1/23. Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma.Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
2/23. Uterine pleomorphic rhabdomyosarcoma in a patient receiving tamoxifen therapy.INTRODUCTION: tamoxifen has been used as adjuvant therapy for the treatment of breast cancer. Its use has been associated with the development of proliferative endometrial lesions such as polyps, hyperplasia, and carcinoma. Mesenchymal tumors including malignant mixed mullerian tumors, endometrial stromal sarcomas, adenosarcomas, and leiomyosarcomas have been more recently described with tamoxifen use. CASE REPORT: This report describes the first case of a pure uterine rhabdomyosarcoma in a patient receiving tamoxifen therapy. DISCUSSION: Although uterine rhabdomyosarcomas are rare tumors and may arise de novo, we discuss the possible role of tamoxifen in the development of these mesenchymal tumors.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
3/23. radiation-induced gastrointestinal stromal sarcoma of the esophagus.Esophageal sarcoma is an uncommon tumor, sporadically reported in the literature. radiation therapy is frequently employed in the treatment of carcinoma of the esophagus, and the increased risk of development of sarcoma arising in irradiated fields is well known. However, to our knowledge, the occurrence of radiation-associated sarcoma of the esophagus after radiation therapy for carcinoma of the esophagus has not been reported. We therefore report the case of a 43-year-old female who developed a gastrointestinal stroma sarcoma 9 years following radiation therapy for esophageal squamous cell carcinoma. The patient underwent resection of her gastrointestinal stromal sarcoma by transhiatal esophagectomy with cervical anastomosis and is doing well 18 months later. The increasing use of radiation therapy for esophageal carcinoma suggests that radiation-associated sarcoma of the esophagus may be seen more frequently in the future.- - - - - - - - - - ranking = 177.19358294837keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
4/23. Low-grade endometrial stromal sarcoma of the ectocervix after therapy for breast cancer.OBJECTIVE: Low-grade endometrial stromal sarcoma is an uncommon, indolent uterine sarcoma that can arise in extrauterine locations. The objective of this study was to report on a previously unpublished site of origin for a low-grade endometrial stromal sarcoma. methods: A case of a low-grade endometrial stromal sarcoma arising in the ectocervix after goserelin hormonal therapy for breast cancer was studied. RESUTLS: Low-grade endometrial stromal sarcoma can arise in the ectocervix even in the absence of endometriosis. CONCLUSION: Low-grade endometrial stromal sarcoma should be included in the differential diagnosis of sarcomas of the ectocervix.- - - - - - - - - - ranking = 9keywords = stromal (Clic here for more details about this article) |
5/23. Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von hippel-lindau disease.Here we report tumor-to-tumor metastases identified in two patients with von Hippel-Lindau (VHL) disease. The first patient had bilateral renal carcinomas and multiple cerebellar hemangioblastomas, and the second patient had a renal carcinoma and multiple hemangioblastomas in the retina, cerebellum and spinal cord. A cerebellar lesion from the first patient and a spinal lesion from the second patient contained two distinct components. The inner part of these tumors consisted of a nested mass of polygonal clear cells that expressed cytokeratin and epithelial membrane antigen, while the outer part of the tumors showed proliferation of capillaries and intervening foamy stromal cells that were negative for cytokeratin and epithelial membrane antigen. The tumors were thus considered to be hemangioblastomas complicated by metastatic lesions of renal cell carcinoma of clear cell type. These cases indicate that tumor-to-tumor metastasis should be considered when hemangioblastoma contains a clear cell carcinoma component in the setting of VHL disease, and that immunohistochemical staining for cytokeratin and epithelial membrane antigen is useful for the diagnosis.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
6/23. Malignant tumors of the small intestine.SUMMARY: Malignant tumors of the small bowel are unusual and account for only 1% to 5% of all gastrointestinal tract malignancies. Thirteen cases of malignant tumors of the small bowel identified at the VAMC in puerto rico from January 1999 to September 2001 and a review of the literature are presented. The mean age of our cases was 67 (range: 45-78). Vague abdominal pain, nausea, vomiting, and melena were the most frequently reported symptoms. The average time from first symptoms to diagnosis was 3.2 months. A positive test for occult blood or hypochromic microcytic anemia was invariably present. hyperbilirubinemia and increased alkaline phosphatase were warning signs that allowed earlier diagnosis in patients with duodenal tumors. Fifty-four percent of the lesions were detected by endoscopic examination while 46% relied on radiographic studies. Eleven had carcinomas and two malignant gastrointestinal stromal tumors. Neither carcinoids nor lymphomas were identified. Our report of thirteen cases of malignant small bowel tumors is unusual and exhibits differences with the previously reported data. The cases were identified in a limited Hispanic population in a short period of time. Aggressive evaluation and a high suspicion of these malignancies should be entertained whenever subtle symptoms and unexplained gastrointestinal blood loss are assessed.- - - - - - - - - - ranking = 35.438716589674keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
7/23. Metastatic ovarian Sertoli-cell tumor: FNA findings with immunohistochemistry.A malignant sertoli cell tumor of the ovary is a rare sex-cord stromal derived neoplasm. Sertoli cell tumors account for approximately 4% of Sertoli-stromal cell tumors (Hsu et al., J Histochem Cytochem 1981;29:577-580). The diagnosis of this entity by fine-needle aspiration and needle core biopsy can be difficult when the differential also includes a metastatic renal cell carcinoma, due to the overlap of both cytological and histological features. Here, we show that immunohistochemical staining on cytological material including alpha-inhibin can be used to differentiate between these two malignant tumors.- - - - - - - - - - ranking = 2keywords = stromal (Clic here for more details about this article) |
8/23. Renal cell carcinoma metastatic to follicular adenoma of the thyroid gland. A case report.BACKGROUND: Renal cell carcinoma is an unpredictable tumor that can recur many years after the original diagnosis and metastasize to uncommon sites, including the thyroid gland. Differential diagnosis from primary thyroid tumor is often difficult both clinically and pathologically. We report a case of metastatic renal cell carcinoma in follicular adenoma of the thyroid gland. CASE: A 48-year-old woman presented with a 3-cm-diameter, palpable mass in the left lobe of the thyroid gland. The patient's history included removal of a left renal mass, which was conventional renal cell carcinoma. Fine needle aspiration cytology smears contained a few small clusters of polygonal cells with abundant, clear cytoplasm and irregular, hyperchromatic nuclei as well as bland-looking thyroid follicle cells and stromal cells. A papillary or follicular growth pattern was not detected. A cell block made from the aspirated sample was composed mainly of clear cells. By immunohistochemical stains, the clear cells were completely negative for TTF-1, thyroglobulin, calcitonin and inhibin while equivocally staining for cytokeratin, CD10 and galectin-3. The histologic diagnosis was renal cell carcinoma metastatic to follicular adenoma of the thyroid gland. CONCLUSION: Renal cell carcinoma metastatic to the thyroid may masquerade as a primary thyroid neoplasm. A history of prior nephrectomy, the presence of unremarkable thyroid follicle cells, the absence of a papillary or follicular growth pattern and immunohistochemical study can help differentiating metastatic renal cell carcinoma from a primary thyroid lesion with clear cell change.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
9/23. A c-KIT codon 816 mutation, D816H, in the testicular germ cell tumor: case report of a Japanese patient with bilateral testicular seminomas.Mutations of the c-KIT gene have been reported not only in gastrointestinal stromal tumors and mast cell tumors, but also in testicular germ cell tumors (TGCTs). In the present study we employed polymerase chain reaction and dna sequencing analysis to characterize the c-KIT gene in a 29-year-old Japanese patient with bilateral testicular seminomas. Direct sequence analyses revealed a single base substitution in exon 17 in one c-KIT allele, resulting in an amino acid substitution of D816H in this mutated allele. This mutation was found in the left, but not in the right, testicular seminoma. This is the first description of a c-KIT gene mutation in a Japanese patient with bilateral TGCT. The mutational analysis of the c-KIT gene seems to provide crucial information for managing TGCT patients not only in europe but also in japan.- - - - - - - - - - ranking = 35.438716589674keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
10/23. High-grade endometrial stromal sarcoma after tamoxifen therapy for breast cancer.A case of high-grade endometrial stromal sarcoma, confined into an intrauterine polypoid growth, in a woman with a history of breast cancer who was treated with adjuvant tamoxifen. Based on the findings, a high-grade endometrial stromal sarcoma was diagnosed. The patient underwent a total hysterectomy and bilateral salpingo-oophorectomy with multiple omental biopsies. Pathological examination on multiple uterine sections showed the absence of residual tumor cells in the uterus. The endometrium showed patterns of glandular cystic hyperplasia. After 14 months of follow-up, the patient is well and free of disease. In deciding if tamoxifen therapy is warranted, all potentially life-threatening adverse events associated with tamoxifen should be considered, including endometrial adenocarcinoma or uterine sarcoma.- - - - - - - - - - ranking = 6keywords = stromal (Clic here for more details about this article) |
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