Abstract:Determination of the primary tumor in periampullary region carcinomas can be difficult, and the pathological assessment and clinicopathological characteristics remain elusive. In this study, we investigated the current recognition and practices for periampullary region adenocarcinoma with an indeterminable origin among expert pathologists through a cognitive survey. Simultaneously, we analyzed a prospective collection of cases with an indeterminable primary tumor diagnosed from 2008 to 2018 to elucidate their … Show more
“…The PRAIO extends along both the bile duct and the main pancreatic duct and is characterized by unique morphological features, different from those of PDAC, dCCA, or other peri-ampullary tumors. However, studies with a larger number of PRAIO cases should be performed towards the establishment of PRAIO as a distinct entity [88].…”
Pancreatic ductal adenocarcinoma (PDAC) and distal cholangiocarcinoma (dCCA) are very aggressive tumors with a high mortality rate. Pancreas and distal bile ducts share a common embryonic development. Hence, PDAC and dCCA exhibit similar histological features that make a differential diagnosis during routine diagnostic practice challenging. However, there are also significant differences, with potential clinical implications. Even if PDAC and dCCA are generally associated with poor survival, patients with dCCA seem to present a better prognosis. Moreover, although precision oncology-based approaches are still limited in both entities, their most important targets are different and include alterations affecting BRCA1/2 and related genes in PDAC, as well as HER2 amplification in dCCA. Along this line, microsatellite instability represents a potential contact point in terms of tailored treatments, but its prevalence is very low in both tumor types. This review aims at defining the most important similarities and differences in terms of clinicopathological and molecular features between these two entities, also discussing the main theranostic implications derived from this challenging differential diagnosis.
“…The PRAIO extends along both the bile duct and the main pancreatic duct and is characterized by unique morphological features, different from those of PDAC, dCCA, or other peri-ampullary tumors. However, studies with a larger number of PRAIO cases should be performed towards the establishment of PRAIO as a distinct entity [88].…”
Pancreatic ductal adenocarcinoma (PDAC) and distal cholangiocarcinoma (dCCA) are very aggressive tumors with a high mortality rate. Pancreas and distal bile ducts share a common embryonic development. Hence, PDAC and dCCA exhibit similar histological features that make a differential diagnosis during routine diagnostic practice challenging. However, there are also significant differences, with potential clinical implications. Even if PDAC and dCCA are generally associated with poor survival, patients with dCCA seem to present a better prognosis. Moreover, although precision oncology-based approaches are still limited in both entities, their most important targets are different and include alterations affecting BRCA1/2 and related genes in PDAC, as well as HER2 amplification in dCCA. Along this line, microsatellite instability represents a potential contact point in terms of tailored treatments, but its prevalence is very low in both tumor types. This review aims at defining the most important similarities and differences in terms of clinicopathological and molecular features between these two entities, also discussing the main theranostic implications derived from this challenging differential diagnosis.
“…So wurde in einer kürzlich veröffentlichten multizentrischen Studie an über 1200 Patienten mit periampullären Tumoren gezeigt, dass einerseits in 13 % der vermuteten Fälle eines Pankreaskarzinoms ein nichtpankreatischer periampullärer Tumor vorlag und andererseits von den vermuteten nichtpankreatischen periampullären Tumoren 21 % postoperativ als Pankreaskarzinom identifiziert wurden [ 8 ]. Eine aktuelle Studie aus Japan berichtet, dass bei über 5 % aller Patienten mit periampullären Tumoren ein nichtidentifizierbarer Ursprung vorliegt („periampullary region adenocarcinomas with an indeterminable origin“, [PRAIO]; [ 3 ]). Diese PRAIOs tragen eigenständige histopathologische Merkmale, sodass das Vorliegen einer zusätzlichen Entität periampullärer Tumoren diskutiert wird.…”
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.
“…In advanced cases, it is extremely difficult to distinguish AC from periampullary carcinoma to the extent that the only diagnosis could be rendered is carcinoma of the pancreatobiliary-ampullary region. Although, the clinical presentation and treatment modalities for ampullary and periampullary tumors are the same, their outcome are quite different with that for pancreatic adenocarcinoma being much worse than for the other tumors [2]. This inspired us to search for the use of an immunohistochemical marker expression to discriminate between AC, pancreatic adenocarcinoma, duodenal adenocarcinoma, and other confusing benign and inflammatory pancreatic lesions.…”
BACKGROUND: Maspin (a tumor suppressor gene) is down-regulated in breast, prostate, gastric, and melanoma. Although it is not detected in normal pancreatic tissue, it is over-expressed in pancreatic cancer suggesting that maspin may play different activities in different cell types. Pancreatic ductal adenocarcinoma (PC) acquires maspin expression through hypomethylation of its promoter.
AIM: Because the discrimination between ampullary and periampullary carcinomas is challenging in advanced cases, this inspired us to search for the use of maspin expression to discriminate between ampullary carcinoma (AC), PC, duodenal adenocarcinoma (DC), and other confusing benign and inflammatory pancreatic lesions.
METHODS: Immunostaining for maspin was performed for 80 pancreaticoduodenal lesions. Sixty cases were malignant: 48 cases of pancreatic epithelial tumor (41 PC and 7 solid pseudopapillary neoplasm), 9 AC, and 3 DC. Twenty cases were non-malignant: 12 inflammatory (chronic pancreatitis), 5 benign neoplastic (serous cystadenomas), and 3 normal pancreatic tissue. Cytoplasmic and/or nuclear staining was considered positive as: Focally positive (5–50% of tumor cells), diffusely positive (>50% of tumor cells), or negative (<5% tumor cells).
RESULTS: Maspin expression (positive/negative), distribution (focal/diffuse), and nuclear expression are significantly different between PC, solid pseudopapillary neoplasm, AC, and DC. PC shows significantly higher expression with more diffuse positivity and more nuclear expression than other malignant groups. Forty cases of PC (40/41) (97.6%) showed positive expression; 28 of them (28/40) (70%) showed diffuse expression and 82.5% (33 cases) showed nuclear and cytoplasmic expression. Only one case (14.3%) (1/7) of solid pseudopapillary neoplasm showed positive focal cytoplasmic expression. Three AC cases (3/9) (33.3%) showed positive focal cytoplasmic expression. Two cases of DC (2/3) (66.7%) showed positive focal cytoplasmic expression. Maspin expression shows significant positive correlation with poor prognostic variables as tumor grade, lymphovascular invasion, T stage of PC. Minority of chronic pancreatitis and benign lesions are maspin positive with significant difference from the malignant groups.
CONCLUSION: Our results suggest that maspin can be of value in differentiating pancreatic adenocarcinoma from ampullary carcinoma, duodenal adenocarcinoma, and other confusing lesions as chronic pancreatitis.
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