Appendiceal goblet cell carcinoma: comparison of classification and staging systems with evaluation of the prognostic role of immunohistochemistry stains

Background: Goblet cell carcinoma (GCC) of the appendix is a unique lesion that exhibits features of both adenocarcinoma and neuroendocrine tumors. Due to the rarity of this cancer, multiple grading (e.g., Tang, Yozu, and Lee) and staging systems (e.g., tumor, lymph nodes, and metastasis [TNM]) have been developed for classification. This study aimed to compare commonly used classification systems and evaluate the prognostic effectiveness immunohistochemical staining may or may not have for appendiceal GCC. Methods: An electronic medical records review of patients who were diagnosed with GCC of the appendix in our hospital system from 2010 to 2020. The data were collected regarding the age at diagnosis, gender, initial diagnosis at presentation, operation(s) performed, final pathology results, current survival status, and year of recurrent disease or death year. Results: Ten patients were evaluated. Seventy percent of the patients were above the age of 50 years at diagnosis. Postdischarge survival ranged from 1 month to 109 months postdiagnosis. Two patients expired from GCC at 13- and 54-months following diagnosis. When comparing the classification systems, Lee categorized more patients as high risk than Tang and Yozu. Immunohistochemical staining was analyzed using four staining methods: Ki67, E-cadherin, Beta-catenin, and p53. Tumor, lymph nodes, and metastasis staging has supportive evidence for worsening prognosis and overall survival secondary to the depth of invasion of the tumor. Conclusion: Tumor, lymph nodes, and metastasis staging may be superior to the other classification systems in predicting overall mortality. Our study demonstrated that immunohistochemistry staining does not appear to have a significant impact in determining the prognosis for GCC of the appendix. (AU)

Vesico-appendiceal fistula in a mucinous adenocarcinoma of the appendix / Fístula vésico-apendicular em adenocarcinoma mucinoso do apêndice

BACKGOUND: A rare case of vesicoappendiceal fistula secondary to mucinous adenocarcinoma of the appendix is presented. CASE REPORT: A 62-year-old man with a one year history of recurrent urinary tract infections. After two months he developed pneumaturia and fecaluria. An abdominal and pelvic computed tomography demonstrated a trans-mural mass in the posterior wall of the bladder with a vesicoenteric fistula leading to the terminal ileum. Laparotomy revealed a tumor arising from the appendix contiguous with the bladder posterior wall. The bladder was opened and a large fistula and tumor on the posterior bladder wall near the trigone was identified. Frozen pathological analysis showed a mucinous adenocarcinoma. En-bloc right hemicolectomy and partial cystectomy, preserving bladder trigone was performed. After manipulating the tumor, grossly leakage of mucinous materials occurred into the pelvic cavity. A peritoneal washing with a mytomicin solution at 42º C was then performed, to prevent peritoneal seeding. The patient had a prolonged postoperative ileus and was discharged at the 15th day. Five months after the procedure the patient was recieving chemotherapy with 5-fluoracil and leucovorin and there was no signs of recurrent disease. CONCLUSION: The presentation with vesico-appendiceal fistula is extremely rare with only a few cases reported in the literature. Knowledge of different types of neoplasm and appropriate treatment allows the surgeon to provide patients optimal care referring to specialized centers whenever appropriate.

INTRODUÇÃO: Apresenta-se raro caso de fístula vésico-apendicular secundária a adenocarcinoma mucinoso do apêndice. RELATO DE CASO: Paciente masculino de 62 anos com história de um ano de infecções urinárias de repetição. Após dois meses desenvolveu pneumatúria e fecalúria, sendo indicada tomografia computadorizada de abdômen que mostrou massa trans-mural na parede da bexiga, com fistula vésico-entérica para região do íleo terminal. A laparotomia revelou tumoração surgindo do apêndice cecal, em continuidade com a parede posterior da bexiga. A mesma foi aberta, sendo identificada grande fistula e material tumoral até próximo ao trígono vesical. A biópsia de congelação identificou adenocarcinoma mucinoso. Realizada hemicolectomia associada à cistectomia parcial em bloco, com preservação do trígono vesical. Houve extravasamento de mucina para a cavidade pélvica pela manipulação. Optado por lavagem peritoneal com solução de mitomicina a 42º C para prevenir disseminação peritoneal. O paciente evoluiu com íleo prolongado pós-operatório, tendo alta hospitalar no15º dia. Cinco meses após o procedimento encontrava-se em quimioterapia com 5-fluouracil e leucovorin, sem sinais de doença recidivante. CONCLUSÃO: A presença de fístula vésico-apendicecal é extremamente rara, tendo poucos casos relatados na literatura. O conhecimento dos diferentes tipos de neoplasias e seus tratamentos adequados permite ao cirurgião oferecer melhor cuidado ao paciente.