aberration is a significant change in the development of leiomyosarcomas.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/23. Synchronous oral leiomyosarcoma and squamous cell carcinoma.An unusual case of synchronous squamous cell carcinoma and leiomyosarcoma of the oral cavity is reported in a patient without any identified environmental risk or predisposing factors. The invasive squamous cell carcinoma involved the tongue, whereas the leiomyosarcoma was located in the soft palate. No immunostaining was found for human papillomavirus or Epstein-Barr virus, and in situ hybridization showed negativity for human papillomavirus dna within the tumor cells. Alterations of bcl -2, c-erb -b2 and Rb oncoproteins were not found immunohistochemically. Overexpression of p53 was detected by immunohistochemistry in both tumors, but p53 gene mutations were not found by polymerase chain reaction. Neither loss of heterozygosity of p53 nor microsatellite instability was detected in this patient. The smooth muscle nature of the leiomyosarcoma was confirmed by immunohistochemical methods. To our knowledge, synchronous smooth muscle and epithelial oral tumors have not previously been reported.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/23. Immunohistomorphologic and molecular cytogenetic analysis of a carcinosarcoma of the urinary bladder.Carcinosarcomas of the urinary bladder are malignant biphasic tumors with an epithelial and a spindle cell component. For the histogenesis of the two components, a biclonal and a monoclonal origin are discussed. We present the immunomorphology and molecular cytogenetics of such a case. The immunohistology of biopsies of the urinary bladder revealed a poorly differentiated urothelial carcinoma (GIII) and a co-existing pleomorphic, spindle cell leiomyosarcoma (GIII). The two components were microdissected and further analyzed for gains and losses of chromosomal material using comparative genomic hybridization. In addition, loss of heterozygosity analyses were included. The tumor components revealed as overlapping core aberrations losses on the short arm of chromosome 9 and on the long arm of chromosome 11. However, both components showed additional aberrations exclusively detected in one of the components. The occurrence of overlapping aberrations strongly argues for a monoclonal origin of this tumor with a common ancestor. The additional aberrations of the components point to an independent and divergent course of tumor progression in both components.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/23. Primary hepatic leiomyosarcoma in a woman after renal transplantation: report of a case.In contrast to malignant lymphomas or skin cancer, smooth muscle tumors including leiomyosarcoma are rarely associated with transplant recipients. We herein present a 33-year-old woman with end-stage renal disease who received a transplant at 27 years of age. Four years after the transplantation, at age 31, she underwent a mastectomy because of primary right breast cancer, which was found to be a 5-mm-sized mucinous carcinoma with no regional lymph node metastasis. Six years after the transplantation, a liver tumor was unexpectedly discovered. An explorative laparotomy revealed a well-encapsulated tumor occupying the posterior portion of the right lobe of the liver. The patient underwent a posterior segmentectomy. Histologically, the tumor possessed intermingling fascicles of spindle cells with eosinophilic cytoplasm and elongated nuclei. Based on an immunohistochemical examination, the tumor cells were positive for the muscle-associated antibody. In addition, rna probes for Epstein-Barr virus were negative based on in situ hybridization. The histologic, immunohistochemical findings were considered to be diagnostic for leiomyosarcoma, which is a low-grade malignancy. Two years after surgery, the patient is doing well with no recurrence of liver tumors or breast cancer.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/23. Pulmonary metastases from a low-grade endometrial stromal sarcoma confirmed by chromosome aberration and fluorescence in-situ hybridization approaches: a case of recurrence 13 years after hysterectomy.Pulmonary metastasis from low-grade endometrial stromal sarcomas (ESSs) occasionally are found after long, disease-free periods, mostly as incidental histological or radiological discoveries. We describe a case of low-grade ESS presenting as nodular pulmonary metastases finally diagnosed by estrogen-receptor staining, cytogenetic and fluorescence in situ hybridization (FISH) analyses, and perusal of the histology of hysterectomy material. An abnormal nodule in the lung field was discovered by means of chest X-ray of a 47-year-old woman. She had been disease free for 13 years after hysterectomy for an alleged leiomyoma. A computed tomographic scan revealed nodules, with fluctuation in size over the 2-year period, in both lungs. Finally the lesion in the left lung was resected, and pulmonary endometriosis was suspected because of the lack of stromal cell nuclear atypia and positive immunohistochemical reactions for estrogen and progesterone receptors. However, a characteristic karyotype was identified cytogenetically: 46, XX, t(7;17)(p15;q11), the translocation of which, specific to ESS, was confirmed by FISH analysis. A final diagnosis of pulmonary metastases from an ESS could be made by reviewing the histology of the previous uterine tumor. In this case, metastatic lesions from an ESS showed a decrease as well as an increase in size, despite the malignant potential. Immunostaining for estrogen and progesterone receptors and cytogenetic and FISH analyses, together with clinical information on the past gynecological history, are valuable diagnostic keys.- - - - - - - - - - ranking = 5keywords = hybridization (Clic here for more details about this article) |
8/23. leiomyosarcoma of the breast: a pathologic and comparative genomic hybridization study of two cases.leiomyosarcoma is an extremely rare form of primary breast sarcoma. We present the pathologic and genetic findings of two cases of leiomyosarcoma of the breast. The patients were 44 and 52 years of age and they presented with circumscribed masses of 3.0 and 4.5 cm, (greatest dimension) respectively. Microscopically, the two tumors showed diffuse proliferation of spindle cells with oval and blunt-ended nuclei arranged in short fascicles or bundles. There was moderate cytologic atypia in both cases, and 6 and 12 mitotic figures per 10 high power fields, respectively. No epithelial component was identified. The tumor cells were strongly immunoreactive for markers of smooth-muscle differentiation, including desmin, muscle-specific actin, and smooth-muscle actin. comparative genomic hybridization analysis showed losses of 10q (two of two cases), 13q (two of two cases), 17p (one of two cases), and gains of 1q (one of two cases) and 17p (one of two cases). The patterns of chromosomal imbalances identified in leiomyosarcoma of the breast are similar to those reported in leiomyosarcoma of soft tissue and uterus and are different from those reported for leiomyoma, indicating that these alterations may be important for development of malignant smooth-muscle tumors regardless of site or organ of origin.- - - - - - - - - - ranking = 5keywords = hybridization (Clic here for more details about this article) |
9/23. Primary hepatic leiomyosarcoma in a child with the acquired immunodeficiency syndrome.We report the first case of acquired immunodeficiency syndrome (AIDS)-related primary hepatic leiomyosarcoma in a 9-year-old girl. The pathologic diagnosis was made on a partial hepatectomy specimen and was confirmed by immunohistochemistry and electron microscopy. No human immunodeficiency virus-related nucleic acid was identified in tumor cells by in situ hybridization. review of the AIDS-related literature reveals a rising incidence of tumors of smooth muscle origin in human immunodeficiency virus-infected patients. This case study details the eighth pediatric AIDS patient with a tumor of smooth muscle origin and represents the 20th and the youngest patient with primary hepatic leiomyosarcoma to be reported in the world literature.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
10/23. A case of nasal leiomyosarcoma: the first comparative genomic hybridization analysis.Leiomyosarcomas are rare in the head and neck region. The treatment of choice is surgical resection. We present a case of leiomyosarcoma in a 50-year-old female patient, arising from the inferior turbinate. The tumor was resected by a transnasal microendoscopic approach and no evidence for tumor recurrence was found during an endoscopic and radiologic follow-up of two years. cytogenetic analysis by means of comparative genomic hybridization revealed chromosomal gains at 4p13p15, 6p21p22, 7q22qter, 9q22qter and chromosomal losses at 10q22qter and 19p12p13.1. With augmented experience, the transnasal approach may be appropriate to resect circumscribed malignant tumors located in the nasal cavity.- - - - - - - - - - ranking = 5keywords = hybridization (Clic here for more details about this article) |
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