“…For pancreatic acinar-MiNEN, Pokrzywa et al (30), found that 515 of 57,804 patients resected for pancreatic malignancy had pancreatic acinar-MiNEN via American National Cancer Base, with an incidence of 0.9% over ten years (2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014). Similarly, in the cohort of Nießen et al (11), the frequency of acinar-MiNEN was 0.8% (5 of 570) of all resected pNENs and 0.14% (5 of 3,580 patients) of pancreatic resections for pancreatic tumors from 2001 to 2019.…”
mentioning
confidence: 71%
“…For pancreatic adeno-MiNEN, Yang et al (5), performed a Chinese single-center study on pancreatic neuroendocrine neoplasms (pNEN) over a study period of 10 years (2002-2012) and showed a frequency of 5.5% (6 of 110) adeno-MiNEN out of all pNEN patients resected at the center. In the study of Nießen et al (11), found that the occurrence of adeno-MiNEN was 0.2% (8 of 3,580) of all pancreatic resections for pancreatic tumors and 1.4% (8 of 570) of all resected pNEN over a study period of 19 years (2001-2019). For pancreatic acinar-MiNEN, Pokrzywa et al (30), found that 515 of 57,804 patients resected for pancreatic malignancy had pancreatic acinar-MiNEN via American National Cancer Base, with an incidence of 0.9% over ten years (2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014).…”
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.
“…For pancreatic acinar-MiNEN, Pokrzywa et al (30), found that 515 of 57,804 patients resected for pancreatic malignancy had pancreatic acinar-MiNEN via American National Cancer Base, with an incidence of 0.9% over ten years (2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014). Similarly, in the cohort of Nießen et al (11), the frequency of acinar-MiNEN was 0.8% (5 of 570) of all resected pNENs and 0.14% (5 of 3,580 patients) of pancreatic resections for pancreatic tumors from 2001 to 2019.…”
mentioning
confidence: 71%
“…For pancreatic adeno-MiNEN, Yang et al (5), performed a Chinese single-center study on pancreatic neuroendocrine neoplasms (pNEN) over a study period of 10 years (2002-2012) and showed a frequency of 5.5% (6 of 110) adeno-MiNEN out of all pNEN patients resected at the center. In the study of Nießen et al (11), found that the occurrence of adeno-MiNEN was 0.2% (8 of 3,580) of all pancreatic resections for pancreatic tumors and 1.4% (8 of 570) of all resected pNEN over a study period of 19 years (2001-2019). For pancreatic acinar-MiNEN, Pokrzywa et al (30), found that 515 of 57,804 patients resected for pancreatic malignancy had pancreatic acinar-MiNEN via American National Cancer Base, with an incidence of 0.9% over ten years (2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014).…”
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.
“…Based on this fact and the miscellaneous terms that have been used (MiNEN, MANEC, MANET), we recommend that the subgroup of non-invasive exocrine neoplasms as non-neuroendocrine counterparts (including MANET) be included in MiNEN. We also recommend that the two components should be reported and graded separately in clinical practice, since different components could have different prognosis (Table 3 ) [ 41 , 42 ]. In our case, the clinical behavior is relatively benign, which is consistent with other composite IPMN-NET and further supports the heterogeneity of outcome depending on the histological subtypes [ 6 , 26 , 27 ].…”
Background
Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are extremely rare. Their pathogenesis and molecular landscape are largely unknown. Here, we report a case of mixed pancreatic intraductal papillary mucinous neoplasm (IPMN) and well-differentiated neuroendocrine tumor (NET) and identify its genetic alterations by next-generation sequencing (NGS).
Case presentation
A fifty-year-old male was admitted into the hospital for evaluation of a pancreatic lesion detected during a routine examination. Abdominal ultrasound indicated a hypoechoic mass of 2.6 cm at the head of the pancreas. Malignancy was suspected and partial pancreatectomy was performed. Thorough histopathological examination revealed a mixed IPMN-NET. In some areas, the two components were relatively separated, whereas in other areas IPMN and NET grew in a composite pattern: The papillae were lined with epithelial cells of IPMN, and there were clusters of NET nests in the stroma of papillary axis. NGS revealed shared somatic mutations (KRAS, PCK1, MLL3) in both components. The patient has been uneventful 21 months after the surgery.
Conclusions
Our case provides evidence of a common origin for mixed IPMN-NET with composite growth features. Our result and literature review indicate that KRAS mutation might be a driver event underlying the occurrence of MiNEN. We also recommend the inclusion of mixed non-invasive exocrine neoplasms and neuroendocrine neoplasms into MiNEN.
ZusammenfassungPeriampulläre Neoplasien sind eine heterogene Gruppe verschiedener Tumorentitäten der periampullären Region, von denen das Pankreasadenokarzinom mit 60–70 % am häufigsten ist. Wie typisch für Pankreaskarzinome zeichnen sich periampulläre Pankreaskarzinome durch ein aggressives Wachstum und eine frühe systemische Progression aus. Aufgrund ihrer besonderen Lage in unmittelbarer Nähe zur Papilla Vateri treten Symptome in eher früherem Tumorstadium auf, sodass die Therapiemöglichkeiten und Prognose insgesamt günstiger sind als bei Pankreaskarzinomen anderer Lokalisation. Trotzdem unterscheiden sich die Therapieprinzipien bei periampullären Pankreaskarzinomen nicht wesentlich von den Standards bei Pankreaskarzinomen anderer Lokalisation. Ein potenziell kurativer Therapieansatz beim nichtmetastasierten periampullären Pankreaskarzinom ist multimodal und besteht aus der Durchführung einer partiellen Duodenopankreatektomie als radikale onkologische Resektion in Kombination mit einer systemischen, meist adjuvant verabreichten Chemotherapie. Bei Patienten mit günstigen prognostischen Faktoren kann hierdurch ein Langzeitüberleben erzielt werden. Zudem wurden mit der Weiterentwicklung der Chirurgie und Systemtherapie auch potenziell kurative Therapiekonzepte für fortgeschrittene, früher irresektable Tumoren etabliert, welche nun nach Durchführung einer neoadjuvanten Therapie oft einer Resektion zugeführt werden können. In diesem Beitrag werden die aktuellen chirurgischen Prinzipien der radikalen onkologischen Resektion periampullärer Pankreaskarzinome im Kontext der multimodalen Therapie dargestellt und ein Ausblick auf mögliche künftige Entwicklungen der Therapie gegeben.
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