Mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla of Vater in a Chinese patient: A case report

Liu, Xu; Li, Dezhao; Sun, Xu; Lv, Guoyue

doi:10.1177/0300060520947918

Cited by 5 publications

(9 citation statements)

References 19 publications

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“…MiNEN is a rare tumor commonly found in the appendix and colorectum in the digestive tract, while MiNENs that occur in the ampulla are very rare. The literature search found only 16 cases,and most of them were case reports[ 1 , 2 , 5 ]. Patients with a MiNEN of the ampulla usually present with nonspecific clinical symptoms, such as abdominal pain, jaundice, nausea, vomiting, and upper abdominal discomfort[ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…At present, the best treatment for MiNEN is not clear[ 5 ]. It has been reported that for MiNEN without distant metastases, radical surgery can be the first choice for treatment, and palliative chemotherapy can be selected when the patient has distant metastases[ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…Among the reported cases of MiNEN in the ampulla, most cases undergo surgical radical resection (pancreaticoduodenectomy). Postoperative chemotherapy regimens are different, such as oxaliplatin-based combination chemotherapy and S-1, and chemotherapy for NEC components is usually recommended[ 2 , 5 ].Among the 3 patients undergoing chemotherapy in this study, the regimen was different from the previously reported regimen. After the tumor recurrence was observed in these patients, they were treated with chemotherapeutic drugs, and the reduction of metastases was observed, suggesting that it may be effective.…”

Section: Discussionmentioning

confidence: 99%

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Mixed neuroendocrine-nonneuroendocrine neoplasm of the ampulla: Four case reports

Wei¹,

Zhang²,

Wang³

et al. 2022

WJCC

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BACKGROUND Mixed neuroendocrine-nonneuroendocrine neoplasm (MiNEN) is a rare tumor that occurs in the gastrointestinal tract and pancreas, usually composed of adenocarcinoma and neuroendocrine carcinoma. MiNEN occurring in ampulla is even rarer. We report 4 cases of MiNEN in ampulla, combined with literature review to summarize the clinical features and treatment of the disease, in order to improve the understanding of the disease. CASE SUMMARY A retrospective analysis was performed in 4 cases of MiNEN of the ampulla diagnosed by pathology from 2014 to 2021. The 4 patients were all male, aged 67-81 years (average 72.25 years). Among them, 2 patients had jaundice, 1 patient had abdominal pain, and 1 patient had jaundice with abdominal pain as the first symptom. All 4 patients underwent enhanced CT or MRI, which all indicated that the tumors were located in the ampulla. Two patients underwent duodenoscopy, and a biopsy revealed ampullary adenocarcinoma. All 4 patients underwent radical pancreaticoduodenectomy. Four cases were followed up: One patient developed severe complications after the operation, his condition deteriorated, and he survived for 1 mo. In the other 3 patients, tumor recurrence was observed during follow-up, and 2 of them survived for 29 mo and 22 mo respectively. One case survived and is still being followed up. CONCLUSION MiNEN of the ampulla are extremely rare, lacking typical clinical symptoms and imaging features, and are usually diagnosed after postoperative histopathological and immunohistochemical examinations. The main treatment is radical surgical resection, which can be combined with chemotherapy. The best method of diagnosis and treatment needs further research.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Mixed neuroendocrine-nonneuroendocrine neoplasm of the ampulla: Four case reports

Wei¹,

Zhang²,

Wang³

et al. 2022

WJCC

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“…We found 19 cases of NENs reported in the literature in a two-year time period, from May 2020 until April 2022 ( Table 1 ) [ 9 , 14 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ].…”

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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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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“…HE-stained sections served for the morphological identification of both neuroendocrine and nonneuroendocrine components. Immunohistochemistry is mandatory to test the nature of the neuroendocrine component, which is also recommended to evaluate the subtype of the nonneuroendocrine (11)(12)(13)15,16,19,20,22,23,25). Preoperative diagnosis was rarely established, and only a few cases reported had been diagnosed through preoperative biopsy with endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) specimens (17)(18)(19)21,24).…”

mentioning

confidence: 99%

Mixed neuroendocrine nonneuroendocrine neoplasms of the pancreas: a case report and literature review of pancreatic mixed neuroendocrine nonneuroendocrine neoplasm

Wang¹,

Lou²,

Qin³

et al. 2021

Gland Surg

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A 36-year-old woman underwent left lateral hepatic lobectomy and tumor enucleation of the pancreatic head due to the diagnosis of mixed neuroendocrine nonneuroendocrine neoplasms (MiNENs).Her past medical history revealed an ill-defined mass in the pancreatic uncinate (approximately 1 cm in size) and liver nodules in segment II, suggesting a pancreatic neuroendocrine tumor (pNET) and liver metastases.After the operation, pathological examination showed that the lesion in the pancreas was MiNEN. The neuroendocrine component consisted of grade 1 (G1) NETs, and the nonneuroendocrine part was welldifferentiated adenocarcinoma. Additionally, in hepatic lesions, the type of pancreaticobiliary duct in moderately differentiated adenocarcinoma indicated the source of tumor cells. We present the following case in accordance with the CARE reporting checklist. MiNENs of the pancreas are extremely rare and heterogeneous malignancies. Owing to the rarity of pancreatic MiNEN, no structured guidelines on preoperative diagnostics, therapy and follow-up exist thus far. Thus, problems in differential diagnosis and appropriate therapy may arise when treating suspected MiNEN patients. We performed a rigorous search on pancreatic MiNEN via PubMed and Web of Science. In total, 167 studies were identified, and 28 cases in 15 studies about pancreatic MiNEN were assessed. All the available information was listed and fully discussed.

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Mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla of Vater in a Chinese patient: A case report

Cited by 5 publications

References 19 publications

Mixed neuroendocrine-nonneuroendocrine neoplasm of the ampulla: Four case reports

Mixed neuroendocrine-nonneuroendocrine neoplasm of the ampulla: Four case reports

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

Mixed neuroendocrine nonneuroendocrine neoplasms of the pancreas: a case report and literature review of pancreatic mixed neuroendocrine nonneuroendocrine neoplasm

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