Abstract

Goblet cell carcinoid represents a unique entity of appendiceal neoplasia. Its pathological features and clinical behavior are distinct from the classic carcinoid tumor as well as primary adenocarcinoma of the appendix. Correct histopathological classification and diagnosis provide guidelines for treatment and prognosis. Morphological transformation of the Goblet cell carcinoid from typical Goblet cell carcinoid to adenocarcinoma morphology is likely associated with accumulation of additional genetic changes that is why subclassification of this group of tumors is needed. Investigation of molecular genetic changes could increase our understanding of this exotic but clinically important group of tumors. We present the case of a patient with metastatic goblet cell carcinoid involving terminal ileum, ascendent colon, ovary, omentum and peritoneal spreading, treated with debulking surgery and chemotherapy (FOLFOX4 regimen) with good response, reduction of disease on CT and PET complete remission. Improvement of clinical symptoms as well as quality of life was reached by combined palliative treatment. Correct diagnostics and therapeutic efforts bring patient benefit even in metastatic setting. Better knowledge of rare tumors and understanding of their biology help improve therapeutic approaches.

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