Pure large cell neuroendocrine carcinoma of the gallbladder, is surgical relentlessness beneficial? A case report and literature review

Abstract

Pure large cell neuroendocrine carcinoma of the gallbladder is a rare disease. However, the prognosis of this aggressive tumor is poor with short survival after diagnosis. We are describing in this manuscript a case of pure large cell neuroendocrine carcinoma in which survival exceeded 26 months, after performing two curative surgeries. We are reporting the case of a 68-year-old woman with a history of recovered right breast carcinoma and operated 6 years later for a completely asymptomatic gallbladder tumor of 31 mm. In this case, curative surgery was performed allowing monobloc resection of the gallbladder and the hepatic segments IVb and V, a lymph node dissection was performed to. The histological examination of the specimen and immunohistochemistry confirms that the tumor was a grade 3 pure large cell neuroendocrine carcinoma of gallbladder with lymph node invasion, the hepatic and biliary surgical margins were free. Postoperative adjuvant chemotherapy was administered and the evolution was eventless until the discovery at 20 months of a lymph node considered being metastatic recurrence. A second surgery was performed allowing removal of three lymph nodes. This time, a different protocol of chemotherapy was administered to our patient who remains alive and without recurrence at 26 months from her first surgery. Surgical relentlessness with free margins resections associated with appropriate chemotherapy probably improves the survival of patients suffering from this rare and aggressive tumor.