Pancreatic involvement in Von Hippel-Lindau disease.
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BACKGROUND: Involvement of the pancreas in Von Hippel-Lindau disease that is a tumor predisposing syndrome mentioned in literature with some morbid and mortal progression. AIMS: For evaluation the faith of the pancreatic involvement in VHL disease we analysed our patient population with VHL disease. MATERIALS AND METHODS: 12 of the 56 patients that were evaluated in our institute with the diagnosis of Von Hippel-Lindau disease had pancreatic involvement. They are periodically examined for 5 years follow up period. Their retrospective analysis was accomplished. RESULTS AND CONCLUSIONS: Pancreatic involvement in our patient population disclosed lesions that were multicysts or serous cystadenomas. During follow up period, we did not observe significant morbidity related to pancreatic involvement. Repeated radiological examination of pancreatic lesions disclosed insignificant modifications such as slight increase or decrease in size. Whereas we considered morbidity and mortality related to renal and central nervous system pathologies in VHL disease. Shortly, even pancreatic involvement in VHL disease requires close clinical follow up, morbidity and mortality in this case is not severe as in renal and the central nervous system involvement. (+info)
Tumour-infiltrating gamma/delta T-lymphocytes are correlated with a brief disease-free interval in advanced ovarian serous carcinoma.
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BACKGROUND: Significant progress has been made in understanding the molecular biology of ovarian carcinoma. Along with the molecular characteristics of cancer, the patient's response to the tumour may also contribute to survival; in particular, the effect of the immune system may play an important role on survival of cancer patients. PATIENTS AND METHODS: We analysed the CD3 positive tumour-infiltrating T cells and direct molecular assessment of T cell receptors (TCRs) gamma and beta in 95 advanced ovarian carcinomas. RESULTS: Gamma/delta T cells are statistically correlated with a brief disease-free interval (P=0.036). CD3 positive tumour-infiltrating T cells are correlated with a brief disease-free interval and with survival (P=0.004 and P=0.0001, respectively). CD3 positive tumour-infiltrating T cells are associated with clinical responsiveness to chemotherapy (P=0.003). CONCLUSIONS: Further studies are required to better understand the role of gamma/delta T cells in ovarian carcinoma, yet these data underline the importance of host immune response to cancer and the need to better study immune mechanisms to modulate the therapeutic treatment of cancer. (+info)
Cell-nonautonomous induction of ovarian and uterine serous cystadenomas in mice lacking a functional Brca1 in ovarian granulosa cells.
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Women with germline mutations in BRCA1 have a 40% risk of developing ovarian cancer by age 70 and are also predisposed to cancers of the fallopian tubes. Given that ovulatory activity is a strong risk factor for sporadic ovarian cancer, we hypothesized that reduced BRCA1 expression might predispose to gynecological cancers indirectly, by influencing ovarian granulosa cells. These cells secrete sex steroids that control the ovulatory cycle and influence the growth of ovarian epithelial tumors. Granulosa cells also secrete mullerian inhibiting substance (MIS), a hormone that inhibits both the formation of female reproductive organs in male embryos and the proliferation of ovarian epithelial tumor cells. We tested this hypothesis by using the Cre-lox system to inactivate the Brca1 gene in mouse ovarian granulosa cells. A truncated form of the Fsh receptor promoter served as the Cre driver. Here, we show that indeed, inactivation of the Brca1 gene in granulosa cells led to the development of cystic tumors in the ovaries and uterine horns. These tumors carried normal Brca1 alleles, supporting the view that Brca1 may influence tumor development indirectly, possibly through an effector secreted by granulosa cells. (+info)
Calcifications in mucinous and serous cystic ovarian tumors.
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Mucinous cystic ovarian tumors sometimes contain calcifications, but the frequency and significance of such calcifications in diagnostic radiology are not well understood. We therefore retrospectively investigated the radiological and histopathological evidence of calcifications in 44 cases of ovarian mucinous cystic tumors (22 benign, 13 borderline, and 9 malignant) and 21 cases of ovarian serous cystic tumors (6 benign and 15 malignant) in which a non-contrast CT scan was performed. The shape and distribution of the calcifications in the mass lesion were assessed both radiologically and histopathologically. Calcifications were noted in 34.1% of mucinous cystic tumors on CT scans and 56.8% in histopathological studies, and they were found in two locations, intramural and intra-cystic, according to the histopathological findings. Intramural calcifications were frequent in benign tumors, and intra-cystic calcifications were frequent in proliferating tumors. Calcifications (psammoma bodies) were noted in 4.7% of serous cystic tumors on CT scans and 14.3% in histopathological studies. CT was not sufficiently sensitive in the detection of intra-cystic calcification in mucinous tumors and psammoma bodies in serous tumors. However, the presence of intramural calcifications may be a good indicator of mucinous tumors. Understanding the frequency and morphology of the calcifications in these neoplasms is one of the keys to making a correct diagnosis. (+info)
Serous cystadenoma of the pancreas: tumor growth rates and recommendations for treatment.
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OBJECTIVE: To define the natural history and optimal management of serous cystadenoma of the pancreas. SUMMARY BACKGROUND DATA: Serous cystadenoma of the pancreas is the most common benign pancreatic neoplasm. Diagnostic criteria, potential for growth or malignancy, and outcomes are not well defined. As a result, management for patients with serous cystadenomas varies widely in current practice. METHODS: A total of 106 patients presenting with serous cystadenoma of the pancreas from 1976-2004 were identified. Hospital records were evaluated for patient and tumor characteristics, diagnostic workup, treatment, and outcome. Twenty-four patients with serial radiographic imaging were identified, and tumor growth curves calculated. RESULTS: Mean age at presentation was 61.5 years and 75% of patients were female. The most common symptoms were abdominal pain (25%), fullness/mass (10%), and jaundice (7%); 47% were asymptomatic. Mean tumor diameter was 4.9 +/- 3.1 cm, which did not vary by location. Tumors <4 cm were less likely to be symptomatic than were tumors > or =4 cm (22% vs. 72%, P < 0.001). The median growth rate in the patients who had serial radiography was 0.60 cm/y. For tumors <4 cm at presentation (n = 15), the rate was 0.12 cm/y, whereas for tumors > or =4 cm (n = 9), the rate was 1.98 cm/y (P = 0.0002). Overall, 86 patients underwent surgery, with one perioperative death. CONCLUSIONS: Large (>4 cm) serous cystadenomas are more likely to be symptomatic. Although the median growth rate for this neoplasm is only 0.6 cm/y, it is significantly greater in large tumors. Whereas expectant management is reasonable in small asymptomatic tumors, we recommend resection for large serous cystadenomas regardless of the presence or absence of symptoms. (+info)
Secretin receptors in normal and diseased human pancreas: marked reduction of receptor binding in ductal neoplasia.
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Receptors for gut hormones, which are often overexpressed in cancer, are clinically relevant for receptor-targeted tumor imaging and therapy. Because the receptors for the gut hormone secretin are poorly characterized, we assessed secretin receptor expression in the main secretin target, the human pancreas. We investigated 58 non-neoplastic pancreases and 55 pancreatic tumors for receptor localization and density by in vitro receptor autoradiography using [(125)I]Tyr(10) rat secretin and for secretin receptor mRNA by reverse transcriptase-polymerase chain reaction. Secretin receptors were highly expressed in non-neoplastic ducts and lobuli and also in lower amounts in ductal neoplasias, including ductal adenocarcinoma, intraductal papillary mucinous tumors, and pancreatic intraepithelial neoplasia. Reverse transcriptase-polymerase chain reaction revealed wild-type receptor mRNA in the non-neoplastic pancreas and both wild-type and spliced variant receptor transcripts in ductal adenocarcinomas. Serous cystic tumors were highly positive for secretin receptors, whereas mucinous cystic tumors were negative. This study is the first to describe the precise secretin receptor distribution in human non-neoplastic pancreas and various pancreatic tumors. High secretin receptor expression in the non-neoplastic ducts reflects the major role of secretin in bicarbonate secretion. Reduced secretin binding in pancreatic ductal tumors may relate to (alternatively spliced) secretin receptor isoforms. Thus, secretin receptors in pancreatic tumors may represent potential clinical targets. (+info)
Serous borderline tumor of the fallopian tube presented as hematosalpinx: a case report.
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BACKGROUND: Compared with their ovarian counterparts, serous borderline tumors of the fallopian tube are uncommon, with limited experience about their clinical behaviour. We present a case of serous borderline tumor of the fallopian tube with unusual presentation and summarise all the published cases to date. CASE PRESENTATION: A case of serous borderline tumor of the fallopian tube in a 34-year old patient is presented, incidentally found during routine gynecologic examination. At laparoscopy the tumor was unusually presented as hematosalpinx and was treated by salpingectomy. Cell-cycle analysis of the tumor tissue revealed a diploid DNA content and a low S-phase fraction. There was no evidence of the disease during the follow-up period of 4.6 years. CONCLUSION: The current case and review of the literature suggest salpingectomy as the optimal treatment for patients with serous borderline tumor of the fallopian tube. (+info)
Molecular pathogenesis of ovarian borderline tumors: new insights and old challenges.
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Ovarian borderline (low malignant potential) tumors are a puzzling group of neoplasms that do not fall neatly into benign or malignant categories. Their behavior is enigmatic, their pathogenesis unclear, and their clinical management controversial, especially for serous borderline tumors (SBT), the most common type of ovarian borderline tumor. Clarifying the nature of borderline tumors and their relationship to invasive carcinoma has puzzled investigators since the category was created over 30 years ago. Much of the confusion and controversy concerning these tumors is due to a lack of understanding of their pathogenesis and an absence of a model for the development of ovarian carcinoma. This review summarizes recent molecular studies of ovarian borderline tumors with special emphasis on the role of SBT in tumor progression and its relationship to ovarian serous carcinoma. (+info)