SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
An autopsy confirmed, primary malignant APUDoma of the liver is described. Immunohistochemical methods demonstrated the presence of glucagon in some 20% of tumor cells. Clinically the 66 year old patient had presented with an abortive form of the glucagonoma syndrome with diabetes mellitus, anemia and profound weight loss.
Explore the source record for details and available documents.
5-HT apudomas are rare. In our country, so far there has been no detailed report about using whole blood 5-HT and urinary 5-HIAA in the diagnosis of apudomas. Measurement of whole blood 5-HT in 87 and urinary 5-HIAA in 44 apudoma patients has been carried out in our laboratory since 1980. The results showed that the values of whole blood 5-HT and urinary 5-HIAA were significantly higher in apudoma patients than those in non-apudoma, postoperative apudoma and normal groups. The value of whole blood 5-HT over 130 micrograms/ml and urinary 5-HIAA over 30 mg/24 h were preliminarily recommended as diagnostic for apudomas.
One should differentiate apudomas, two-component tumors (a combination of apudoma with a non-endocrine tumor), apudoma-like tumors and non-endocrine ones with admixture of APUD cells among the tumors with cells possessing the properties of APUD cells. Apudomas in their turn are represented by microapudomas and high- and low-differentiated macroapudomas. Routine light microscopy in most cases provides for apudoma verification, estimation of its differentiation and tissue origin, occasional identification of a secreted hormone.
Findings at CT, MR imaging and angiography in 29 patients with pancreatic apudomas are presented. CT and angiography were performed in all the patients, MR imaging in 10. Twenty-five patients underwent laparotomy. The ability of the different imaging methods to localize the primary tumor and detect hepatic metastases was compared. CT and MR imaging depicted the primary tumor in 79% and 88% of the cases respectively, angiography in 72%. Enhancement patterns at CT and signal intensity variations at MR imaging were unspecific parameters regarding different types of tumors. CT and MR imaging were considerably more accurate than angiography in evaluating local tumor resectability. Angiography revealed more numerous small hepatic metastases (< 0.5 cm) than CT or MR imaging, but missed metastases in the left lobe in 5 patients. MR imaging seems promising in localizing and characterizing pancreatic apudomas, but further evaluation is needed before any conclusion can be drawn. At present dynamic incremental CT seems mandatory in the evaluation of pancreatic apudomas. Angiography is of value for preoperative and preembolization vascular mapping, and in localizing small pancreatic apudomas not shown at CT or MR imaging. Faster dynamic thin slice CT scanning and fast-sequence MR imaging may increase the sensitivity of detecting small primary pancreatic apudomas.
APUDomas are rare tumours originating from a variety of endocrine cells localized in different organs. Acute complications from APUDomas usually result from the increased biosynthesis and release of bioactive amines or polypeptide hormones by the tumour. Less frequently, bleeding or compression by the tumour can occur requiring emergency surgery. Increased gastrin production by gastrinomas is the cause of ZES (peptic ulceration and diarrhoea) by gastrin effects on gastric acid secretion. Volume depletion, hypokalaemia, severe bleeding, duodenal perforation, oesophageal stricture and pyloric stenosis are the most dramatic complications. Treatment of these complications and their prevention has been facilitated by the availability of antagonists to H2 receptors and H(+)-K+ proton pump. These medications should control acid output in every patient with ZES. Frequent manifestations of carcinoid tumours, VIPomas and medullary thyroid carcinomas are flushing and diarrhoea. Octreotide, a long-acting somatostatin analogue, has markedly changed the management of these patients, their symptoms decreasing in severity or disappearing in most cases. Octreotide has also been used with success in the prevention and treatment of the carcinoid crisis, a dreaded complication of carcinoid tumours. A better understanding of the pathophysiology of APUDomas has enabled new treatment designs which have considerably ameliorated the quality of life of patients affected by these tumours; efforts must be continued to affect their life expectancy.
Eighteen patients with various apudomas metastatic to the liver underwent hepatic artery embolization. In one group of 14 patients, hepatic artery embolization was the only antineoplastic therapy used during the study period. Three deaths occurred in this group. A partial remission of the hepatic metastases occurred in eight of the 11 living patients. In addition to hepatic artery embolization, a second group of four patients received concurrent hepatic artery infusions of chemotherapeutic agents. A partial remission occurred in three of these patients. Hepatic artery embolization may offer significant palliation for patients with apudomas metastatic to the liver by reducing the tumor bulk and thus decreasing the production of pharmacologically active substances secreted by functioning tumors. The impact of this treatment on the median survival time of patients with apudomas could not be demonstrated.
Previous reports of endocrine activity of glomus jugulare tumors have been of pheochromocytomas but not carcinoid apudomas. This paper is to alert the otolaryngologist to the presence of carcinoid apudomas in glomus jugulare tumors. A patient with a long history of explosive diarrhea, facial flushing, and violent headaches, the symptom of carcinoid syndrome, was cured after removal of his large glomus jugulare tumor. The diagnosis of carcinoid syndrome is made with a good history and confirmed with a 24 hour urine collection for 5 Hydroxy indole acetic acid (5 Hiaa). a series of patients treated at the Johns Hopkins Hospital for glomus jugulare tumors is reviewed for the presence of endocrine activity and the literature on endocrine active glomus jugulare tumors is presented. Preoperative evaluation for the presence of both pheochromocytoma and carcinoid apudoma is recommended for all patients with glomus jugulare tumors.
Apudomas are neoplasms known to produce many biologically active amines. Some of these tumors have been known for 70 years, but over the last 15 years a wealth of new knowledge has been obtained. This study reports experience with apudomas over an 8-year period (1975-1983) at The Piedmont Hospital. There were 159 tumors with the potential for producing various ectopic hormones as well as biologically active amines, including 112 oat cell carcinomas, 37 carcinoid tumors, 4 paragangliomas, 2 pancreatic islet cell tumors, 2 medullary thyroid carcinomas, 1 pituitary adenoma, and 1 pinealocytoma. Of the 112 oat cell carcinomas, only 2 per cent had hormone-related symptoms. Of the carcinoid tumors, 12 were found in the appendix, 9 in the small intestine, 9 in the lungs, 4 in the colon and 2 in the rectum. Nineteen of the 37 carcinoids had hormone-related symptoms but none had a classic carcinoid syndrome. A third group of apudomas included several of relative rarity, the paragangliomas, the medullary thyroid carcinomas, the pancreatic islet cell tumors, pituitary adenomas, and pinealocytomas. Of this latter group, only the pancreatic islet cell tumors had symptomatology that could be related to ectopic hormone production.
Uteric apudoma found inside the cervical canal polyp is described. Histologically, it was a combination of structures characteristic of apudomas of different levels and differentiation. Problems of histogenesis of cervical apudomas are discussed.
An oat cell carcinoma occurring in the esophagus of a 62-year-old man is described. A strong argyrophilia of the oat cells together with the presence electron microscopically of neurosecretory type granules in their cytoplasm suggests a derivation of this tumor from the cells of the APUD series, and the tumor is diagnosed as apudoma. An aberrant columnar epithelium on the esophageal surface is presumed to be the source of its origin. A rapid postoperative course of the patient despite a rather circumscribed growth pattern of the primary tumor appears to characterize the clinical aspect of this case. An apparent accumulation of the cases with esophageal apudoma in Japan is discussed.
The pathology of APUDomas involves two main areas. The first is the biochemical aspects of these tumours and the use of biochemical tests to investigate a patient suspected of having an APUDoma, and the other is the histopathology of these tumours. This review is devoted to the histopathological considerations.
It is tempting to speculate that genetic studies of the human APUDomas, particularly those of a hereditary nature, may ultimately provide valuable clues to the molecular basis of malignant transformation in cells of all types, uncover the mechanisms responsible for tumor progression, and perhaps decipher the signals important in the differentiation of normal neural crest-derived tissue. Generally, several strategies have been used in the genetic analysis of these tumors with success. These include (1) cytogenetic examination of recurring chromosomal abnormalities in hopes of pinpointing critical neighboring growth regulatory sequences important in tumor evolution, (2) identification of dominant acting oncogenes in tumor cells, (3) search for recessive inactivated suppressor genes that may regulate cell growth by analyzing tumors for loss of heterozygosity (LOH), and (4) genetic linkage studies of kindreds affected with familial APUDomas to identify and subsequently characterize the predisposition gene using a positional or functional cloning approach. The results of these strategies as they have been employed in the investigation of cutaneous malignant melanoma (CMM), the dysplastic nevus syndrome (DNS), and the multiple endocrine neoplasia (MEN) syndromes are summarized.
The concept of the APUD system and the APUDomas associated with it has evolved significantly since Pearse's description in the 1960s. Part of this evolution has been an understanding of the relationships between the APUD system and the central and autonomic nervous systems. The APUD system now referred to as the diffuse neuroendocrine system, can be linked to the central nervous system and autonomic nervous system by genetics, embryology, cellular characteristics, anatomy, interaction of the systems, and the immune system. Awareness of these relationships may enable clinicians to better understand APUDomas and lead to better methods of detection of these tumours and their treatment.
The different diagnostic imaging modalities available for determining the location of the various APUDomas are discussed with reference to their advantages and disadvantages. The ability to image these lesions, and to be confident in their role in the underlying pathophysiology, and clinical neuroendocrine syndrome causing symptomatology and illness, has proved to be the key to successful treatment. In many instances it is not the diagnosis that is in question, but it is the extent of disease, the location of the lesion, or whether the anatomical abnormality under study is responsible for the complicating symptoms being considered, that are the crucial questions. The ability to locate APUDomas by a variety of direct and indirect imaging or regionalizing modalities, represent a magnificent advance in the management of these tumors. It should, however, be kept in mind that no one modality or diagnostic method can uniformly be relied upon. It is evident that a multimodal, interactive medical team approach is essential to the successful overall management of patients afflicted with these fascinating tumors.
Pancreatic apudomas are not common but are frequently curable. Thus, it is important for every clinician to be fully aware of the varied clinical syndromes that suggest their presence. The availability of specific radioimmunoassays has made confirmation of the diagnosis relatively simple. Advances in the techniques for staining the different cell types have led to the recognition that many of these tumors are mixed, and that the general term "pancreatic apudoma" is appropriate. Pancreatic endocrine tumors, as examples to "nature's experiments," have yielded considerable insight into the possible physiologic effects of the various peptides they produce. It is to be hoped that further study of tumors such as the somatostatinoma and PPoma may yield further information about these enigmatic compounds.
Prospective screening was carried out in 12 members of three families with multiple endocrine adenopathies, type I (MEA,I) and in 14 patients with no multiple endocrine adenopathies with and without other endorcinopathies. Elevated basal and responsive (after a meal) plasma concentrations of a relatively new candidate-hormone, human pancreatic polypeptide (hPP), were associated with pancreatic apudoma tumors in three asymptomatic patients with multiple endocrine adenopathies, type I. Two of these patients had excision of the tumors that resulted in normal plasma hPP concentrations postoperatively. Both tumors contained hPP predominantly by immunocytochemistry; one, a pure pancreatic polypeptide apudoma, was studied extensively demonstrating also by radioimmunoassay a high content of hPP and negligible amounts of insulin, glucagon, somatostatin, vasoactive intestinal polypeptide and gastrin. In this patient plasma concentrations of other polypeptides including insulin, glucagon, somatostatin, vasoactive intestinal polypeptide, gastrin, parathyrin, thyrocalcitonin, prolactin, corticotropin, growth hormone, thyrtropin and amine, serotonin, were within normal limits. The other patient, after excision of an hPP-detected pancreatic mixed hPP-gastrinoma, also became eugastrinemic postoperatively. Normal basal plasma hPP concentrations, but with exaggerated hPP responses to a meal in 11 patients, were associated with various combinations of islet cell hyperplasia, antral G cell hyperplasia with moderate hypergastrinemia and parathyroid hyperplasia. The patients with multiple endocrine adenopathies who have demonstrated this type of increased hPP response to a meal have not been operated on but are at risk for islet hyperplasia. Four of the 12 patients with multiple endocrine adenopathies, type I, with both normal basal and normally responsive hPP concentrations have no evidence as yet of pancreatic involvement.
A variety of cells found in the pituitary and pineal glands, sympathetic nervous system and adrenal glands, the gut, pancreas, thyroid (C-cells), chemoreceptors (type I-Cells), lungs (P-cells), skin (melanocytes) and the urogenital tract have a common origin from the neural crest. These cells are programmed for neuro-endocrine function and, as a group, can be regarded as one of the physiological control systems. They secrete a variety of amine and peptide hormones and have common cytochemical characteristics from which the term APUD cell is derived. Tumours of these cells are referred to as 'apudomas' and may synthesise not only their own hormones but also those which are normally produced by other APUD cells. The relevant physiological properties of some of the peptides which have been described relatively recently are discussed and the principal clinical syndromes produced by the APUDomas are described.