Report of two cases of large cell neuroendocrine carcinoma of duodenal ampulla with contrasting outcomes following pancreaticoduodenectomy according to the use of adjuvant chemotherapy

Imamura, Naoya; Nanashima, Atsushi; Hiyoshi, Masahide; Fujii, Yukihiko

doi:10.1016/j.ijscr.2017.01.031

Cited by 7 publications

(6 citation statements)

References 18 publications

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“…Ampullary LCNECs in the literature are limited to 24 case reports before ours, as identified by our review to date ( Table 2 ) [ 11 , 18 , 24 , 26 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 ]. Only 14 cases were pure LCNECs [ 24 , 26 , 56 , 57 , 59 , 60 , 61 , 62 , 63 , 66 ], while the other 11 had other components [ 11 , 18 , 26 , 59 , 60 , 65 , 66 , 68 ]. Many of these patients had early recurrence with metastases, especially in the liver [ 24 , 56 , 58 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 68 ].…”

Section: Discussionmentioning

confidence: 99%

“…Only 14 cases were pure LCNECs [ 24 , 26 , 56 , 57 , 59 , 60 , 61 , 62 , 63 , 66 ], while the other 11 had other components [ 11 , 18 , 26 , 59 , 60 , 65 , 66 , 68 ]. Many of these patients had early recurrence with metastases, especially in the liver [ 24 , 56 , 58 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 68 ].…”

Section: Discussionmentioning

confidence: 99%

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Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

et al. 2022

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Ampullary large-cell neuroendocrine carcinomas (LCNECs) are extremely rare, and available data are limited on case reports. They present with jaundice, non-specific abdominal pain, or weight loss, imitating adenocarcinoma. Their incidence increases due to the improved diagnostic techniques. However, preoperative diagnosis remains challenging. We report the case of a 70-year-old man with a history of metabolic syndrome, cholecystectomy, and right hemicolectomy, presenting with jaundice. Laboratory results showed increased liver biochemistry indicators and elevated CA 19-9. Esophagogastroduodenoscopy revealed an ulcerative tumor on the ampulla of Vater, and the biopsy revealed neuroendocrine carcinoma. Although computed tomography (CT) detected enlarged regional lymph nodes, the positron emission tomography (PET) showed a hyperactive lesion only in this area. Pylorus-preserving pancreatoduodenectomy with R0 resection was performed. Pathologic evaluation of the 3.1 × 1.9 cm tumor revealed an LCNEC with immunohistochemical positivity at Synaptophysin, EMA, CD56, and cytokeratin CK8/18. The Ki-67 index was 45%. Two out of the nine dissected lymph nodes were occupied by the neoplasm. The patient was discharged home free of symptoms, and adjuvant chemotherapy with carboplatin + etoposide was initiated. A comprehensive review of the reported cases showed that the preoperative biopsy result was different from the final diagnosis in few cases, regarding the subtypes. Conventional radiology cannot identify small masses, and other methods, such as endoscopy, magnetic resonance cholangiopancreatography (MRCP), and FDG-PET scan, might aid the diagnosis. Diagnosis is based on histology and immunohistochemical markers of the surgical specimens. The treatment of choice is pancreatoduodenectomy, followed by adjuvant chemotherapy. However, recurrence is frequent, and the prognosis remains poor.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

et al. 2022

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“…Their recommendation is pancreaticoduodenectomy (PD) together with lymph node dissection for a better overall survey [ 7 ]. On the other hand, recent literature emphasizes more on the histopathologic features of the tumor (differentiation grade, small or large cell type) rather than ‘TNM’ or ‘ENETS’ (European Neuroendocrine Tumor Society) staging systems in predicting the overall course and treatment strategy [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Oncology preferred close follow-up with a conservative approach instead of giving oncotherapy. In a study presented by Naoya Imamura et al [ 8 ], adjuvant chemotherapy after radical resection performed for LCNECs was found with better the overall survey without any relapses in the long term. In ‘The NANETS Consensus Guidelines for the Diagnosis and Management of Poorly Differentiated (High-Grade) Extrapulmonary Neuroendocrine Carcinomas’ which was conducted by Strosberg et al [ 9 ], adjuvant chemotherapy (4–6 cycles of cisplatin or carboplatin and etoposide) is recommended regarding the post-operative treatment of poorly differentiated NECs.…”

Section: Discussionmentioning

confidence: 99%

Aggressive clinical course of large cell neuroendocrine carcinoma of ampulla of Vater

Sönmez

Buyuker

Girgin

et al. 2019

North Clin Istanbul

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A 78-year-old male patient with history of righ hemicolectomy due to adenocarcinoma was admitted by the complaint of epigastric discomfort. Laboratory data showed increase in liver biochemistries (aspartate aminotransferase (AST): 159 IU/L, alanine aminotransferase (ALT): 235 IU/L, alkaline phosphatase (ALP): 350 IU/L, gamma glutamyl transferase (GGT): 911 IU/L, total bilirubin: 1.55 mg/dl and direct bilirubin: 0.82 mg/dl). Endoscopic retrograde cholangiopancreatiography (ERCP) done after the gastrointestinal (GI) upper endoscopy was compatible with tumoral lesion, and biopsy confirmed 'neuroendocrine carcinoma'. Pylorus-preserving pancreaticoduodenectomy (PPPD) had been performed with R0 resection. Pathologic evaluation revealed 1.5 cm tumor of large cell neuroendocrine carcinoma (LCNEC). Five months later, biyopsy of suspicious lesions in liver had been documented as 'high grade neuroendocrine carcinoma metastasis'. He was referred to oncology for chemotherapy but unfortunatelly he had expired three months later. Large cell neuroendocrine carcinoma (LCNECs) of ampulla of Vater may have aggressive clinical course despite radical resections involving lymph node dissections. Small tumor size and lymph node negativity are not reliable factors for this tumor type.

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“…Various investigations that can be performed for the diagnosis of NET are upper gastrointestinal endoscopy which is the gold standard [6]. Ultrasound abdomen, endoscopic ultrasonography (EUS) and EUS-fine-needle aspiration and biopsy (EUS-FNAB) can also be done along with other radiological modalities such as computerised tomography (CT) and magnetic resonance imaging (MRI) [8,9]. Surgical intervention includes Whipple's procedure or pancreaticoduodenectomy with Roux-enY anastomosis.…”

Section: Introductionmentioning

confidence: 99%

Neuroendocrine Carcinoma of Duodenum—an Uncommon Tumour at an Unusual Site

Dewan

Bhat

Prasad

et al. 2018

Indian J Surg Oncol

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Neuroendocrine carcinoma rarely occurs in the duodenum, and most cases of neuroendocrine carcinoma in the duodenum show rapid progression of the disease. Such cases have poor prognosis even with radical surgery with or without chemotherapy with low 5-year survival rate. We present a case of a 52-year-old man who presented with abdominal pain of 1-month duration and one episode of vomiting. Upper gastrointestinal endoscopy revealed polypoidal lesions in the first and second part of the duodenum. Whipple's procedure was performed. Diagnosis of poorly differentiated neuroendocrine carcinoma was made with extension to pancreas with peripancreatic lymph node metastases. The patient expired on post operative day 17 following cardiac arrest.

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Report of two cases of large cell neuroendocrine carcinoma of duodenal ampulla with contrasting outcomes following pancreaticoduodenectomy according to the use of adjuvant chemotherapy

Cited by 7 publications

References 18 publications

Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

Aggressive clinical course of large cell neuroendocrine carcinoma of ampulla of Vater

Neuroendocrine Carcinoma of Duodenum—an Uncommon Tumour at an Unusual Site

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