Glucagon, gastrin, and carcinoid tumors of the duodenum, pancreas, and stomach: polypeptide "apudomas" of the foregut.
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Endocrine tumors arising sporadically or as a manifestation of the multiple endocrine neoplasia type I syndrome (MEN I) have been shown to have mutations on chromosome 11. These mutations can be detected at the molecular level by loss of heterozygosity (LOH) for DNA markers from chromosome 11. This study represents one of the largest collections of sporadic endocrine tumors in which LOH was systematically assessed on chromosome 11 for the loci flanking the proposed MEN I region. DNA was isolated from 39 endocrine tumors and probed with 7 DNA probes spanning the region of chromosome 11q13 from the loci PYGM to INT-2. Eleven tumors demonstrated LOH at any two loci in this region. The remaining 28 tumors showed no LOH or were noninformative at these loci. Thus, nearly 30% of these tumors showed LOH in the region (from PYGM to INT-2) that is thought to contain the MEN I gene(s). Previous studies of sporadic endocrine tumors have suggested that these tumors may arise via the same mechanism as tumors of the MEN I syndrome. Namely, these sporadic tumors are thought to result from mutations leading to genetic loss on the long arm of chromosome 11, thereby inactivating a possible tumor-suppressor gene (or genes). These findings strongly support the hypothesis that sporadic pancreatic endocrine tumors share a similar etiology of tumorigenesis with tumors of the MEN I syndrome, which principally involves deletion of a tumor-suppressor element (or elements).
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Multiple endocrine neoplasia, type 2b, is a designation that has been proposed for the combination of medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and somatic abnormalities. The neural lesions produce a characteristic diffuse or nodular enlargement of lips, tongue, and buccal mucosa. Microscopically, the lesions are composed of unencapsulated masses of hypertrophied and elongated nerves in which are found rare ganglion cells. The histologic similarity between the oral mucosal lesions and the alimentary tract ganglioneuromatosis in the syndrome suggests that the term ganglioneuromatosis is the most appropriate designation for the oral lesions. The oral lesions signal high risk for two potentially lethal neoplasms--medullary thyroid carcinoma and pheochromocytoma. Thus, patients having the characteristic findings are in urgent need of repeated evaluation of thyroidal C-cell and adrenal medullary function.
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Radionuclide imaging has been helpful to oncologists in the initial evaluation and follow-up of patients with primary and metastatic disease. With the introduction of ultrasound and computed tomography, there has been some reduction in the number of radionuclide imaging procedures. These imaging modalities should be used to complement one another. With the introduction of single-photon emission computed tomography in clinical nuclear medicine, there will be a significant improvement in the sensitivity, accuracy, and quantitative ability to detect abnormal lesions. There are intensive efforts in the development of tumor imaging using new agents. Specific antibodies for surface tumor antigens can be labeled with radioisotopes and used as tools for screening primary tumors and metastatic lesions. Therefore, for the present, radionuclide imaging will continue to contribute to clinical oncology.
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No good randomized studies exist for many types of endocrine tumors to prove that surgery increases the survival of patients with these tumors. However, many case reports and our own experience suggest that it does. Therefore, surgery may be performed to potentially increase the survival of patients with metastatic and localized malignant endocrine tumors. Subsequently, these tumors may still recur locally or distantly. Therefore, surgery is usually a palliative procedure but is occasionally curative. Because endocrine tumors secrete hormonal substances resulting in particular syndromes, debulking surgery may be necessary to control hormonal syndromes. The location of a tumor may cause mass symptoms that can also be relieved by surgery. Thus, many patients with malignant endocrine neoplasia benefit from aggressive surgery. The surgeon must determine the resectability of the tumor as well as the operative morbidity and benefit to the patient before proceeding.
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A patient with Von Hippel-Lindau's disease was followed from the time of diagnosis to the time of his death 13 year later. He was asymptomatic when the diagnosis was made, although a hemangioblastoma of the medulla was found on angiography. The patient's course and autopsy are described and the features of this disease are reviewed.
During the past ten years, endoscopic adrenalectomy has emerged as the standard of treatment of benign diseases of the adrenal gland. Although no randomised trial exists comparing open with endoscopic adrenalectomy, the endoscopic approach has proven to be safe with lower morbidity and shorter hospital stay compared with open surgery. For parathyroidectomy several different minimal invasive techniques have been described (i.e. fully endoscopic, videoscopic assisted, and focused using mini-incision). Prerequisits for all of these approaches are a positive preoperative localisation of the adenoma, an intraoperative parathormone testing, and the respective surgical experience in the minimal invasive technique. Proponents of the videoendoscopic assisted thyroidectomy mention the enhanced exposure of the surgical field to be beneficial. However, drawbacks of this technique are the fact that a minority of patients qualify for this approach and that the learning curve is quite long for the surgeon. The experience with the laparoscopic approach to endocrine pancreatic tumours is still limited. Good indications are insulinomas that are located anteriorly or within the tail of the pancreatic gland. Nowadays, laparoscopic enucleation of such tumours and tail resections become feasable and safe.