Case 212 - Discussion

Uploaded: 2008-05-07, Updated: 2008-09-09

 

 

CK7

CK20

CD56

Synaptophysin

 

EMA

 

Diagnosis: Appendiceal goblet cell carcinoid tumor. Extending into mesoappendiceal adipose tissue, cecal mucosa, adjacent cecum and cecal mesenteric adipose tissue, and serosal surface of ileum . No evidence of malignancy within 6 of 6 pericolonic lymph nodes.

Appendiceal goblet cell carcinoid (GCC) tumor

Carcinoid tumors are rare and are mostly located in the gastrointestinal tract (67.5%) and in the bronchopulmonary system (25.3%). Carcinoid tumors account for about 85%of all appendiceal tumors seen in surgical pathology. The prevalence of appendiceal carcinoid is between 0.3 - 0.9% in patients undergoing appendectomy. Dr McCusker reported 227 goblet cell carcinoids (13.8%) among 1645 appendiceal malignancies, along with 613 cases (37%) of mucinous adenocarcinoma, the most frequent diagnosis [1]. The average diagnostic age is between 38 and 49 years for malignant lesions, and goblet cell carcinoids tend to present at a later age of about 52 years. The Northern Ireland Neuroendocrine Database includes 517 carcinoid tumours of the appendix, midgut and lung diagnosed since 1975. 114 of these were appendiceal carcinoids, 16 of which were goblet cell in nature. Based on the review of 57 published papers encompassing nearly 600 diagnosed patients, the mean age of presentation for GCC of the appendix was 58.89 years with equal representation in both males and females.

 

Appendiceal GCC is composed of adenomatous groups of goblet cells containing predominantly vacuolated cytoplasm with basal round to crescentic nuclei containing a single small nucleolus. Many cells resembled the signet ring type. Less conspicuous were cells with pink granular cytoplasm and round nuclei.  The tumor typically consists of nests of goblet cells that are slightly larger than normal goblet cells,  and these tumors are thought to arise from a pluripotent cell (stem cell) as intestinal APUD cell with endodermal origin with divergent neuroendocrine and mucinous differentiation. Argyrophil cells are found in nearly 88% of GCCs and argentaffin cells in nearly 50% of GCCs. The demonstration of argentaffin or argyrophil cells in a high percentage supports the classification of GCC within the spectrum of carcinoids.

 

GCCs were subdivided into two types: the goblet cell type and the tubular type. Goblet cell type is more common than tubular type. Goblet cell type is composed of smooth-bordered nests, usually four or more cells. The tubular type is composed of small, discrete acini or tubules lined by a single layer of cuboidal or columnar cells. Both types possess the essential criteria for classification as GCC. The tubular type is more closely related than goblet cell type to the usual appendiceal carcinoid in both histological and prognostic characteristics and is probably an evolutionary stage in the development of the goblet cell type. Cells are usually uniform with pattern of infiltration being nests, rosettes, or clumps without a distinct lumen. As the cells are distended with mucin, they have crescentric nuclei arranged around the periphery of the clumps, resembling goblet cells or signet-ring cells.

 

GCC shows diffuse and strong positivity to CK19 and CK20, the reactivity to CK7 is variable [2, 3].  The tumor cells are commonly positive for NSE, chromogranin A, Synaptophysin and CEA.

 

REFERENCES

  1. Arnold R, McCallion K, McGailie C. Goblet Cell Carcinoids of the Appendix. Ulster Med J 2006; 75 (1) 40-45.

  2. Alsaad KO, Serra S, Schmitt A, Perren A, Chetty R. Cytokeratins 7 and 20 immunoexpression profile in goblet cell and classical carcinoids of appendix. Endocr Pathol. 2007 Spring;18(1):16-22.
  3. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97(4):93459.

  4. Arch Pathol Lab Med. 2001;125:386390