Appendiceal carcinoids range from well-differentiated endocrine tumor to well-differentiated endocrine carcinoma, while poorly differentiated (small cell) carcinoma has not been described in this site. We report herein a case of mixed intestinal-type adenocarcinoma associated with a small cell carcinoma arisen in a 35-year-old woman and clinically presenting as an appendiceal abscess. The resected tumor histologically appeared as a biphasic lesion composed of a nonmucinous adenocarcinoma closely juxtaposed with a poorly differentiated (small cell) endocrine carcinoma. The subsequent right hemicolectomy was unremarkable, but one pericolic lymph node showed a metastatic deposit consisting of the adenocarcinoma only. The patient thus underwent a chemotherapeutic protocol for colorectal cancer, and she is alive and well at the 65-month follow-up. Immunohistochemically, the adenocarcinoma strongly stained for cytokeratin 20 and carcinoembryonic antigen, while the endocrine component displayed a dot-like positivity for pan-cytokeratins and chromogranin. Of note, both components did not stain with CDX2 and p53. At genotypic analysis by microsatellite instability, both components shared many microsatellite alterations as well as a normal p53 gene setup, although small cell carcinoma harbored additional alterations. Clinical and molecular findings led us to consider this lesion as a clonal tumor in which the endocrine component seems to derive from a progressive differentiation of the adenocarcinoma following a glandular-to-endocrine sequence.

Primary mixed adenocarcinoma and small cell carcinoma of the appendix - A clinicopathologic, immunohistochemical, and molecular study of a hitherto unreported tumor / Rossi, Giorgio; F., Bertolini; G., Sartori; N., Bigiani; A., Cavazza; M., Foroni; R., Valli; G., Rindi; DE GAETANI, Carmela; G., Luppi. - In: THE AMERICAN JOURNAL OF SURGICAL PATHOLOGY. - ISSN 0147-5185. - 28:(2004), pp. 1233-1239. [10.1097/01.pas.0000128666.89191.48]

Primary mixed adenocarcinoma and small cell carcinoma of the appendix - A clinicopathologic, immunohistochemical, and molecular study of a hitherto unreported tumor

ROSSI, Giorgio;DE GAETANI, Carmela;
2004

Abstract

Appendiceal carcinoids range from well-differentiated endocrine tumor to well-differentiated endocrine carcinoma, while poorly differentiated (small cell) carcinoma has not been described in this site. We report herein a case of mixed intestinal-type adenocarcinoma associated with a small cell carcinoma arisen in a 35-year-old woman and clinically presenting as an appendiceal abscess. The resected tumor histologically appeared as a biphasic lesion composed of a nonmucinous adenocarcinoma closely juxtaposed with a poorly differentiated (small cell) endocrine carcinoma. The subsequent right hemicolectomy was unremarkable, but one pericolic lymph node showed a metastatic deposit consisting of the adenocarcinoma only. The patient thus underwent a chemotherapeutic protocol for colorectal cancer, and she is alive and well at the 65-month follow-up. Immunohistochemically, the adenocarcinoma strongly stained for cytokeratin 20 and carcinoembryonic antigen, while the endocrine component displayed a dot-like positivity for pan-cytokeratins and chromogranin. Of note, both components did not stain with CDX2 and p53. At genotypic analysis by microsatellite instability, both components shared many microsatellite alterations as well as a normal p53 gene setup, although small cell carcinoma harbored additional alterations. Clinical and molecular findings led us to consider this lesion as a clonal tumor in which the endocrine component seems to derive from a progressive differentiation of the adenocarcinoma following a glandular-to-endocrine sequence.
28
1233
1239
Primary mixed adenocarcinoma and small cell carcinoma of the appendix - A clinicopathologic, immunohistochemical, and molecular study of a hitherto unreported tumor / Rossi, Giorgio; F., Bertolini; G., Sartori; N., Bigiani; A., Cavazza; M., Foroni; R., Valli; G., Rindi; DE GAETANI, Carmela; G., Luppi. - In: THE AMERICAN JOURNAL OF SURGICAL PATHOLOGY. - ISSN 0147-5185. - 28:(2004), pp. 1233-1239. [10.1097/01.pas.0000128666.89191.48]
Rossi, Giorgio; F., Bertolini; G., Sartori; N., Bigiani; A., Cavazza; M., Foroni; R., Valli; G., Rindi; DE GAETANI, Carmela; G., Luppi
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/306928
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