Repeat liver resection for pure large cell neuroendocrine carcinoma of the gallbladder: a favorable outcome

Abstract

Background The pure large cell type is a rare variant of primary neuroendocrine carcinoma of the gallbladder. Few reports have mentioned extended survival. Although a multimodal treatment has been described in the treatment of such rare disease, redo liver resection has not yet been mentioned. Case report A 67-year-old lady was found to have poorly differentiated, high grade, pure large cell neuroendocrine tumor of the gallbladder after cholecystectomy for gallstones. After the diagnosis, staging workup showed a lesion in segment IVB/V of the liver, and chromogranin was elevated (982 mcg/L). The patient underwent central inferior hepatectomy and wedge excision of a lesion in segment III (discovered intra-operatively), with hilar lymphadenectomy. Three months after the first liver resection, she developed a new liver lesion II/III and underwent left lateral liver resection. The patient remained disease-free for 4 months following the second liver resection but then developed recurrent liver disease and was started on chemotherapy. Further progression led to multi-organ failure and death at 26 months from initial diagnosis. Conclusion This is the first reported repeat liver resection in such a rare disease that has led to extended overall survival. We suggest that a group of selected patients with this rare malignancy, and liver-limited disease, may benefit from repeated liver resection.

Neuroendocrine tumors of the gallbladder (Review)

“…Surgical treatment. Advantages and disadvantages of the various treatment options discussed are listed in Table I: Due to its complexity, gallbladder cancer must be treated surgically by experienced biliary tract physicians and pathologists (42). GB-NEC is characterized by high malignancy, early lymphatic metastasis (the N2 lymph node metastasis rate is significantly higher compared with that of patients with adenocarcinoma in the same period) and a poor prognosis when compared with all other types of gallbladder cancer (20).…”

Large Cell Neuroendocrine Carcinoma of Gallbladder: A Case Report

“… 6 Neuroendocrine tumours of the digestive system have been classified based on mitotic figures and the Ki-67 index, regardless of the origin, size, or anatomic extent of the tumours. 7 As per WHO 2019 updates, GB NEC encompasses small-cell and large-cell types of poorly differentiated NETs with mitotic rate >20mitoses/ 2 mm 2 and/or Ki-67 index >20%. 3 The diagnosis of GB-NEC is based on typical morphological features and immunohistochemistry markers (chromogranin A, synaptophysin, and neuro-specific enolase); pathological results determine tumour grade and staging.…”

“… 5 Very few pure LCNEC have been reported in the literature with a median survival of 8 to 10 months, so it becomes very difficult to propose the best management plan. 3 , 7 Still, surgical resection improves overall survival and is the cornerstone in the management of GB-NEC. 5 For neuroendocrine tumours of GB, cholecystectomy could be enough if detected in early stages (in situ and T1 tumours).…”

“… 3 , 5 Completion surgery is the standard practice for cases diagnosed post cholecystectomies. 7 Due to its aggressive behaviour, repeated liver resection is also offered in patients with metastasis confined to the liver and with a good performance scale. 3 , 7 Platinum-based postoperative adjuvant chemotherapy (cisplatin or carboplatin plus etoposide) is the main chemotherapeutic regimen to improve the prognosis for those in advanced stages with satisfactory results.…”

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

“…Abutaka et al had reported a case of pure large cell GBNEC where relentless surgery after a first R0 resection of an NEC of the gallbladder, followed by two hepatic metastasectomy with lymph node dissection had prolonged survival to 26 months [ 18 ].…”