Goblet cell carcinoids of the appendix: immunophenotype and ultrastructural study.
BACKGROUND
Goblet cell carcinoids of the appendix are rare neoplasms with uncertain biological behavior.


OBJECTIVE
The aims of our study were to evaluate the immunophenotype of this neoplasm with cell cycle/cell proliferation markers and to understand their histogenesis with ultrastructural analysis using conventional carcinoids as a frame of reference.


METHODS
Clinical data and archival pathologic material of all goblet cell carcinoids of the appendix recorded by the Saskatchewan Cancer Registry between 1970 and 1998 were reviewed and evaluated by light microscopy, histochemistry, immunohistochemistry, and electron microscopy.


RESULTS
Seven cases of goblet cell carcinoids were identified among 110 cases of conventional carcinoids of the appendix. Histopathology revealed widespread infiltration of the periappendiceal fat in all cases, with extensive perineural invasion. The cells stained strongly positive for mucicarmine, periodic acid-Schiff, periodic acid-Schiff diastase, Alcian blue, cytokeratin, and carcinoembryonic antigen. Most cases were positive for synaptophysin. Increased expression of cell proliferation markers and cell cycle markers was observed. Expression of p53 was strong in one case. Electron microscopy demonstrated the presence of mucinous vacuoles of varying sizes and occasional membrane-bound neuroendocrine granules.


CONCLUSIONS
Goblet cell carcinoids of the appendix arise from a pluripotent cell with divergent neuroendocrine and mucinous differentiation. These neoplasms are widely invasive; they demonstrate a high cellular proliferation rate and dysregulation of the cell cycle with up-regulation of cyclin D1 and p21, and down-regulation of p16. Complete removal of the tumor is recommended because of the unpredictable biological behavior of this tumor, which includes delayed local recurrences and lung metastases.
Department of Pathology, Royal University Hospital, University of Saskatchewan, Saakatoon, Canada.
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Kanthan
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Saxena
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SC
Kanthan
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001063:Appendiceal Neoplasms / Q000737:chemistry / Q000601:surgery / Q000648:ultrastructure*; D014408:Biomarkers, Tumor / Q000032:analysis; D002276:Carcinoid Tumor / Q000737:chemistry / Q000601:surgery / Q000648:ultrastructure*; D019938:Cyclin D1 / Q000032:analysis; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D005260:Female; D020397:Goblet Cells / Q000737:chemistry / Q000648:ultrastructure*; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D019394:Ki-67 Antigen / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000032:analysis; D009490:Neurosecretory Systems / Q000648:ultrastructure
D014408:Biomarkers, Tumor; D019394:Ki-67 Antigen; D009363:Neoplasm Proteins; D019938:Cyclin D1