gastrins and Appendiceal-Neoplasms

Topics: Appendiceal Neoplasms; Carcinoid Tumor; Carcinoma, Small Cell; Cholecystokinin; Digestive System; Duodenum; Endocrine Glands; Fetus; Gastric Mucosa; Gastrins; Humans; Ileum; Peptides; Rectal Neoplasms; Respiratory System; Secretin; Substance P; Vasoactive Intestinal Peptide

Gastrointestinal carcinoids are derived from the diffuse intestinal endocrine system and may produce amines and many peptides, including serotonin, chromogranin A (CGA), and tachykinins. Most peptide hormones are synthesized as bigger prohormones, which are processed to smaller active hormones by prohormone convertases (PCs). A total of 35 cases of gastrointestinal carcinoids, including gastric, duodenal, small intestinal, appendiceal, and large intestinal carcinoids, were immunocytochemically stained for serotonin, CGA, and PC 1/3 and 2, in order to colocalize CGA and PCs in the carcinoids. All carcinoids were positive for CGA and PCs. Carcinoids that stained strongly for CGA were generally weakly stained for PCs and those weakly staining for CGA were more strongly stained for PCs in the majority of the small and large intestinal tumors. Gastrointestinal carcinoids were positive for CGA and PCs, and the presence of PCs may suggest that the conversion of peptide prohormones to smaller peptide hormones occurs in gastrointestinal carcinoids. PCs immunocytochemistry may be added as a new phenotypic characterization for gastrointestinal carcinoids.

Topics: Adult; Aged; Appendiceal Neoplasms; Aspartic Acid Endopeptidases; Carcinoid Tumor; Child; Chromogranin A; Chromogranins; Duodenal Neoplasms; Female; Gastrins; Gastrointestinal Neoplasms; Humans; Immunohistochemistry; Intestinal Neoplasms; Intestine, Large; Intestine, Small; Male; Middle Aged; Proprotein Convertase 2; Proprotein Convertases; Serotonin; Stomach Neoplasms; Subtilisins

The diagnosis of adenocarcinoid (mucinous/goblet cell carcinoid) is usually unexpected by both clinicians and pathologists. We report here the case of a 74-year-old man with gastric lymphoma (B-cell MALToma) diagnosed by endoscopy, who was found on exploratory laparotomy also to have extensive intraabdominal involvement by adenocarcinoid, arising from the ileum and/or appendix. The patient died two years after diagnosis with bladder outlet and small bowel obstruction due to diffuse metastases. In addition to mucin positivity, immunohistochemical stains demonstrated the tumor to be positive for chromogranin, synaptophysin, serotonin, gastrin, and glucagon. Of histogenetic interest, some individual neoplastic cells appeared to be positive for both mucin and chromogranin, and this was confirmed by the electron microscopic finding of microvilli, intracytoplasmic mucin droplets, and neurosecretory granules involving the same neoplastic cells. This also appears to be the first reported case of adenocarcinoid associated with lymphoma and demonstration of histochemical/immunohistochemical and ultrastructural evidence of cellular components with dual mucinous adenocarcinoma and neuroendocrine features, and the second reported case to have prostatic metastases.

Topics: Adenocarcinoma; Aged; Appendiceal Neoplasms; Biomarkers, Tumor; Carcinoembryonic Antigen; Carmine; Chromogranins; Coloring Agents; Gastrins; Glucagon; Helicobacter Infections; Helicobacter pylori; Humans; Ileal Neoplasms; Immunohistochemistry; Laparotomy; Lymphoma, B-Cell, Marginal Zone; Male; Metaplasia; Microscopy, Electron; Neoplasms, Multiple Primary; Prostatic Neoplasms; Serotonin; Stomach Neoplasms; Synaptophysin

In order to compare histologic subtypes and endocrine profiles, immunohistochemical and silver stains were performed on 120 appendiceal carcinoids. Forty-three were predominantly insular; 21 were mixed insular, glandular, and trabecular; 33 were goblet cell; 17 were tubular; and five were clear cell carcinoids. Insular, mixed, and clear cell carcinoids were generally diffusely argentaffin and positive for chromogranin, neuron-specific enolase (NSE), and serotonin. Occasional tumors of insular or mixed patterns had scattered cells that stained weakly for glucagon, calcitonin, adrenocorticotrophic hormone (ACTH), somatostatin, cholecystokinin (CCK), human pancreatic polypeptide (HPP), or gastrin. Most had S-100-positive sustentacular cells. Less than half were positive for carcinoembryonic antigen (CEA). Many were cytokeratin-positive, but often focally. Goblet cell carcinoids contained few endocrine cells, but these were strongly argentaffin and positive for serotonin in nearly all, and positive for HPP in almost a third. Tubular carcinoids lacked argentaffinity and serotonin but were diffusely and strongly positive for glucagon. All goblet cell and tubular carcinoids were diffusely positive for CEA and cytokeratin. Somatostatin stained strongly in a single tumor, which had psammoma bodies and was in a patient with neurofibromatosis. In all groups, argentaffinity correlated with serotonin positivity, and argyrophilia with chromogranin positivity, although the latter was somewhat more sensitive. We conclude that among appendiceal carcinoids, the endocrine content varies according to histologic subtype.

Topics: Adrenocorticotropic Hormone; Appendiceal Neoplasms; Carcinoembryonic Antigen; Carcinoid Tumor; Cholecystokinin; Chromogranins; Diagnosis, Differential; Follow-Up Studies; Gastrins; Glucagon; Humans; Immunohistochemistry; Keratins; Pancreatic Polypeptide; Phosphopyruvate Hydratase; S100 Proteins; Serotonin; Somatostatin

Topics: Adult; Appendiceal Neoplasms; Carcinoid Tumor; Gastrins; Humans; Male; Postoperative Period; Serotonin