CARCINOID TUMORS OF THE APPENDIX IN CHILDHOOD.
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OBJECTIVE: The clinicopathologic and immunohistochemical features of 11 pseudomyxoma peritonei (PMP) cases were studied to determine pathologic diagnosis, site of origin and prognosis. METHODS: Clinical files of 11 cases of PMP were reviewed with follow up. The changes in mucinous tumors of peritoneum and primary tumors under microscope and immunostaining were reviewed. RESULTS: Eleven cases (8 women, 3 men) are reported. The patients age ranged from 36 to 76 (average 56). One died 2 years after operation, and one case was lost. The remaining 8 cases were alive 1 to 60 months postoperatively. Of the 11 cases, 8 cases had appendiceal mucinous neoplasm of the 11 cases, and 5 women had synchronous ovarian mucinous tumors; colon mucinous adenocarcinoma was present in one case with synchronous ovarian mucinous tumor; simple ovarian mucinous tumors were present in two cases. Immunostainings were consistent on mucinous tumors of appendix, ovary and peritoneum in the same case. CONCLUSIONS: To diagnose the PMP, the type of tumor should be considered whether it is benign, low malignant or malignant. The appendix neoplasm is closely related to PMP. The prognosis depends greatly on the growth speed of the mucinous neoplasm.
Epithelial neoplasms of appendix are infrequent, and their pathological features are not fully characterized. We collected 33 cases of appendiceal tumors and examined immunohistochemically the expression of cytokeratins (CK, CK7, and CK20), mucin core protein (MUC1, MUC2, MUC5AC, and MUC6), E-cadherin, chromogranin A, and p53 protein. Gene analysis of TP53 was also conducted on exons 5 to 8. Clinically, mucinous tumors were predominant in females. Immunohistochemically, all the tumors expressed CK20, whereas CK7 was positive in one third of the cases. Similarly, MUC2 was expressed in all the tumors, whereas MUC1 and MUC5AC were detected in about a half of the cases. Although chromogranin A-positive cells are generally sparse in normal appendix, they were more common in mucinous tumors than in nonmucinous tumors. Contrary to the previous data reported (Mod Pathol 2002;15:599-605), mucinous carcinoma exhibited a higher frequency of p53-positive cells (mean 29%) compared with mucinous adenoma (2.8%) (P < .001), whereas nonmucinous tumors showed high levels of p53-positive cells to similar extent (51%-67%) in both adenoma and carcinoma. The high expression of p53 protein coincided with the presence of mutations in multiple sites of TP53 gene in mucinous tumors. This is the first report that characterized the immunophenotypic profile of appendiceal epithelial neoplasms with an emphasis of a higher frequency of p53 positivity in mucinous carcinoma cases compared with mucinous adenoma in the appendix.
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Pseudomyxoma peritonei is an overused and underspecified condition that has garnered much attention in the historic literature. In recent years, this condition has been convincingly linked to appendiceal mucinous neoplasms, yet there has been insufficient attention to the histologic characteristics, classification, and differential diagnostic considerations of these neoplasms when encountered by the surgical pathologist. This review provides a coherent approach to the diagnosis and classification of appendiceal mucinous tumors and the peritoneal implants associated with the pseudomyxoma peritonei syndrome with emphasis on differential diagnostic considerations and recommendations for the final pathology report.
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