1/38. 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) |
2/38. c-kit immunocytochemical staining in the cytologic diagnosis of metastatic gastrointestinal stromal tumor. A report of two cases.BACKGROUND: Gastrointestinal stromal tumor (GIST) is a distinct group of mesenchymal neoplasms recently shown to exhibit differentiation toward interstitial cells of cajal (ICC). C-kit (CD117), an immunocytochemical marker consistently expressed in normal ICC, is demonstrable in 81-100% of GISTs. We report two cases wherein immunocytochemical staining for c-kit aided in the diagnosis of metastatic GIST in the liver. CASES: Two patients, a 37-year-old female (case 1) and a 76-year-old male (case 2), presented with multiple nodules in the liver. They had a history of small bowel GIST resected 11 and 1 year earlier, respectively. Fine needle aspiration of the liver nodules showed loose aggregates or fascicles of spindle cells with elongated to oval nuclei, rare paranuclear vacuoles and eosinophilic cytoplasm. The spindle cells showed minimal (case 1) to moderate nuclear pleomorphism (case 2), with occasional mitotic figures seen in case 2. Immunocytochemical staining revealed strong and diffuse staining for c-kit; it was negative for actin, desmin, CD34 and S-100 protein. Thus, a diagnosis of metastatic GIST was rendered. Histologic review of the primary small bowel GISTs of both cases and the subsequently resected liver nodules in case 1 confirmed the diagnosis. CONCLUSION: Metastatic GIST may pose diagnostic problems due to its broad morphologic spectrum and variable cytologic atypia; in particular, distinction from leiomyosarcoma and other mesenchymal tumors is difficult. The diagnostic difficulty is compounded when the prior history of gastrointestinal tumor is not available or forgotten and when GIST is the initial presentation of the tumor. C-kit is a highly sensitive and reliable immunocytochemical marker that can aid in the diagnosis.- - - - - - - - - - ranking = 212.38866034712keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
3/38. Case report of heterotopic bone formation in metastatic carcinoma of the colon.The case of an 83-year-old woman, who was operated on for an adenocarcinoma of the sigmoid colon and died with retroperitoneal matastases, is described. The post mortem examination showed widespread heterotopic bone formation in these metastases. Gastrointestinal cancers and their metastases are liable to calcify and ossify, and they do so more frequently than other malignant epithelial tumors. A search through the literature led to the discovery of 35 other cases of this type. The highest frequency of heterotopic bone formation occurs in cancers of the distal portion of the large intestine and in pulmonary and lymph node metastases. The results of the present case support the view that bone formation derives from the metaplasia of stromal fibroblasts into osteoblasts. The knowledge that gastrointestinal cancers can calcify and ossify has a definite diagnostic relevance for the radiologist and gastroenterologist.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
4/38. trisomy 8 as sole karyotypic aberration in an ovarian metastasizing sertoli-leydig cell tumor.Sertoli-Leydig cell tumors (SLCTs) represent a rare group of sex-cord stromal tumors of the ovary of unknown pathogenesis. We report a SLCT of intermediate differentiation with peritoneal recurrence and lymph node metastasis 12 months after removal, including cytogenetic analysis by comparative genomic hybridization and fluorescence in situ hybridization, which showed trisomy 8 as sole unbalanced karyotypic aberration. Our results provide evidence that a simple numeric chromosomal abnormality in SLCT may be associated with a malignant phenotype and suggest that the molecular pathogenesis of SLCT may be different from ovarian granulosa-stromal cell tumors.- - - - - - - - - - ranking = 2keywords = stromal (Clic here for more details about this article) |
5/38. Positron emission tomography for detecting clinically occult surgically resectable metastatic ovarian cancer.BACKGROUND: The specific indications for PET imaging in patients with ovarian cancer have yet to be precisely defined. While PET has limited sensitivity for detecting small-volume (<1 cm) metastatic disease, accurate identification of larger tumor nodules may have a significant impact on clinical management and the selection of patients for cytoreductive surgery. CASES: The cases of two patients with suspected recurrent Stage IIIC serous ovarian cancer based solely on elevated CA125 levels and one patient with an apparent Stage IC poorly differentiated ovarian sex cord-stromal tumor who had macroscopic surgically resectable disease (>1 cm) identified by PET after negative or equivocal computed tomography are presented. CONCLUSION: PET imaging may be a useful technique for identifying potentially surgically resectable, macroscopic metastatic ovarian cancer when computed tomography findings are negative or equivocal.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
6/38. Malignant phyllodes tumor of the breast metastatic to the parotid gland diagnosed by fine needle aspiration biopsy. A case report.BACKGROUND: phyllodes tumor (cystosarcoma phyllodes) is a rare fibroepithelial neoplasm of the breast. Malignant phyllodes tumor is characterized by an infiltrative border and marked degree of hypercellular stromal overgrowth with > 5 mitoses per 10 high-power fields. Distant metastasis occurs in 10-20% of patients with malignant phyllodes tumor. The most common sites of distant metastases are the lungs, bone and abdominal viscera. Although theoretically any organ may have metastasis, the parotid gland has not been documented before in the English-language literature. CASE: A 40-year-old, Caucasian woman with a history of malignant phyllodes tumor of the left breast presented with a mass on the right side of the parotid gland. Fine needle aspiration biopsy of the mass revealed abundant discohesive spindle cells showing moderate nuclear pleomorphism with occasional mitoses. No epithelial elements were seen. A diagnosis of malignant spindle cell tumor consistent with metastatic malignant phyllodes tumor was made. histology confirmed the cytologic diagnosis. CONCLUSION: Fine needle aspiration biopsy is accurate and efficient in conjunction with clinical information in the diagnosis of malignant phyllodes tumor of the breast metastatic to the parotid gland.- - - - - - - - - - ranking = 1keywords = stromal (Clic here for more details about this article) |
7/38. 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 = 52.847165086779keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
8/38. Isolated abdominal wound metastasis from a gastrointestinal stromal tumor.BACKGROUND: gastrointestinal stromal tumors (GIST) frequently recur even after complete resection. The typical pattern of failure from GISTs is both local and distant with hepatic and peritoneal metastases being most common. Isolated abdominal-wall recurrence from GISTs has not been previously described. AIM OF THE STUDY: To report an isolated abdominal-wound recurrence in the absence of widespread disease in a patient with GIST. methods: Case report of a GIST and isolated abdominal-wound recurrence after laparoscopic-assisted en bloc resection. RESULTS: Elderly male patient presented with an isolated abdominal wall incisional recurrence 18 mo after gastric resection and adjuvant imatinib mesylate therapy for a high-grade GIST. CONCLUSIONS: Complete resection of gastrointestinal stromal tumors followed by imatinib therapy may alter the extent of recurrence.- - - - - - - - - - ranking = 265.2358254339keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
9/38. Molecular response of gastrointestinal stromal tumour after treatment with tyrosine kinase inhibitor imatinib mesylate.Bleeding from the tumour site is not uncommon during the treatment of gastrointestinal stromal tumours with imatinib mesylate. It might represent an early reaction of highly vascularised tumour tissue to receptor blockade. Although often requiring emergency surgery, this is not necessarily a deleterious sign. Slow tumour regression and cystic tissue alteration may follow. Using immunohistochemistry and consecutive resection specimens, it was shown that the number of mitoses decreased significantly and MIB-1 as a marker of cell proliferation could no longer be detected. In the few tumour cells still present, the magnitude of expression of the pathognomonic marker CD117 remained unchanged. Decreases in the size of tumours responding to imatinib mesylate cannot be expected to meet the world health Organisation or RECIST (response evaluation criteria in solid tumours) criteria. This underlines the necessity of functional imaging by positron emission tomography, contrast enhanced magnetic resonance imaging, or magnetic resonance spectroscopy to assess the response to treatment.- - - - - - - - - - ranking = 746.74608676368keywords = gastrointestinal stromal tumour, gastrointestinal stromal, stromal tumour, stromal (Clic here for more details about this article) |
10/38. Malignant gastrointestinal leiomyosarcoma and gastrointestinal stromal tumor with prominent osteoclast-like giant cells.CONTEXT: One case of leiomyosarcoma and one case of gastrointestinal stromal tumor with prominent osteoclast-like giant cells have so far been reported in the digestive tract. OBJECTIVE: To ascertain the clinicopathologic features and biologic behavior of these tumors, we report 3 additional cases of leiomyosarcoma of the gastrointestinal tract and one malignant gastrointestinal stromal tumor. DESIGN: Histologic and immunohistochemical examinations were performed. Clinical and follow-up data were recorded, and the literature was reviewed. RESULTS: The age of the patients ranged from 50 to 68 years (mean, 62 years). One of the lesions arose in the stomach, one in the ileum, and 2 in the colon. Three tumors showed a strong positivity for muscle actin and desmin and were diagnosed as leiomyosarcomas, 2 of them showing spindle cells and 1 of them showing epithelioid cells. The fourth tumor reacted strongly positive for c-Kit (CD117) and vimentin, and it was diagnosed as an epithelioid malignant gastrointestinal stromal tumor. All tumors were characterized by numerous osteoclast-like giant cells that were unevenly distributed and that, using immunohistochemistry, reacted strongly with CD68. CONCLUSIONS: Malignant stromal tumors with osteoclast-like giant cells of the gastrointestinal tract are rare entities, are more commonly of a myogenic origin such as leiomyosarcoma, and seem to have an aggressive behavior.- - - - - - - - - - ranking = 370.93015560745keywords = gastrointestinal stromal, stromal (Clic here for more details about this article) |
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