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Goblet cell adenocarcinomas (GCAs) are an exceedingly rare subtype of tumors, almost exclusively occurring in the appendix, and characterized by features overlapping both adenocarcinomas and neuroendocrine tumors (NETs), which has historically led to confusion and varied nomenclature. This study presents a comprehensive review of the literature highlighting particularities of this type of malignancy, starting from a rare case of a 54-year-old female operated on in our clinic for an appendiceal tumor, initially suspected to be a mucinous neoplasm based on colonoscopic biopsy, which was ultimately confirmed to be goblet cell adenocarcinoma on both intraoperative frozen section and definitive pathological examination. Exhibiting signs and symptoms associated with an abdominal mass, she underwent a right hemicolectomy with partial omentectomy for locally advanced, high-grade, invasive goblet cell adenocarcinoma of the appendix with lymphatic macro metastases and epiploic invasion, categorized as AJCC stage IVb carcinomatosis. The patient received FOLFOX adjuvant. Six months later, she required reoperation due to the progression of carcinomatosis, which was again confirmed histopathologically. A second-line oncological protocol comprising irinotecan, capecitabine, and bevacizumab was initiated. Given the rarity of GCAs and the absence of a consensus on nomenclature, classification, and diagnostic criteria, we conducted a comprehensive literature review to highlight current trends related to this entity, including its classification within different systems (Tang, Yozu, WHO, AJCC), as well as the therapeutic surgical approaches—ranging from simple appendectomy to extensive multiorgan resection, cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC), and the use of systemic therapy. Adhering to these recommendations will enhance communication among pathologists, surgeons, and oncologists regarding the natural history and prognosis of this rare malignancy. Full article

Background and Clinical Significance: Neuroendocrine expressing goblet cell adenocarcinomas (GCAs) are uncommon clinically aggressive tumours of the digestive system, originating almost exclusively in the ileocecal appendix. GCA’s singularity comes from its amphicrine nature, expressing both neuroendocrine and exocrine characteristics. The case report’s objective is to raise awareness of this neoplasia’s possible extra-appendiceal localisation by showcasing a GCA involving the cecum with no detectable appendiceal tumour. Case Presentation: The authors present a case of GCA with neuroendocrine expression in an 82-year-old male patient with severe anaemia and comorbidities who underwent a right colectomy and had no histopathological evidence of appendiceal tumour involvement. Immunohistochemical testing was performed using synaptophysin, chromogranin A, neuronal specific enolase, CD56, CDX-2, CK20, CEA, MUC2 and Ki67, thus establishing the final diagnosis of high-grade extra-appendiceal goblet-cell adenocarcinoma of the cecum, G3. The patient died on postoperative day 26 due to pneumonia and acute renal failure in a chronic renal disease context. Conclusions: Extremely few cases of extra-appendiceal GCA have been reported. Appendiceal evaluation with the exclusion of this possible origin should be mandatory in such cases for a correct classification. These tumours do not benefit from any official management protocols concerning clinical evaluation, and their treatment is commonly based on the tumour’s stage, as in classical adenocarcinoma. Full article