1/65. Neuroendocrine carcinoma of the uterus. Neuroendocrine carcinomas of the cervix and endometrium were reviewed. They have been variously designated as carcinoid, argyrophil cell carcinoma, apudoma, small cell carcinoma, oat cell carcinoma, endocrine carcinoma, and neuroendocrine carcinoma, the last-mentioned term being preferred in this chapter. Adenocarcinomas with neuroendocrine cells are occasionally encountered in the cervix and endometrium. It is generally questioned whether they should be included in the spectrum of neuroendocrine carcinomas, although differential diagnosis between some such tumors of the gastrointestinal tract and neuroendocrine carcinoma is reported to be difficult. Since the majority of neuroendocrine carcinomas of the cervix are highly aggressive, it is important to establish the neuroendocrine nature in the cervical carcinomas. In addition to the characteristic histologic features and argyrophil stainability, immunohistochemical demonstration of several neuroendocrine markers may be helpful in diagnosing neuroendocrine carcinoma of the cervix. Ultrastructural demonstration of neurosecretory granules is almost decisive in establishing the tumor's neuroendocrine nature, but it is not applicable in all cases. Neuroendocrine carcinomas of the cervix have been treated by surgery, radiation therapy, and chemotherapy, but optimal treatment methods have not yet been established because of the rarity of the tumor. Finally, we have described a typical neuroendocrine carcinoma of the cervix and reported some data regarding its experimental study. ( info) |
2/65. apudoma and subcorneal pustular dermatosis (Sneddon-Wilkinson disease). The subcorneal pustular dermatosis (SPD) is a rare dermatosis. IgA monoclonal gammapathy is the most frequently associated disease with the SPD. We report a case of SPD in a patient with metastatic apudoma, an association not previously described. The rare nature of both diseases suggests that this association is not fortuitous. ( info) |
| A metastatic pancreatic polypeptide tumour is described which presented clinically with diabetes mellitus. This is the first case to present this way. ( info) |
4/65. Oat cell carcinoma (apudoma) of the esophagus: a case report. 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. ( info) |
5/65. Clinically silent gross hypergastrinaemia from a multiple hormone-secreting pancreatic apudoma. A patient is described who had a malignant pancreatic islet cell apudoma secreting corticotrophin (ACTH) and melanocyte-stimulating hormone (MSH), both of which were clinically active, and very large quantities of immunoreactive gastrins, which were biologically active but clinically silent (normal gastric acid secretion and no peptic ulceration). The presence of parietal cell antibodies, with no increase in the plasma concentrations of hormones which can inhibit gastric acid secretion (secretin, GIP and VIP), suggests that many of the of the parietal cells may have been blocked by the autoantibodies. ( info) |
6/65. Cyclical release of vasoactive intestinal polypeptide (VIP) from a pancreatic islet cell apudoma. A patient is described who presented with the classical symptomatology and profound electrolyte disturbance of the Verner-Morrison syndrome due to a pancreatic apudoma secreting vasoactive intestinal polypeptide (VIP). diagnosis was confirmed by plasma VIP as measured by a radio-immunoassay technique now available. It is suggested that the cyclical nature of the symptoms in this case was due to cyclical release of VIP from the tumour in response to an unknown stimulus. perfusion studies confirmed the excess secretory state of water, sodium and chloride in the small intestine. Symptoms were completely abolished by surgery and the progress is being monitored by means of serial plasma VIP estimations to detect any early recurrence of metastatic disease. ( info) |
7/65. Multiple-hormone producing lung carcinoma. Endocrine and immunohistochemical studies were performed in a patient with lung cancer associated with gynecomastia. Elevated level of human chorionic gonadotropin (hCG) in plasma and mild hyperadrenocorticism were demonstrated by hormone assays. Postmortem examination proved the existence of anaplastic small cell carcinoma of the lung mixed with a feature of chorioepithelioma. The presence of significant amounts of adrenocorticotropic hormone (ACTH), beta-melanocyte stimulating hormone (beta-msh), calcitonin, gastrin, hCG, hCG-alpha, hCG-beta and human chorionic somatomammotropin (hCS) in tumor tissues was demonstrated by radioimmunoassays, bioassay and immunohistochemical techniques. We present here a unique case of multiple hormones producing tumor elaborating both hormones of amine precursor uptake and decarboxylation (APUD) series (ACTH, beta-msh, calcitonin and gastrin) and of placental origin (hCG, hCG-alpha, hCG-beta and hCS). ( info) |
8/65. Mucin-producing carcinoid ("composite tumor") of upper third of esophagus: a variant of carcinoid tumor. An unusual upper esophageal polypoid tumor was found to be predominantly a carcinoid tumor and in part a mucin-producing adenocarcinoma. A review of literature showed this to be a unique tumor in this location. ( info) |
9/65. Hormone producing pancreatic islet cell carcinoma: changing clinical presentation. A patient with pancreatic islet cell carcinoma demonstrated spontaneous remission and recurrence of hyperinsulinism and disappearance of elevated plasma motilin levels. Despite evidence for gastrin production by the tumor initially, the zollinger-ellison syndrome was not diagnosed until three years after initial presentation. diarrhea and steatorrhea could be attributed to hyperchlorhydria rather than to direct intestinal effects of elevated cirulating gastrin, gastric inhibitory peptide or motilin. Pancreatic islet cell carcinomas, considered as a type a APUD cell proliferation, frequently produce more than one hormone; the pattern of hormone secretion may differ with time and clinical manifestations may change accordingly. ( info) |
10/65. liver transplantation for primary and secondary hepatic apudomas. Four patients underwent liver transplantation in the Cambridge/King's College Hospital programme for malignant primary and secondary apudomas, secreting various peptide hormones and uncontrollable by standard treatment techniques. After transplantation all patients had excellent symptomatic relief and specific peptide hormone levels fell to normal ranges. Two patients remain alive and well after 38 and 22 months, the latter with recurrence of slight tumour-related symptoms and elevation of the specific secretory product. The other two patients died from chronic graft rejection 7 and 8 months after transplantation. ( info) |