Korean J Pathol.  2010 Feb;44(1):101-105.

Primary Extrapulmonary Small Cell Carcinoma of the Appendix: A Case Report

• Park JH
• Park KJ
• Roh YH
• Roh MS

Abstract

Primary extrapulmonary small cell carcinoma (EPSCC) of the appendix is an extremely rare entity, and there has been only one previous report on this in the English medical literature. We report here on the first Korean case of EPSCC of the appendix in a 55-year-old woman. The patient had no history of an identified pulmonary tumor, and she presented with constipation and lower abdominal pain. The patient underwent right hemicolectomy with regional lymph node dissection and bilateral salphigo-oophorectomy. The histology of the entire appendiceal tumor revealed pure EPSCC with diffuse immunoreactivity for pancytokeratin, cytokeratin 7, cytokeratin 20, CD56, thyroid transcription factor 1, c-kit and carcinoembryonic antigen, and there was focal weak immunoreactivity for chromogranin A and synaptophysin. After the second cycle of chemotherapy, the condition of the patient gradually deteriorated due to cancer peritonei and the patient died 7 months later. EPSCC of the appendix is a distinctive clinicopathological entity that displays highly aggressive behavior and an unfavorable outcome.

Keyword

Appendix; Carcinoma, small cell

MeSH Terms

Abdominal Pain
Appendix
Carcinoembryonic Antigen
Carcinoma, Small Cell
Chromogranin A
Constipation
Female
Humans
Keratin-20
Keratin-7
Lymph Node Excision
Middle Aged
Nuclear Proteins
Synaptophysin
Thyroid Gland
Transcription Factors
Carcinoembryonic Antigen
Chromogranin A
Keratin-20
Keratin-7
Nuclear Proteins
Synaptophysin
Transcription Factors