Routine Transcervical Thymectomy in MEN-1 Patients
Multiple Endocrine Neoplasia Type 1 (MEN-1)Thymic CarcinoidA case series was built after review of available literature by searching four databases (PubMed, Embase, Medline and Cochrane Library) for observational studies or case reports on routine prophylactic TCT for MEN-1 and the development of thymic carcinoids.
Clinical Effectiveness of Serum Chromogranin A Levels on Diagnostic of Pancreatic Neuroendocrine...
Non Functioning Pancreatic Endocrine TumorChromogranin A (CgA) is a glycoprotein with a molecular weight of 49 to 52 kDa produced by chromaffin cells of the adrenal medulla, enterochromaffin-like (ECL) cells, and endocrine cells of the stomach and pancreas, and it is the precursor to several functional peptides including vasostatin and pancreastatin. Importantly, CgA can be measured in the serum or plasma or detected within the secretory vesicles as a general diagnostic biomarker for neuroendocrine tumors (NETs), and plasma CgA levels also provide information regarding tumor burden and response to treatment. It has a sensitivity and specificity between 27% and 81%. Some studies have noted an association between CgA concentrations and tumor location or degree of differentiation. It has also been proposed that plasma CgA levels are more frequently elevated in well-differentiated tumors compared with poorly differentiated tumors of the midgut. Some other clinical series have provided evidence of an association between plasma CgA levels and the extent of disease, tumor burden, or presence of metastases, and high baseline levels of CgA are suggestive of a poor prognosis. However, there exist still controversies the effectiveness of serum CgA levels on diagnostic relevance, treatment response after surgical resection or sandostatin analog, clinicopathologic features of pancreatic neuroendocrine tumors (PNETs). To date, moreover, a precise association between CgA levels and survival has not been clearly demonstrated, although a number of studies suggest that this relationship may exist. There, especially, is no relevant data on value of serum CgA level for clinical usefulness in Korean population.
B-Receptor Signaling in Cardiomyopathy
CarcinomasAmyloidosis37 moreWe hope to determine the importance of different genes (including B receptors) in anthracycline-induced cardiomyopathy. This has important benefits to patients exposed to anthracyclines, as this could help determine whether certain individuals have increased susceptibility to cardiac injury.
Studies of Inherited Diseases of Metabolism
Multiple Endocrine NeoplasiaDiseases of mineral metabolism such as familial multiple endocrine neoplasia type 1 (FMEN1), familial hypocaliuric hypercalcemia (FHH), familial hyperparathyroidism (FH), and pseudohypoparathyroidism (PHP) are known as hereditary abnormalities. Meaning these conditions are passed from parents to their children through genes. These specific conditions result in abnormal levels of calcium in the blood. This study was designed to help researchers understand more about the genes that are responsible for these disorders. By learning more about the genetic process involved in hereditary abnormalities, new tests and treatments can be developed. Subjects for this study will be members of families that have had relatives diagnosed with a disease of mineral metabolism. Participants will be asked to give blood samples for DNA extraction. DNA is the part of cells that carries genetic information. The DNA will be analyzed and the results given to the subjects. Genetic counseling will be provided to subjects to aid in interpreting their results....
Assessing the Patient Experience in Cancer Care
Brain NeoplasmBreast Neoplasm8 moreCommunication is an important component of comprehensive cancer care impacting patient satisfaction, adherence, and quality of life. The wide array of issues addressed in cancer clinical interactions makes communicating about a broad range of topics (including quality of life, communication, symptom control, complementary/alternative therapies, costs, treatment burden, prognosis, anxiety, side-effects, sexual function, palliative care options, etc.) especially interesting and potentially challenging. Some of these topics may not be routinely addressed in the clinical interaction or may require consultative support from other members of the comprehensive cancer care team. One frequently overlooked critical element in research on communication between cancer clinicians, their patients, and their primary care clinicians is describing real-time consultations between patients and their clinicians. These interactions provide rich material for assessing key psycho-social dynamics and identifying issues that patients find important in their care. In order to devise systems of care that optimize the patient experience, it is critical that clinicians and researchers understand, appreciate, and systematically characterize the richness and complexity of the decision-making process in routine cancer consultations between cancer patients and their treating clinicians. This study seeks to assess the patient experience in cancer care by observing patients and their physicians in their clinical interactions and following them for several months to see how their care went. By describing in-depth the conversations and experiences of patients in these clinical interactions, this study will lay the foundation for practice-based interventions to optimize patients' interactions with their cancer care teams.
Study of Molecular Pathways in Medullary Thyroid Carcinoma and Correlation of Molecular Data With...
Medullary Thyroid CarcinomaMultiple Endocrine Neoplasia Type 2Background: Medullary thyroid carcinoma (MTC) is a rare malignancy, occurring either as a sporadic disease (75% of cases), or in a hereditary pattern as multiple endocrine neoplasia (MEN) type 2 (MEN2A or MEN2B) or familial medullary thyroid carcinoma (FMTC). The MTC arises from the neural crest C-cells and in hereditary cases the first pathological disorder is C-cell hyperplasia (CCH) Most patients with MTC have advanced disease at the time of diagnosis. Chemotherapy and external beam radiotherapy have been minimally effective. Molecular targeted therapeutics (MTTs) and other receptor kinases in patients with advanced MTC have demonstrated activity. Despite some clinical responses, the collection of tumor tissues and autologous normal tissues has been virtually non-existent. Thus, laboratory studies defining affected molecular targets and downstream pathways, and molecular data providing direction for future clinical trials has yet to occur. Data from molecular studies of tumor tissue of hereditary or sporadic MTC patients will assist in predicting clinical behavior and the biology of MTC in predicting response to a given MTT, and in designing combination clinical trials. Objectives: Clarify how normal molecular pathways are altered by mutations in the RET protooncogene. Including additional genetic mutations and unidentified chromosomal translocations. Correlate results from molecular analyses of MTC tissue with patient s clinical course. Define how the molecular and clinical data will be useful in designing targeted therapy for patients with MTC. Eligibility: Patients must have confirmed diagnosis of C-cell hyperplasia, primary MTC, or metastatic MTC with archived pathology specimens available at Washington University. Design: Paraffin blocks of MTC tissues from archival samples at Washington University Department of Pathology will be selected. H&E slide from selected tissue blocks will be examined for molecular study suitability. Necessary tissue samples from blocks will have molecular studies, including, gene arrays, array comparative genomic hybridization, immunohistochemistry, and sequencing. Retrospective chart review will occur to obtain relevant clinical information.
Diagnostic Accuracy of Preoperative Diagnostic Procedure in the Assessment of Lymph Node Metastases...
Non Functioning Pancreatic Endocrine TumorPancreatic Neuroendocrine Neoplasms (PanNEN) are a heterogeneous group of neoplasms that arise from the endocrine cells of the pancreatic gland. Non-functioning (NF-PanNEN) represents the most common forms and do not produce syndromes due to hormonal hypersecretion. Several prognostic factors have been demonstrated for NF-PanNEN. The presence of nodal metastasis and lymph node ratio are widely considered predictors of disease-free survival and even the number of positive nodes has been found to be associated with recurrence. In addition to traditional imaging exams, diagnostic work-up should include a Positron Emission Tomography/CT with 68Ga labeled somatostatin analogues, which have shown a high sensitivity and specificity while 18F-FDG PET can be associated for evaluating the possible presence of a high-grade component. Moreover, pancreatic endoscopic ultrasound (EUS) is usually part of the preoperative staging both for imaging details and cytological sampling. Therefore an accurate identification of nodal metastases preoperatively may have important implications for the extent of surgical resection and lymphadenectomy and even for a prognostic outcome. In this study the investigators will evaluate prospectively the accuracy of these diagnostic exams in detecting the lymph node status. Patients with sporadic NF-PanNEN who are candidates for surgical resection will undergo CE-CT scan, 68Ga DOTATOC (and eventually 18F-FDG) PET/MRI and EUS with FNA/B. Reference standard for defining the presence of nodal metastases is represented by pathological examination on the specimen.
Biomarkers for Angiogenesis in Renal Cell Carcinoma and Neuro-endocrine Tumours.
CarcinomaCarcinoma4 moreThe primary objective of this study is to analyse the concentration dopamine and serotonin in thrombocytes of patients with renal cell carcinoma and neuro-endocrine tumours compared to the concentrations of these catecholamines in healthy volunteers. The concentration dopamine and serotonin in thrombocytes with and without medication will also be evaluated.
An Analysis of Presentation and Outcome Following Treatment of Pancreatic Endocrine Neoplasms
Pancreatic Endocrine NeoplasmsA single institutional study of Pancreatic Endocrine Neoplasms over 18 years.
Genetic Evaluation of Families With Endocrine Cancers
Pancreatic CancerThymic Cancer2 moreThis study is being conducted to identify altered genetic factors that may exist and influence endocrine cancers in unrelated MEN1 families with different cancers. A grading system will be developed for endocrine cancers, including pancreatic cancers, thymus gland cancers, parathyroid disease and MEN1 syndrome as low-risk and high-risk to improve screening and timing of surgery.